Therapeutic ultrasound for the treatment of glaucoma.

We present a summary of the results obtained on 1142 treatments of 1072 eyes treated in our hospital during the last 5 years. Ultrasound has been used to treat 843 eyes with refractory glaucoma and also to restore failed trabeculectomy filtering blebs in 229 eyes. The single treatment success rate with success defined as an IOP > or = 20 mm Hg was 59% at one year and 46% at 3 years. In cases of failed blebs, ultrasound treatment performed 3 to 12 weeks after trabeculectomy revived the blebs of 82% of the phakic eyes when 5 FU subconjunctival injections were performed in the following days.


Holmium laser sclerostomy in glaucoma management: a new tool for an old procedure.

A THC: YAG laser (thulium, holmium, chromium doped YAG crystal) was used to create thermal sclerostomies in 49 glaucomatous eyes of 46 patients. The laser is a long-pulsed (300 microseconds), compact, self contained, solid state laser operating in the near infrared (2.1 mu). A 1 mm conjunctival stab incision was made 12 mm away from the sclerostomy site to allow entry of a specially designed 22-gauge (712 mu) optic probe that delivers energy at a right angle to the long axis of the fiber. Probe insertion produced minimal disturbance of the conjunctiva. Pulse energies of 80 mJ tot 120 mJ were used with a repetition rate of 5 pulses/sec. Total energy levels to produce full-thickness sclerostomies ranged from 1.4 to 7.2 J. Subconjunctival 5-fluorouracil injections were administered in 46 eyes. Estimated probability of success was 74% at 6 months and 67% at 12 months. Mean intraocular pressure of successful cases was 13.3 mmHg at both both 6 months & 12 months. Twelve cases failed within the initial 6 months, and two additional cases failed by 12 months.


Morphological aspects of normal optic disc and early signs of glaucomatous alterations.

A group of 110 eyes with untreated ocular hypertension, 94 being non-exfoliative and 16 exfoliative and in addition 10 glaucomatous eyes were selected for this study. The optic nerve head was examined by ophthalmoscopy and a drawing was made of the estimated cupping. The disc pallor was measured from disc photographs in a double masked manner at the same time. The possibility to detect early glaucomatous damage by a single examination will be discussed and the two methods will be compared. This study indicates that an increasing vertical diameter of disc pallor might have a predictive value.


Biomorphometry and histomorphometry of the optic disc with special reference to the parapapillary region.

Glaucomatous optic nerve damage is typically associated with intrapapillary changes such as neuroretinal rim loss. In this study, parapapillary chorioretinal atrophy was evaluated in 691 normal eyes, 1081 glaucomatous eyes and 31 eyes with ocular hypertension. It was significantly larger and occurred more often in the glaucomatous eyes (parapapillary atrophy area: 1.07 +/- 0.83 mm2) than in the normal eyes (0.55 +/- 0.64 mm2) or the eyes with ocular hypertension (0.55 +/- 0.37 mm2). These differences were significant also for eyes with moderate glaucomatous damage (0.86 +/- 0.62 mm2). Parapapillary chorioretinal atrophy was associated with shallow glaucomatous cupping, diffuse nerve fiber loss, markedly tessellated fundus and only moderately elevated intraocular pressure. It increased with decreasing neuroretinal rim area. It showed a spatial correlation to neuroretinal loss inside the optic disc. In unilateral glaucoma, it was larger in the affected eye than in the unaffected eye. Differentiated into zones Alpha and Beta, it correlated clinico-histologically with a complete loss of retinal pigment epithelium cells and incomplete loss of photoreceptors (Zone Beta) and pigmentary and structural irregularities of the retinal pigment epithelium (zone Alpha). Parapapillary chorioretinal atrophy is associated with glaucoma.


Measurement of retinal nerve fiber layer thickness and its functional correlations with the visual field.

We have developed a technique for stereophotogrammetric measurements of retinal nerve fiber layer thickness at the disc margin. This technique has a reproducibility ranging from 3.9% to 6.0% in normal, ocular hypertensive and high pressure open angle glaucomatous eyes. The presence of a halo or scleral crescent had no effect on the reproducibility of the measurements. The measurements have shown that ocular hypertensive eyes have about an 18% decrease of retinal nerve fiber layer thickness compared to normals. High pressure open angle glaucomatous eyes have about a 43% decrease in retinal nerve fiber layer thickness compared to normals. This technique allows the development of maps of retinal nerve fiber layer thickness so that localized changes at the disc margin can be detected. The thinnest nerve fiber layer occurs primarily in the temporal segment. This technique has also correlated measurements of nerve fiber layer thickness with other measurements of the optic disc, particularly showing a significant correlation with area of pallor as well as measurements of the width of optic disc cup at about halfway between surface and the bottom of the cup. A significant correlation with visual field loss has been shown in high pressure open angle glaucomatous eyes, primarily with the temporal quadrant of the retinal nerve fiber layer and the nasal visual field. The rate of the visual field change in primary open angle glaucoma has been correlated with the initial nerve fiber layer thickness, with the greater the retinal nerve fiber layer thickness the greater the rate of the visual field loss during follow-up. These findings suggest that retinal nerve fiber layer thickness discriminates between normal, ocular hypertensive eyes as well as those with open angle glaucomas and may be useful in following patients with open angle glaucoma prior to development of the visual field loss.


Color vision and contrast sensitivity tests in early diagnosis of primary open-angle glaucoma.

Color vision was examined by the Farnsworth 100 hue test in 48 eyes with ocular hypertension (OHT). Thirteen eyes had a general loss of color discrimination and 8 had a blue-yellow axis. Spatial contrast sensitivity was tested in 31 eyes with OHT by the Mentor B-VAT and was reduced in 8 of them. Temporal contrast sensitivity was explored in 40 eyes with OHT by the Flickersyst&m and showed abnormal responses in 11 eyes. According to the lack of specificity of the responses and the overlapping of the results of normal eyes and OHT eyes, those tests are not useful for classifying an individual patient. Such investigations however have the interest to show that macular mediated functions may be impaired early in glaucoma.


Contribution of pattern electroretinogram and pattern visual evoked potential in early diagnosis of primary open angle glaucoma.

Pattern electroretinogram (PERG) combined with visual evoked potential (VEP) has become increasingly useful in clinical assessment of the anterior visual pathways. In regard to the PERG dependence on ganglion cell activity, we studied monocular PERG in 36 patients (71 eyes) with intraocular hypertension (6 eyes under topical beta-blockers) and in 10 patients (18 eyes) with advanced glaucoma, to estimate the potential effect of increased IOP on ganglion cells. We recorded simultaneous VEP to evaluate the retino-cortical conduction time. Referring to normal values obtained from 19 eyes (12 subjects), we found clear-cut PERG abnormalities in 20 eyes among ocular hypertensive patients with increased IOP. They were more altered in the advanced glaucomatous group. Two or three control exams were performed in 10 patients every 6 months: however such a short-time follow-up seems too limited to define the ERG prognosis value in this pathology. Comparing the PERG with the clinical routine assessments, we discuss its place in the diagnosis and in the follow-up of patients with ocular hypertension.


[Psycho-functional and objective tests applied to early diagnosis of POAG, indications for general ophthalmologists and therapeutic incidences].

The early diagnostic of glaucoma (before the appearance of visual field defects) can be made in some cases with the help of certain objective tests and, to a lesser degree, with subjective or psychophysical tests. Retinal nerve fiber layer photography is certainly the most rewarding objective test, providing the technique has been mastered and there is a clear fundus view without chorio-retinal atrophy. The identification of nerve fiber defects is an almost sure sign of glaucomatous damage in the absence of other pathology. An abnormally small neuroretinal rim area can also be an indicator of glaucoma. However, no conclusion can be drawn if the value is within the wide range of normal limits. Electrophysiological tests in glaucoma patients at the present time give results that show too much variability and overlap with those of normals to be of practical value. The same can be said for subjective tests such as spatial and temporal contrast sensitivity (the flicker test, for example). The specificity and sensitivity of the tests are too weak to be useful for the diagnosis of glaucoma in 1992.


[Importance of the role of dose rate on tumor induction in rats after radon inhalation].

Lung cancer can be induced in rats, by radon daughter products, after exposure as low as 25 WLM (80 mJ.h.m-3) protracted over 4 to 6 months with a dose rate of 100 to 150 WL (2 to 3 mJ.m-3). The incidence of lung cancer is not increased and is equal to that of controls when the same cumulated dose is protracted over 18 months at 2 WL (0.042 mJ.m-3).


Evolutionarily conserved promoter motifs and enhancer organization in the mouse gene encoding the IIB myosin heavy chain isoform expressed in adult fast skeletal muscle.

We have isolated the mouse gene for the MHC isoform expressed in adult type IIB (fast-contracting, glycolytic) skeletal muscle fibers, and determined the DNA sequence of the promoter region. This sequence represents the first example of a promoter for a gene encoding an adult-specific isoform of a mammalian skeletal MHC. The proximal 200 bp of the promoter contains several sequence motifs which are identical or very similar to homologous motifs found in the promoters of a family of chicken skeletal MHC genes. Of these, two novel AT-rich sequences may be important for regulation of the promoter. A second feature of the mouse IIB MHC promoter sequence concerns a number of sequence motifs located at ca. -1,000 bp which are organized in a similar fashion in the IIB MHC promoter and a homologous region of promoter of the mouse muscle creatine kinase gene.


[Effect of elastin peptides on cell proliferation].

Elastin peptides were shown to act on a cell membrane receptor coupled to a G-protein, phospholipase C, and its activation increases IP3 and DAG and opens receptor-dependent Ca(++)-channels. As some growth factors also produce similar modifications in intracellular Ca++, we wanted to explore the effect of elastin peptides on cell proliferation using 3H-thymidine incorporation and cell counting. The concentration of peptides needed for the stimulation of cell proliferation varied between large limits (1 microgram/ml to 10 mg/ml) according to the origin of the cells and the nature of the peptides. The proliferation of CCL 39 chinese hamster lung fibroblasts was enhanced in a dose-dependent fashion in the concentration range of 3 to 10 mg/ml. The proliferation of human skin fibroblasts was enhanced in the concentration range of 0.5 to 3.3 mg/ml and inhibited at higher concentrations. This effect depended little on the average molecular weight (MW) of the peptide preparation, high MW peptides (average 75 kDa) and lower MW peptides (average MW 10 kDa) were both efficient approximately to the same extent. It appears probable that only a small fraction of these peptides possesses this growth promoting property; other sequences might have the opposite effect. The conformation of the peptides may also play an important role. Human sera contain circulating elastin peptides in the concentration range of 1.0 to 10 micrograms/ml, increasing in obstructive arteriopathies and in some hyperlipidemias. It appears therefore that the above findings may have physiopathological significance in the regulation of cell proliferation in normal and pathological conditions.


Early detection of heart transplant rejection using cardiac echography combined with the assay of glycosylated residues in plasma by proton NMR spectroscopy.

Early diagnosis of acute cardiac graft rejection by non-invasive methods is required for medical, organizational, psychological and economic reasons. We have monitored 18 heart recipients over a period of 2.5 years using endomyocardial biopsies (EMB), cardiac Doppler-echography (CDE) and proton NMR spectroscopy assay of plasma glycosylated residues. Diastolic parameters of CDE and assay of the glycosylated residues by NMR spectroscopy respectively detect 42 and 45% of the acute low grade (mild or moderate) histological rejections. The combination of the two methods allows the detection of 65% of rejections. The strategy combining plasma NMR spectroscopy and echography is pertinent to the non-invasive detection of acute cardiac rejections with low histological grade.


[Lethal effect of boron neutron capture reaction on human uveal melanoma cells in culture incubated with borophenylalanine].

Cell cultures obtained from human uveal tumours have been used as experimental model to study the lethal effect consecutive to the neutron capture reaction on boron incorporated into cells as borophenylalanine. An irradiation with a neutron fluence of 6 x 10(9) n cm-2 reduced the number of viable cells by about 30%.


[Description of cell line established from human thyroid papillary cancer and secreting human chorionic gonadotropin hormone].

One of the difficulties in characterization of the oncogenes involved in thyroid carcinogenesis is the production of cell lines. Arising from a poorly differentiated thyroid papillary carcinoma we have established a cell line synthesizing the thyroglobulin and human chorionic gonadotropin (alpha and beta subunits) (HCG) hormones. These cells will allow research of the oncogenes involved or potentially involved in thyroid papillary carcinomas and evaluation of the role of the autocrine secretion of HCG.


[Study of the role of negative regulator, AcSer-Asp-Lys-Pro (AcSDKP) in the phenomena of cellular proliferation].

The intra and extracellular kinetics of AcSDKP levels in cell culture have been studied. A close correlation was observed between the minimal level of intracellular AcSDKP (a negative regulator of cell proliferation) and the initiation of DNA synthesis. The return to initial levels of intracellular AcSDKP when the rate of DNA synthesis decreases, suggests a role for the tetrapeptide in homeostasis during cell growth. The return to normal values was not observed in preliminary studies on cell lineages during uncontrolled proliferation.


Nucleus reticularis thalami participates in sleep spindles, not in beta rhythms concomitant with attention in cat.

In the awake cat, characteristic electrocortical synchronized oscillations (beta rhythms) can develop on the parietal cortex in specific behavioural situations such as during attentive fixation of a target. These activities differ from other synchronized rhythms which accompany slow sleep ("spindles") and are more widespread. It is shown here through single unit recording from the thalamic nucleus reticularis (RET), that the latter structure participates in spindles (as already postulated by other authors), but not in the beta rhythms which seem to depend on a more restricted thalamic focus in the posterior thalamic nucleus. These data thus support the idea that RET plays a role in slow-wave sleep but not in the cognitive operations involved in focussing attention.


[Sequential analgesia. Technical aspects].

Combined spinal and epidural anaesthesia was described by P. Moulaert. After searching the epidural space, a spinal needle is introduced through the Tuohy needle for performing the intrathecal block; then an epidural catheter is put for the postoperative analgesia. The author from his own experience of this regional anaesthesia proposes modifications of the Tuohy and spinal needles to improve the technique.


[Does the differential block by the epidural route provide a diagnostic aid in chronic pain?].

Sixty-two patients with chronic pain below the waist level were evaluated with a differential epidural block performed after an overall somatic and psychological assessment. The pain and neurophysiologic variations under block were compared with the somatic and psychological findings. No consistent relationship was found between pain variations and sympathetic sensory effects of the graduated epidural block. The presence of neurological diseases and depressive or hysterical disorders influenced particularly the pain responses to the block. It is concluded that the differential epidural block is not sufficient to affirm the somatic origin of a chronic pain and must be always confronted with the somatic and psychosocial findings.


[Evolution of the blood levels of propofol administered by continuous perfusion during extracorporeal circulation].

The pharmacokinetics of propofol administered in continuous infusion was studied in 10 patients without left ventricular insufficiency during extracorporeal circulation (ECC) with hemodilution, for aortocoronary bypass. After a dosage of 1.5 mg.kg-1 during anaesthetic induction, the blood level was 4,800 micrograms.l-1. Under continuous infusion levels remained very high: they decreased by 40% during EEC induction and rose more than 10% when artificial ventilation started again. These modifications can be explained by physiological variations induced by EEC (non pulsated flow, redistribution, vasoconstriction, hemodilution, hypothermia) and they lead to adapt dosages in this type of anaesthesia.


[Secondary migration of a central venous catheter linked to an implantable port].

The secondary migration of the distal part of a central venous catheter with implantable port is rarely described. This study reports a case in which the distal part of a catheter with implantable port moved spontaneously, a month after its implantation in a branch of the internal jugular vein without any other complication. The discussion is about modalities, frequency and explanation mechanisms of the complications due to central catheters. The conclusion insists on systematical radiologic control of the position of implantable port with central venous access, and in case of bad working or any sign of thrombo-phlebitis.


[A fatal gas embolism during a hysteroscopy-curettage].

A 26 year old woman without any pathological history except metrorrhagia underwent a hysteroscopy under epidural anaesthesia and uterine cavity dilatation by saline solution. A respiratory distress and cardoffirculatory arrest occurred at the end of the procedure. Despite several attempts of cardiorespiratory resuscitation the patient deceased. The diagnosis retained was a gas embolism caused by blast effect from reintroduction of the hysteroscope into the uterine cavity. Different mandatory security measures are evoked.


[General practitioners' experience in a mass screening campaign of colorectal cancers].

In a mass screening campaign of colorectal cancer, general practitioners play an essential role for obtaining a high rate of participation from people. It is important to analyze the reasons for the participation and their actual experiences in the campaign. Out of 71 GPs participating in the campaign, 67 have been interviewed by a sociologist and 51 GPs (76%) considered themselves "motivated" for participating after an instruction course in small groups. Sixteen GPs were sceptical about the outcome or not highly motivated. However thirteen of them participated by moral obligation vis à vis their patients or for not being different from their colleagues. All GPs appreciated being actively involved in the organization of the screening campaign and considered that the public had been well informed. Such information covered the information leaflet, the press campaign at the beginning of the campaign and the recall letter for those who had not been consulted during the time when the test was distributed by GPs. The main constraint was the time spent to convince the patients to do the screening test and explain its importance. The additional time, 5 to 10 minutes per consultation was important to the GPs. They thought it was fair to be paid for their active participation. These data suggest that it is possible to motivate GPs for a mass screening campaign, on the condition that they are actively involved and participate in an instruction course for small groups.


[Aggravation of hypoxemia in supine position in myotonic dystrophy].

Myotonic dystrophy (MyD) involves a variety of systems. Respiratory disorders are common, namely elevation of diaphragm, alveolar hypoventilation, aspiration pneumonia and sleep apnea. We evaluated respiratory involvement. The subjects were 11 patients with MyD. Also 6 patients with limb girdle muscular dystrophy (LG) were examined to be compared with MyD. Both groups had the similar activities of daily living. All of them never complained of dyspnea. Arterial blood gas studies were performed in supine position and standing position. A new evidence was found that hypoxemia was aggravated and alveolar-arterial oxygen pressure difference was increased in supine position in MyD. Next, pulmonary function tests were done in supine position and sitting position. Functional residual capacity (FRC) were more reduced in supine position in MyD compared with LG. The value to subtract closing capacity from FRC was negative in supine position in MyD, showing closing phenomenon. We propose the mechanism of the aggravation of hypoxemia may be the following. The reduction of FRC caused by respiratory muscle involvement brings out the closing phenomenon. Abnormal uneven distribution of ventilation-perfusion ratio happens and then hypoxemia is worsened in supine position in MyD.


[X-ray computed tomographic scans of lower limb and trunk muscles in facioscapulohumeral muscular dystrophy].

X-rays computed tomographic (CT) scans of muscles of the lower limbs and the trunk in 14 patients with facioscapulohumeral muscular dystrophy (FSH) were studied. The CT scans showed that the affected muscles were decreased in density and size. The laterality of muscular involvement was sometimes observed. The muscular lesions in the lower limbs showed proximal distribution. In the thigh, the hamstrings were affected first, the adductor muscles second, and then the muscular involvement progressed to the quadriceps femoris muscle. In the lower leg, the gastrocnemius and soleus muscles were relatively spared as compared with the tibialis anterior muscle. In the lumbar girdle, the abdominal muscles were involved first, the gluteal muscles second, the back muscles third, and the psoas major muscle were relatively spared. The muscular weakness of this distribution exacerbated lumbar lordosis. The neck muscles were less affected than those of the lumbar girdle. The CT scans in FSH demonstrated the characteristic pattern of muscular involvement, which differed from the inherited muscular diseases such as Duchenne muscular dystrophy, myotonic dystrophy, and others.


[Analysis of different aspects of the health systems in Tunisia].

The health care system in Tunisia is composed of two sectors, public and private. Given the social and economic development level of the country, it can be said that the hospitals, health centers and workforce devoted to health are not severely limited. However, these structures are under-equipped and finance management is not relevant. The establishment of a National Health Service would render all the system more efficient.


[A hereditary ataxia associated with hypoalbuminemia and hyperlipidemia--a variant form of Friedreich's disease or a new clinical entity?].

The patients belonged to three different families and were products of consanguineous marriage. The neurological symptoms and signs in these patients began in infancy or childhood and included gait disturbance, horizontal nystagmus, distention tremor of the hands, muscular wasting and sensory impairment of the hands and legs. CT-scan and/or MRI showed atrophy of the cerebellum. Serum biochemical analyses revealed hypoalbuminemia with hyperlipidemia. There were no abnormalities in the heart, liver, kidney, gastrointestinal tract, or endocrine systems. The autopsy revealed degenerative changes in the spinal cord including posterior column and lateral pyramidal tract, as well as in the peripheral nerves and cerebellar cortex. Although we have speculated that the disease presented here would be a clinical variants of Friedreich's disease, it would make a new clinical entity because there was no report about the association to hypoalbuminemia and hyperlipidemia with spinocerebellar degeneration.


[Antibodies to GM1 ganglioside in motor neuron disease--in comparison with demyelinating neuropathy].

We studied serum antibodies to GM1 ganglioside by enzyme linked immunosorbent assay (ELISA) in 55 patients with motor neuron disease (MND) composed of 36 ALS and 19 lower motor neuron disease (LMND), 44 patients with demyelinating neuropathy (DN) composed of 29 Guillain-Barré syndrome (GBS) and 15 chronic inflammatory demyelinating polyneuropathy (CIDP), and 21 healthy controls. High levels of serum antibodies against GM1 were confirmed by thin-layer chromatography overlay procedure. In MND group, the mean level of anti-GM1 IgM antibodies was not significantly elevated in comparison with controls. There was no significant difference in anti-GM1 antibodies between ALS group and LMND group, while anti-GM1 IgM antibodies in DN group, especially in GBS group, were significantly elevated (p < 0.001). High levels of anti-GM1 IgM antibodies (greater than the mean level plus 3 standard deviations of controls) were detected in 9 patients (6 with ALS and 3 with LMND) with MND (16.4%) and 16 patients (11 with GBS and 5 with CIDP) with DN (36.4%). Serum antibodies to GM1 reacted with GD1b ganglioside in only one patient with MND and 10 patients (8 with GBS and 2 with CIDP) with DN. Anti-GM1 IgG antibodies were elevated significantly in DN group. There was no correlation among anti-GM1 IgM antibodies and both duration and severity of illness in MND. In some patients with MND, levels of anti-GM1 IgM antibodies became high in the advanced stage. It is unclear whether these antibodies are primary manifestation or consequence of motor neuron disease.(ABSTRACT TRUNCATED AT 250 WORDS)


[Clinical significance of carotid and ocular bruits in cerebrovascular disease].

We investigated the clinical significance of carotid and ocular bruits in 250 consecutive patients with cerebrovascular disease (CVD). The incidence of bruits was compared with that in 100 age- and sex-matched neurological controls without CVD. In the cerebrovascular disease group, carotid bruits alone were found in 12 patients, both carotid and ocular bruits in 7, and ocular bruits alone in 2. Carotid bruits were found only in 3 controls. We then evaluated cerebrovascular disease in the 25 patients (16 men and 9 women) who had bruits. The patients ranged from 55 to 81 years in age (mean: 70.6 years). The patients with carotid bruits alone constituted the largest group, and those with ocular bruits alone were the smallest group. Carotid bruits were heard and abnormal blood flow was observed in 19/28 arteries of the 21 patients who underwent echo-flow studies. Carotid artery stenosis/occlusion was detected in 24/31 arteries (77%) in 23 patients who underwent digital subtraction angiography (DSA). Thus, the sensitivity was 0.77 and specificity of a carotid bruit 0.91, respectively. In 9/10 patients with unilateral ocular bruits, ipsilateral or contralateral carotid artery stenosis/occlusion was detected. Diminished cerebral blood flow was observed in 10/15 patients with bruits who underwent SPECT. Of these 8 had reduced cerebral blood flow ipsilaterally to the bruit. Blood flow was reduced in the carotid artery territory in all of the patients, and watershed reductions were commonest. Among the 25 patients, some showed neurological semiology of the vertebrobasilar territory in addition to that of the carotid territory.(ABSTRACT TRUNCATED AT 250 WORDS)


[Gene expression in lactate dehydrogenase-A subunit deficiency].

A 33-year-old female complained of muscle pain and stiffness after severe exercise from the age of nine. Her sister also had similar symptoms. Consanguinity was found in her parents. Neither muscle wasting nor weakness was detectable. The activity of LDH in the muscle was decreased less to than 8% of normal value. The isoenzyme pattern of the muscle LDH revealed only one band of B4. The levels of blood lactate did not rise on anaerobic exercise, while a marked increase of pyruvate was found. Northern blot analysis showed that the ratio of LDH-A transcript to beta-actin transcript in the patient was similar to that in a normal subject. RNA preparations were reverse-transcribed, amplified by a polymerase chain reaction (PCR), and separated by electrophoresis. The size of PCR product corresponding to exon 6 was decreased. The nucleotide sequence of this product was determined and a 20 bp deletion was found. This mutation results in a frame-shift translation and premature termination. The predicted incomplete LDH-A subunit contains only 259 instead of 331 amino acids. Immunofluorescence for LDH-A subunit could be seen within the cytoplasm and on the surface membrane of the muscle fibers in our patient as well as control subjects by the immunohistochemical studies. These findings suggest that LDH-A mRNA is transcribed in a truncated form and an enzymatically inactive protein is produced in the patient's muscles.


[Widespread tau abnormality in a case of cortico-basal degeneration].

A case with cortico-basal degeneration was reported with special reference to the immunohistochemical study. A 59-year-old housewife noted tremor and clumsiness of her left hand. On the initial examination she showed the hyperreflexia of the upper extremities and jaw jerk, parkinsonian symptoms such as Myerson sign, parkinsonian gait and rigidity in the left arm. She showed pronounced forced grasping in the left hand. At the age of 60 she showed a WAIS scale with verbal IQ of 99 and performance below the scale. She could not copy hand postures. Tremor was aggravated by action or anxiety, more prominent on the left hand. There was some incoordination on the finger-nose testing of left arm and on the knee-heel testing of both legs. She also showed homolateral dyskinesia. She had a left Babinski sign and sensory testing was normal. A CT scan showed slightly enlarged ventricles (Fig. 1). At the age of 61 she could not understand simple requests and speak few words spontaneously, showing severe dysarthria. There were palilalia and motor impersistence. CT scan showed more widening of the lateral ventricles. At the age of 62, she had lingual dyskinesia and tapping on her upper lip provoked myoclonic jerk on her arms. She died of pneumonia at the age of 65 years, 6 years from the onset. The brain weighed 1190 g. There were bilateral old subdural hematomas on the right parietal and occipital lobe and the left parietal lobe. There was atrophy of frontal and superior parietal region.(ABSTRACT TRUNCATED AT 250 WORDS)


[A cervical collar therapy for non-progressive juvenile spinal muscular atrophy of the distal upper limb (Hirayama's disease)].

Non-progressive juvenile spinal muscular atrophy of the distal upper limb (Hirayama's disease) occurs predominantly in men in the second decade with unilateral weakness of the fingers and hand, and atrophy of the hand and forearm. The symptoms generally progress for 3-4 years after onset and then stop. Since the second decade is a very important period starting one's career, early stopping of the progression and possibly the improvement are essential. On the bases of the neuroradiological studies, a forward displacement of the posterior wall of the dural canal at the lower cervical level on neck flexion is presumed to be the main pathogenetic mechanism in this disease. On neck flexion, the spinal cord is compressed anteroposteriorly at C7 and C8 segmental levels. Pathological studies suggested that chronic microcirculatory disturbances in the spinal cord as the result of repeated flexion of the neck might produce necrosis of the anterior horns. We used a cervical collar for the prevention of neck flexion in 14 patients in the early stage and compared the results with those of 18 patients who had natural courses. In the therapy group, muscle weakness of the affected hand improved in 5 patients and the progression of the disease was stopped in 8 patients with the duration of 1.8 +/- 1.2 years, whereas the duration of progression of the natural course group was 2.9 +/- 1.6 years. Only one patient who could not tolerate the use of collar showed a slight progression. These results suggest that the cervical collar is a useful therapeutic tool in this disease.


[Migrant sensory neuritis--electrophysiological and pathological study].

Migrant sensory neuritis, which was first proposed by Wartenberg, is very uncommon and only a few case reports have ever been published. We described one case of migrant sensory neuritis and discussed the pathogenesis of this disease. A 44-year-old man noticed numbness in the lateral aspect of the dorsum of the left foot in February 1985. Physically, there was hypoesthesia in the region of left sural nerve with positive Tinel's sign. During the next 4 years, pain, tingling sensation or hypoesthesia appeared in the regions of the right superficial radial nerve, right axillary nerve, left intercostal nerve, left lateral plantar nerve, digital nerve of the right second digit, left saphenous nerve, right superficial peroneal nerve, left superficial radial nerve, bilateral ulnar nerves and bilateral median antebrachial cutaneous nerves one after another in a migrating fashion. Tinel's sign was also positive at the right superficial radial nerve. In some occasions, decreased deep tendon reflexes were observed, but there had been no muscle weakness. Some nerves showed complete recovery, but others showed persistent involvement. Some nerves were affected repeatedly. Laboratory examination failed to clarify underlying disease except for mild liver dysfunction. Electrophysiological study showed reduced amplitude of the sensory nerve action potential (SNAP) of right sural nerve, left ulnar nerve, right superficial radial nerve and digital branch of right median nerve with preserved sensory nerve conduction velocity. SNAP of left sural nerve was absent. These findings mean the cause of the sensory disturbance is axonal degeneration rather than segmental demyelination. There were no abnormalities in motor nerve conduction study.(ABSTRACT TRUNCATED AT 250 WORDS)


[A case of fibromuscular dysplasia presenting with Wallenberg syndrome, and developing a giant aneurysm of the internal carotid artery in the cavernous sinus].

A 25-year-old man developed Wallenberg syndrome (WS). At that time his carotid angiography was normal. When he was 28 years old, he suffered from retinal artery embolism in the left eye. At the age of 30 years, he had an acute onset of abducens nerve palsy in his right eye. The carotid angiography showed a giant aneurysm at the cavernous sinus portion in the right internal carotid artery. At his age of 38, the right oculomotor, trochlear and trigeminal nerves were involved. A vertebral angiography revealed a bead-like formation, and a diagnosis of fibromuscular dysplasia (FMD) was made. An intensive angiographic examination revealed many stenotic or dilated lesions in the carotid, vertebral, coronary, renal, and hepatic arteries. A sural nerve biopsy specimen revealed that the sural vein was involved. In Japan only one case of FMD presenting with WS is known. FMD should be under consideration as an underlying disease, when WS occurred in younger patients with few risk factors. In this patient an angiography revealed no abnormality in the cavernous sinus portion of the internal carotid artery, when he suffered from WS. However, eight years later he was proved to have a giant aneurysm in the cavernous sinus portion. In conclusion, we support the hypothesis that aneurysm may originate from angiographically normal arterial wall in FMD.


[Two cases of recurrent optic neuritis (OPN) and acute transverse myelopathy (ATM) with associated anticardiolipin antibodies].

We investigated anticardiolipin antibodies (aCL) by enzyme linked immunosorbent assay with adding aCL-cofactor in two cases of recurrent OPN and ATM patients. These two patients had similar clinical features with ATM and OPN during their clinical courses. They were supposed to be suffered with multiple sclerosis (MS), although cranial MRI was normal and oligoclonal IgG band (OCB) was consistently absent in the cerebrospinal fluid. Positive aCL is suggestive that this disease may be a disorder associated with aCL with different etiology and pathogenesis from other MS patients. Serologic testing for aCL with aCL-cofactor should be warranted for MS patients, especially for those showing OPN and ATM during the clinical course, because in orientals the incidence of ATM and OPN is relatively high among MS.


[A case of posterior choroidal artery infarction].

The posterior choroidal artery supplies the lateral geniculate body, the posterior thalamus and the caudate body. Currently, a few cases of infarction in this arterial territory have been reported. This is a case of 59-year-old male, exhibiting left homonymous hemianopsia and left hemiparesis. Clinically it was impossible to make a diagnosis of infarction in this area. We were able to determine that it was a posterolateral choroidal artery infarction rather than an anterior choroidal artery infarction using 1.5 T-magnetic resonance imaging (MRI). The MRI scan clearly disclosed the areas of infarction, which included the lateral geniculate body, the posterior thalamus and the caudate nucleus body. Additionally, cerebral angiography revealed an occluded proximal portion of the right posterior cerebral artery (P2) and a patent right anterior choroidal artery. Anatomically, the areas surrounding the lateral geniculate body are supplied by both the anterior and the posterolateral choroidal arteries. However, the posterolateral choroidal artery infarctions are believed to be rare due to usual dominance of the anterior choroidal artery. Presently, with the use of MRI scanning, these areas can be easily visualized. Therefore, the posterolateral choroidal artery infarction can be diagnosed more easily. It is conceivable that more cases will be accurately diagnosed using this tool.


[Multifocal axonal motor neuropathy associated with anti-ganglioside antibodies].

We report a patient with asymmetrical patchy weakness of the limbs, and with autoantibodies against gangliosides GM1, GD1b, asialo GM1. Although electrophysiological studies did not reveal conduction block, treatment with prednisolone resulted in clinical improvement. A 52-year-old man was admitted to Kyoto University Hospital, because of gait disturbance. Neurological examination revealed a patchy distribution of weakness in the limbs. Deep tendon reflex was normal at the right knee, and was depressed at the right biceps. Other deep tendon reflexes were absent. There was a slight decrease in vibratory sensation in the distal portions of the lower extremities. Routine laboratory studies, heavy metal screen, vitamin, cryoglobulin, coproporphyrin and delta-amino levulinic acid in urine, and the protein value of the cerebrospinal fluid were normal. Head and neck MRI, and myelography were normal. Immunofixation electrophoresis showed IgM lambda M-protein in serum. Thin-layer chromatography with immunostaining showed his serum IgM reacted with GM1, GD1b, and asialo GM1. ELISA (Enzyme Linked Immunosorbent Assay) demonstrated high titers of anti GM1, GD1b and low titer of anti asialo GM1. Motor conduction studies showed no demonstrable conduction block, normal conduction velocities and the low amplitudes of CMAP. Sensory conduction studies showed no abnormalities except for slightly decreased amplitude of SNAP in sural nerve. Electromyography showed active denervation in extensor digitorum communis muscle, tibialis anterior muscle and left biceps brachii muscle. Muscle biopsy specimen revealed large and small group atrophy and there was perivascular mononuclear infiltration at one point.(ABSTRACT TRUNCATED AT 250 WORDS)


[Abulia: a case of cerebral infarction in the bilateral genua of internal capsules].

A patient was presented with an outstanding symptom of abulia due to cerebral infarcts in the bilateral genua of internal capsules. A 53-year-old woman, generally in good health and active, had no contributory medical history except for hypertension. She was well until August 20, 1988, when she was noted to have become taciturn and absent-minded. In the morning, she got up and went to work as usual. Although she worked without trouble, she hardly talked with her colleagues. After getting home from work, she would lie down without doing any housework, and this was continued on the following day. However, she had no physical problems. She was thus admitted to a hospital on August 22. Lethargy and urinary incontinence were apparent for a few days. Thereafter she became awakeful and could take care of herself. She sat on her bed all the time, and could talk normally with her daughter. She was referred subsequently to the Department of Neurology, Hyogo Prefectural Tsukaguchi Hospital on August 30. On examination, the patient was alert, polite and cooperative with no physical abnormalities except for high blood pressure. Neurological examination indicated the patient to be attentive and well-oriented. Cranial nerves and eye movements were normal except for slight anisocoria and sluggish pupils. There were no muscle weakness, extrapyramidal signs, or cerebellar signs. Deep tendon reflexes were normal. Babinski signs and forced grasping were not noted. A neuropsychological study showed the patient not to be demented, aphasic, or apraxic.(ABSTRACT TRUNCATED AT 250 WORDS)


[Treatment of Guillain-Barré syndrome with "axonal" involvement by plasma exchange].

A 19-year-old man with Guillain-Barré syndrome (GBS), who had been bedridden, dramatically improved after plasma exchange despite electrophysiological evidence of severe axonal damage. We commenced the therapy at 24 days after the onset of illness. Each trial of plasma exchange improved the strength clinically and rapidly increased the amplitude of the compound muscle action potential. Four months later, muscle strength recovered to normal except for mild weakness of the distal limb muscles. The time course of such a prompt recovery precludes axonal regeneration as its underlying mechanism in our patient. We postulate that some humoral factor might have reversibly depressed the axonal excitability, causing clinical symptoms which mimic axonal degeneration. Plasma exchange is a choice of treatment even for patients with GBS showing electrophysiological findings suggestive of axonal involvement.


[A case of syringomyelia with proximal dominant muscle weakness and without superficial sensory disturbance].

A 43-year-old woman had noticed muscular weakness in her arms for four years before her admission. Muscle weakness and atrophy were prominent in the bilateral deltoid muscles, but muscular strength was almost unimpaired in the bilateral forearms and intrinsic muscles. There was no sign of sensory impairment except vibratory sensation. EMG revealed neuropathic NMU. X-P of the cervical spine showed enlargement of the spinal canal diameter, and MRI of the spinal cord revealed a large syrinx. On the basis of metrizamide CT and cranial MRI, a diagnosis of syringomyelia with Chiari malformation (type I) was made. Despite the presence of a large syrinx extending from C 1 to Th 11, the only detectable neurological sign was proximal weakness of the upper extremities simulating myopathy.


[Paraproteinemia and motor neuron disease].

In some patients, motor neuron disease (MND) is associated with a paraprotein, raising the question that there may be a relationship between MND and monoclonal gammopathy. We analysed the presence of monoclonal immunoglobulin in the serum from 53 patients with MND using immunofixation in cellulose acetate membrane. The incidence of paraproteins in 6 cases (11.3%) of patients with MND exceeds values found with cellulose acetate membrane supports in reported populations (below 1%), even when age was also considered. Monoclonal components were IgG (33%), IgM (33%) and IgA (33%). In six cases, four showed typical changes of amyotrophic lateral sclerosis and other two patients were spinal progressive muscular atrophy (SPMA) in autopsy. No malignancy was detected in all cases. These results corroborate the concept of a probable association between MND and benign monoclonal gammopathy (plasma cell dyscrasia). A patient with SPMA had transient elevation of IgG-GM1 antibodies. Other five patients with M-proteinemia did not show elevated serum anti GM1 antibodies.


[Rhythmic skeletal myoclonus without palatal myoclonus].

We reported a case of 89-year-old woman showing rhythmic skeletal myoclonus mainly on the right upper limb. This myoclonus appeared five days after the cerebral infarction. It was seen constantly both at rest and in posture, and decreased during voluntary movement. When the patient was under emotional stress, it spread to the submandibular, neck and trunks of upper limb. During sleep, this movement completely disappeared. There was no myoclonus in palato-pharyngo-laryngo-oculo-diaphragmatic muscle group. In the examination of the surface electromyography, the movement was not reciprocal between extensor and flexor muscles, and its cycle was about 3.5 Hz. It was different from the intention tremor because it did not increase during the movement phase on the finger nose test. The examination of MRI revealed a small infarction including right dentate nucleus and right superior cerebellar peduncle, and from which an infarction of the superior cerebellar artery territory was considered. Only a few cases of rhythmic skeletal myoclonus without palatal myoclonus have been reported in the literature. All of these cases had small infarction of the same region as the above case. Their myoclonus began 5 to 15 days after the onset of cerebral infarction. These periods were markedly shorter than that of intention tremor and palatal myoclonus. This fact suggest that the rhythmic skeletal myoclonus has a different mechanism from that of the palatal myoclonus.


Neurogenic arthropathy. Differential diagnosis.

Neurological affections responsible for secondary arthropathic pathology are: tabes, syringomyelia, diabetes mellitus, congenital insensitivity to pain syndrome, alcoholism, leprosy. Each of the affections shows predilection for specific joints: syringomyelia the shoulder, tabes the hip and knee, diabetes mellitus the foot, congenital insensitivity to pain the lower limb, alcoholism the shoulder and knee. The authors discuss two cases of hip arthropathy in previous dorsal myelic fractures.


Pathologic features of neurogenic arthropathy.

"Neurotraumatic" and "neurovascular" theories are still debated as a basis for the pathogenesis of neurogenic arthropathy. The observations of pathologic features described in the present study lead to the conclusion that both mechanisms, loss of deep sensation and neurovascular reflex inducing increased blood flow and active bone resorption, may contribute to the development of complete joint disorganization.


Radiographic and therapeutic aspects of neurogenic arthropathy.

The authors describe the typical radiographic aspects of neurogenic arthropathy based on an examination of 127 cases observed at the Rizzoli Orthopaedic Institute. Surgical indications for the various joints affected are discussed, and the results obtained after treatment are reported.


Cellular reactions in hip prosthesis loosening.

Based on the results obtained in histological examinations carried out on periprosthetic tissues in a large series of cases of prosthetic hip joint explants, the authors analyze the cellular events that may occur in loosening phenomena as compared to what occurs in the paraphysiological repair process that is observed in the stable prosthesis. Like other authors, they believe that the principal role in the mechanism of loosening is played by macrophages which are recalled in a large number, at times together with multinucleate giant cells, at the bone-implant interface, after micromovements of the prosthesis and the formation of wear particles have occurred. The macrophages would be capable of favoring resorption of the periprosthetic bone tissue, producing areas of osteolysis in which the transmission of the mechanical stress of loading is modified. The ensuing prosthetic instability increases wear phenomena, causes a greater amount of osteolysis, and, in a vicious cycle, loss of the relationship between bone and implant, and, thus, prosthetic loosening. Finally, the authors report a hypothesis on the pathogenesis of the phenomenon, based on which non-physiological stress, associated with wear and eventually infection, leads to loosening.


Biomechanics of cemented and cementless prostheses.

The success of the cemented arthroplasty is due primarily due to excellent initial stability, intimate contact with the prepared bone and the isoelastic properties provided by the cement. In an experimental in vitro study, the initial stability of both the cemented and uncemented femoral components was measured and it was found that they were both very stable in simulated single limb stance (maximum motion of less than fifty microns). However, in simulated stair climbing, the uncemented components were more unstable than the cemented components (maximum micromotion of 76 microns for cemented and 280 microns for uncemented), and this relative instability of the uncemented femoral components could compromise the bone ingrowth. Studies on femurs retrieved at autopsy from patients who underwent cemented total hip arthroplasty two week sup to seventeen years earlier and were functioning well, have shown that the failure of cemented femoral components is initiated primarily by mechanical factors, consisting of debonding at the cement-prosthesis interface and fractures of the cement rather than lack of bone ingrowth or fibrous tissue formation at the interface. Thus the problems with using cemented femoral components involve the poor strength of the cement-prosthesis interface and the cement, while the problems with cementless components involve the difficulties in precisely machining the femoral canal, and providing rigid stability as well as accurate fit.


Considerations on ceramic prosthesis explants.

The authors report data obtained for 11 cases of explants of prostheses having ceramic joint surfaces. The wear phenomena observed in 9 prostheses, including 1 case with breakage of the ceramic head, was moderate: 20-22,000 debris per mu 2. In 2 cases the wear was massive, with a concentration of debris in the peri-implant tissues 5-10 times greater (100,000-218,000 debris per mu 2). An explanation for the trigger mechanism of wear phenomena is proposed: the cases of massive wear are related to loosening of the acetabulum. A histological examination of the bone tissue surrounding a stem covered with alumina (Al2O3) carried out in three cases allowed us to observe an area of demineralization measuring approximately 500 mu of tissue in contact with the ceramic coating. The osteomalacia could be caused by the diffusion of alumina ions.


Clinical experience with primary cemented total hip arthroplasty.

Results obtained with 433 cemented prostheses with original design, implanted at Exeter since 1969, are presented. Clinical and radiographic findings were evaluated on 88% of cases at 7,4-13, 4-16, 4 follow-ups. Average age at surgery was 66,7 years. Sixty-two (14,3%) prostheses have been revised. Stem loosening was evident in 13 (3%) cases, of which 11 were revised. Socket loosening was evident in 21 patients (4,9%), all revised. Due to sepsis 7 prosthetic were explanted (Girdlestone) were done. Stem sinking > 2 mm into the cement mantle was observed at 16,4 years follow-up in 15% of cases, with no relation to pain. None of the patients showed a complete radiolucent line around the stem. The biomechanical relevance of the tapered design of a smooth and collarless stem for the cemented implant is discussed.


Clinical experience with primary cementless total hip arthroplasty.

One hundred-eleven patients (121 hips) treated with cementless total hip arthroplasty (Harris-Galante, Zimmer) were clinically and radiographically reviewed at an average follow-up of 67 months (55-79). 9,1% of the stems presented signs of loosening and 5 stems (4,1%) had to be revised. None of the acetabula required revision surgery for loosening. One socket was revised due to recurrent dislocation. Clinical results were evaluated according to Harris protocol: excellent 75,2%, good 12,4%, fair 5%, poor 3,3%. Ten (7,9%) intraoperative fractures of the proximal femur were observed: in 2 cases stem instability consequently occurred. Endosteal cortical erosions, not clinically evident, were observed in 8,3% of stable stems. A foreign body biological reaction to polyethylene or metallic debris is supposed as cause of erosions.


Borderline indications for use of cement in total joint replacements.

In order to delineate the borderline indications for the cemented and uncemented total hip replacements, we need to evaluate the existing success rates with these procedures. The long term success rates with cemented total hip replacements done using an intramedullary methylmethacrylate plug, doughy Simplex bone cement introduced in a retrograde fashion via a cement gun, and collared cobalt-chrome femoral components with rounded corners were reviewed at eleven year follow-up. The patients ranged in ages from 20 to 84 years (mean 57 years). Among the 105 patients in the group followed up at a minimum of 10 years 97% of the femoral components remained rigidly fixed. These results suggest that the cemented femoral components give satisfactory results to the vast majority of patients over eleven years. Further improvements in the use of cement for prosthetic fixation were made since then. The strength of the cement-prosthesis interface is now considerably enhanced by precoating the prosthesis industrially with a thin layer of methacrylate and or by using texture prosthesis. The strength of cement is considerably improved by techniques such as centrifuging the cement after mixing, or mixing the cement in vacuum. These techniques appear promising and may further enhance the fixation of the prostheses to the skeleton.


Radiographic evaluation of HDPE cemented and cementless Lord and An.C.A. screwed acetabular models.

A total of 187 alumina screwed porous-ceramic coated sockets (An.C.A.), 48 screwed smooth-surfaced Lord sockets, and 251 cemented polyethylene sockets were radiographically evaluated at an average follow-up of 30, 51 and 96 months respectively. After 6 years the Lord prostheses revealed a 38% incidence of loosening, similar to that observed for cemented sockets 10-12 years after surgery. The An.C.A. prostheses revealed radiographic loosening equal to 12% (6 cases) in the first 50 implants, and only 0.7% in the remaining 137 cases: overall, the An.C.A. acetabular prosthesis revealed an index of radiographic loosening equal to 3.3% (7/187). To guarantee "osteointegration" of the porous coating of An.C.A. sockets optimal stability must be obtained when the prosthesis is screwed in. Because the mid-term follow-up for this clinical experience is relatively short (30 months), an opinion on the reliability of the screwed "porous" sockets must await confirmation.


Bone remodeling in experimental total hip arthroplasty.

Experimental studies of cementless porous-coated total hip arthroplasty indicate that the most important design variable for femoral remodeling is stem stiffness. In the long-term (2 years) in the canine model, other variables including the presence, type and placement of the porous coating did not significantly affect the pattern of bone remodeling when tested with metallic stems. The basic pattern of bone remodeling was characterized by proximal cortical atrophy, and distal cortical and medullary bone hypertrophy. The use of low stiffness stems altered this pattern, leading to reduced proximal bone loss and no distal cortical hypertrophy.


Femoral bone remodeling: clinical experience with cemented and cementless total hip arthroplasty.

A radiographic study of femoral bone tissue remodeling around hip arthroplasty was carried out simultaneously in two series totalling 224 cementless and 77 cemented prostheses. Remodeling was analyzed by detailed visual evaluation of the radiographic assessment. The results show that the phenomenon of bone remodeling is still evolving more than 5 years after surgery. Bone demineralization of the proximal femur is more evident in cementless prostheses than in those stabilized with acrylic cement (24% vs. 12%). The preoperative state of mineralization significantly influences bone remodeling.


Charnley total hip prostheses: clinical and radiographic results after 10 to 18 years.

The work reviews 76 Charnley cemented total hip prostheses applied between 1970 and 1977 using a traditional cementation technique. Long-term results after an average of 12 years (minimum 10 and maximum 18) were excellent or good in 79% of the cases, fair in 2.6% and poor in 7.9%. The revision rate was 10.5% Cases characterized by radiographic signs of loosening were studied in detail, and correlations with clinical and radiographic data searched for.


Indications and limits of hybrid hip prostheses (cementless acetabulum and cemented stem).

The authors report the indications and short-term results obtained in a series of 99 total hip prostheses with a screwed acetabulum and cemented stem. The principles which inspired their choice and the criteria used to select patients are discussed. The results obtained, evaluated both clinically and radiographically, are satisfactory, particularly with regards to percentage of pain symptoms in the femur.


Cemented hip prostheses: modular screw system.

The long-term results of 21 patients treated with modular screw cementless hip prostheses are reported. An examination of periprosthetic osteogenesis and function evaluated by gait analysis revealed good incorporation of the prosthesis and good clinical results in all of the cases studied.


Identifit: a silicone mould used to intraoperatively construct a cementless femoral stem.

A new system named Identifit to construct a femoral stem for use in hip arthroplasty is presented: a silicone mould of the femoral canal is made, and while the surgeon proceeds to insert the acetabulum, in a laboratory located annexed to the surgical theater the mould is used to make a titanium stem in the same shape. The stem is anatomical with a mean value for bone to prosthesis contact equal to 94%. The first results obtained seem to confirm the theoretical effectiveness of this system.


Rehabilitation treatment in cemented and cementless prostheses.

Rehabilitation after hip arthroplasty is aimed at improving the results obtained with surgery and allowing the elderly patient to resume a normal daily life as quickly as possible. The correct use of the prosthesis positively influences its duration. Full weight-bearing on the limb submitted to surgery is allowed at different times: days 7-8 if the prosthesis is cemented. When the prosthesis is cementless the proper time for full weight-bearing is established by the orthopaedist, and it is usually allowed on days 30-40. Walking initially makes use of a walker, followed by parallel, and finally antibrachial support.


[Sleep disorders in psychiatric diseases. Epidemiological aspects].

Very few epidemiological surveys have specifically studied relationships between sleep disturbances and psychiatric diseases. In this review, we preferred to use the classification proposed in 1979 by the Association of Sleep Disorders Centers. It includes four main categories: insomnias, excessive sleepiness, troubles of the wake/sleep schedule and parasomnias. Evaluating psychiatric disorders among general populations is easier owing to DSM III and DSM III-R criteria, but there are not equivalent criteria in evaluating sleep disorders. It is almost impossible to realize polysomnographic recordings in large samples, therefore sleep disorders are to be detected by questionnaires. It has been shown that there is a good correlation between self-reports and polysomnographic recordings among clinical and general samples. The prevalence of insomnia, defined as difficulties of initiating and maintaining sleep, is estimated between 9 and 31%. It is higher among women, elderly people, separated and divorced subjects, and low educational levels' groups. It has to be noticed that polysomnographic records of some subjective insomniacs are not different from those of good sleepers, sleep latency excepted. These subjective (and not objective) insomniacs have high scores in anxiety scale, depression scale, or psychologic distress. Insomnia is more frequently noted amongst subjects with psychiatric diagnoses, especially major depressive disorders and anxiety disorders. Depressive disorders are present in 21-40% of insomniacs versus 0-1% of non-insomniacs, and anxiety disorders in 13-24% of insomniacs versus 3-10% of non-insomniacs. In depressive disorders, sleep alterations are frequently noted: they are difficulties of initiating and maintaining sleep, decreasing proportion of slow-wave sleep, decreasing time of REM (rapid eye movement) sleep and REM sleep latency, and increasing density of REM sleep. Of these modifications, the last two ones seem to be specific for depression. The relationships between sleep, aging and depression are more complex than previously noted. For example, differences between depressed and non-depressed subjects depend on the age of the population. The prevalence of hypersomnia is lower than the insomnia's. It varies between 2 and 4%. It is more frequently noted among young people, and never married subjects. Two specific aetiologies must be looked for: sleep apnea syndrome and narcolepsy. These diagnoses are respectively found in 45% and 24% of hypersomniacs examined in American Sleep Centers. Hypersomnias are objectived by the Multiple Sleep Latency Test, which measures the physiologic sleep tendency.(ABSTRACT TRUNCATED AT 400 WORDS)


[Sleep disorders in psychiatric diseases. Clinical and polygraphic data].

This article considers first the relationships between sleep disturbances and psychiatry in general. It shows the interest of sleep recordings in psychiatric diseases as a complementary examination, in order to clarify a diagnosis or the selection of a treatment. Sleep disturbances in the main psychiatric disorders are described: brain organic diseases, thymic disorders, schizophrenia. Modifications of sleep are also found in many other psychiatric disturbances such as anxiety or neuroses. Polysomnography is an excellent functional examination of the brain and has an increasing importance in psychiatry.


[Parasomnia].

The parasomnias comprises a group of disorders that intrude into the sleep process and are not primarily disorders of sleep and wake states per se. These disorders are manifestations of central nervous system activation usually transmitted through skeletal muscle or autonomic nervous system channels (ICSD 1990). The parasomnias are divided into four groups: Arousal disorders (sleepwalking, night terrors, and confusionnal arousal), sleep-wake transition disorders (rhythmic movement disorder, sleep starts), REM sleep parasomnias (nightmares, sleep paralysis, hallucinations, and REM sleep behavior disorders), and other parasomnias (sleeptalking, nocturnal enuresis, and nocturnal bruxism.


[Sleep apnea syndrome: psychiatric aspects].

The prognosis of sleep apnea syndrome (SAS) is dominated by cardiovascular complications. However, this disease is concerned with psychiatric field through specific clinical patterns. Nocturnal respiratory arrests are responsible for hypoxic and hypercapnic events including sleep fragmentation, reduction of slow wave sleep and rapid eye movement sleep. Alertness disturbances and sleep disorders are the most common functional complaints. Memory and cognitive disorders, personality changes and anxiodepressive syndromes may occur but are often neglected due to the increase of frequency with age together with the frequency of SAS; clinicians should consequently be aware of the fact that such troubles may be related to sleep respiratory disorders. In this case polysomnography should be performed before prescribing hypnotics, tranquilizers or sedative antidepressants.


[Consumption of benzodiazepines in a university hospital center].

Benzodiazepine consumption has been studied in an inpatient population of a hospitalo-universitary center. The different user wards were classified by their cost or the importance of their benzodiazepine use. In a second step, the authors studied the prescription in the 6 most consumer medical wards. The most prescribed benzodiazepines were lorazepam and dipotassium clorazepate (27 and 23% respectively). In these six wards, on the day of the study, 48% of the 227 inpatients were taking benzodiazepines. Fourteen out of them were taking more than one of these drugs. In 80% of the cases, the patient was asking for the prescription. Out of the 110 inpatients found to have taken a benzodiazepine on the day of the study, 74 had already regularly used it during the years before hospital admission, mainly women (64%) and old people. Finally, out of the 227 inpatients studied, the hospitalization is a possible inducer of the benzodiazepine intake and dependence in 16% of the patients. The results are discussed against the background of other studies concerning benzodiazepine consumption.


[Cesarean section: descriptive epidemiology. Increase in frequency of indications for cesarean section].

The use of caesarean section is constantly increasing in all countries of the western world. This article considers various aspects of the phenomenon, from the most frequent justifications for the increase in caesarean sections, to the consequences of this practise on the health of the mother and of the fetus/newborn infant. The often-suggested link between the increase in the number of caesarean births and the decrease of the perinatal mortality rate is also discussed. The caesarean section is potentially capable of offering large benefits, but at the same time is not without risk for the mother and the child. In order to reduce this risk meticulous attention must be paid to the anesthesiological and surgical techniques employed and surgery must be used only when absolutely necessary.


[Bulimia and epidemiology].

Despite the multiplication of the epidemiological studies related to bulimia, the prevalence of the syndrome remains difficult to estimate, due to the variance of the results. 1 to 17% of the students will be bulimic. Numerous methodological imperfections may explain this disparity. In the studies the populations differentiate in accordance with age, social or economical level or ethnic group. Furthermore many of them were not randomly selected. Additionally, a few studies draw a comparison between the respondents and the sample from which they were drawn. This comparison is very important when the response rates are low. These two factors weaken the representation of the studies. All but one of the studies evaluated subjects by means of self-report questionnaires. The terms used are often not precise enough and there is a risk that individual subjects will interpret them idiosyncratically. Moreover, a lot of these questionnaires were pioneered in the studies, and reliability and validity data are lacking. The screening instruments "BITE" and "BULIT" have been formally evaluated, although they have not been used for the epidemiological evaluations. The BEQ has a good reliability, but has a low rate of identifying false positives. Studies using these self-report questionnaires operationalize the DSM III, the DSM III-R or the Russell criterias. As far as bulimia is concerned, there exist a small relationship between DSM III and DSM III-R. Furthermore, the DSM III criteria for bulimia have been criticized for being overinclusive. The inclusion in the DSM III-R of "methods of weight control" and "a persistent overconcern with shape and weight" contribute to decrease the prevalence of the disease.(ABSTRACT TRUNCATED AT 250 WORDS)


[The impact of AIDS on mortality in Italy].

Using the official mortality data, collected by ISTAT, and the data of the Italian Register of AIDS cases, in Istituto Superiore di Sanità, three arguments have been developed: 1) Study of causes according to which ISTAT could classify deaths from AIDS: the leading cause appears to be "Deficit of Cell Immunity" (I.C.D. 279.1). 2) Study of the under-estimation of official data with respect to those of the specific system. Under-estimation decreased over the years, reaching 20% in 1987 and 6% in 1988. 3) Analysis of the general mortality trend in all of Italy and in particular metropolitan areas, with a high concentration of cases, such as Rome and Milan; there is evidence of a possible influence of AIDS deaths on mortality of young age classes particularly notable among single males.


[Psychiatric hospitalization in public wards and in private clinics. A comparative study on a population of a district of Turin from 1981 to 1988].

After the 1978 psychiatric reform law, public inpatient facilities (SPDC) and private clinics, with a higher number of available beds, coexist in the metropolitan area of Turin. Presented data, concerning the population of a specific catchment area, allow a comparison between the admissions in the two kinds of facilities. Patients hospitalized in 1981 were followed up to 1989, evaluating their contacts with the Community Mental Health Centre (SSM). A higher number of admissions in private clinics emerges, but there is no progressive increase during the considered period. The SPDC has evolved towards a sort of 'specialization' in psychotic syndromes; lengths of stay, moreover, were shorter. Public services (SPDC and SSM) seem to play a limited role in meeting the demand for psychiatric care expressed by the considered population.


[Economic efficiency and effectiveness in health care: the economist's perspective].

The debate on the efficiency of the health care system in Italy requires a developing dialogue between clinicians, epidemiologists and health economists. In this perspective the meaning of economic efficiency and its relationship with effectiveness are presented, either at production unit level either at system level. Application of criteria of efficiency is not independent from value judgements. The conflict between clinical freedom and controls on health care expenditure have ever been and it may not be eliminated, but compounded by expliciting the criteria of choice and by defining the responsibilities of health policy makers and clinicians. An example is represented by the recent Oregon's experiment. In the agenda of the future change of the Italian National Health Service, mechanisms of cost-effectiveness resource allocation criteria are suggested to be included.


[Indications for x-rays of the skull and cervical spine: an open question].

During the first six months of 1990, a study was carried out on a group of patients examined at the Neuroradiology Department of a Neurological Institute for skull and cervical spine X-rays. Kind of disturbances, the examination prescribed and patients' places of origin were taken into consideration. We studied 174 patients: 108 suffering from headache, 28 from cervical arthrosis, and the others from miscellaneous diseases. Male/Female ratio (M/F) was lower in the group affected by headache; the age was more advanced in the group affected by cervical arthrosis. Skull X-rays were usually normal in headache patients; cervical X-rays were not informative in cervical arthrosis patients. We can conclude that only through a different relationship between the prescribing physician and the Radiologist it will be possible to approach the justification and optimization criteria of medical radiological exposure.


[Mortality of psychiatric patients. A retrospective cohort study of in-patients at the Psychiatric Hospital of Reggio Emilia].

The authors report the results of historical cohort study in long-term patients of psychiatric hospitals in Reggio Emilia. The cohort was formed by 790 patients hospitalized before 1978, and has been followed-up until 31/12/'89. The results of the study are: 269 subjects deceased (34%); 117 discharges (14.8%) and 411 (52.1%) still in hospital on 1/1/'90. An excess mortality was observed in the cohort. Mortality appears to be particularly high among young patient and females.


Stabilization of severe proliferative diabetic retinopathy by long-term treatment with SMS 201-995.

Growth hormone and growth factors have been implicated in the pathogenesis of diabetic retinopathy. Hypophysectomy has been proposed as a treatment for proliferative diabetic retinopathy unresolved by panretinal photocoagulation (PPC). SMS 201-995, a long acting somatostatin analogue which slows down growth hormone secretion, may provide a non-invasive therapy for these rare cases. To assess this possibility, we studied the feasibility and efficiency of long-term SMS 201-995 treatment in diabetics. SMS 201-995 was injected subcutaneously with a continuous pump system at a dose of 400 micrograms/d into 4 insulin dependent diabetic patients suffering from proliferative diabetic retinopathy progressing despite a pan-photocoagulation. The mean age of these patients was 29 +/- 3 years and mean disease duration 18 +/- 3 years. Treatment periods lasted from 6 to 20 months (mean 15 months). Mean 24-hour growth hormone levels decreased by 57% after only one month of treatment (7.4 +/- 1.9 mU/l to 3.2 +/- 0.9 mU/l). The decline continued up to the third month. After the sixth month, signs of resistance to the drug were noted. The frequency of 24-hour GH peaks over 10 mU/l followed a parallel pattern. No rebound was observed when the treatment was progressively discontinued. In 2 patients neovascularization stopped. In the other 2 the process regressed. In all treatment had beneficial effects on the retina. Overall visual acuity improved (7.8 +/- 0.8/10e vs 5.5 +/- 0.8/10e). These effects were obtained within 3 to 6 months. Glycosylated haemoglobin levels did not change (8.8 +/- 1.3% to 9.0 +/- 0.8%). Insulin doses decreased 41% (46.5 +/- 1.7 U/d to 27.3 +/- 3.0 U/d). No severe hypoglycaemia occurred.(ABSTRACT TRUNCATED AT 250 WORDS)


[Action profile of a semisynthetic human isophane zinc insulin compared with customary isophane insulin].

Addition of zinc to a protamine-isophane insulin should probably allow to lengthen its duration of action. Therefore we compared the action of this new type of insulin (NPH Zn) with that of a classical protamine-isophane NPH insulin (NPH); both were manufactured by the same firm. Five healthy volunteers were connected to an artificial pancreas. 0.6 U/Kg of insulin was injected subcutaneously in the low left quarter of the abdominal wall. Insulin action was monitored by the glucose needs delivered by the artificial pancreas working in euglycemic glucose clamp mode. Each insulin were tested in every subject at a ten day interval. The peak plasma insulin levels were observed after 2 h 30 for the NPH insulin (40 +/- 17 microU/ml) and after 3 h (47 +/- 22 microU/ml) for the NPH Zn insulin. The glucose needs reached their top levels after 2 hours (102 +/- 19 mg.kg-1 x 15 min-1) for NPH insulin and after 3 hours (98 +/- 28 mg.kg-1 x 15 min-1) for NPH Zn; the glucose needs decreased on a parallel way for both insulins but a one hour delay for NPH Zn insulin was observed. Addition of zinc to isophane NPH insulin slightly modifies the pharmacokinecinetic parameters, moving forward one hour in the glucose delivery.


HLA DQA1 and DQB1 study in Algerian type 1 diabetes families.

The distribution of HLA class II alleles associated with insulin-dependent diabetes mellitus (Type 1) in the Algerian population is poorly known. We have typed 36 Algerian Type 1 diabetic probands and their families using DQA1 and DQB1 oligonucleotide probes. Fifty-nine parental haplotypes non transmitted to diabetic offspring served as controls. The frequencies of DQA1 and DQB1 alleles and haplotypes and their associations with Type 1 diabetes were, except minor differences, similar to those reported in French. Susceptibility DQA1 (Arg52+) and DQB1 (Asp57-) alleles were significantly increased among patients versus controls (90% vs 53%, RR = 8.4, p < 10(-6), and 94% vs 64%, RR = 9.4, p < 10(-5), respectively). 85% of Type 1 diabetics versus 34% of control haplotypes were either DR3DQw2 or DR4DQw8 susceptibility haplotypes (DQA1 Arg52+, DQB1 Asp57-) (RR = 10.8, p < 10(-7). 75% of the probands vs 14% of the controls (RR = 18, p < 10(-5)) and 73% of affected siblings versus 24% of unaffected siblings (RR = 8.4, p < 0.02) possessed a genotype composed of these two susceptibility haplotypes in the homozygous or heterozygous state. 42% of the probands were DR3DQw2/DR4DQw8, corresponding to Hardy-Weinberg expectations. The lack of excess of heterozygotes could be due to the consanguine families in this sample, as among the patients with consanguine parents the frequency of DR3, 4 heterozygotes was lower (27% vs 48% in non-consanguine patients, NS) and that of DR3 homozygotes increased (45% vs 12%, respectively, p < 0.03).(ABSTRACT TRUNCATED AT 250 WORDS)


[Insulin autoantibodies and insulin-induced antibodies in children with insulin-dependent diabetes].

The aim was to study the time course of development of insulin antibodies during the first months of treatment in diabetic children, under human insulin therapy, and to see whether the presence of insulin autoantibodies influenced the subsequent binding to insulin. Anti-insulin antibodies were measured using a radio-binding assay in 16 diabetic children, aged 4-13 years, before the first insulin injection and at regular intervals until the 9th month of treatment. Insulin autoantibodies were detected in 11 out of the 16 children at the time of diagnosis. Binding to insulin increased significantly after one month of treatment in these children, and after 2 months in the children with no insulin autoantibodies at diagnosis. After 2 months insulin therapy, all the children demonstrated antibodies against insulin. Insulin binding at 9 months was not correlated to the baseline values. Anti-insulin antibodies develop rapidly and frequently under human insulin, and are not influenced by the presence of insulin autoantibodies.


Catheter ablation of accessory pathways in patients with Wolff-Parkinson-White syndrome.

Fifty-two patients with Wolff-Parkinson-White syndrome underwent transcatheter ablation. All patients were symptomatic. Eighteen had documented episodes of atrial fibrillation, 14 of which also had reentrant tachycardias; the remaining 34 had only episodes of reentrant tachycardias. Forty-nine patients had both anterograde and retrograde conduction through the accessory pathway; 3 had retrograde conduction alone; 2 patients had 2 accessory pathways and 1 had 3. All patients were resistant or intolerant to at least 2 antiarrhythmic drugs. All patients were treated with radiofrequency current. Ablation was considered successful if the anterograde and retrograde conduction were completely abolished. Ablation was considered unsuccessful if ablation of only 1 pathway in patients with 2 or more accessory pathways and/or modification of the accessory pathway conduction without interruption was achieved. Accessory pathway ablation was successfully performed in 46 out of 52 patients (88%). Fifty out of 56 accessory pathways were effectively ablated (89%). Thirty-eight required a single session of ablation and 8 additional patients were successfully ablated during a second session. The number of radiofrequency current applications ranged from 2 to 13 (mean 4.1 +/- 2.5). The mean duration of the sessions was 4.30 +/- 1.50 hours (range 2.30 to 9). The mean radiation exposure for session was 55 +/- 25 minutes (range 20 minutes to 2.30 hours). Complications were observed in 2 out of 52 patients. One patient had a transient II degree type 1 atrioventricular block; another patient with severe arterial hypertension had a mild hemorrhagic stroke with complete neurological remission. FOLLOW-UP. Forty-five out of the 46 patients in whom ablation was successful were asymptomatic for arrhythmias during a mean follow-up of 8 months (range 4 to 16), without antiarrhythmic treatment, and without reappearance of preexcitation. One patient showed reappearance of preexcitation at electrocardiogram one month after the ablation, followed by an episode of reentrant tachycardia; this patient underwent a second successful ablation session. Our results show that ablation techniques have high success rates with no serious complications.


[Radiofrequency transcatheter ablation of anomalous left atrioventricular pathways: the role of the transseptal approach].

In this study we used two different approaches in radiofrequency catheter ablation (RFCA) of the left free wall atrioventricular accessory pathway (AP): the retrograde transaortic (TAo) approach and the transseptal (TSA) one. Our aim was to evaluate the success rate and the duration of the two procedures. From May 1, 1991 to April 30, 1992, 33 pts (23 M, 10 F; mean age 38 +/- 16 years, range 14-66) with left free wall atrioventricular AP were selected among a 57 patient population, in which RFCA was performed for arrhythmias related to the AP. In 20/33 pts (61%) stable ventricular pre-excitation was present, while in 4/33 (12%) it was intermittent; in the remaining 9/33 pts (27%) only retrograde conduction through the AP was documented. In the majority of the pts (26/33) a diagnostic electrophysiologic study was performed immediately before the ablation procedure, during the same EP test. A 7 F steerable large tip catheter was used for energy delivery. In 8/33 pts, RFCA was performed by using only the TAo approach; other 7/33 pts underwent RFCA with a TSA technique after one completely unsuccessful retrograde TAo ablation and, in the remaining 18/33 pts, the TSA approach was used electively and continuously from January 1992. Overall, the TAo procedure has been carried out in 15 cases, while the TSA one in 25 cases. In the latter group, the ablation catheter was positioned against the left atrioventricular groove through a patent foramen ovale in 5/25 cases (20%), while a TSA puncture was needed in the remaining 20 cases. After successful ablation, the observation period was prolonged up to 60 min. Complete AP ablation was achieved in 31/33 pts (94%), while the remaining 2 pts underwent surgical cryo-ablation after unsuccessful TAo procedure. Among the three different subsets of pts, the success rate was as follows: 40% (6/15 cases) by using TAo technique, 100% (7/7 cases) by TSA after one unsuccessful attempt with the TAo technique, and 94% (17/18 cases) after single elective TSA; in the only case where the first elective TSA procedure failed, a second attempt was successful. The duration of the whole electrophysiologic test was 4.0 +/- 1.3 hours for the TAo approach vs 3.3 +/- 0.9 hours for the TSA one (p < 0.05). The mean fluoroscopy time was significantly (p < 0.05) shorter in pts who underwent elective TSA (43 +/- 27 min), than in pts who underwent only TAo approach (68 +/- 42 min) or both TAo and TSA approach (157 +/- 54 min). No complication during or after the procedure was observed in any case. In RFCA of left free wall atrioventricular APs, the TSA approach seems to be as safe as the TAo approach. In this preliminary experience, the success rate and the short duration of single elective TSA procedure suggest that this can be used as a first-choice approach in these pts.


[Endomyocardial biopsy in heart transplantation: the experience of the Naples Center].

From January 1988 through August 1992, 38 cardiac transplants were performed. 385 endomyocardial biopsies (EMBs) from 32 cardiac allograft recipients were investigated. In 21 (5.45%) EMBs the specimens were inadequate for the diagnosis. In the remaining 364 EMBs the grades of acute rejection were: minimal in 213 (58.51%) EMBs, mild in 132 (36.26%), mild/moderate in 12 (3.3%) and moderate in 7 (1.93%). The most reliable histologic feature of acute rejection was the myocyte necrosis or damage in presence of pironinophilic mononuclear cell infiltrate, so our therapeutic protocol requires myocyte damage to perform an additional treatment, which was performed in 7 cases (1.93%). An intermediate grade mild/moderate, was introduced to classify the EMBs in which the myocyte necrosis was scant or not clear. In these patients another EMB was performed after 3 or 5 days. One patient died of chronic rejection 17 months after the transplant. Changes not related to acute rejection were: ischemic early or late necrosis; changes related to previous biopsy site; subendocardial infiltrate of mononuclear cells (Quilty's alteration); focal, diffuse or perimyocytic fibrosis and artefacts as contraction bands; nuclear or cytoplasmic vacuolar alterations. Some of these changes occasionally made the diagnosis hard or the specimens inadequate for the diagnosis. In spite of these diagnostic difficulties, the EMB is a safe and reliable invasive investigation which plays an important role in the management of rejection in cardiac allograft recipients.


[The PTCA of venous grafts: the immediate and long-term results].

From June 1981 to September 1991, 30 coronary angioplasty procedures were performed in 25 patients with lesions of saphenous vein grafts. The mean time between bypass surgery and PTCA was 63.1 months (range: 2-168 months). The clinical indications for PTCA were: stable angina in 25 cases; unstable angina in 2 and silent ischemia in 3. The treated lesions were localized at the proximal anastomosis site in 12 cases, in the midshaft in 11 and at the distal anastomosis in 10. Twenty-five procedures (83.3%) were successful with a mean residual stenosis of 24% +/- 9%; 1 procedure was unsuccessful and 4 were complicated (13.3%) (1 death in the catheterization laboratory; 1 non Q wave acute myocardial infarction, and 2 emergency bypass operations). The only factor predicting the immediate result was the stenosis morphology (p < 0.05). LONG-TERM CLINICAL FOLLOW-UP. The 20 patients with a first successful PTCA were followed up clinically for a mean period of 36.3 months (range: 1-120 months). There were no deaths nor acute myocardial infarctions. Ten patients (50%) remained completely asymptomatic for the follow-up period. Ten subjects (50%) had recurrence of angina, treated medically in 4 (20%) and with a repeat PTCA in 6 (30%) (for restenosis in 5 cases and for disease progression in 1). Three (15%) of these patients with a repeat PTCA had a new recurrence and were sent to surgery. Sixty-five percent of the patients where asymptomatic at the last clinical control after one or repeat PTCA. We did not find any statistically significant predictor of events (angina) at follow-up. In our experience PTCA for lesions of saphenous vein grafts is a feasible procedure with acceptable initial success and a favourable long-term outcome.


[The adenosine test in association with 99m-technetium sestamibi tomoscintigraphy in the diagnosis of coronary pathology].

The exogenous adenosine is able to provoke a coronary vasodilation, which is the same as the one provoked by the papaverine and greater than the one provoked by the dipyridamole. We report our experience in using exogenous adenosine in association with technetium-99m-sestamibi tomoscintigraphy for a diagnostic test on the coronary artery disease (CAD). We considered 22 patients (18 male and 4 female, mean age 57 years) affected by angiographically demonstrated coronary artery disease (stenoses > or = 50%). Adenosine was infused at a dosage of 0.070 mg/kg/min for 3 minutes and, if well tolerated, the dosage was increased to 0.140 mg/kg/min; in the third minute of the major dosage the radioisotope was injected. The myocardial perfusion imaging at rest was evaluated on the following day. The test was completed on 21 of the 22 patients, and 20 of the latter were subjected to the maximal dosage of the adenosine infusion. Clinically irrelevant adverse effects were observed in 20 cases; only one patient developed a II degree type 1 AV block. Angina occurred in 19 patients. Coronary angiography demonstrated significant stenosis of 35 vessels: the left anterior descending (LAD) in 14 patients, the left circumflex (LCx) in 8 and the right coronary artery (RCA) in 13. In detecting CAD, the test in our study demonstrated a sensitivity of 85% in the LAD disease, of 89% in the LCx disease and of 77% in RCA disease. The adenosine infusion associated with technetium-99m-sestamibi tomoscintigraphy demonstrated an elevated incidence of adverse effects which are of short duration and clinically irrelevant. The method was shown to be highly sensitive in detecting the CAD.


[Heart valve involvement in systemic lupus erythematosus: an echocardiographic study].

The purpose of this study was to evaluate the spectrum of morphologic and functional cardiac involvement in a selected population of patients with systemic lupus erythematosus (SLE) by means of echocardiography. Thirteen patients (2 male and 11 female) affected by SLE, mean age 41.9 years (range, 21-64), underwent M-Mode, two-dimensional and Doppler echocardiography. Eleven patients had renal disease and 3 of them were undergoing dialysis. One patient had findings of active disease. Six patients had systemic hypertension. None had a history suggestive of rheumatic fever or infective endocarditis. At echocardiographic study nine patients demonstrated findings of valvular involvement. These alterations were defined, according to the echocardiographic features, in two types: vegetation (verrucous Libman-Sacks endocarditis) and thickening. Vegetations were present in 6 patients, involving the mitral valve in all six and the aortic valve in three. The mitral valve vegetations were more frequent on the subannular portion of the posterior leaflet. Seven patients had valvular thickening: involvement of both mitral and aortic valve was present in five, and isolated mitral or aortic valve lesions in the remaining two patients. Combined valvular vegetation and thickening were observed in 4 patients. Eight patients had mild valvular dysfunction on Doppler examination: five isolated mitral regurgitation, two combined mitral and aortic regurgitation and one combined mitral stenosis and regurgitation. In agreement with previous reports, our study shows that valvular involvement in SLE is relatively frequent. Echocardiography can identify additional patterns of valvular lesions different from the known "verrucous Libman-Sacks endocarditis". The degree of valvular dysfunction is not important.


[The closure with a double-umbrella prosthesis of recanalized ducts after surgical ligation].

Four patients of 5, 27, 27 and 62 years of age, who previously underwent surgical ligation of ductus arteriosus (respectively 3, 17, 19 and 17 years before), came to our observation because of recanalization of the duct. In three cases the duct was an isolated finding, whereas in the oldest patient the duct ligation had been accompanied by implantation of a Starr prosthesis in aortic position. The diagnosis of residual shunt was based on the auscultation of a continuous murmur at the left subclavicular space and on typical signs of patent ductus evidenced by Doppler interrogation. In all cases the diagnosis was confirmed by angiography; the minimal diameter of the ducts varied between 2.5 and 4 mm. Three umbrellas of 17 mm and one of 12 mm diameter were employed for the closure of the residual shunt. The implantation of the double-umbrella device was obtained with the routine transfemoral venous approach. In the youngest patient, in order to facilitate the progression of the transseptal catheter through the duct, it was necessary to create a circuit with the guide wire from the femoral vein to the contralateral femoral artery. In all four patients the shunt was completely abolished. The oldest patient underwent replacement of a malfunctioning aortic prosthesis three days after implantation of the double-umbrella device; the surgical approach was this way significantly simplified. The implantation of a Rashkind double-umbrella device is the method of choice for closure of residual shunts through the duct after surgical ligation.


[Paroxysmal atrial arrhythmias in arrhythmogenic cardiomyopathy of the right ventricle with a familial character: the role of right atrial involvement].

Arrhythmogenic right ventricular cardiomyopathy presents a rather frequent familial occurrence. In the present study the cases of two brothers (25 and 30 years old, respectively) presenting with the peculiar finding of early involvement of right atrium, are described. Both subjects were investigated because of recurrent complex arrhythmias, including supraventricular (sustained atrial flutter, atrial tachycardia) and ventricular tachyarrhythmias (very frequent premature beats, nonsustained ventricular tachycardia). The associated echo-angiographic aspects of the right atrium and right ventricle were those typically described in arrhythmogenic right ventricular cardiomyopathy. The familial occurrence of simultaneous right atrial and ventricular involvement, to the best of our knowledge not previously described, can likely be due to the similar phenotypic expression of the same genotype. The prognostic significance of atrial involvement in right ventricular arrhythmogenic dysplasia is still to be defined and the clinical follow-up of these two cases may contribute to it.


[Acute pulmonary edema and a state of shock in a female patient with a penbutolol-treated pheochromocytoma].

A woman with "mild arterial hypertension" had a cardiogenic shock a few minutes after taking the first tablet of penbutolol. Subsequently, a suprarenal pheochromocytoma was discovered (and surgically excised, with stable recovery).


[A posttraumatic pseudoaneurysm of the radial artery. Its diagnostic assessment by duplex color Doppler].

A twenty-nine-year-old woman, one week after a traumatic skin laceration on the territory of the distal part of the radial artery, developed a pulsating mass suggesting a pseudoaneurysm of the radial artery. Using the duplex color Doppler echography, we clearly demonstrated the anatomical damage, the presence of the false lumen and the continuity of the vessel. These observations were of great assistance for the subsequent surgical treatment.


[Paradoxical embolism: a case].

We report a case of pulmonary embolism complicated by paradoxical embolism (which is rarely diagnosed in life). Foramen ovale patency is a relatively common condition and this, together with the presence of pulmonary embolism, increases the probability of paradoxical embolism.


Electrophysiological study on jaw-opening reflex recorded from digastric muscle in Parkinson's disease and primary cranial dystonias.

We investigated digastric reflex excitability in normals and in patients with extrapyramidal disorders such as primary cranial dystonias and Parkinson's disease. Relationships between exteroceptive suppression of masseter muscle and digastric reflex were also investigated in some cases. Digastric reflex hyperexcitability was observed in dystonic patients when compared to normals and parkinsonian patients. Furthermore, some patients with cranial dystonia presented absence of exteroceptive suppression reflex in masseter muscle with enlarged digastric response. These results indicate hyperexcitability of the digastric reflex and abnormal agonist-antagonist muscle co-contraction in chewing reflexes of patients with cranial dystonias. This shows that digastric reflex is an important electrophysiological investigation to explore the physiopathological mechanisms of primary cranial dystonias.


Feeding ecology of Saguinus bicolor bicolor (Callitrichidae: Primates) in a relict forest in Manaus, Brazilian Amazonia.

This study is part of a long-term ecological study of habitat and dietary requirements of the pied bare-face tamarin (Saguinus bicolor bicolor). One group was studied for 11 months in an area of secondary forest in a suburb of Manaus, Amazonas, Brazil. Three main vegetation types occurred inside the group's home range (12 ha): capoeira, older secondary forest and campinarana (white sand forest). The tamarins ate fruits (21 species), flowers (1 species), exudates (4 species), and arthropods (insects and spiders). They spent 14.3% of total activity time seeking and eating animal prey, and 9.9% feeding on plant material, mostly fruits. In general, fruits consumed were ripe, small and succulent. Trees used for feeding were low and had small crown diameters. Three plant species (Protium aracouchinni, Myrcia cf. fallax, and Couma utilis) were used intensively during the three seasons covered by the study period. The concentrated use of 3 fruit species, each for an extended period (one fruiting species per season), provided the tamarins with a regular food supply. Tamarins consumed exudates from holes in the bark of trees of the families Anacardiaceae and Vochysiaceae, as well as gum exuded from seed pods of Mimosaceae. Exudates were exploited during the dry season and at the beginning of the wet season. Group travel was primarily based on routes connecting the fruiting trees exploited, with foraging for animal prey occurring during travel. Tamarins searched for arthropods on trunks, branches and leaves and in trunk holes. The foraging and feeding tactics displayed by S. b. bicolor are closely linked to morphological characteristics (small size and weight, claw-like nails) that allowed access to energy-rich resources (arthropods and plant exudates) in different strata of the vegetation.