[Surgical treatment of biliary lithiasic disease in elderly patients].

A transversal retrolective analysis of all patients undergoing surgical treatment for cholelithiasis and choledocholithiasis between 1980 and 1987 was performed. The aim of the study was to investigate risk factors for the development of postoperative complications and mortality in elderly patients. A group of 187 patients above 70 years old were comparatively analyzed with 962 younger patients (total group: 1,149). Medical records were evaluated with emphasis to the diagnosis, associated diseases, characteristics of the surgical treatment, postoperative complications and mortality. Fifty percent of the elderly patients presented one or more concomitant diseases which significantly contributed to their operative risk. Acute cholecystitis, choledocholithiasis and cholangitis were more frequently found in aged patients (p < 0.05). In this group more patients also required bile duct exploration (p < 0.005). Major complications occurred in 16% and nine developed wound infection (5%). Operative mortality was 5.3%. In contrast, operative mortality of patients under 70 years of age was 1.5% and postoperative complications occurred infrequently. Acute cholecystitis, congestive heart failure, a history of myocardial infarction, and liver insufficiency were found as specific risk factors in the elderly.


[Usefulness of the 14C-urea marked test in the detection of Helicobacter pylori in patients with dyspepsia].

This study was undertaken to evaluate the usefulness of the urea test (UT) in the detection of Helicobacter pylori (HP) in dyspeptic patients. The UT was done in 105 patients with dyspepsia who underwent endoscopy and biopsy. Hematoxylin-eosin and Warthin Starry tissue stains were performed for HP detection, and the resulting microscopic findings were considered as the gold standard. The sensitivity, specificity, and positive and negative predictive values of the UT were of 95%, 63%, 85% and 83% when compared to the hematoxylin-eosin stain-related findings, and 99%, 79%, 93% and 96% when compared to those related to the Warthin Starry stain. We conclude that the UT is a simple, non-invasive and useful diagnostic alternative to detect HP in patients with dyspepsia.


[Usefulness of clinical, radiologic, and endoscopic studies in chronic diseases of the terminal ileum: analysis of 36 cases].

With the purpose of defining which is the most frequent chronic pathology of the terminal ileum in a reference center (INNSZ), and establish the diagnostic accuracy of the preoperative procedures used, 36 resection specimens were reviewed histopathologically. The diseases found in decreasing frequency were: Crohn's disease, tuberculosis, carcinoids, lymphomas, endometriosis and leiomyomas. Seventy-seven percent of the cases were benign and the rest malignant. The number of cases in which the preoperative diagnosis was right or included among the differential diagnosis was as follows: clinical study 44%, radiological study 48%, endoscopical study 32% and histological study by means of endoscopic biopsy 20%. The most frequent differential diagnosis were Crohn's disease, tuberculosis and intestinal lymphoma. It is concluded that chronic disease of the ileum represents frequently a diagnostic problem due to their clinical, endoscopical and radiological similarities which may only be solved by histological analysis of the surgical specimens.


[Littre's hernia with appendix in its contents. Presentation of a case].

Littre's hernia is defined as any hernial sac which contains a Meckel's diverticulum. It has been reported in association with inguinal or femoral hernias principally and this hernia is rare but well described. A case is reported of a 44 years old man programmed for repair of a right inguinal hernia and we found incidentally that the sac contained a Meckel's diverticulum and the appendix. We reviewed the literature about this pathology and comment the management. It is concluded that the best management of Meckel's diverticulum is intestinal resection and the hernia, where the diverticulum was found, should be repaired.


[Physico-chemical properties of ursodeoxycholic acid and its usefulness in hepatopathies].

The authors of this article reviewed the physico-chemical properties of bile acids, with particular attention to ursodeoxycholic acid. Based on this information they explain the rational for treating some chronic liver diseases, like primary biliary cirrhosis and primary sclerosing cholangitis with bile acids. Finally they discussed the results of the different clinical trials of ursodeoxycholic acid in chronic liver diseases.


[Knowledge, application, and diffusion of results of clinical research: the case of porto-systemic encephalopathy].

The objectives of this survey were to evaluate the medical knowledge, acceptance and application of research results, and to identify the main sources of information in our environment. Portal-systemic encephalopathy (PSE) was the specific health problem addressed. Three hundred seventy-seven attendants to the National Mexican Gastroenterology Week (december 14-19, 1991) were surveyed. The results point out: 1) Gaps in PSE knowledge, since 30% or more incorrect answers were obtained for some PSE manifestations and therapeutic measures; 2) A persistent use of measures with doubtful efficacy, which is exemplified by the regular use of neomycin by (80%) of the respondents, 3) frequent influence of the availability of therapeutic measures (68%), as the satisfaction with therapeutic response (66%) on PSE treatment patterns, and 4) important role of continuing education courses and/or scientific meetings (> 66%) and medical journals (> 57%) as sources of information.


[Hepatic surgery: 10 year experience at the Salvador Zubirán National Institute of Nutrition].

Techniques for surgical liver resections have improved in the last years. The results obtained with liver resections in 67 patients with benign and malignant tumors in a referral hospital for liver diseases in Mexico are analyzed retrospectively, with the aim of knowing the results obtained with such problems in our country. For benign diseases we operated on 36 patients with mean age of 45 years (26 were women), we had one operative death. In 31 patients with operations for malignant tumors (16 were primary liver tumors), the mean age was 52 years and 17 were men; there were eight operative deaths. Major hepatic resections are justified, the results are very satisfactory in benign disease; a major proportion of complications have to be accepted in patients with malignant disease; however, the results are good if we realize that those patients will die in few months if the tumor is not removed.


[Laparoscopic cholecystectomy. Analysis of 50 cases].

Biliary lithiasis is considered a significant health problem. Traditionally open cholecystectomy has been considered the gold standard procedure for symptomatic cholecystitis. Laparoscopic cholecystectomy has recently emerged as an alternative, but its usefulness in community hospitals is still being evaluated. Herein we reported our experience in 50 patients treated for 7 months (August of 1991, to February of 1992) by laparoscopic cholecystectomy. It has been necessary to convert one case into an open surgery. There has been no mortality. Seven patients developed right shoulder pain postoperative, it was controlled with minor non addictive analgesics. One had umbilical hematoma, another patient had a superficial phlebitis, and another one developed urinary retention. 43 patients (86%) had had 12 to 24 h hospital stancy, and were back to their normal activities in 7 days; during a 7 months follow-up no complications have been reported. We concluded that laparoscopic cholecystectomy is a safe procedure and can be done by the average general surgeon in community hospitals in a selected patient population. However, we strongly support a continuous monitoring of a protocol for patient management for record-keeping-purposes and as an educational tool.


[Diazepam versus midazolam as pre-endoscopic medication].

A prospective randomized double blind study was carried out on 60 patients undergoing elective gastrointestinal endoscopy. Patients in group A were sedated with diazepam (0.15 mg/kg) and those in group B received midazolam (0.10 mg/kg). There were no differences between the groups in age, sex, previous drug intake, level of anxiety prior to the procedure and from the endoscopist point of view the easiness to perform the studies. The group of patients who received midazolam (B) showed a significantly greater sedation, had less pain in the site of injection, their retrograde amnesia was more effective and tolerated much better the overall procedure (p < 0.05). There were no complications in either group. The cost per dosage was 2:1 greater with midazolam. It is concluded that midazolam has some advantages over diazepam used for sedation of patients undergoing gastrointestinal endoscopy. The time for recovery and cost are its disadvantages.


Contrast sensitivity in clinical practice.

We collected and evaluated the results of contrast sensitivity (CS) examination by means of Vistech chart with an arranged testing distance 208 and 420 cm covering spatial frequencies 1.15-27.25 cycles/degree (c/deg). Our test was comprised of normal population and patients with chronic renal insufficiency including the group of waiting patients, dialysed patients and dialysed patients after neuroretinopathy and also patients after kidney transplantation and those with Alport's syndrome. We gave our attention to the results of visual acuity (VA) and contrast sensitivity (CS) examination in patients after surgery for detached retina, aphakic patients and patients with artephakia. We examined and evaluated CS in patients with intraocular hypertension. All patients reached the VA values 6/9-6/6. 1. The results of examination of 100 healthy persons of different age displayed significant differences in age groups covering all spatial frequencies between the groups 21-50, 51-60 and 61-80 years whereas in sets of higher age we registered differences in the region of medial spatial frequencies only. These data served us to create control groups in the individual partial groups. 2. Patients with chronic renal insufficiency have CS significantly lowered. These examinations suggest that there is a certain relation between renal and retinal functions and that the dialyzation treatment is not able as yet to compensate fully all changes evoked by renal insufficiency. A clear tendency to normalize CS after renal transplantation is suggestive of a certain reversibility of these changes. This is valid for transplant patients with a clear lens. If opacity of the posterior cortex of the lens occurs after a long-time cortisone treatment, a substantial fall in the CS curve is registered in all spatial frequencies in spite of the VA being 6/9-6/6. Patients with neuroretinopathy have CS always significantly disturbed. These changes are reversible although this reversibility is not complete. The new way of dialyzation treatment secures a relatively rapid normalisation of pathological changes in the fundus and repair of subjective functions. At the same time we came to the conclusion that the prognostic outlooks of these patients have become distinctly better as far as their subjective visual functions are concerned. 3. Patients after surgery for detached retina displayed in all cases in the operated eye highly reduced CS in median and low spatial frequencies simultaneously with a statistically significantly lower threshold visual acuity and reduced slope of the acuity function in the diseased eye.(ABSTRACT TRUNCATED AT 400 WORDS)


[Phlegmonous colitis].

Phlegmonous inflammation of the digestive tract is a rare lesion related in the majority of the previously described cases to systemic infections and chronic hepatic diseases. Both process may promote gastric and intestinal loss of the mucosal local defenses mechanisms against bacterial invasion. The term phlegmonous enterocolitis or gastritis defines an acute inflammatory process with purulent or nonpurulent character, that selectively damages the gastric, small and large intestines submucosal layer. The intestinal lesions are more frequently located in the small portion, followed by the colonic involvement. In the present paper we describe the postmortem findings and clinical course of a case with phlegmonous colitis in a 53 years old woman with cirrhosis and S pneumoniae septicemia.


Our experience with management of epiphyseolysis in children and adolescents.

A total of 58 children is referred to with fractured growth plates of the distal forearm, proximal humerus, radial condyle of humerus, ulnar epicondyle of humerus, distal tibia, proximal tibia and the distal femur. The related injuries are divided in accord with Salter-Harris classification. The treatment of epiphyseolyses is concerned together with summarizing its results and the appropriate experience. In addition, the indications for both conservative and surgical approaches are delimitated in treating these injuries.


[Endoscopic resolution of acute dilatation of the colon secondary to edema of colonic anastomosis].

Acute colonic dilation due to anastomotic edema is rare and is not always solved with conservative treatment. Endoscopic colonic decompression was carried out in 3 patients completing the procedure with a transrectal drainage. No complications resulted from the procedure and all 3 patients did well, once motility was re-established.


Surgical treatment of vesico-renal reflux in children aged up to 3 years.

A total of 36 children age-ranged from 2 up to 34 mos (17 boys and 19 girls) with previous surgery for the vesico-renal reflux has been evaluated. This disorder had most commonly been classified to be of higher degree (IV. and V. 45%) and often showed no response on repeated conservative treatment with even stronger antibiotics. The boys (27 ureters) and girls (31 ureters) underwent the 32 antireflux operations after Anderson-Glenn and those 26 after Politano-Leadbetter, respectively. In 6 ureters, their resection and modelling after Hendren was necessary to be done. On post-operative check-out, the 6 post-antireflux Anderson-Glenn's plastic ureters revealed on miction cystography the persistent vesico-renal reflux re-operated subsequently in accord with Politano-Leadbetter. From them, in 2 ureters the post-operative stricture has occurred in the site of ureter-to-bladder healing that was transitorily secured with percutaneous nephrostomy.


[Pancreatic resections at general hospitals in Torreon, Coahuila].

A retrospective study of nine patients with pancreato-biliary neoplasias were operated in several general hospitals in Torreon, Mexico. Six had pancreatic adenocarcinoma, two ampullary carcinoma and one with common bile duct benign adenoma. We had a morbidity of 55% (5/9); three cases with pancreatic fistula (resolved with nutritional support and general measures) two had obstruction of gastricyeyuno anastomosis (one required surgical management). One patient (11%) died of massive pulmonary embolism. We have now the possibility to perform an earlier diagnosis with update invasive and non invasive diagnostic studies such ERCP, computed tomography and angiography. We are proud to have in our hospitals, intensive care units and well trained surgeons that allow us to perform such kind of specialized surgery.


Reconstruction of the penis following necrosis from circumcision used high frequency cutting current.

Penis necrosis secondary to circumcision by an electrical scalpel in a 2 years and 2 months-old boy occurred. After healing of the electric burn only stumps of the erectile bodies and strictured urethral meatus remained. The penis shaft reconstruction by corpora mobilisation and by detachment of the crura from the pubo-ischial rami was effected. This procedure gained 6 cm of a new length of the penis. To prevent readhesions to the puboischial rami the penis was buried in a scrotal tunnel. Six months later the denuded shaft was resurfaced with one split thickness skin graft and a zigzag seam on the ventral side was made. The patient voids with good stream, has spontaneous erections and nocturnal emissions. During 10-years follow-up after the reconstruction two complications, namely a stricture of the urethra and a slight dorsal tethering of the penis caused by a scarred pubic skin were treated. The problems of the a reconstruction type and resurfacing of the penis in childhood are discussed.


An educational "treatment plan" model for the general practice residency.

The need to match residency program experiences with the interests, skills, and personal goals of residents is influenced by many factors. A structured system, the Individualized Educational Plan, is described which provides a formalized means of addressing each of these needs. The development of an educational plan, similar in concept to the development of a formal treatment plan for patients, provides the basis for the system. The system provides both the program director and the resident with the means to evaluate in detail the resident's progress and the program's performance in helping the resident achieve competency.


Detecting cardiac valvular pathology in patients with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is associated with multiple cardiac complications, including valvular damage and an increased risk of bacterial endocarditis. The purpose of this study was to evaluate prospectively a group of patients with SLE for the presence of valvular abnormalities in order to assess their candidacy for antibiotic prophylaxis prior to invasive dental procedures. Of the 43 participants, 19 (44%) had echocardiographic evidence of valvular pathology; however, only seven (16%) had a physical exam consistent with pathologic valve anatomy or function. Because of the high percentage of SLE patients with valvular abnormalities, and the poor sensitivity of the physical exam, referral to a cardiologist for echocardiography is suggested prior to invasive dental care for patients with SLE. If cardiac valvular pathology is detected, antibiotic prophylaxis should be considered.


Informed consent and the geriatric dental patient.

Dental professionals are increasingly aware of their ethical responsibility to permit patients to participate in treatment decisions through the informed consent process. At the same time, growing numbers of geriatric patients present special challenges that can jeopardize the use of informed consent. Consequently, the use of informed consent with geriatric patients warrants special analysis due to complicating factors such as patient passivity and questionable competency. This article establishes the relationship between the ethical principle of autonomy and informed consent while examining factors that must be present in order for a valid informed consent to be obtained. Additionally, the article addresses informed consent issues regarding competent patients, geriatric patients with fluctuating or uncertain competency, and patients who are clearly incompetent.


Pica: a peculiar behavior with oral involvement.

Pica is a behavior commonly displayed by children, pregnant females, and individuals with mental retardation. A population of institutionalized adults with mental retardation was assessed for pica. Of this population, 10.8% were identified with this behavior. Also, this article discusses aspects of pica and describes how this behavior can affect the mouth.


Treatment outcomes for specific subtypes of dental fear: preliminary clinical findings.

In order to examine the clinical treatment outcome for dental fears, we examined 111 active patient records retrospectively using an 80-item structured format. Subjects were from three fear subtypes: needle phobic (N = 15), gagger (N = 11), and non-specific dental fear (N = 85). Mean subtype Dental Fear Survey scores ranged from 72.3 to 81.0. Patients received either behavioral therapy alone (N = 85) or behavioral therapy with a nitrous oxide or IV sedation adjunct (N = 26). Mean psychological treatment time was 3.9 hours (SD = 2.8 h) and did not differ by fear subtype (p > 0.05). All patients successfully completed initial dental treatment. Relapse of fear requiring additional psychological treatment occurred only in the non-specific fear subtype (rate = 11.2%). Patients receiving pharmacologic adjuncts to behavior therapy (nitrous oxide) experienced less relapse.


Technique for construction of dental molds for high-dose-rate remote brachytherapy.

High-dose-rate remote (HDR) brachytherapy eliminates radiation exposure hazard to personnel, allows for optimum dosage planning (dosimetry), and delivers high radiation dose close to the tumor in only a few minutes. The catheters can be incorporated into a fixed geometry within a mold, allowing for repeated treatments without the need for repeated implantation of radioactive isotopes or repeated dosimetry. This article presents the use of HDR brachytherapy in three patients to illustrate the advantages of HDR brachytherapy for the treatment of head and neck cancers in the following circumstances: (a) for outpatient therapy, (b) as an addition to or potentiation of external beam irradiation, (c) for preservation of function of regional structures, (d) for optimization of dosimetry, and (e) for palliation. It requires close interaction among the radiation oncologist, head and neck surgeon, and the dental oncologist and/or maxillofacial prosthodontist in order for optimum benefit for the patient to be achieved.


Are dental practices less productive with older patients? Comparison of alternative output measures.

This study assessed the effects of increased visits by older dental patients on alternative measures of time- and dollar-based practice productivity. The Minnesota Dental Practice Analysis System was used for analysis of data from 31 practices (12,818 patients visits) reporting increased visits by older adults between June, 1980, and December, 1984. Results suggested that the productivity effects associated with older patient visits were highly dependent on the practice output measure selected. While increased visits by patients aged 70 and over were associated with increased monetary productivity per patient visit, time and monetary productivity per practice day were found to decline. An association between increased proportions of older patients visits and increased dentist minutes per visit appeared to account for this. This econometric analysis tends to support the notion that dental practices are less productive with older patients.


An in vitro analysis of a sustained release system for the treatment of denture stomatitis.

This study investigated the feasibility of a sustained-release delivery system for the treatment of denture stomatitis using four antifungal agents (chlorhexidine, clotrimazole, fluconazole, and nystatin) incorporated into a tissue conditioner (Lynal) at zero (control), low, medium, and high concentrations. Hardness of all experimental groups increased with both concentration and time, but remained within the range of clinical acceptability (Shore A < or = 49). Mean tensile strength increased in all experimental groups and was significantly stronger than the control for at least one concentration level of each drug, p < or = 0.05. All failures for tensile-strength testing were characterized as cohesive. All drugs demonstrated release from the tissue conditioner matrix and inhibition of growth of C. albicans that was either total, dose-related, or related to incubation time prior to inoculation. All controls supported growth.


Modified orthodontic treatment goals in a patient with multiple complicating factors.

This paper reviews orthodontic care in patients who are in nontraditional categories. Specific orthodontic management of a patient who had severe hemophilia, seropositivity for anti-HIV Ab, and Hepatitis B surface antigen is reviewed. The importance of defining acceptable treatment goals in these patients is of paramount importance.


The reality of dental CAD-CAM: hype or hope?

Dental CAD-CAM has become a clinical reality. It holds great promise to help provide a higher level of service to the patient, while allowing the dentist to spend more time on patient needs than on the mechanics of restoration production. Still, this technology is not suited for every dental office. This paper discusses the technique of CAD-CAM, its potential and its limitations so the dentist can better evaluate the appropriateness of this new modality for his or her own office.


A chemomechanical system for caries removal and glass ionomer cement to restore fixed partial denture abutments.

When a fixed partial denture fails due to recurrent caries under the casting of the abutments, a remake process usually requires a great deal of cooperation, multiple lengthy appointments, and financial resources. Many patients with special needs may not have the cooperation, tolerance, and resources to support such treatment. This paper describes an alternative method that utilizes a chemomechanical system for caries removal followed by a conventional restorative material to treat those patients.


Personality patterns of Texas dentists: a study using the bi/polar theory.

Are individuals with certain personality styles uniquely attracted to dentistry? A recent study with 218 Texas dentists using the Bi/Polar Inventory of Core Strengths showed that the dentists were more thinking (57.3%) than risking, profoundly more practical (78.0%) than theoretical, and more independent (67.9%) than dependent. Compared with the general population, dentists were significantly more thinking, practical and independent. Of the eight personality styles, Pattern II (thinking-practical-independent) (29.4%) was the only pattern significantly over-represented among the dentists compared with the general population. However, Pattern I (thinking-practical-dependent) (17.4%) was also over-represented at the trend level. Significantly under-represented among the dentists were Pattern V (risking-practical-dependent) (6.9%) and Pattern VI (risking-theoretical-dependent) (3.2%). Results suggest at least some personality influence on attraction to dentistry.


Family member and guardian acceptance of dental services for nursing home residents.

The decision to utilize dental treatment often rests with a nursing home resident's family member or guardian, and factors associated with family member/guardian acceptance of dental services for residents have not been identified. This paper reports the results of a study which found that nearly 66% of nursing home residents' next of kin identified themselves as being primarily responsible for making health care decisions for the residents. Among these 109 resident/next of kin pairs, utilization of dental services by the residents was associated with younger next of kin, next of kin with higher levels of education, and next of kin who perceived dental need for the resident. In addition, residents who were female, physically mobile, not mentally alert, dentate, and had fewer years of education had dental services accepted for them at a higher rate than did residents who were male, physically immobile, alert, edentulous, or had more years of education. Findings suggest that more effort should be directed at educating the next of kin of nursing home residents in the value of dental care among the elderly.


Development of the principle of guided tissue regeneration.

Guided tissue regeneration (GTR) is a new treatment principle in surgical therapy, developed from the result of studies in experimental animals. It implies that only those types of cells with the capacity of producing regeneration are allowed to invade the surgically treated lesion during healing. This is accomplished by the placement of a physical barrier with excludes undesirable types of tissue. Healing of periodontal bony lesions can be obtained predictably by treatment according to the principle of guided tissue regeneration but several studies have suggested that the same principle can also be applied successfully in other fields of dentistry. In implantology, the method has been used for immediate placement of implants into extraction sockets, for healing of peri-implant bony defects and for augmentation of atrophic alveolar ridges.


Guided tissue regeneration and GTAM for periodontal regenerative therapy, ridge augmentation and dental implantology.

In dentistry, the goals of regenerative therapy are to gain new attachment around natural teeth, improve the aesthetics and ridge form in cases of collapsed or deformed ridges and increase the amount of available bone for osseointegrated implants. The purpose of this paper is to describe the dental and periodontal applications of the principles of guided tissue regeneration (GTR) and guided tissue augmentation material (GTAM) therapy. Successful results can be achieved around periodontally diseased natural teeth, however, the procedures are not fully predictable at this time. Correct membrane placement and proper choice of defect configuration in the use of GTR can enhance clinical attempts to gain new attachment. Regenerative therapy can be utilized to augment edentulous ridges and improve ridge-pontic relationships as well as improve aesthetics in ridge abnormalities. Edentulous ridges augmented by GTR can have increased amount of bone height and width for endosseous implant placement. In areas where non-mobile implant fixtures have lost some osseous support, GTR has demonstrated successful gain of new bone support.


Implant prosthodontics--an adjunct in periodontal prosthesis. Part I and II. Diagnosis, treatment planning, and sequencing of therapy.

Periodontal prosthesis can be defined as "those restorative and prosthetic endeavors that are absolutely essential in the treatment of advanced periodontal disease". The sequence of therapy is planned so that the basic contributing factors of inflammation and trauma from occlusion are brought under control. Experimental and clinical research over the last decade has shifted the focus of periodontics towards increased use of guided tissue membrane techniques. Today, as a result of the longitudinal studies on the viability of endosseous implantology, implants used as an integral part of Periodontal Prosthesis now offer the patient and dentist a more stable and predictable restoration. These articles focus on the comprehensive treatment of the partially edentulous situation. The first two parts address diagnosis, treatment planning and treatment sequencing in the overall management of the compromised dentition.


Biocompatibility and biofunctionality of implanted materials.

The mechanical and chemical properties of metals, such as titanium, titanium-based alloys and cobalt-based alloys, and ceramics, such as Bioglass and calcium phosphate, make them suitable for implant applications. However, several factors affect the biologic response to these implanted materials. The predominant tissue found at the implant interface is affected by implant stability, material biocompatibility, and implant design and implant placement into the surgical site. Improvements in implant design and surface preparation may improve implant longevity and fixation for all implants materials.


Monitoring the periodontal microbiota as an adjunct to periodontal therapy: rationale, interpretation of test results and application to patient management.

Recent developments in our understanding of the relationship of the oral microbiota to periodontal diseases have led to improved approaches to controlling the pathogenic influence of certain bacterial species. In addition to non-specific control of bacterial accumulations in the sulcus region, antibiotics may be useful in suppressing persistent species with pathogenic potential. This approach is indicated in cases that do not respond adequately to mechanical plaque control measures or certain categories of periodontal diseases likely to be caused by a specific infection. Monitoring the composition of the microbiota before, during and after antimicrobial therapy is helpful in the selection of appropriate antimicrobial agents, monitoring the effectiveness of the therapy and detecting the return of undesirable microorganisms.


Physiology of osseous and fibrous integration.

The adaptation of bone to dental implants by rigid osseous integration is believed by many to be the hallmark of clinical success. However, many implants that are slightly mobile with a fibrous interface also function well in clinical application. Physiologically, why does bone respond by rigidly integrating one implant and producing a fibrous interface with another? The healing mechanism for rigid osseous integration is well known and accepted. Conversely, the development of a fibrous interface is incompletely understood. This article suggests a unified concept of the interrelationship between fibrous and osseous integration.


The biology and morphology of the implant-tissue interface.

The replacement of teeth in fully partially edentulous patients with dental implants has become an acceptable standard of care. The improved efficacy and success of dental implants has been the result of rigid adherence to the prerequisites for osseointegration. This direct bone-to-implant contact has been described histologically and the success or failure of dental implants may depend upon the relative percentage of this contact. Connective tissue and epithelial interactions with implant surfaces have also been characterized, however, to a lesser degree. Connective tissue morphology was shown to be dependent upon the type of implant surface, i.e. smooth versus porous. The epithelial interactions with implants seems to be similar to those around teeth. The formation of a peri-implant sulcus lined coronally with a sulcular epithelium and more apically with a junctional epithelium has been demonstrated. A better understanding of the interactions between the implant surfaces and the surrounding tissues may enhance treatment results.


GMS syndrome: a new dominant condition with goniodysgenesis, mental retardation, and short stature.

We describe a mother and daughter with a distinct phenotype that is different from previous reports. This is likely to constitute a new syndrome for which we propose the mnemonic GMS for G goniodysgenesis, M mental deficiency, and S short stature. The pattern of occurrence is compatible with either autosomal dominant or X-linked inheritance.


Rett syndrome: a search for gene sources.

A series of 77 Swedish females with classical Rett syndrome were genealogically traced as far back as possible, in most cases to 1720-1750, or 7-10 generations. Details were collected concerning approximately 8,000 ancestors. Common ancestry was seen in 2 pairs of females with Rett syndrome. Thirty-nine of the 77 Rett females were traced to 9 small and separate rural areas, and 17 pairs even came from the same farm or homestead. The common origin was found equally often among descendants of the father as of the mother. In 9 cases, the father came from one and the mother from another of the 9 specific "Rett areas." These observations, combined with the finding of a raised rate of consanguineous marriages in the paternal as well as in the maternal ancestry, point to a genetic transmission. Analyses of parental ages at birth and of birth order gave normal results.


Deletion of chromosome 15pter-->q11.2 due to t(Y;15) in a boy with Prader-Willi syndrome.

Chromosome analysis of lymphocytes from a patient with the clinical presentation of Prader-Willi syndrome showed the presence of 45 chromosomes, including a der(Y) resulting from an unbalanced t(Y;15)(q12;q11.2). In situ hybridization using DYZ3 and DYZ2 showed positive signals at the paracentromeric region on the short arm and at the heterochromatic region of the long arm of the Y chromosome, respectively. The Prader-Willi syndrome in this patient is caused by the deficiency of a very small region involving 15cen-->q11.2.


Kenny-Caffey syndrome in two sibs born to consanguineous parents: evidence for an autosomal recessive variant.

We report on 2 sibs with manifestations of the Kenny-Caffey syndrome born to normal, consanguineous parents. Clinical manifestations included dwarfism, internal cortical thickening and medullary stenosis of tubular bones, poorly ossified skull bones, and hypocalcemia. The younger of the two died during a tonic convulsion. The older had neonatal hypoparathyroidism and is now a short intelligent, 1-year-old child. This family gives new support to the existence of an autosomal recessive variant of the syndrome.


Phenotypically dissimilar hypophosphatasia in two sibships.

Autosomal dominant and autosomal recessive forms of hypophosphatasia have been reported; generally the clinical picture runs true to form in families. In each of 2 kindreds, 2 sibs were clinically affected by hypophosphatasia to a markedly different extent. One set of sibs showed the lethal (perinatal) and infantile forms. The other showed the dental and adult forms. In both families there was consanguinity, albeit distant, and clinical expression in sibs supporting autosomal recessive inheritance.


Thanatophoric dysplasia in monozygotic twins discordant for cloverleaf skull: prenatal diagnosis, clinical and pathological findings.

We present male monozygotic twins with thanatophoric dysplasia (TD) type I concordant for long bone abnormalities and discordant for cloverleaf skull. The twins were the product of the second pregnancy of unrelated parents, with advanced paternal age. Prenatal diagnosis and postmortem examination showed severe rhizomelic shortness of limbs, bowing of the long bones with "telephone-receiver" femora in both twins, and cloverleaf skull and hydrocephalus in one of them. It is now accepted that most of cases of TD, such as in the present report, represent an autosomal dominant mutation with a high new mutations rate.


Clinical, biochemical, and neuropsychiatric evaluation of a patient with a contiguous gene syndrome due to a microdeletion Xp11.3 including the Norrie disease locus and monoamine oxidase (MAOA and MAOB) genes.

Norrie disease is a rare X-linked recessive disorder characterized by blindness from infancy. The gene for Norrie disease has been localized to Xp11.3. More recently, the genes for monoamine oxidase (MAOA, MAOB) have been mapped to the same region. This study evaluates the clinical, biochemical, and neuropsychiatric data in an affected male and 2 obligate heterozygote females from a single family with a submicroscopic deletion involving Norrie disease and MAO genes. The propositus was a profoundly retarded, blind male; he also had neurologic abnormalities including myoclonus and stereotopy-habit disorder. Both obligate carrier females had a normal IQ. The propositus' mother met diagnostic criteria for "chronic hypomania and schizotypal features." The propositus' MAO activity was undetectable and the female heterozygotes had reduced levels comparable to patients receiving MAO inhibiting antidepressants. MAO substrate and metabolite abnormalities were found in the propositus' plasma and CSF. This study indicates that subtle biochemical and possibly neuropsychiatric abnormalities may be detected in some heterozygotes with the microdeletion in Xp11.3 due to loss of the gene product for the MAO genes; this deletion can also explain some of the complex phenotype of this contiguous gene syndrome in the propositus.


Deletion of the proximal short arm of chromosome 8.

We report on a 5-month-old boy with a de novo interstitial deletion of the proximal short arm of chromosome 8 (p21p11.2). He manifested bilateral cleft lip and palate, and apparent hypogonadism. Four previous case reports with similar deletions (p11.1p21) were associated with hypogonadotropic hypogonadism [Beighle et al., Hum Genet 38:113-121, 1977] and hereditary spherocytosis (HS) [Chilcote et al., Blood 6:156-159, 1987; Kitatani et al., Hum Genet 78:94-95, 1988; Lux et al., Nature 345:736-739, 1990]. Our patient has no demonstrable red blood cell abnormality, suggesting that the gene for HS is located in the region 8p11.1 to 8p11.2.


Ectopia lentis and aortic root dilatation in congenital contractural arachnodactyly.

Congenital contractural arachnodactyly (CCA) was described by Beals and Hecht as an autosomal dominant disorder distinct from Marfan syndrome and comprising joint contractures, arachnodactyly, scoliosis, and a distinct "crumpled ear" deformity. While the disorder is similar to Marfan syndrome, it was split from it due to the distinct physical appearance of the patients and, more importantly, the lack of heart and eye findings. Since the original report, several CCA patients have been found to have mitral valve prolapse, structural cardiac anomalies, and occasionally aortic root dilatations similar to those seen in Marfan syndrome. We report on a patient with CCA with bilateral ectopia lentis and aortic root dilatation. Our review of the literature of CCA showed that serial echocardiography and careful eye examinations have not become a standard of medical practice in this condition. Partly this may be due to a lack of documented cases of CCA having severe ectopia lentis and cardiac complications. This patient underscores the need for periodic eye and echocardiographic evaluations of all CCA patients.


Stable ring chromosome 21: molecular and clinical definition of the lesion.

Ring chromosome 21 results in deletions of chromosome 21. We report on a cytogenetic and molecular analysis of a 4-generation family segregating a stable ring chromosome 21 in 4 relatives. To investigate the molecular structure of the ring chromosome, we have analyzed the DNAs of the transmitted ring in a mother and her daughter. The daughter presented at the age of 2 years with severe growth retardation and microcephaly, whereas her mother had microcephaly but normal intelligence. High resolution chromosome analysis of both cases showed the ring chromosome to be r(21)(p13q22) resulting in deletions of 21p and 21q22. The molecular content of the ring chromosome was determined using quantitative Southern blot analyses of 5 random DNA sequences and 4 expressed genes assigned to chromosome 21 and mapping in the region of q22.3. We have shown that collagen type VI, alpha 2 (COL6A2,) S100 protein, beta polypeptide (neural), (S100B), and D21S44 are present in only one copy in both ring carriers, while CRYA1, CBS, D21S43, D21S42, D21S41, and D21S39 are present in two copies. These data and the breakpoints defining the deletion in these patients show that deletion of COL6A2 and S100B is compatible with normal function and confirm the physical map of 21q22.3 by placing COL6A2, S100B, and D21S44 in very distal 21q22.3. Patients with such small deletions provide unique models for understanding the biological and clinical significance of aneuploidy for specific expressed genes.


De Barsy syndrome: report of a case, literature review, and elastin gene expression studies of the skin.

Several "progeroid" syndromes have now been identified. The De Barsy syndrome is an autosomal recessive syndrome of dwarfism, mental deficiency, an "aged" appearance at birth, abnormal elastic fibers on skin biopsy, and lax skin, large helices, eye abnormalities, lax joints, hypotonia, and athetoid posturing. We report one case and review 11 cases from the literature. To understand the abnormal appearance of the elastic fibers on biopsy, we performed elastin gene expression studies on fibroblasts cultured from our patient's skin. Molecular hybridization studies revealed reduced elastin mRNA steady-state levels as compared with age matched control individuals. Assuming normal rates of mRNA translation, reduced elastin synthesis would occur. Diminished dermal elastin content could explain the altered cutaneous elasticity, decreased elastic fibers in the skin, and many clinical manifestations of individuals with this condition.


Alagille syndrome with de novo del(20) (p11.2).

We report on an Arab boy with Alagille syndrome and a de novo deletion of the short arm of chromosome 20 with a 46,XY, del(20)(p11.2) chromosome constitution. Other reported cases are briefly reviewed.


Congenital hypothalamic hamartoma syndrome: nosological discussion and minimum diagnostic criteria of a possibly familial form.

We report on congenital hypothalamic hamartomas, discovered at autopsy in 3 unrelated fetuses. In the first 2 patients, the tumor was associated with skeletal dysplasia only. In the third patient, it was part of a non-random congenital malformation association, suggestive of Meckel syndrome. In one family, a previous boy died soon after birth with similar craniofacial and skeletal abnormalities. As far as we know, the association between isolated skeletal dysplasia and congenital hypothalamic hamartomas has not yet been documented in the literature. Nevertheless, a spectrum of skeletal abnormalities has been described in association with congenital hypothalamic "hamartoblastoma" and a constellation of variable visceral malformations under the eponym of "Pallister-Hall syndrome" (PHS). A detailed analysis of the PHS reported cases shows that only skeletal dysplasia and oro-facial abnormalities are present constantly. They show similarities with those found in our first 2 cases. These findings prompt us to consider skeletal dysplasia and oro-facial abnormalities as common denominator and minimum criteria required to define a nosologically distinct, possibly familial entity, which we suggest calling "congenital hypothalamic hamartoma syndrome" (CHHS).


Genetic analysis of cleft lip with or without cleft palate in Madras, India.

We performed a genetic analysis of 331 non-syndromic cleft lip with or without cleft palate (CL +/- P) proband families ascertained in Madras, India. Predictions of the multifactorial threshold (MF/T) model are tested; goodness-of-fit tests of the MF/T model and complex segregation analysis are also utilized to clarify the genetic etiology of CL +/- P in this study population. There was little evidence for the MF/T model. The most reasonable conclusion from mixed model analysis is that of a major locus with reduced transmission probability. This is not altogether surprising if manifestation of CL +/- P also depends on in utero exposure to harmful environmental agents during the critical period of facial development, as suggested by Melnick et al. [1980] and demonstrated in an animal model of CL +/- P [Melnick et al., 1981]. Further the results in the Madras population are quite similar to those in other populations of Europe and Asia.


Autosomal dominant inheritance of tetramelic monodactyly.

We report on tetramelic monodactyly with autosomal dominant inheritance. The propositus and all affected relatives had only digital malformations as determined by physical examination or history. Since it could not be determined that the involved digits of the propositus or relatives were fifth fingers and toes, we conclude this to be the first report of this type of hand and foot malformation with autosomal dominant inheritance.


Head circumference of children with Down syndrome (0-36 months)

This study provides statistically appropriate head circumference reference curves for males and females with Down syndrome (DS) from birth to 36 months of age. A total of 239 males and 182 females from five study populations, yielding a combination of cross-sectional and longitudinal data, were used for the analysis. The method of least squares was used to test the fit of the growth model y = a+bx+c[log(x + 1)], where x is age in months. These standardized curves should provide information of value in the medical, physical, and developmental management of children with DS.


Cockayne syndrome: review of 140 cases.

To define diagnostic criteria for Cockayne Syndrome (CS) and to identify in detail the complications of the condition, a comprehensive review of 140 cases of CS was performed. Criteria required for the diagnosis include poor growth and neurologic abnormality; other very common manifestations include sensorineural hearing loss, cataracts, pigmentary retinopathy, cutaneous photosensitivity, and dental caries. The mean age of death in reported cases is 12 3/12 years, though a few affected individuals have lived into their late teens and twenties. Prenatal growth failure, congenital structural eye anomalies, severe neurologic dysfunction from birth, and the presence of cataracts within the first 3 years of life are predictors of severe disease and early death. In contrast with other disorders of chromosome or DNA repair, cancer has never been reported in a classical CS patient, and there appears to be no predisposition to infectious complications. The wide spectrum of symptoms and severity of the disease suggest that biochemical and genetic heterogeneity exist. CS is an uncommon but devastating genetic condition which will be better understood as the biochemical interrelationships between DNA replication and repair, and between growth, homeostasis, and oncogenesis are unraveled.


Tracheal agenesis with diaphragmatic hernia.

A case is presented of a newborn infant with Type III tracheal agenesis and a right diaphragmatic hernia which represents the first report of this occurrence. Associated multi-organ anomalies and possible embryologic relationships are discussed.


Progressive bilateral nasal alar collapse: a dominantly inherited trait.

We describe a 47-year-old woman with progressive bilateral collapse of the alae nasi first noted at age 16 years. Her dizygotic twin daughters have similar nasal collapse beginning at age 20 years. This condition appears to be inherited as a dominant trait. Although plastic surgical correction has been successful for a phenotypically similar condition due to trauma, surgical correction must be considered cautiously in individuals with an atraumative, possibly inherited, progressive form of the disorder.


Pentalogy of Cantrell and associated midline anomalies: a possible ventral midline developmental field.

Five cases of the Pentalogy of Cantrell (PC), ascertained through the Baltimore-Washington population-based study of infants with congenital cardiovascular malformations, represent a regional prevalence of 5.5/1 million liveborn infants for this disorder. Three of these patients had cleft lip with or without palate. Review of the reported literature of the Pentalogy of Cantrell and various combinations of the anomalies within the spectrum of this pentad suggests that the PC defines a specific midline ventral developmental field. Cleft lip with or without cleft palate and encephalocele tend to specifically associate with ventral midline anomalies within the spectrum of PC. These associations might either illustrate the previously observed tendency of specific occurrence of certain combinations of midline defects or represent defined subunits of the midline developmental field.


Agonadism in a 46,XY patient with CHARGE association.

We report on an infant girl born with findings of CHARGE association who proved to be a genetic male (46,XY) on cytogenetic study. Further investigation of the genitalia demonstrated partially female internal organs but absence of gonads by ultrasonography, hormone studies, and absence of ZFY by DNA probe of Yp. Pelvic exploration confirmed lack of gonadal tissue and uterus. Facial phenotype was compatible with CHARGE appearance.


Long-acting local anesthetics in dentistry.

Long-acting local anesthetics have proved to be effective for the suppression of both intraoperative and postoperative pain. They are useful for lengthy dental treatments and for prevention of severe pain following many types of surgical procedures. Although the currently available long-acting local anesthetics for dentistry have minimal side effects in the doses usually employed, there are potential problems. Bupivacaine, for example, can cause significant cardiac depressant and dysrhythmogenic responses. Etidocaine has less pronounced effects on the cardiovascular system, but its use may be associated with inadequate control of intraoperative bleeding. A new long-acting local anesthetic, ropivacaine, appears to offer advantages over either of the currently used long-acting agents.


Ketamine: a review of its pharmacologic properties and use in ambulatory anesthesia.

The administration of intravenous agents is the most commonly used method in Canada and the United States to produce sedation or general anesthesia for dental procedures. Ketamine, a dissociative anesthetic, has several advantageous physical, pharmacokinetic, and pharmacodynamic properties. It can be used to induce anesthesia, sedation, analgesia, and amnesia. Ketamine can maintain functional residual capacity, induce bronchodilation, and avoid cardiovascular depression. However, adverse effects have been demonstrated, such as cardiovascular stimulation and unpleasant emergence phenomena, both of which may be modulated by supplementation with benzodiazepines. An increase in the use of ketamine for ambulatory anesthesia has recently been advocated. This review of the literature supports the use of ketamine as an effective agent for selected anesthetic procedures.


Serum potassium after enflurane-succinylcholine induction of anesthesia in children receiving rectal midazolam as premedication.

The administration of succinylcholine causes an increase in serum potassium (K+) concentrations in healthy patients. The purpose of this study was to investigate serum K+ changes following intravenous succinylcholine in children and to evaluate the effect of rectal midazolam pretreatment on these changes. Forty healthy children between the ages of 2 and 7 yr, and who were to undergo oral surgical procedures under general anesthesia were randomly assigned to receive either placebo (saline) or 0.25, 0.35, or 0.45 mg/kg midazolam administered rectally as premedication 30 min before induction of inhalational anesthesia. Blood was drawn after induction with enflurane and at 1, 2, 3, 4, and 5 min after administration of 1 mg/kg succinylcholine to determine changes in serum K+. Although the results indicate a significant increase in serum K+ after succinylcholine in all groups, midazolam pretreatment failed to cause any observable attenuation in the hyperkalemic response.


Responses to intravenous sedation by elderly patients at the Hokkaido University Dental Hospital.

Geriatric patients who undergo intravenous sedation require careful intraoperative management, because respiratory and circulatory depression usually accompanies the administration of recommended adult sedative doses. This study examined results when a single benzodiazepine, diazepam or flunitrazepam, was carefully titrated to a clinical endpoint of conscious sedation. A total of 335 cases was divided into seven age groups. Mean sedative doses gradually decreased with age. The 60- to 69-yr group required about 75% of the adult recommended dose, the 70- to 79-yr group required 40% to 60%, and the 80- to 89-yr group required 30% to 45%. Pulse oximetry values also declined with age; respiratory depression was observed mainly in elderly patients. Declines in blood pressure after benzodiazepine administration were not correlated with age. The rise in blood pressure normally observed intraoperatively was suppressed both in young as well as old patients. We conclude that intravenous conscious sedation in elderly patients reduces stress-induced cardiovascular stimulation and that respiratory depression may occur at even low sedative doses.


Anesthetic effect of EMLA occluded with Orahesive oral bandages on oral mucosa. A placebo-controlled study.

The efficiency of a topical anesthetic occluded with Orahesive Oral Bandage was investigated. Experimental pain was provoked by needle insertions into two palatal test areas in 20 healthy subjects. Pain, estimated on a 100-mm visual analogue scale (VAS), decreased significantly from 23.5 mm to 10.5 mm at the greater palatine foramen and from 51.5 mm to 35.0 mm at the incisive foramen after application of a eutectic mixture of local anesthetics (EMLA). No significant change in pain perception was obtained after placebo application. The EMLA cream and the Orahesive Oral Bandages were well accepted by the subjects, as only two out of 20 subjects experienced slight gagging reflexes and only three considered the taste unpleasant. No other adverse reactions were observed. Occlusion of topical anesthetics seems to be a useful technique for achieving superficial mucosal anesthesia.


Comparison of propofol infusion and isoflurane for maintenance of anesthesia for dentistry in mentally retarded patients.

A continuous infusion of propofol following an induction dose of 2 mg/kg was compared with thiopental/isoflurane for the induction and maintenance of anesthesia in 20 mentally retarded outpatients undergoing routine dental procedures. The infusion rate of propofol and the concentration of isoflurane were adjusted to maintain the heart rate and blood pressure within +/- 25% of the baseline values. Postoperative wakefulness was assessed using a 100-mm visual analogue scale at the time of extubation and at 5, 10, 15, 30, 60, 90, and 120 min after extubation. Both agents provided adequate anesthesia for the treatment, and no major adverse reactions occurred. Recovery was more complete during the first hour after extubation in the propofol group, and these patients were discharged earlier.


Adverse reactions associated with norepinephrine in dental local anesthesia.

Two cases are presented in which a 40-yr-old woman and a 20-yr-old woman developed severe headaches after injection of the local anesthetic Xylestesin-F (3% lidocaine with 1:25,000 norepinephrine). It is suggested that these severe headaches may have been associated with acute transient hypertensive episodes following injection of the local anesthetic. A review of the literature indicates that norepinephrine, at least at a concentration of 1:25,000, should be avoided because of its potential for cardiovascular disturbances.


[Adverse prognostic influence of diabetes mellitus and hyperglycemia on the clinical course of cerebral infarction].

We accessed the potential impact of diabetes mellitus and hyperglycemia on early prognosis in a study of 109 cases of CT proved brain infarction. Main end-points of this study were 30-days case-fatality rates and the probability of early discharge or prolonged hospitalization. Proportions of patients exhibiting diverse evolutions were compared by Fisher's test and, when the number of observations permitted, by chi-square test. Diabetes mellitus was diagnosed by history in 15.6% (17 patients). In 86 cases analysed within 72 hours of admission, hyperglycemia (glucose level higher than 110 mg/dL) was detected in 67.4% (including all 14 diabetic patients (35.3% vs 21.7%; p = 0.18), but these patients probably were discharged early less frequently (23.5% vs 51.1%; p = 0.06). Compared to the normoglycemic patients, hyperglycemic patients also fared worse, when considering altogether the early discharge and case fatality rates and the need for prolonged hospitalization (p = 0.06). They also exhibited a nonsignificant increase in early case fatality rate (31% vs 15.4%; p = 0.21) and a markedly reduced chance of obtaining early hospital discharge (37.9% vs 65.4%; p = 0.03). Case fatality rates were maximal in diabetic hyperglycemic patients (42.9%) and seemed intermediate in non-diabetic hyperglycemic patients (27.3%). Considering the three end-points evaluated, the apparent differences between non-diabetic hyper and normoglycemic patients did not reach statistical significance (p = 0.19). These results suggest that both diabetes mellitus and hyperglycemia "per se" have deleterious effects on the clinical evolution of patients with cerebral infarction.(ABSTRACT TRUNCATED AT 250 WORDS)


In non-diabetic patients, the appearance of hyperglycemia in the acute phase of stroke is related to the extension of cellular injury, and hence to the physiologic stress response. In animal models of ischemic insult, the deleterious effects of hyperglycemia depend heavily on the production of lactic acid "via" activation of the glycolytic anaerobic pathway. The abnormal production of lactic acid and consequent tissular acidosis appear mainly in the early post-reperfusion period, or in states of marked but partial reduction of blood flow. A direct reduction of cerebral blood flow and, perhaps, the production of a hyperosmolar state may contribute to worsening of the ischemic injury. In diabetic patients, previous hemorrheologic and microcirculatory changes, and a greater susceptibility to infections may additionally reduce the chances of complete recovery after stroke.


Follow-up study of macrocephalic children with enlargement of the subarachnoid space.

Eighteen macrocephalic children with enlargement of the subarachnoid space (ESAS), with or without mild ventricular dilatation, were followed prospectively to a mean age of 56 months. All were born at term, with uneventful neonatal period and negative tests for congenital infections. There were 17 boys and 1 girl and the mean follow-up period was 46 months (8-58 months). The initial neurologic evaluation, between ages of 2 to 33 months, disclosed abnormalities in 2 cases. At the follow-up one was still abnormal and the other had a normal neurological examination. Another child, who had a normal neurological examination at the age of 5 months, at the age of 7 years and 7 months had an IQ of 77. Thus the abnormality rate at follow-up was 11%. The OFC returned to the normal range in 45% of the children at the follow-up period. There were no cases of intracranial hypertension. One infant had subdural taps performed at the age of 13 months that disclosed a fluid with the same characteristics as the CSF. All the children had a CT-scan performed at the beginning of the study that revealed a large subarachnoid space; in 77% it was associated with mild ventricular dilatation. Eleven had CT-scans repeated, during the study period, which showed resolution of the process in 3 cases, improvement in 2, and unchanged in 6. We conclude that enlargement of the subarachnoid space in macrocephalic children is often a benign entity. ESAS and macrocephaly will still be present in the majority of children in the long-term follow-up.


[Intermittent prophylaxis in febrile convulsions with oral diazepam].

Intermittent prophylaxis with oral diazepam is presented as an optional treatment for febrile seizures. This proposition is justified by the severe side effects of the currently used chronic anticonvulsant drug therapy in febrile seizures (phenobarbital and valproate). Nineteen patients aged between 3 months and 5 years were treated. They had either simple or complex febrile seizures. Sixteen patients had at least one prognostic factor for recurrence of febrile seizures: first febrile seizure before 15 months of age, positive family history for epilepsy or febrile seizures, occurrence of a complex febrile seizure or abnormal neurological examination. Three patients had none (cases 8, 12 and 13). We recommended 2.5mg b.i.d. for children younger than 12 months, 5mg b.i.d. for children older than 12 months and younger than 3 years, and 7.5 b.i.d. for children older than 3 years. The results showed that only one patient had febrile convulsions while taking adequate diazepam dosage. Transient side effects occurred in 36.8% of the cases.


[Electroneurography of the facial nerve: methods of stimulation and records].

In this study it is, intended to establish the more appropriate points to register the compound action potential from m. frontalis m. orbicularis oculi and nasolabial fold muscles. The optimum site for stimulating electrodes and differences between the first and the last compound action potentials after 20 consecutive stimuli delivered at 1 Hz were analysed.


[Cytopathologic diagnosis of neuroepithelial tumors by the smear technique].

We studied 198 cases of neuroepithelial tumours with intra-operative diagnosis made by smears comparing the accuracy rates with the final paraffin section diagnosis. In 90.6% of cases the final diagnosis obtained on paraffin preparations was similar to that made on smears. In the group of cases with cytological diagnosis not confirmed by paraffin sections the majority of cases were unlike to affect immediate neurosurgical management, and represent mainly differences in grading of astrocytomas and mixed gliomas. The cytological diagnostic criteria are discussed together with the main difficulties for the interpretation of this valuable approach to intra-operative neurosurgical diagnosis.


Interleukin-6 and granulocyte macrophage-CSF in the cerebrospinal fluid from HIV infected subjects with involvement of the central nervous system.

We detected the cytokines interleukin-6 (IL-6) and granulocyte macrophage-CSF (GM-CSF) by ELISA in the CSF and serum of 30 HIV-infected patients classified as AIDS dementia complex (ADC), and 20 subjects with other neurological diseases (OND). We have found a high incidence of detectable IL-6 and GM-CSF in the CSF of ADC patients compared with OND patients. No statistical differences were observed between both groups for serum IL-6 and GM-CSF levels. These results suggest an intrathecal synthesis of these cytokines and a possible involvement in the pathogenesis of ADC.


Evidence of preferential female prevalence of HTLV-I associated tropical spastic paraparesis in Bahia-Brazil.

In order to evaluate the prevalence of HTLV-I infection and its association with tropical spastic paraparesis (TSP) in Bahia, a Northeastern State of Brazil, CSF and sera from TSP patients and CSF and/or sera from some selected groups of individuals were studied. The results seem to indicate a higher prevalence of HTLV-I infection in women than men with TSP and among individuals of HIV risk groups. Some alterations of routine analysis of CSF can suggest HTLV-I infection in TSP patients.


HTLV-I associated myelopathy in Salvador (northeastern Brazil).

Recent studies of tropical spastic paraparesis have confirmed the existence of human T-cell leukemia virus type-1 (HTLV-I) in several tropical areas of the world. In order to determine the role of HTLV-I as an etiologic agent of myelopathies in Salvador, we conducted a clinical and serological study in 43 patients with non-traumatic and non-tumoral myelopathies. We found 9 patients with HTLV-I associated myelopathy (HAM) which points to a new endemic area of HAM.


[Recurrent bacterial meningitis].

The study of CSF fistulae, and especially those involving otolaryngological anomalies, must be based on the search for the causative problem of recurrent meningitis. Congenital malformations, post-traumatic and post-operative situations or even diseases involving the cranial bones are basic causes that should be studied. Currently, cranial trauma is the most usual cause of CSF fistulae, with the possibility of recurrent bacterial meningitis.


[Enteral hyperalimentation in acute cranio-cerebral injuries: replacement or diet therapy?].

The authors raise a discussion on nutritional needs of severely traumatized patients under the currently knowledges about the pathophysiology of trauma, particularly in head injury patients. Special attention to the hypermetabolic and hypercatabolic states is given. Some considerations about dietary glucose and protein interactions are made. Finally, the authors expose the diet composition currently used in their institution. Routes and volumes for diet administration are commented. Questions about further studies in some areas of this field are also stated.


[Spinal cord neuroschistosomiasis: clinical and laboratory evaluation of 5 cases].

Schistosomiasis mansoni is an endemic disease in Brazil. Cases of nervous system schistosomiasis have been referred in our medical literature for the past forty years. In this report five cases of schistosomiasis of the spinal cord are studied. A review of the literature has been made and some clinical, laboratory and therapeutical aspects are discussed.


[Meningioma of the olfactory groove and breast neoplasms: report of 2 cases].

Two cases of olfactory groove meningiomas are presented in patients with breast cancer. Predominance of breast cancer and meningiomas in the age decades of the forties and the fifties, presence of steroid hormones receptors in both and prevalence of meningiomas in women are referred as significant correlations. Examination of the breast of women with meningiomas is recommended. Tomographic images are sometimes atypical, and a surgical intervention may be necessary.


Cortico-basal ganglionic degeneration. A case report.

The case of a Brazilian patient with cortico-basal ganglionic degeneration (CBGD) is presented. Since three years ago, a 71-year old male displays asymmetric ideomotor apraxia, gait apraxia, cortical sensory impairment, myoclonus, limp dystonia and rigidity. His mental status is spared. There is neither consanguinity nor similar cases in his family. The differential diagnosis of CBGD is discussed. A brief review of the literature is made stressing the clinical and pathological features of CBGD. This disease is poorly known and probably underdiagnosed. Its diagnosis can be safely made based on clinical grounds.


[Adrenoleukodystrophy: clinical and histopathological study of a case associated to the use of abortifacient agents in the second month of pregnancy].

The authors report a case of adrenoleukodystrophy in a 8 years old male patient whose mother has taken several abortive drugs during the first three months of pregnancy. The disease was progressive starting with auditory, visual and mental disturbances, followed by neurovegetatives and motor changes with convulsion and fetal position. At the final stage the patient became blind, deaf, quadriplegic and dementiated. Death resulted from lung infection. The diagnosis was confirmed by the CSF, electrophysiological, radiological and necropsy findings. Necropsy changes in the brain and adrenal cortex are detailed.


[Fibrous dysplasia of the temporal bone and cerebral occlusive vasculopathy: report of a case].

Fibrous dysplasia is a benign disease with unknown etiology. Skull attempt may cause neurological disorders. The diagnostic can be made through radiological examination. A case of a patient with right temporal bone involvement with facial palsy, right parietal lobe infarctions and elevated anticardiolipin antibody titers is presented. Cerebral angiography showed occlusion of several cerebral arteries. Post mortem examination displayed recent myocardial infarction, ischemic cerebral softening, and generalized arteriosclerosis. The simultaneous occurrence of fibrous dysplasia and a probable anticardiolipin syndrome is commented.


[Aneurysm of the distal portion of the posterior and inferior cerebellar artery in a child].

The case of a 7-year-old boy presenting with recurrent episodes of subarachnoid hemorrhage due to a distal posterior inferior cerebellar artery aneurysm (PICA), successfully operated, is reported. The low incidence of intracranial aneurysms in the first decade of life and the rare occurrence of distal PICA aneurysms are unusual features of this case. The theories regarding the origin of intracranial berry aneurysms are discussed.


[Cystic oligodendroglioma and positivity of reactions for cysticercosis: report of a case].

A case of a 32-year old male patient with complaints of frontal headache progressive decrease in visual acuity, altered behaviour, and positive results of immunological tests for cysticercosis performed on the cystic and cerebrospinal fluids is presented. After several clinical and surgical proceedings, the frontal craniotomy was indicated and a multi-lobulated cystic tumor was excised. Biopsy material revealed an oligodendroglioma invading the degenerated membrane of cystic wall. Some aspects related to the possible mechanisms involved in the association of oligodendroglioma with neurocysticercosis in the presented case are discussed. Three different types of conclusions may be reached: (1) neurocysticercosis may have acted as an oncogenetic factor for the oligodendroglioma; (2) the glycoprotein nature of the antigens of gliomas and cysticercosis and the similarity in the molecular weight range of their polypeptides may be responsible for the positivity of the reactions for cysticercosis in the cystic fluid; or (3) the association of oligodendroglioma with cysticercosis may be a simple coincidence. The present study strengthens the opinion that other pathologies should be looked for when clinical treatment of cysticercosis does not follow the expected course.


[Clinical psychopathological types associated with smoking].

Dependence and withdraw syndromes related with tobacco are mentioned. Clinical forms and types associated to tabagism considered are: psychopathic, borderline, neurotic, narcisic, psychotic, depressive, hedonic, and bulimic. Psychopathological and etiopathogenic factors of tabagism are listed.


[The neurologist and the medical literature: the process of endless education].

To keep up to date with new advances in medical care and in the understanding of the disease process which affects their specific patients neurologists need to develop strategies for continuing education. They must be learners throughout their careers and the learning must be self-directed, active and independent. The best way to reach this goal is to devote regular time to the medical literature. However as the great volume of journal literature precludes clinicians from reading all of it, special tactics of scanning, selecting and reading medical articles are necessary. Priority should be given to original reports, meaning first-hand accounts of planned investigations, as they go through expert critical review before publication. This safeguard improves their clarity and usually keeps flawed research away. Furthermore one should read only original articles that have direct bearing on his own clinical practice and with scientific rigor making the results likely to be true. Employment of these rules may reduce the relevant literature to an affordable size. Extraction of these articles depend on the regular surveillance of high-yield, high-circulation general medical journals and the specialty and subspecialty journals which are relevant to one's practice. Review articles may be helpful either as an initial approach to a subject whose current knowledge is beyond one's usual domain or as a source of references of original articles. Meta-analysis or the integration and statistical combination of the results of previous research may be a further and a most important advantage of reviews.


A study on the action of two calcium channel blockers (verapamil and flunarizine) upon an experimental model of tardive dyskinesia in rats.

Tardive dyskinesia (TD), a serious complications of neuroleptic chronic use, has no effective therapy yet. We performed an experiment to study the action on TD, of the calcium channel blockers (CCB) drugs, verapamil and flunarizine. We obtained the TD model in rats, administering haloperidol for a 21-day period. After this, the stereotyped movement induced by apomorphine was rated. The CCB drugs were administered in acute (in the 28th day) and chronic (for 8 days, after the 25th day) experiments. Acutely, verapamil increased the stereotyped behaviour, and promoted a reduction of it in the chronic experiment. The results suggest that CCB drugs should be tested in clinical trials of TD.


Electrocardiographic findings in acute cerebrovascular hemorrhage. A prospective study of 70 patients.

Seventy patients with hemorrhagic stroke were prospectively evaluated regarding the electrocardiographic abnormalities observed within the first 48 hours of the ictus. Group I comprised 55 patients with spontaneous cerebral hemorrhage, and group II 15 patients with subarachnoid hemorrhage. Patients taking cardiac drugs (beta blockers, calcium-channel blockers, inotropic drugs) or with severe metabolic/electrolyte disturbances were excluded. The most common ECG abnormality was a prolonged Q-Tc interval: group I, 37 (67.2%); group II, 8 (53.3%). Only 4 (7.2% patients of group I and no patient of group II had a normal ECG. No relation was found between the site of the intracerebral hematoma and the occurrence of any particular ECG change. A prolonged Q-Tc may be related to the development of severe cardiac arrhythmias observed in some patients with acute cerebral hemorrhage.


[Extradural hematoma: comparative radiological study between comatose and non-comatose patients].

A series of 129 patients harbouring extradural hematomas was analysed considering the neurological state immediately before operation as the most consistent variable. Seventy eight patients were considered to be comatose (Group I) and 51 were noncomatose. Among the comatose group, 30 were investigated with computerized tomography (23.3% mortality, 50% good results), 31 were submitted to angiography (48.3% mortality, 38.7% good results), and 17 were operated based on the neurological examination and skull radiography (47% mortality, 35.2% good results). The presence or absence of skull fracture and the density of hematoma did not change the final outcome. Associated intracranial lesions increased the mortality and lowered the good results in both groups. Frontal hematomas (10 cases) in the comatose group were associated with high mortality (52.6%) due to bad neurological state (Glasgow 3-5) and to isolated or multiple intracranial associated lesions (6 patients).


[Incidence of peri-intraventricular hemorrhage in preterm newborn infants with birth weight less than 1500 gms: evaluation of brain ultrasonographic studies and necropsy].

From January to December 1986, 120 preterm infants were born with birth-weight under 1500 gm. Eighty-five infants were included in the study group either because they had a cranial sonographic evaluation or a necropsy. The mortality rate was 70% for the study group, but was 52.5% for the whole period. The sonographic examination was performed in 41 infants. The incidence of periventricular-intraventricular hemorrhage (PIVH) was 42.3%. Twenty-three cases had PIVH grade II (63.8%), 10 grade I (27.7%), and 3 (8.3%) grade IV. Thirteen infants had sonographic and necroscopic examinations. We observed a total agreement between sonographic and necroscopic diagnosis in 9 cases (69%). Two cases were classified as partial agreement as they differed only in the grading of PIVH, whereas in two cases there was a false negative diagnosis in the ultrasound examination. The interval between the examinations in these two false negative cases was 53 and 20 days, respectively. We conclude that the incidence of PIVH found in our study is comparable to that of the literature and that cranial ultrasonography is a sensitive neuroimage technique for the diagnosis of PIVH in the newborn preterm infant.


[Neonatal bacterial meningitis: etiological agents in 109 cases during a 10 year period].

The etiology of purulent meningitis was investigated in 109 newborn infants admitted in a neonatal intensive care unit throughout a ten year period. Bacterial pathogens were isolated from the CSF in 57 (52.2%) neonates. There was a predominance of Gram-negative bacilli isolated in 38 (34.9%) neonates. Gram-positive cocci were isolated from CSF in only 12 (11.0%) neonates. Microorganisms associated with nosocomial septicemia and meningitis in neonates--Klebsiella sp, Salmonella sp. Enterobacter sp, Pseudomonas sp, Flavobacterium meningosepticum and Serratia marcescens--were responsible for presumptive etiology in 38 (49.3%) among 77 patients with positive cultures in "closed sites". They were isolated from 22 (57.0%) neonates with prior hospitalization but only from 12 (34.3%) neonates coming directly from their households (chi 2 = 4.08; p < 0.05). The mortality rate was significantly higher in patients with positive CSF cultures (47.4%) in comparison to patients with negative cultures (18.4%) (X2 = 5.01; p < 0.05). It is possible to conclude that Gram-negative bacilli, many of them of hospital origin, are the major pathogens in this study. An improvement on neonatal health care and a scrupulous control of neonatal nosocomial infections are recommended.


[Immunosuppressive treatment in multiple sclerosis].

An open, retrospective, uncontrolled study was carried out to evaluate efficacy and tolerability of immunosuppressive therapy used in the period 1982-1990 in patients with clinically definite multiple sclerosis. Of 42 patients 43% were treated with one drug, and the others with more than one. Tolerability was evaluated after at least 6 months of therapy in 6 patients on prednisone, 26 on azathioprine, 17 on methyl-prednisolone and 15 on cyclophosphamide pulses. Reversible mild or moderate side-effects were noted in 54% of the patients on azathioprine, leading to change in dose or withdrawal. There were similar side-effects in 83% of the patients on prednisone, 40% of those on cyclophosphamide, and in 12% of those on methylprednisolone. Efficacy was evaluated in 15 patients with a follow-up of at least 2 years, measured by objective scales. The results indicated therapy appeared to arrest progress of disability. We conclude that this study supports the use of immunosuppression in selected cases with rapidly evolving neurological deficits.


[Comparative study of immunoenzyme tests ELISA-G and ELISA-M, indirect immunofluorescence, and complement fixation in the diagnosis of human cysticercosis].

A comparative study of four immunological tests used for anti-Cysticercus antibodies detection--Enzyme-Linked ImmunoSorbent Assay IgG (ELISA-G) and IgM (ELISA-M), indirect immunofluorescence (RIFI) and complement fixation (RFC)--was made in serum and cerebrospinal fluid (CSF). 539 patients with symptoms suggesting cysticercosis, 450 relatives of these patients and 133 normal people (control group) were examined. 1122 serum samples and 120 CSF samples were analysed by ELISA-G and RIFI, 83 sera and 60 CSF also by RFC, and 28 CSF by ELISA-M. 5.2% serum samples were reagent in ELISA-G and RIFI, and 3.5% of them had discordant results. All control group sera were negative. The same tests in CSF were positive in 16.7% and had discordant results in 7.5%. ELISA-G and RIFI in serum and CSF had concordant results in 89.6% (17.7% were positive). ELISA-G, RIFI and RFC had concordant results in 54.2% sera (16.9% positives) and in 81.7% CSF (11.7% positives). When ELISA-G and RIFI were negative, RFC was positive in 41.0% sera and 11.7% CSF. ELISA-G and ELISA-M had concordant results in 78.6% CSF. When these results were discordant ELISA-G was positive in 10.7% and ELISA-M in another 10.7%. It is necessary to use concomitantly several immunological tests for anti-Cysticercus antibodies detection in serum and in CSF, in attempting to reach correct diagnosis.


[Epilepsy and public education].

The social problems experienced by persons with epilepsy become increasingly apparent to those who work closely with group associations. The objective of our study was to obtain information on personal attitudes and knowledge about epilepsies. Primary and high-school teachers and professors (medical and non-medical areas) were submitted to a questionnaire which was designed to obtain this information. We concluded that most professors (including those of medical areas) do not know enough about epilepsies. We believe that problems which are perceived by epileptics regarding social aspects may be softened through improved education of the community in general. Continued education of medical practitioners, professors, teachers, patients and their families leads to a positive attitude towards epilepsies, which are essential and may help to improve life quality and the patient's medical treatment.


[Stereotaxic biopsy for primary lymphomas of the central nervous system].

Primary lymphomas of the CNS are rare. However the incidence of these lesions has increased recently. These tumors are often situated in the thalamus and basal ganglia, but can be multifocal. The treatment for primary CNS lymphoma is radiotherapy alone or in association with chemotherapy. Because the associated morbidity is very low stereotactic biopsy is an ideal method for determining the histologic diagnosis in patients with suspected CNS lymphoma. The authors present a study of 49 patients with primary CNS lymphomas in which a stereotactic biopsy was performed.


[Plasmapheresis in neurology. Critical analysis of indications and protocols].

Therapeutic plasmapheresis (plasma exchange) in neurology is a matter of discussion. We review the main articles on clinical trials, both controlled and uncontrolled, trying to establish the efficacy and safety of the procedure. Humoral aspects involved in the genesis of the disorder play an important role for its indication in the present time. In myasthenia gravis indications for plasmapheresis are established: in the preparation for thymectomy, in patients with respiratory insufficiency and requiring respiratory prosthesis, after long immunosuppression therapy with rebound effect, when all other forms of therapy failed, and in neonatal myasthenia gravis with special technics. We discuss the indication of plasmapheresis in Guillain-Barré syndrome, multiple sclerosis, chronic inflammatory demyelinating polyneuromyelopathy, HTLV-1 myelopathy, HIV peripheral neuropathy and myelopathy, paraproteinemias, polymyositis, and dermatomyositis. Indications for other diseases are mentioned. Reference is made to the results we observed in 31 cases followed consecutively at Hospital dos Servidores, Rio de Janeiro, from June 1984 to June 1990. In our series 26 were myasthenic patients, 4 patients presented Guillain-Barré syndrome, and 1 multiple sclerosis. Therapeutic plasmapheresis protocols suggested by us are summarized.


Coma and death in unrecognized Wernicke's encephalopathy. An autopsy study.

Eleven out of 36 autopsied cases of Wernicke's encephalopathy had developed coma. None of these patients had the diagnosis during life. There were six men and five women with ages ranging from 26 to 50 years (mean 36.6). Seven of these patients were heavy drinkers, three exhibited signs of severe malnutrition, whereas one was being evaluated for a disseminated gastric cancer and one was in treatment of hyperemesis gravidarum. Two patients were brought to the hospital after found unconscious at home. Neuropathological examination disclosed gross changes in the mammillary bodies in eight cases and microscopic changes in all cases. In one case there was atrophy of the anterior superior part of the vermis. Petechial hemorrhages were observed particularly in the walls of the third ventricle. Microscopically there were in addition to hemorrhages, glial proliferation, endothelial hypertrophy and necrosis of nerve cells and myelin. Central pontine myelinolysis was observed in one case. Wernicke's encephalopathy is a clinically underdiagnosed condition. Coma may mask its classical clinical picture or even be the sole manifestation. Although coma points to a poor outlook it may be reversed by thiamine administration. Any patient with coma of unknown etiology should be given parenteral thiamine.


[Krabbe's disease (globoid cell leukodystrophy). Apropos of 5 cases].

A clinical study on five cases of Krabbe's disease (globoid cell leukodystrophy) was performed. A final diagnosis was done either with post-mortem study (two cases) or by enzymatic assays carried on cultured fibroblasts (two cases). Peripheral nerve biopsy for electron microscopy was performed in all cases, and the ultrastructural alterations characteristics of Krabbe's disease were always found. The authors emphasize the suggestive clinical and laboratory data which enable the diagnosis of Krabbe's disease in the absence of the ultrastructural exam of peripheral nerve, or the enzymatic assays not performed in this country.


[Spinal cord cysticercosis: report of 2 cases, review of the literature, and comments on its pathogeny].

A case of spinal intramedullary cysticercosis in a 13-year-old Brazilian female and a case of spinal leptomeningeal infestation by cysticercosis in a 51 year-old Brazilian female are presented. A review of 95 published cases of medullar cysticercosis since 1856 shows the incidence of this condition. Extramedullary forms are explained by the downward migration of larvae from the cerebral to the spinal subarachnoid space and most larvae are expected to be stopped in the upper portions of the spinal canal due to peculiarities of the anatomy of the spinal cord.


[Intracranial dissecting aneurysms of the posterior circulation: report of 3 cases and review of the literature].

Dissecting aneurysms of cerebral arteries are unusual causes of stroke. The carotid system is the commonest site of this pathology, the vertebral arteries are less involved and dissection of the basilar artery is rare. The authors report three cases of arterial dissection of the vertebrobasilar system, two of the vertebral arteries and one of the basilar artery. An extensive review of the literature is presented. The clinical picture of dissection of vertebrobasilar system was inespecific but pain was a prominent symptom, though had not occurred in the site of the arteries involved. The pain was suggestive of subarachnoid hemorrhage. Associated or risk factors were mild trauma, migraine and high blood pressure. The angiographic findings were suggestive, however just the "double lumen" has been considered pathognomonic. The prognosis is variable. It was benign in case 3, left sequela in case 2, and case 1 rebleed fatally.


[Traumatic extradural hematoma in childhood and normal early computed tomography: report of 2 cases].

The cases of two children with traumatic extradural hematoma with an early normal CT scan are reported. Consciousness impairment occurred in them respectively 20 and 60 hours after admission, and CT at this occasion evidenced extradural hematoma. Serial neurological examinations are recommended for children with large traumatic cephalohematoma associated or not to skull fracture and in whom an initial early CT was normal, since they can lately develop extradural hematoma.


Traumatic callosotomy.

In a 33 years-old epileptic woman with a traumatic impact to the vertex, clinical and radiological studies (skull X-R, CT and MRI) disclosed an extensive callosal section under the topography of the falx, associated to fronto-basal contusions. There was a significant improvement in the epileptic syndrome. No interhemispheric disconnection syndrome could be determined, which is compatible with the posterior regions of the corpus callosum having been spared. This is, to the best of our knowledge, the most extensive callosal injury documented by MRI to date.


[Progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of a case and review of the literature].

Progressive supranuclear palsy (PSP) was first recognized as a distinct syndrome by Richardson, Steele and Olszewski roughly a quarter century ago. Subsequent clinical experience has corroborated and enlarged their original observations. PSP has become familiar as a chronic progressive disorder with extrapyramidal rigity, bradykinesia, gait impairment, bulbar palsy, dementia and a characteristic supranuclear ophthalmoplegia. It is a significant cause of parkinsonism and its etiology remains obscure. The case of a patient from Santa Catarina who presented definite clinical evidences of this syndrome is reported. This is the first description in this southern Brazilian State, where at least 50 more patients should exist, if we may extrapolate the prevalence rate of this condition in developed countries to this well developed area of Brazil. A review of the literature was undertaken with emphasis on recent clinical and therapeutic aspects of PSP.


Acute central nervous system infection by Trypanosoma cruzi and AIDS.

The acute infection of the CNS by Trypanosoma cruzi acquired by blood transfusion is uncommon. The concomitance of AIDS in the patient reported shows the importance of cellular immunity in restriction of this parasite, and reinforces the problem of blood transfusion in endemic zones.


Machado de Assis's epilepsy.

Machado de Assis (1839-1908) is considered the most important Brazilian writer and a great universal literary figure. Little is know about his medical, personal and family history. He hid his "disease" as much as possible. Machado referred to "strange things" having happened to him in his childhood. He described seizures as "nervous phenomena", "absenses", "my illness". Laet observed a seizure and described it as: "... when Machado approached us and spoke to me in disconnected words. I looked at him in surprise and found his features altered. Knowing that from time to time he had nervous problems, ... and only permitted Machado take the Laranjeiras Street car, when I saw that he was completely well". A photographically documented seizure is shown. Alencar wrote, "The preoccupation with health was frequent: either he was having the consequences of a fit or was foreboding one". It is clear that Machado presented localized symptomatic epilepsy with complex partial seizures secondarily generalized of unknown etiology. The seizures which began in infancy or childhood had remission in adolescence and then recurred in his thirties and became more frequent in his later years. His depression got markedly worse with age. In our opinion, the greatest consequence of Machado's epilepsy, was his psychological suffering due to the prejudice of the times. Despite this Machado showed all his genius, which is still actual and universal.


Clinical and morphological study of calf enlargement following S-1 radiculopathy.

Calf enlargement following sciatica is a rare condition. It is reported the case of a 28-year-old woman who complained of repeated episodes of lower back pain radiating into the left buttock and foot. One year after the beginning of her symptoms, she noticed enlargement of her left calf. X-ray studies disclosed L5-S1 disk degeneration. EMG showed muscle denervation with normal motor conduction velocity. Open biopsies of the gastrocnemius muscles were performed. The left gastrocnemius muscle showed hypertrophic type 2 fibers in comparison with the right gastrocnemius. Electron microscopy showed mildly increased number of mitochondria in these fibers. A satisfactory explanation for denervation hypertrophy has yet to be provided.


[Hemifacial spasm and basilar impression associated with Arnold-Chiari deformity. Report of a case].

The authors report a case of symptomatic basilar impression and Arnold-Chiari malformation being presented as the first symptom of hemifacial spasm. The surgical treatment of the malformation resulted in improvement of the clinical manifestation with reduction of the hemifacial spasm. The need for the aetiological therapy for the hemifacial spasm is emphasized, before symptomatic treatment with botulinum toxin is tried.


[Partial motor status epilepticus. Primary form provoked by eye closure].

We report the case of a young female patient with photosensitive primary epilepsy who presented partial motor status epilepticus provoked by the act of shutting the eyes. Clinical, EEG and neuroimage data are presented and discussed.


[Macroprolactinoma at 6 years of age: diagnostic difficulties].

Prolactinoma is rare in childhood and adolescence. The earliest known diagnosis was reported in a patient at 8 years of age. We report the case of a sellar tumor found in a 6 years old girl. After a long symptomatic period she was brought for treatment. At this time she had partial papillar atrophy, hyperprolactinemia, and diminished reserve of growth hormone and cortisol. Due to rapid visual deterioration, she was submitted to a frontotemporal craniotomy for suspected craniopharyngioma. The tumor tissue immunohistochemistry was positive for prolactin. It is concluded that prolactinoma must be considered in differential diagnosis of sellar tumors in childhood since it benefits from a less aggressive therapy.


Danazol. A new perspective in the treatment of HTLV-1 associated myelopathy (preliminary report).

We investigated the efficacy of danazol treatment in eight patients with HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP). Treatment with danazol yielded clinical improvement of urinary control and gait disturbances in 7 out of the 8 patients. The improvement was noted within 15 days of danazol administration. Analysis of factors of relevance to the clinical improvement with danazol showed that the beneficial response was preferentially found in females.


Advanced trauma life support. A time for reappraisal.

The experience of Advanced Trauma Life Support training received by three anaesthetists is discussed with particular reference to the teaching of airway management, the grade of staff who should attend the present courses and the relevance to the British hospital system. We conclude that these courses are useful but limited by their inflexibility and failure to recognise the difference in skill mix in the British setting.


Improving public health care: lessons on governance from five cities.

Policy-oriented investigations into public health care delivery have been limited, especially during the Reagan era of competition and profit-based health care, when the inner city was essentially forgotten. In this study, policymakers toured five urban public health care systems in different parts of the country to promote consideration of a new governance for Chicago and Cook County's complicated and uncoordinated care for the medically indigent. A comparison of patterns of governance revealed strengths and weaknesses of each model. Local leadership and the political will to evolve a system of care, with clear connections between the public and private sectors, account for each city's relative success in addressing mounting needs of inner-city populations.