A one and half year old boy was brought with status epilepticus to emergency ward and treated with Lorazepam and Phenytoin. The child was the first issue of a non-consanguineous marriage, who was born after full term uneventful pregnancy. He had global developmental delay in the milestones. His fine and gross motor skills were consistent with 8-month level; and language and social skills were consistent with a 6-month level. There was no family history of seizures or neurological illness. At the age of 5 months, he developed generalized tonic–clonic seizures without fever for the first time and till the present admission he had three episodes of seizures (not on anticonvulsants). On examination, his head circumference was 48.2 cm (>95th centile) with frontal bossing [], weight 9 kg and height 72 cm. He did not have any neurocutaneous markers. On neurologic examination, cranial nerves were normal, muscle strength and tone were normal, and deep tendon reflexes were brisk with bilateral plantar extensors. His fundus examination did not show any abnormality. Examination of other systems was unremarkable. His blood counts, serum electrolytes, renal and liver functions, blood glucose, serum calcium, phosphorous, magnesium, serum lactate, serum ammonia, cerebrospinal fluid (CSF) analysis, Electroencephalogram (EEG), and arterial blood gases were normal. His computed tomography (CT) scan of brain and urine chromatography was also normal. MRI brain with contrast: Diffuse white matter paucity and signal alteration predominantly involving bilateral frontal cortex also involving caudate nuclei, genu of internal capsule on T2-weighted with relative sparing of the temporo-occipital lobes. Magnetic resonance (MR) spectroscopy study of bilateral fronto-parietal cortex white matter shows decreased N-acetyl aspartic acid ratio values [].


A 26-year-old female patient presented with recurrent left breast abscess with palpable lump and multiple discharging sinuses attended our outdoor clinic in January 2010. According to the patient, the lump had been present for the past 24 months when she had 7 months pregnancy, the patient was treated with anti tubercular drugs[] and after delivery she left antitubercular treatment (ATT) for 2 months then restarted again and completed full ATT regimens. Again, she developed sinuses in her left breast 6 months ago []. She had no past history of weight loss or fever and no history of breast malignancy or TB in her family. On physical examination, her weight was 45 kg. The left breast was very tender and diffused irregular mass was present, mainly involved the lower quadrants. Their overlying skin was discolored, with multiple sinuses discharging a dirty yellow colored fluid. On fine needle aspiration cytology (FNAC), smear disclosed aggregated of epithelioid histocytes occasional multi nucleated histiocytic giant cells, on an inflammatory background consisting of polymorphs, lymphocytes and some plasma cell along with fair number of squamous epithelial cells. Zeihl Neelsen (ZN) stain was positive for acid fast bacilli (AFB). Culture by Lowenstein-Jensen medium (LJ Medium) was negative for Mycobacteria after eight weeks incubation. Interferon gamma releases assay gave negative result at standard cutoff by manufacture.[] In view of again developed breast abscess with AFB, possibility of treatment failure was considered as relapse case restarted category II treatment under DOTS (Directly Observed Treatment Short Course). On three months follow-up, lymph node enlargement had regressed and abscess was resolved, though there was recurrence of small abscess after 1 month. Finally, she completed the treatment and was cured of TB infection.


A 40-year-old female was referred to our centre for management of left breast pain with purulent discharge in April 2010. There was no history of recent pregnancy or lactation or a history of breast trauma. She had noticed a painful mass in her left breast one year ago. Again from last 20 days she noticed a visible skin lesion on the breast with purulent discharge. She had no history of previous ATT intake, tubercular infection in her family, diabetic mellitus (DM) and weight loss or fever. She received at least 3 courses of antibiotic therapy by local physician but no therapeutic effect was noticed before she was referred to our centre. On physical examination, a painful nodular mass was detected near to the nipple of the left breast along with a discharging sinus. Based on the ultrasonography and mammography results, the patient underwent FNAC with a suspicion of malignancy/TB or any pyogenic infection. Diagnostic FNAC smear showed group of epithelioid cell and multinucleated histolytic giant cell on a background of mixed inflammatory cell infiltrate composed of neutrophilic polymorphs lymphocyte and histocytes in conjunction with positive AFB in pathology. AFB was also positive by ZN satin in microbiology. Mycobacterium culture was positive after 3 weeks incubation. Interferon gamma releases assay in tube (IGRA-IT) was positive. Chest radiography was intact and there were no changes compatible with TB or sarcoidosis. On the basis of laboratory evidence, the patient was put on standard four ATT category I regimen (2HRZE)3(4HR)3.[] The discharge was stopped and a healed sinus tract was seen after three months subsequent follow-up.


A 29 year-old woman complained moderately painful pus discharge from incision site in right breast for last 3 months attended our outdoor clinic in February 2011. Incision and drainage for a lump was done at nearby hospital and treated with general antibiotics which were not responded and pus was persisted. She had last child birth 6 years earlier. No history of TB/chemotherapy of ATT was reported. She had no history of breast cancer in her family but had family history of TB to her grandfather. Mammography examination revealed a hypoechoic lesion about 0.7×0.72×0.69 cm, small rounded, with 0.18 ml volume which was noted in the inferior medial quadrant of right breast. After one month hypoechoic space occupying lesion size was increased to 1.02×0.9×0.84 cm and a volume of 0.40 ml. The patient underwent an open breast biopsy; the lump was completely excised under local anesthesia. Surgical finding was irregular cystic swelling in lower inner quadrant of right breast filed with thick and pultaceous materials communicating with lactiferous duct with surrounding fibrosis. She received intravenous augmentin 625 mg thrice daily with supplements. A sample of the pus/tissue was sent to the microbiology and histopathology laboratory. ZN microscopy, LJ culture was negative and bacterial culture and sensitivity were sterile after 48 hour of aerobic incubation. But histopathology disclosed granulomatous mastitis in breast lesion with presence of defined granulomas composed of epithelioid and multinucleated langhans giant cells. The patient was immediately placed on ATT category I regimen (2HRZE)3(4HR)3.6 After six months of follow up, wound was healed without any pus discharge and 7 kg weight gain.


A day before being applied to us (Istinye Public Hospital, Istanbul, Turkey, 2008), the patient (6-year-old male) with his two brothers had been taken to a physician with complaints of nausea and abdominal pain. Five milligram dose of metoclopramide had been prescribed to the patient. After receiving the first dose of medication, the contraction and retention of neck, contractions of chin and arms occurred within half an hour in the patient who used metoclopramide. The patients were brought to our clinic. The patient's conscious was clear, but he was agitated and restless. Pulse was 102/min; blood pressure was 85/45 mmHg. Respiratory rate was 27/min as normal. There were speech dysfunction, trismus, and dystonic movements of sleeves. Involuntary movements gradually increased and opistotonus developed. Results of complete blood count (CBC), liver and kidney function tests and electrolytes were within normal values.


The patient was 8 years old and male. The story was same with his brothers. Five mg dose of metoclopramide had been prescribed to the patient. On physical examination, conscious of the patient was clear but he was agitated and restless. Pulse was 95/min; blood pressure was 90/50 mmHg. Respiratory rate was 25/min as normal. There were speech dysfunction, trismus, and dystonic movements of arms. Results of CBC, liver and kidney function tests, and electrolytes were normal.


The patient was 15 years old and male. The story was same with his brothers. Ten mg doses of metoclopramide had been prescribed to the patient. Conscious of the patient was clear but he was agitated and restless. Pulse was 92/min; blood pressure was 125/85 mmHg. Respiratory rate was 22/min as normal. There were speech dysfunction, trismus and dystonic movements of arms. And involuntary movements gradually increased and opistotonus developed. Results of CBC, liver, and kidney function tests and electrolytes were found as normal. Examination of the other systems was also normal.
We considered that acute dystonic reaction originated from metoclopramide, because the complaints of three brothers began suddenly onset and there were no similar complaints before. Therefore, biperiden 5 mg (IM) was performed to all three cases in order to treat them. The symptoms disappeared dramatically after 10 min from drug application. Patients were followed in observation room and there were no additional developing problem after the observation. Therefore, he was discharged to come back for control. The family was informed about the issue.


A 40-year-old male presented to the emergency department with complaints of irrelevant talk, abnormal behavior, and disorientation for four days. He had multiple generalized tonic clonic convulsions two days back. On admission he was afebrile, anxious, restless and confused with doubtful neck rigidity since he kept himself stiff and was non-cooperative during examination. He had tremors in both hands, pulse 106/min, blood pressure 146/96 mm of Hg and respiratory rate of 20/min. His remaining general physical examination was unremarkable. History revealed him to be an alcoholic for 12 years with psychological and physical dependence, with abstinence for five days. His childhood history was unremarkable. He had no past history of any medical or surgical illness except frequent cough and occasional fever for few weeks for which he took cough syrups and antipyretics directly from chemists without any consultation with doctor. He was a salesman in a grocery shop with difficulties in saving his job because of frequent absenteeism. His wife reported him to be a happy type of person during the initial three-four years of starting alcohol with friends. He gradually became alcohol-dependent, irritable, abusive and frequently aggressive to the wife and both children of 10 and 12 years of age. There was no history of any promiscuous behavior or sexually transmitted disease.
On mental status examination (MSE) he had altered sensorium with poor concentration, disorientation to time and place, short-term memory impairment, slurring of speech, increased psychomotor activity and irritable mood.
On the basis of history and MSE a diagnosis of alcohol withdrawal delirium was made and patient admitted. He was started on chlordiazepoxide 25 mg four times a day with adequate fluids and multivitamins. His hemoglobin was 12.5 gm/dl, total leucocyte count 6700/mm3 with 80% polymorphonuclear cells and 20% lymphocytes. Erythrocyte sedimentation rate was 48 mm per h and random blood sugar was 124 mg/dl. Serum bilirubin total was 1.8 mg/dl and direct was 0.9 mg/dl, Alanine amino transferase ALT was 58 and aspartate amino transferase (AST) was 97 IU/L. Renal function test, serum electrolytes, thyroid function test, lipid profile, HIV test, electrocardiogram and computed tomography scan of head were unremarkable. During second and third day of admission his psychomotor activity slightly decreased to normal. Abdominal ultrasound showed fatty liver. On fourth day morning he developed rigidity of limbs, mutism with apathetic look, open eyes and fixed gaze but no emotional responsiveness. His stuporous state was not associated with posturing or waxy flexibility. Injection lorazepam 4 mg twice a day was added to reduced dose of chlordiazepoxide 25 mg twice a day considering his condition as unusual catatonic manifestation of alcohol withdrawal. Reviewing his temperature charts revealed that he had been having an evening rise of temperature during all these days. On reexamination he had neck rigidity. Physician reference was sought and the case was transferred to the medical ward with a clinical diagnosis of alcohol withdrawal with coexistent meningoencephalitis. On further investigation his chest X-ray showed bilateral miliary shadows and cerebrospinal fluid (CSF) examination revealed protein 150 mg%, CSF sugar – 60 mg%, TLC- 60 cells/high power field, differential leucocyte counts- 100% lymphocytes, adenosine deaminase 18 U/L. Diagnosis was then revised to tubercular meningoencephalitis and treatment switched to antituberculous therapy and injection dexamethasone followed by oral steroids. After three days of starting antitubercular treatment he showed significant improvement and was discharged on Category One antitubercular treatment with advice to follow up treatment from his nearby directly observed treatment supervised (DOTS) centre as the patient was from a remote area. He did not agree to take any anticraving drugs because of poor financial status. He kept his commitment of fortnightly counseling sessions up to three months with complete abstinence and then was lost to follow-up.


A previously healthy 9-yr-old boy with a cough and sputum since 7 days and a fever since 3 days visited the outpatient department (OPD) of Wonju Medical Center. He took cough medication for 2 days before admission but did not take any antibiotics. He was an elementary school student, and there was no recent history of sick contacts, travel, or illness.
Auscultation of the lungs revealed coarse crackles with right side rhonchi. Laboratory results showed that complete blood cell count, white blood cell differential count, electrolyte level, liver function tests, and urinalysis were normal. C-reactive protein (CRP) was 12.0 mg/L (reference range: 0-5 mg/L). The chest radiograph showed pneumonic consolidation on the right upper lung field. Sputum culture showed growth of normal flora. Sputum smears were negative for acid-fast bacilli, and a tuberculin skin test was negative. Blood culture showed no growth after 7-day incubation. The serologic test for detecting anti-mycoplasma IgM antibody (SERODIA®-MYCO II, Fujirebio, Tokyo, Japan) was negative, but the patient's cold agglutinins titer was 1 :128. A PCR test of a nasopharyngeal swab specimen was positive for M. pneumoniae. Gene amplification of M. pneumoniae was performed by MyGenie 32 Thermal Block (Bioneer, Daejeon, Korea), and we used 20 µL of high fidelity PCR premix (AccuPower®Premix, Bioneer, Daejeon, Korea) as the reactive agent. PCR primers were designed for detecting known M. pneumoniae single point mutations (A2063G, A2064G, T2611C, and C2617A). In this case, the resistant strains displayed a point mutation in the 23S rRNA: A-to-G transition mutation at nucleotide 2063.
Initial treatment with flomoxef was given intravenously (70 mg/kg/day, divided into 3 doses). The result of PCR for M. pneumoniae was positive, and roxithromycin (7 mg/kg/day, divided into 2 doses) was added on the 2nd day of hospitalization. On the 5th day of hospitalization, neither respiratory symptoms nor follow-up chest radiography findings improved. Treatment was, therefore, switched from roxithromycin to azithromycin (10 mg/kg/day, once a day for 3 days). On the 9th day of hospitalization, clinical symptoms and chest radiography findings improved, so the patient was discharged. Four days later, he was symptom-free with no detectable complications on OPD follow-up. The M. pneumoniae isolated from this patient carried an A2063G mutation.


A 70-year-old male patient was diagnosed with autoimmune pancreatitis in May 2004. At that time, the serum IgG was mildly increased to 1,780 mg/dL (normal, 694 to 1,618 mg/dL). Until October 2009, he had been receiving steroid treatment, and he had a history of recurrence of autoimmune pancreatitis in November 2005.
In July 2010, a follow-up chest computed tomography was performed and showed abnormal findings with multiple enlarged lymph nodes in the bilateral mediastinal and both hilar areas as well as a small irregular nodular and patchy ground-glass opacity in both of the upper lobes and in the right lower lobe of the lung (). A physical examination showed a 2 cm skin nodule on his left forearm. The laboratory findings were as follows: IgG, 1,900 mg/dL (normal, 694 to 1,618 mg/dL); IgG4, 3.56 g/L (normal, 0.06 to 1.214 g/L); and serum amylase, 258 U/L (normal, 30 to 110 U/L).
A biopsy of the skin nodule revealed that it was a subcutaneous lymph node (). This lymph node showed effacement of the lymph node structure with a dense lymphoid infiltrate with marked sinus expansion by histiocyte-like cells with intermediate to large round nuclei, vesicular chromatin, and ample cytoplasm containing small phagocytosed lymphocytes, red cells, and plasma cells (emperipolesis). The plasma cells were abundant both in the sinus and in the medullary cords. The immunohistochemical stain showed that the histiocytes were positive for CD68 and S-100 protein. Numerous IgG4-positive cells (approximately 200 IgG4 cells/high power field) were also noted. A bronchoscopic biopsy of the peribronchial lymph nodes was performed and showed a few non-caseating granulomas that were suspicious for dust granulomas with approximately 20 IgG4-positive plasma cells. The patient recovered with steroid therapy.


A 54-year-old woman complained of abdominal fullness in February 2009. She had undergone breast-conserving surgery and axillary lymph node dissection for left breast cancer in October 2000, and had subsequently developed left pleural effusion () in July 2006. Thoracic drainage with intrathoracic administration of OK-432 had failed to control the pleural effusion, which contained no cancer cells. The patient experienced variable amounts of pleural effusion and was followed on an outpatient basis for the next 30 months.
The patient then developed abdominal distention with elevated serum levels of carcinoembryonic antigen (CEA) and carbohydrate antigen (CA) 15-3 (). Positron emission tomography/computed tomography (PET/CT) showed bilateral ovarian tumors () with ascites. Systemic chemotherapy (weekly paclitaxel 80 mg/m2) and intra-abdominal chemotherapy (carboplatin 100 mg/m2) with repeated aspirations (4,000-8,500 mL) failed to control the ascites. Bilateral oophorectomies were performed to provide a definitive diagnosis of the ovarian tumors and to establish some therapeutic clues for controlling the ascites. The right and left ovaries were weighed 15 and 250 g (left tumor), respectively and were lobulated. No tumor dissemination was observed in the abdomen and the ascites contained no cancer cells cytologically. Pathological examination showed that the tumors were composed of solid and luminal structures () and were positive for estrogen receptor (), progesterone receptor, cytokeratin (CK)-7, and gross cystic disease fluid protein-15 (), and negative for HER2 overexpression and CK20. These pathological findings suggested that the bilateral ovarian tumors originated from the patient's breast cancer.
The patient was discharged 9 days after surgery. The left pleural effusion disappeared () and serum CEA and CA 15-3 levels decreased below the normal upper limit (). The patient remained well under aromatase inhibitor therapy with no further recurrence for 40 months after the oophorectomies.


The patient was informed that the case would be presented for publication and she agreed.
A 41-year-old lady met with a car accident in June 2009 and was admitted to the hospital with the chief complaint of chest and back pain and restrictions of movements of right shoulder. During the accident, the patient was knocked down by a car when she was riding a motorcycle and trying to cross a street. She hit the ground with her right shoulder which suffered internal rotation and abduction. There was no history of coma, nausea, vomiting, respiratory distress, and shortness of breath.
Physical examination showed slight swelling and tenderness on the right shoulder. Sternoclavicular joint deformity [] was also spotted. Piano key sign and thoracic squeeze sign were both positive. Active abduction and flexion were limited to 60°, while there was limitation of extension and adduction. Passive abduction was restricted to 90°. There was bruising at the back.
Radiographic examination showed dislocation of right acromioclavicular joint and sternoclavicular joint, multiple rib fractures, and minor right pleural effusion []. 3D CT reconstruction [] of the injured clavicle demonstrated posterior dislocation of the lateral end of the clavicle and anterosuperior dislocation of the sternoclavicular joint.
Open reduction and internal fixation was performed under general anesthesia through a 10-cm curved incision from acromian and a 5-cm horizontal incision on the right sternoclavicular joint. After exposure, superioposterior dislocation was at the acromio claviculer joint, while the medial end of the clavicle was found subluxed anteriorly and upward. Moreover, coracoclavicular ligament avulsion was also spotted. The sternoclavicular joint was fixed first. To relocate the clavicle, we firstly removed the adherent dense fibrous connective tissue in the joint space. Next, with a satisfied reduction of the clavicle, in the sternoclavicular joint, an oblique T-type steel plate was transfixed by four screws, while at the same time another lag screw was inserted from the superior surface of clavicle toward sternum []. The acromioclavicular joint was fixed with two Kirschner wires (3.0mm). The wires were inserted from the lateral end of acromion, parallel to the articulation []. Repair of the articular capsule and surrounding ligaments was then done.
After wound closure, the patient was asked to wear an abduction device for a month. Passive motion exercises began after 1 week while the active ones began 2 weeks later. Removal of both Kirschner wires and plate was performed under general anesthesia 6 months after surgery. X-ray conducted 1 week [], 6 months [], 1 year [] and 2 years [] after the operation showed stable fixation and good relationship of both acromioclavicular and sternoclavicular joints. At 2 year followup, the patient had an abduction of approximately 150°, forward flexion of 160°, backward extension of 60°, and normal rotation [Figure –]. Besides, the power of the shoulder girdle muscles recovered and no instability of the dislocated joints was noted.


We present a case of 54 year old male patient who got fist punched in the right eye by a coworker half an hour prior. He complained about the loss of vision in the right eye.
He did not loose consciousness, have dizziness, or vomiting. The patient presented with both upper and lower lid haematoma and small subconjunctival hemorrhage. Fluorescein staining was negative, anterior chamber and lens was clear. Intraocular pressure was normal. Retina and optic nerve head appeared normal on fundoscopy (Figure ). The vision was “counting fingers at 1 meter” in the right eye and 0.5 without the correction in the left eye on the Snellen chart. “Mydriacyl bottle cap or Red desat” subjective test indicated color perception dysfunction of the right eye (Figure ). Relative afferent pupillary defect (RAPD) was positive, but mild in the right eye. We immediately performed ocular ultrasound which was normal (Figure ) and orbital X ray which showed no signs of bone fracture. We decided to treat the patient immediately with the megadoses of steroids following the protocol suggested by Cerovski () (Figure ).
The first dose was methylprednisolone in intravenous doses of 30 mg/kg, followed 2 hours later by a dose of 15 mg/kg, and 3 additional doses after every 6 hours of 15 mg/kg of methylprednisolone i.v. Patient also received steroid eyedrops 4 times a day. The following day patients vision began to improve, BCVA (best corrected visual acuity) being 0.3 in the right eye. And the 3 rd day BCVA was 0.5. On the forth day it improved to 0.7, stenopeic 1.0. On the second day VEP (visual evoked potentials) test was performed and it showed P100 wave of lower amplitudes and latency delay in the right eye, and was normal in the left eye. CT orbital scan was also done on the second day and it did not show any pathology (Figure ). Patient came for a follow up visit after seven days and the vision was normal.


A 20-yr-old man, 173 cm in height and 58 kg in weight, underwent a microdiscectomy for a symptomatic right-sided disc herniation at the intervertebral space, confirmed by magnetic resonance imaging in 2002. Routine laboratory studies were unremarkable with a hemoglobin (Hb) level of 14.1 g/dL and hematocrit (Hct) of 41.6%. Anesthesia was induced with 250 mg thiopental, 100 mg succinylcholine, 25 mg atracurium and maintained with 1.5% isoflurane in 50% oxygen and 50% nitrous oxide with a fresh gas flow 2 L/min. A routine microdiscectomy was carried out in the prone position on Wilson's frame under general anesthesia. During operation the discectomy the surgeon felt some momentary downward slippage of the pituitary rongeur into the deep intervertebral space. At that moment unexplained profuse bleeding occurred in the disc space. Shortly after this event blood pressure (BP) suddenly decreased from 110/80 mmHg to 80/60 mmHg. It became normalized soon after the injection of ephedrine 10 mg and atropine 0.5 mg and the bleeding stopped spontaneously. In the recovery room, the patient experienced transient hypotension (90/60 mmHg) and tachycardia (95 bpm/min) which again responded well to volume replacement. On the first day after the surgery operation the patient complained of abdominal discomfort and wound pain. BP was 100/70 mmHg, HR 92 bpm/min and body temperature 38.4℃. Hb and Hct was 11 g/dL and 32% respectively. Neurologic examination revealed normal motor tone in his lower extremities. Physically, the abdomen was mildly distended with tenderness in the left lower quadrant of the abdomen on a physical examination. Bowel sounds were decreased. Apparently he was stabilized with volume expansion.
On the second day after the operation he started to complain of increasing abdominal pain in the left quadrant with dizziness and became restless and very pale. At that time BP was 100/60 mmHg, HR 100 b/min and body temperature 38.5℃. Hb was 9.2 g/dL and Hct was 26.9%. Abdominal examination revealed decreased bowel sounds and the left femoral and deep peroneal artery pulsation was absent on palpation. Three hours later, Hb became 6.8 g/dL and Hct was 19.7%. Four units of blood were transfused for stabilization. An urgent Doppler sonogram showed no flow in the common iliac artery suggesting segmental obstruction. Contrast-enhanced CT also revealed a low attenuation perfusion defect in the common iliac artery at the level of L4-5 with a moderate amount of retroperitoneal hematoma (). There was no leakage of contrast but a few small air densities were present around the obstructed artery probably coming out from the perforated anterior spinal ligaments. Angiography of the abdomen demonstrated a complete obstruction of the left common iliac artery, located about 2 cm distal to the aortic bifurcation (). The distal portion of the obstructed artery was filled with contrast material by collateral circulation through the internal and external iliac arteries. Fifty five hours after the L4-5 discectomy, an emergent laparotomy was performed. Anesthesia was induced with 250 mg thiopental, 100 mg succinylcholine, 25 mg atracurium and maintained with 1.5% isoflurane in 50% oxygen and 50% nitrous oxide with a fresh gas flow 2 L/min. During the operation, BP and HR were stable and the surgical field revealed a moderate retroperitoneal hematoma. There was no active bleeding. A small defect was observed in the anterior longitudinal ligament overlying the right side of the L4-5 disc space. The laceration was located at the posterior aspect of the left common iliac artery, made by a partial puncture with a pituitary rongeur. It was packed with thrombus and adhered densely to the punctured anterior longitudinal ligament at the surgical level so that it was spontaneously sealed. The arterial injury was repaired by the excision and interposition of an 8 mm polytetrafluoroethylene graft with the removal of the retroperitoneal hematoma. The postoperative course was uneventful and the patient remains in good condition over 10 yr later.


A 19-year-old boy, first born to second-degree consanguineous parentage, presented with fatigue, myalgia, pains and aches in the extremities, especially after sustained exercise, muscle cramps and progressive weakness of the proximal lower limbs of 2 years duration, which was gradually worsening over the past 6 months. He had no history of wasting, twitching of muscles, hypertrophy of muscles, myoglobinuria, fluctuations or diurnal variations. There was no history of respiratory distress, bulbar weakness, cranial nerve disturbances, episodes of encephalopathy or cardiac symptoms. There was no family history of similar illness.
Examination revealed a young boy of thin and tall build with predominant skeletal markers of Marfan syndrome with arm span of 12 cm more than his height (arm span 182 cm, height 170 cm). He also had myopia, high arched palate, kyphoscoliosis [] and wrist sign positivity [] (tip of the thumb covering the entire fingernail of the fifth finger when wrapped around the contralateral wrist) and moderate pectus excavatum []. He also had striae in skin over the back. He had neck flexor weakness, wasted small muscles of hands with proximal muscle weakness of both lower limbs and generalized areflexia, exaggerated lumbar lordosis and waddling gait. There was no ectopia lentis or cardiac or pulmonary involvement.
On evaluation, serum creatinine phosphokinase was mildly elevated (343 IU/L).Tandem mass spectroscopic analysis revealed normal levels of free and acylcarnitine species. Cardiac evaluation with ECG and ECHO were normal. There was no evidence of aortic root dilatation. Thyroid profile and serum lactate were normal. Nerve conduction study was normal. X-ray to rule out protrusio acetabulae and magnetic resonance imaging to look for dural ectasia was not done.
Left biceps muscle biopsy was subjected to hematoxilin eosin (HE) [], modified Gomori trichrome (MGT), periodic acid Schiff's (PAS), oil red O and a battery of enzyme histochemical stains (succinic dehydrogenase [SDH], nicotinamide adenine dinucleotide tetrazolium reductase [NADH-Tr], adenosine triphosphotase [ATPase, pH 9.5, 4.6, 4.3] and acid phosphatase). Morphologically, skeletal muscle tissue showed preserved architecture, normal endo and perimysial components and polygonal fibers with mild variation in diameter. Moderate numbers of myofibers in each of the fascicle showed multiple fine vacuoles giving a sieve-like appearance. These fibers were present in between normal-appearing fibers, giving a mosaic pattern. SDH and NADH-Tr revealed these fibers to be intensely stained and red granular (Ragged red) on MGT []. The vacuoles were positive to oil red O and negative to PAS and acid phosphatase. Tiny pieces of skeletal muscle tissue fixed in 3% glutaraldehyde embedded in araldite for electron microscopy showed distortion of filamentous pattern, presence of lipid vacuoles and aggregates of mitochondria of varying sizes with altered mitochondrial cristae [].
The patient was started on a low dose of carnitine and Co Q. His muscle pain decreased and his neck and proximal muscle weakness of lower limbs improved over the next 2 months. He fulfilled 5/7 of the systemic score in Ghent criteria of Marfan syndrome, although there was no family history of Marfan syndrome. In view of the lack of genetic analysis and family history, the patient does not fulfil Ghent criteria but the patient does have clinical markers of Marfan syndrome and needs close follow-up as evolution of more organ involvement is age dependent. Neck weakness in LSM is reported in one patient[] and association with skeletal markers of Marfan is not reported in the literature.


A 36-year-old male patient was admitted to our hospital with the complaint of non-specific chest pain. He had a history of smoking and hypertension, potential risk factors for coronary artery disease. Also his father at the age of 46 years had died suddenly with unclear etiology. At the time of the evaluation, the patient had been on antihypertensive therapy with beta blocker for 2 years. The chest pain was not typical for angina pectoris but the patient was very nervous due to his family history.
The physical examination did not reveal any abnormal findings. Blood pressure was 140/90 mmHg and pulse 72 beats/min. Cardiac and lung auscultation were normal. The electrocardiogram (ECG) and chest X-ray were normal. Routine blood and biochemical laboratory tests were within normal limits. Transthoracic echocardiograpic examination revealed normal left ventricular size and contractility. The left ventricular ejection fraction was 65%. For further evaluation an exercise test was required but this was not possible as the patient had recently undergone knee surgery for meniscopathy. Therefore, to evaluate chest pain and to detect or rule out coronary artery disease, the patient underwent computed tomography coronary angiography (CTCA). CTCA was performed using a 64 slice CT scanner (Siemens Sensation 64, Germany). Scan parameters were as follows: Slice collimation, 32 × 2 × 0.625 mm; rotation time, 0.33 mins; tube voltage, 120 kV; tube current, 600 mA; and pitch, 0.2. The average heart rate was 71 bpm during the scan. The scan time was 7 sec. CT angiography was triggered automatically by the arrival of the contrast bolus (automatic bolus tracking). A prescan was taken at the level of the aortic root and a region of interest (ROI) was placed on the ascending aorta. As soon as the signal density level in the ascending aorta reached the predefined threshold of 120 Hounsfield units (HU), the scan started. We injected 80 ml nonionic contrast medium (Iomeron 400/ml; Iomeprol, Bracco, Italy) at a flow rate of 5 ml/s in the left antecubital vein. This was followed by a 40 ml saline chaser bolus at a flow rate of 4 ml/s to wash out contrast from the right ventricle. During the scan, the ECG was recorded simultaneously. The reconstruction interval for the coronary arteries with the fewest motion artifacts was determined (images at 75% of the R-R interval) and used for further analysis. For reconstruction of axial images, we used a slice thickness of 0.75 mm and a slice width of 0.5 mm. A medium soft-tissue reconstruction kernel (B30f) was used for reconstruction. For post-processing, an external workstation (Leonardo, Siemens, Germany) was used. In addition to the transverse source images, multiplanar reformations (MPRs), curved MPR images, maximum intensity projections (MIPs), and volume rendered (VR) images were utilized for the evaluation. VR reconstructions depicted the vascular anatomy well and were used for 3-dimensional (3D) orientation.
Coronary CTA demonstrated an anomalous left anterior descending artery (LAD) arising from the same ostium with the right coronary artery, which coursed downwardly along the interventricular sulcus, and another LAD arising from the left main coronary artery, which spread to the anterior wall of the left ventricle toward the left ventricular apex [Figures and ]. The right coronary artery (RCA) and the left circumflex artery (LCX) were normal. There were no significant coronary stenoses or occlusions. However, the anomalous LAD was coursing between right ventricular outflow tract and aorta which should be considered to be critical because of the potential to provoke myocardial ischemia or even sudden cardiac death []. These CTCA findings were consistent with double LAD, with one vessel arising from the ostium of right coronary artery and the second one arising from the left main coronary artery. The patient was informed about the anomaly and the potential complications. He did not want to undergo a surgical revascularization. He is now on follow up with no signs of myocardial ischemia.


Ms X, a 55-year-old married Caucasian woman was admitted to psychiatry ward following an overdose of prescribed medication in conjunction with alcohol. This was her first contact with psychiatric services although she had been on citalopram for a long period from her general practitioner. She experienced low mood, suicidal ideation, reduced appetite, poor sleep, and tearfulness following her divorce and removal of her adopted son from her care. She had a difficult childhood and was sexually abused by her father over a long period of time. The sexual abuse was associated with high negative emotions and a lot of anger. About 3 years ago, she suffered from seizures and cerebrovascular accident resulting in weakness in right leg and dysphasia. Couple of months ago, she started drinking alcohol in moderation in order to cope with her feelings of distress and low mood due to divorce and her son's removal from her care. There was no history suggestive of any illicit drug abuse in this lady.
During her current admission, Ms. X disclosed that she had multiple and distressing spontaneous orgasms since her childhood following sexual abuse by her father. These orgasms would generally be coupled with her feeling angry on any account. However, she stopped experiencing these orgasms with the initiation of citalopram but would experience them again if she did not take citalopram for a few days. Unfortunately, when she was hospitalized following cerebrovascular accident about 3 years ago, citalopram was discontinued in error and she started re-experiencing these multiple orgasms. Apart from hypertension, she did not have any other risk factors to have cerebrovascular accident at the age of 52. She could have up to 70 orgasms in a day and felt totally exhausted. She did not demand citalopram out of embarrassment and shame for 3 months and continued to suffer till she was restarted on citalopram following which the orgasms disappeared.
Ms. X was diagnosed to be suffering from adjustment disorder brief depressive reaction based on her current psychosocial stressors and symptoms (F43.20 of ICD-10). She settled fairly quickly on the ward and improved in her low mood and stopped feeling suicidal and was discharged on citalopram (40 mg daily) and her other medications for her physical health problems (bisoprolol 10 mg mane, levetiracetam1 g twice a day, tramadol 100 mg twice a day).


A 65-year-old male presented to our clinic for cataract surgery. He was a known case of diabetes mellitus since 5 years that was well controlled with oral antidiabetic drugs. The preoperative fasting and postprandial blood sugar status was found to be within normal limits. The preoperative best corrected visual acuity (BCVA) was 20/120 in the right eye and 20/80 in the left eye. The preoperative slit lamp biomicroscopic examination revealed posterior subcapsular cataract in both eyes. Fundus examination using indirect ophthalmoscope was normal in both eyes. Specular microscopy revealed a cell density of 1984/mm2 in the right eye and 1934/mm2 in the left eye, and coefficient of variation (CV) of 0.35 in the right eye and 0.32 in the left eye. Ultrasonic pachymetry revealed central corneal thickness of 0.57 mm in the right eye and 0.58 mm in the left eye.
Patient underwent phacoemulsification and intraocular lens implantation under peribulbar anesthesia, using a temporal scleral tunnel approach in his right eye. A dispersive viscoelastic (sodium chondroitin sulfate, 4%; sodium hyaluronate, 3%) was used during the surgery considering the low cell count. No intraoperative complications were noted. On postoperative day 1, the BCVA in the right eye was 20/400. Slit lamp examination revealed diffuse corneal edema and DM detachment extending from the temporal sclerocorneal tunnel wound and involving most of the cornea. Anterior segment optical coherence tomography (OCT) was done for documentation []. The central corneal thickness was unrecordable. Considering the large extent of DM detachment, a decision was taken to do descemetopexy. On the same day, 14% C3F8 was injected into the anterior chamber of the right eye through inferotemporal limbal paracentesis track in the operation room. Visual acuity and central corneal thickness gradually improved to 20/40 and 0.586 mm, respectively, over a period of 3 weeks.
Two months after the first surgery, the patient underwent phacoemulsification and intraocular lens implantation in his left eye. The surgery was performed by the same surgeon with extreme caution, using a temporal scleral tunnel approach. A dispersive viscoelastic (sodium chondroitin sulfate, 4%; sodium hyaluronate, 3%) was used during the surgery. No intraoperative complications were noted. On first postoperative day, the visual acuity in his right eye was 20/40 and in the left eye was counting fingers at 3 feet. Slit lamp examination revealed diffuse corneal edema and DM detachment involving most of the cornea in his left eye. Anterior segment OCT was done for documentation []. The central corneal thickness was 0.896 mm in the left eye. On the same day, descemetopexy was done using 14% C3F8, injected into the anterior chamber through inferotemporal limbal paracentesis track. Postoperative period was uneventful. Visual acuity and central corneal thickness improved gradually to 20/30 and 0.596 mm, respectively, over a period of 3 weeks. In both eyes, C3F8 bubble took about 2 weeks to disappear completely from the anterior chamber. Anterior segment OCT photograph was taken in both eyes 3 weeks postoperatively [Figs. and ].


An 85-year-old Kurdish man lived in Iran, had been referred for elective coronary angiography and was admitted because of typical chest pain but with mild intensity. One year ago he had experienced an episode of chest pain accompanied by troponin elevation that had been considered acute coronary syndrome. However, he had not been underwent coronary angiography, with any reason that we do not know. After that event, he did not have typical coronary symptoms until current presentation. He was scheduled for angiography because of typical chest pain and old electrocardiographic (ECG) changes.
Initially, His physical exam was unremarkable, ECG showed normal sinus rhythm with Q waves and inversion of T waves in II, III and aVF leads without any acute or new findings. Chest X-ray revealed the bulge aortic knob contour (). Routine transthoracic echocardiography, including the suprasternal view, showed dilated ascending aorta (4.6 cm) () and mild commissural aortic regurgitation preserved left ventricular systolic function. During catheterization, the left Judkins catheter passed without any problem, but we could not pass the right Judkins catheter, hence pigtail catheter was inserted and aortic root angiography showed the localized bulging of ascending aorta, but we were not sure whether it is dissection with true aneurysm or pseudoaneurysm? The findings on coronary artery angiography were unremarkable.
Multislice spiral CT angiography revealed aneurysmal dilation of ascending aorta from sinotubular junction with the saccular aneurysm at the proximal part of arch with a 37 mm per 56 mm maximum diameter (). There was extension of thrombosis into the proximal part of right brachiocephalic artery, but ostia of both arteries did not involve with the aneurysm. The patient had been referred for surgery.
During surgery we found the dissection of ascending aorta till distal to brachiocephalic artery (transverse arch involvement). Dissection flap was composed of some parts of intima and media layers (). Replacement of ascending and transverse aorta was performed with interposition tubular graft 30 and valve sparing. The operation and recovery was uneventful.


A 58-year-old woman presented to the emergency department after she awoke in the morning with cough. She went to sleep with a chewing gum in her mouth and when she awoke, the gum was no longer present. She reported sleeping many times with chewing gum in her mouth without any problems. She denied any other significant medical history and was a lifelong non-smoker. She denied dyspnea or fever, but reported some substernal chest discomfort and minimal blood tinged phlegm. Her presentation prompted the performance of a chest roentgenogram [] and computed axial chest tomogram []. The prior was unremarkable, but the latter confirmed the presence of a foreign body in the left proximal mainstem bronchus. The patient then underwent a flexible bronchoscopy through an endotracheal tube, with the use of a 5.2-mm Olympus Flexible Video Bronchoscope (Olympus America, Center Valley, PA, USA). A foreign body, identified as chewed gum, was noted in the proximal left mainstem bronchus. Repeated attempts to remove the gum with forceps and a basket were unsuccessful, with only small pieces being removed. We then decided to try to remove the gum with the use of the cryotherapy probe. The ERBE cryotherapy system (Erbokryo, ERBE Cryosurgery, Tübingen, Germany) was utilized, with a 1.9-mm cryotherapy probe. This allowed us to successfully remove en-bloc the entire chewing gum. The patient tolerated the procedure well and was discharged the next day with no complications, and with resolution of symptoms. Four weeks later during a routine follow-up, she reported no further problems.


Mr. T, a 28-year-old unmarried male, from a lower socioeconomic status family of Dehradun, India; presented to the Outpatient Department of the Institute of Human Behavior and Allied Sciences (IHBAS); An Institute providing Quality Care in Mental Health, Neurosciences and Behavioral Sciences in Northern India; with abnormal behavior, which was unmanageable at home. He was admitted at IHBAS; on detailed assessment, it was found that the patient was a diagnosed case of G6PD Deficiency since his childhood. He had 13 years of psychiatric illness, which was of insidious onset. It was a continuous course, with several exacerbations, and the duration of the last exacerbation was for one-and-a-half months preceding the index admission. His illness was characterized by muttering to self, unprovoked aggressive behavior, irritability, over-talkativeness, which was difficult to be interrupted, and claiming that the rays of the sun and moon enter his brain and make him hit his family members and destroy the household articles. He would also claim that planets Mars and Saturn were exerting a bad impact on his physical health and making him physically weak. He also statedthat these two planets would insert various thoughts into his mind and would take away his thoughts from his mind, against his wishes, following which his mind would go blank for a few minutes. The patient would also frequently complain of hearing voices of Pakistani troops marching toward Dehradun, his native place; in clear consciousness through his ears and he would hear voices of Pakistani soldiers telling him that they would kill him and destroy his nation. The patient would be distressed due to these voices and on several occasions would be found responding to these voices. He also used to claim that he possessed special powers, due to which he had cured his father's diminished vision, and would also claim to cure his earache. His other family members shared none of these beliefs. During exacerbations of his illness, he would donate the fruits and vegetables of his shop to people, at times he would even donate the day's earning to beggars, and would also smoke bidis excessively. His sleep would also decrease by about three hours a day and he would eat more food than his usual pattern.
During all these 13 years, the patient had never been asymptomatic, although the intensity and frequency of his symptoms would decrease with treatment. He had both affective as well as psychotic symptoms all through his illness. There was a past history of repeated hemolytic crises in the form of bleeding from the gums, till 12–13 years of age, but there was no history of recent hemolytic crisis. Since 1993, the patient had been tried on three different classes of antipsychotics, in adequate doses, for adequate durations, but with poor response. Mood stabilizers were also tried, with some improvement in symptoms. He had a history of three prior admissions and also had a family history of psychotic illness in his mother and two elder sisters, who were maintaining well on treatment. The patient's birth and developmental history was uneventful and he had a well-adjusted, pre-morbid temperament.
On physical examination, black discoloration of the gums of both upper and lower jaws was present and the rest of the physical examination was within normal limits. On mental status examination; his psychomotor activity and rate of speech were increased; reaction time was decreased, and there was pressure of speech. Affect was euphoric, with full range and reactivity, thought content revealed delusion of persecution, delusion of control, delusion of grandiosity, and delusion of thought insertion and thought withdrawal. He also had second and third person auditory hallucinations, with impaired judgment and poor insight into illness. All routine investigations were within normal limits (Hb-14g / dl, PCV-40.6%, MCV-98.6fl, MCH-32.4pg, MCHC-32.9g / dl, S.Bilirubin-0.88mg / dl, and no RBCs were detected in the urine examination).
Following discussions during the hospital stay, he was diagnosed as a case of Schizophrenia Paranoid Subtype, as per ICD-I0.[] However, a differential diagnosis of Schizoaffective Disorder Manic Subtype was also entertained, in view of the prominent manic symptoms during the course of illness. During his stay of three weeks, he gradually improved and was discharged. The patient later dropped out of follow-up.


Mrs. A, a 48-year-old homemaker, presented in 2008 with a three-year history of involuntary movements of the trunk and the hands, which had worsened over the previous 7-8 months. She had predominantly choreic movements of both upper limbs. She also had behavioral changes such as irritability and aggression of recent onset. Family history was not available. On examination, ocular saccades were slow; she had generalized choreiform movements involving hands legs, lips and the trunk; dystonia of hands and feet and ataxic gait. MRI showed caudate atrophy. Neuropsychological assessment showed diffuse fronto-parietal and temporal involvement. A genetic analysis for Huntington's disease was performed after obtaining written informed consent which showed CAG repeat expansion. Patient was started on Lithium 150 mg, once daily, along with Carbamazepine 200 mg twice a day.
The movements have not worsened after starting Lithium and the behavioral symptoms improved completely. Patient is on regular follow up for the last four years and is maintaining the same improvement.


Mr. B, a 58-year-old male, presented in 2001 with a history of abnormal movements. These movements had started at the age of 35 years and were slowly progressive. A clinical diagnosis of Huntington's disease was made. At the time of presentation, the movements were disabling, irregular, would worsen with anxiety and disappear during sleep. Patient had a significant family history of HD; involving both parents and 7 out of 9 siblings, totally 9 affected first-degree relatives. On examination, patient was noted to have severe generalized chorea involving the hands, legs, trunk and the neck. CT scan showed atrophy of caudate. A genetic analysis showed a pathological CAG repeat expansion in Huntington's gene. The movements continued with a variable course over the next 8 years.
Patient was admitted in 2009 with a worsening of movements over the previous 6 months and weight loss of about 6 kg. Patient was evaluated for medical causes of weight loss. All investigations including prostate specific antigen and chest X-ray were normal and the weight loss was attributed to nutritional causes. Patient was prescribed dietary modifications to prevent further weight loss. Patient was started on Carbamazepine 200 mg twice a day for control of movements. Patient was also started on Lithium 150 mg at night. Patient reported a decrease in severity of the movements which continued following discharge. Family members reported that the patient was able to eat well and had no behavioral symptoms. However, he still needs support for some activities of daily living. The patient has been on follow up for the last three years and maintains status quo.


Mrs. C, a 51-year-old lady presented in 2002 with history of psychiatric symptoms for the previous 8 years. Onset was sudden with symptoms of being anxious, accusing the family members of neglect, and being violent toward family members. Patient also had history of non-rhythmic quasi-purposive movements of hands which she attributed to anxiety. Family history revealed that her mother had movement disorder and an early death. On examination, patient C had choreic movements of the limbs, anxiety and depressive symptoms. A diagnosis of HD was made which was confirmed by genetic analysis, which showed expanded CAG repeats in the Huntingtin's locus. Patient was initially started on Carbamazepine 400 mg and Clonazepam 4 mg/day. Over the next one year, patient developed psychotic symptoms like delusions of reference and persecution toward her family members and was also started on Quetiapine up to 300 mg. Two years later, patient had sudden deterioration in symptoms with worsening of chorea and onset of cognitive decline–memory disturbances and apraxia, which made her completely dependent for all activities of daily living. During this time, she also developed significant mood swings. She was started on Lithium 300 mg. For the last 3 years, patient has been on a combination of Lithium 300 mg, Carbamazepine 800 mg, Quetiapine 300 mg and Clonazepam 2 mg/day. Patient's husband reported no further deterioration over 3 years in cognition or movements in the patient. However, she continued to be dependent on husband for activities of daily living and eventually died due to cardiac arrest.


A 37-year old male surgeon presented with three days of progressive muscle pain and weakness. Five days prior he had fever, myalgias and sore throat. His fever and sore throat resolved after two days, but he continued to have muscle pain that evolved into diffuse muscle weakness, greatest in his distal upper extremities. This disabled him from performing surgery. He denied any recent trauma or strenuous exercise. He also denied any medical history or medication use prior to his illness, except for ibuprofen three days prior to his presentation. He reported multiple sick contacts, including sick patients and his four-month old son.
His vital signs and general examination were normal. He had no rashes or joint pain. His forearm muscles and intrinsic muscles of the hand were tight and tender to touch. His neurological examination was notable for 5/5 strength throughout except bilateral wrist flexion and extension (4/5), finger flexion (3/5), finger extension (2/5) and thumb adduction (3/5). His reflexes and sensation were normal.
Laboratory values were generally unremarkable with the exception of CK (1,504 ng/ml). Pertinent negative virologic tests included RSV by PCR, parvovirus B19, EBV, CSF bacterial culture, HIV, Influenza A by PCR, Influenza B by PCR, and CSF Influenza by PCR.
He was given IV fluids and ibuprofen. One day after presentation, the swelling and tenderness improved. Two days later his symptoms had nearly resolved. Examination revealed only subtle 5-/5 weakness in bilateral forearms and hands. CK decreased to 752 ng/ml and serum creatinine remained stable and within normal limits. Nerve conduction and needle EMG (proximal, distal and paraspinal muscles) studies were normal. A few days later he was able to return to work, including performing surgeries.


A 25-year-old male presented with four days of progressive weakness and muscle pain. Initially he noticed myalgias in his chest and biceps. Two days prior to presentation he noticed forearm pain and hand weakness. One day prior to presentation he noticed lower extremity weakness and pain, limiting his ability to ambulate. Two weeks prior to presentation he had been prescribed amoxicillin for a sore throat and fever, however he was not tested for streptococcus or influenza at that time. He denied prior medical problems, trauma, routine medication use or recent strenuous exercise.
Vital signs and general examination were normal. He had no rashes or joint pain. Bilateral arm and forearm muscles were tight and tender. Strength was decreased bilaterally in upper and lower extremities, including wrist extension and flexion (4/5), grip strength (4-/5), hip flexion (3/5), hip extension (5-/5), knee flexion (4-/5) and knee extension (5-/5). His reflexes and sensation were normal.
Laboratory values were generally unremarkable with the exception of a CK of 3,575 ng/ml. Pertinent negative virologic tests included CSF bacterial culture, HIV, Influenza A by DFA, Influenza B by DFA, and CSF Influenza by PCR. An MRI of the cervical spine was normal.
He was given IV fluids and ibuprofen. The day following presentation his weakness continued to progress and involved truncal weakness. Needle EMG study (distal, proximal and paraspinal muscles) showed only one run of positive waves in the thoracic paraspinal muscles. Two days after presentation he had dramatic improvement. His strength returned to 5/5 except in his left upper extremity strength, which improved to 4+/5 in wrist flexion, wrist extension and finger extension. The swelling and tenderness also improved. His CK decreased to 975 ng/ml and serum creatinine remained stable and within normal limits.


A 45-year-old male presented with four days of progressive weakness and pain. He first noticed trouble manipulating small objects with his fingers. The weakness in his hand progressed to the point that he was no longer able to perform his job as a cable installer. He reported his forearms were tender to the touch. The patient's wife had confirmed H1N1 influenza four weeks prior, requiring hospitalization. He also had a fever, cough and sore throat shortly after his wife was sick, but did not seek medical attention. He had otherwise been in his usual state of good health with no medication use or recent trauma.
Vital signs and general examination were normal. There was no evidence of skin rash. His muscles were tender to the touch. His neurological examination was notable for decreased strength to grade 4+/5 in bilateral upper extremities, hip flexors and hip extenders. Reflexes and sensory examination were normal.
Laboratory values were generally unremarkable with the exception of a CK of 600 ng/ml. Pertinent negative virologic tests included CSF bacterial culture, Influenza A by PCR, Influenza B by PCR, and CSF Influenza by PCR.
The day following admission his strength had returned to 5/5 and the muscle soreness had resolved. His CK declined to 410 ng/ml and serum creatinine remained stable and within normal limits. He had been given IV fluids and ibuprofen.


A 79-year-old female presented with a sore throat and non-productive cough for 10 days, a rash for 7 days, and fever for 4 days. The rash was painful and initially began on the palms of both hands with subsequent proximal spread to the torso. At the same time, the patient developed a similar rash on the plantar aspects of both feet with progression to the lower legs. The patient also reported a rash within her mouth that she noticed on the day of presentation.
The patient's medical history was significant for PFTC (diagnosed 2 months prior to admission) with metastases to bilateral lungs, ribs, and clavicles. She underwent a total thyroidectomy 1 month prior to admission and has since been receiving radioiodine therapy. Her family history was significant for multiple first-degree family members with thyroid carcinoma of unknown pathology.
On physical exam, the vital signs were remarkable for a fever of 100.6°F. The remainder of the exam was significant for the rash that manifested as distinct and intact tender pustules of varying (5~7 mm, diameter) sizes as well as several erythematous halos with surrounding edema. These findings were present on the dorsal and palmar surfaces of the hands () and extended to the mid-radial/ulnar region. The rash was also present on the abdomen, bilateral heels, and on the mucosal surface superior to the lower labial frenum.
Initial laboratory results revealed leukocytosis (17,100 cells/mm3) and neutrophilia (80%). A Group A streptococcal throat culture and anti-streptolysin O (ASLO) titers were positive with an ASLO of 894 IU/ml, and an erythrocyte sedimentation rate (ESR) of 100. Anti-neutrophil cytoplasmic antibody, anti-nuclear antibody, varicella- zoster direct fluorescent antigen, a syphilis screen, and blood/viral/wound/urine cultures were all negative. In light of the patient's painful rash on the hands, an X-ray of the left hand was performed and demonstrated osteoarthritic changes in the digits.
Upon admission, the patient was empirically started on vancomycin for possible methicillin-resistant Staphylococcus aureus infection. The patient remained febrile with an increase in the leukocytosis (29,200 cells/mm3), and ceftriaxone was added on hospital day 4; initial and subsequent blood and urine cultures were repeatedly negative. Given the persistent rash and fever, biopsies of the upper extremity lesions were taken and dermatopathology demonstrated superficial perivascular dermatitis with deeper sections revealing pustular vasculitis consistent with SS (, pathology showing SS). Following the histopathologic diagnosis of SS, prednisone was started with improvement in pain and swelling noted by the patient. Within 1 day of starting prednisone, the patient became afebrile. She received 10 days of steroid therapy, which resulted in resolution of the diffuse pustules on follow-up visits.


A 33-year-old man presented with recurrent episodes of abdominal pain. He had experienced intermittent epigastric pain for about 10 months, which developed into pain of the entire abdomen for 3 months. The abdominal pain was not associated with any other symptoms and resolved spontaneously, so he did not seek medical attention. There was no significant past medical or family history. Laboratory findings on admission were unremarkable. Abdominal computed tomography revealed slightly dilated loops of small bowel and a hypo-dense, irregularly contoured polypoid mass in the ileum (). Radiological features were suggestive of intussusception. However, there was a difficulty in finding the cause of the ileo-ileal intussusception in this patient. He had no history of previous operations or malignancy and was relatively healthy. During the operation to address the intussusception, a dimple was noted in the antimesenteric ileal wall, 40 cm proximal to the ileocecal valve. Gentle reduction of the intussusception demonstrated a large polypoid mass attached to a stalk at the corresponding mucosa of the dimpled area. Segmental resection of small bowel was performed. Grossly, the resected specimen showed a large pedunculated mass (6×4×3 cm) with surface ulceration. The stalk portion was connected to adjacent mucosa. On sectioning, the mass was homogenous, soft, and yellow with some white fibrotic tissue in the stalk (). The preliminary gross diagnosis was a submucosal lipoma. Microscopic findings revealed that the mass consisted of mature fat cells with scattered pancreatic acinar and ductal cells in the periphery. The mucosa overlying the mass was gastric type epithelium and showed focal ulceration.
The proper muscle was obliterated. The final diagnosis was a lipoma within an inverted Meckel's diverticulum (). The patient was discharged on postoperative day 7 without any complications.


A five-year-old girl was scheduled for surgical closure of an atrial septal defect under cardiopulmonary bypass. After routine preuse check, anesthesia was induced by inhalation of sevoflurane, using Jackson Rees’ modification of the Ayre's T-piece (JRMATP). Induction and tracheal intubation were uneventful. The lungs were ventilated using a circle breathing system and volume-controlled ventilation (VCV). The capnogram showed an increasing trend of end-tidal carbon dioxide (EtCO2; 45 mmHg) with high inspired carbon dioxide (FiCO2; 12 mmHg). Soda lime in the canister was fresh and did not show any change in color. Increasing the flow of fresh gas had no effect on FiCO2. As the source of the problem could not be identified, the breathing system was changed to JRMATP and anesthesia was continued. Later, the anesthesia machine was replaced and further management of the case was uneventful.


A nine-year-old boy was scheduled for the release of a burn contracture in the leg. Anesthesia was induced with propofol and a size 2.5 laryngeal mask airway (LMA) was inserted. Anesthesia was maintained with isoflurane using a circle breathing system, with the patient breathing spontaneously. The capnogram showed a progressive increase in EtCO2 with elevated FiCO2 (up to 8 mmHg), not altered by increasing the flow of fresh gas. Soda lime appeared unaltered. When ventilation was assisted manually, EtCO2 decreased to normal levels and touched baseline during inspiration. The problem recurred whenever the child was allowed to breathe spontaneously without assistance. Anesthesia was continued with assisted ventilation and further management was uneventful. On close inspection, the flexible leaflet of the expiratory UDV appeared minimally deformed. After the end of the procedure, the UDV was tested and found to be incompetent.


A four-year-old boy was scheduled for cleft palate repair, as the third patient on the list. After managing two adult patients earlier, the corrugated tubing of the circle breathing system was changed to the pediatric type and tested for leaks. Anesthesia was induced with sevoflurane using JRMATP. After tracheal intubation, the circle breathing system was used with pressure-controlled ventilation (PCV). After a few breaths, FiCO2 increased to 9 mmHg []. Soda lime was only partly discolored. The flow rate setting in the ventilator (i.e., the flow rate used to achieve the set inspiratory pressure, an equivalent of Pramp or ‘rise time’) was increased and FiCO2 became zero. With manual ventilation, FiCO2 increased whenever the tidal volume was delivered slowly and decreased with rapid delivery of tidal volume. We noted that the leaflet of the expiratory UDV was deformed and part of it remained open during inspiration []. The valve was closing fully with rapid delivery of tidal volume (or higher flow rate). The flow waveform on the ventilator showed minimal flow during inspiration []. The flow sensor was not direction sensitive, and was showing both the normal expiratory flow and the abnormal reverse flow on the same side of the Y-axis (the ventilator was not equipped with any alarm to indicate reverse flow). Later, the expiratory UDV was tested and found to be incompetent. The machine was used in subsequent patients after replacing the defective valve leaflet. The problem recurred after a few days and the new leaflet was also found to be deformed.
On checking all anesthesia machines with similar UDVs, the inspiratory UDVs were found to be deformed and incompetent in some machines, although no problem was reported during clinical use. The matter was reported to the manufacturer and they concluded that the problem was caused by the thinness of the valve leaflet (0.3 mm). All the leaflets in UDVs were replaced with thicker ones (0.5 mm). No similar problems have been reported since then.


A 3-year-old boy presented with a left side facial deformity at the outpatient department. Parents reported that the swelling started as an insidious and was slowly enlarging, remarkably noticeable since birth. [] On examination a soft mass infiltrating the left cheek skin from the inferior mandibular border to the orbital rim and left labial commissure. The boundaries of the lesion were ill defined and it was non-pulsatile. The underlying facial skeleton was not involved. Clinically, the patient had a prominent, large nevus on the left cheek. The oral mucosa appeared normal. A working diagnosis of lymphangioma was made, and it was decided to observe the lesion annually.
As the child continued to grow, a progressive accelerated growth in the size of the left cheek was observed. Radiologically there was hypertrophy of all facial bones in the region, including zygoma, maxilla and mandible. When he was 4 years, a biopsy of the upper lip was made for a diagnosis, revealing a diffuse, poorly circumscribed, unencapsulated, lobulated, fatty infiltration consisting of mature fat cells without any cytological abnormalities. Nerve and muscular fibers and thin-walled blood vessels were observed throughout the adipose tissue. Neither lipoblasts nor signs of malignancy were found. A diagnosis of CDIL- F was made based on the clinical, radiological and histopathological features. Surgical reduction of the lipomatosis area was planned and executed. In the process most of the nevus was also removed. Only a minor portion of the nevus remained along the surgical margin [].
Two more surgical debulking of the lipomatosis was carried out at age of 5 years and 7 years of age []. Currently the boy is 8.5 years old [] and presented with clinical evidence of hypertrophy of the left masseter, the left medial pterygoid muscle, the left hemi-tongue, and the soft tissues of the left cheek. The left zygoma and inferior orbital rim was also hypertrophied [Figures –]. The findings were confirmed by computed tomography. No ophthalmological, neurological (left cranial nerves V and VII were intact), vascular or respiratory problems were present. Psychomotor development till date was completely normal, and no other fatty infiltration was discovered on examination of the rest of the body. The patient is being followed up.


A 30-year-old female patient presented with complaints of fever, breathlessness, dull-aching, continuous pain, and dragging sensation on the left side of the abdomen since 3 months. Also, there was history of loss of weight and appetite. Past history was insignificant. On examination, abdomen was slightly distended with a palpable mass reaching up to the umbilicus, 6-7 cm from the left costal margin. The mass was tender, firm, irregular and fingers could not be insinuated below the ribs. A systemic examination was normal. Routine investigations revealed leucocytosis (Total leukocyte count=12,000/mm3) with eosinophilia (7%, absolute eosinophil count=840/mm3) and raised erythrocyte sedimentation rate (ESR) (35 mm in first hour). An X-ray study of both abdomen and chest was found to be normal. Ultrasonography of the abdomen showed enlarged spleen with multiple cystic areas, together measuring 18×15×11 cm. []. A computed tomography (CT) scan showed a large mass measuring 17.5×15×10 cm, containing air fluid levels and heterogeneous contents with predominant low-density areas, elevating left dome of the diaphragm []. Other abdominal organs were found to be normal. Therefore, the clinical diagnosis of the cystic lesion of spleen was made.
Left subcostal exploratory laparotomy was performed revealing huge cystic mass occupying lower pole of spleen. Splenectomy was performed and sent for HPE. Tab. Mebendazole 200 mg/kg/day was started on fifth post operative day and continued after surgery for three months.
Grossly, the splenectomy specimen measured 17×14×10 cm. The posterior aspect showed a cystic cavity having a white and smooth cyst wall, filled with multiple, variable-sized daughter cysts in a flower-shaped arrangement []. The hematoxylin and eosin (H and E)-stained slides showed a cyst wall comprising of three layers []. The cystic contents consisted of mixture of multiple infolded membranes, fragmented hooklets, and debris which were part of hydatid sand. Multiple scolices of Echinococcus granulosus were also seen []. Definitive diagnosis of a hydatid cyst was, hence, made.


An 18-year-old man with a history of asthma was admitted to a local hospital because of fever, profound headache, weight loss, red eyes, blurred vision, and generalized muscle aches. All symptoms resolved spontaneously within 1 month before a classifying diagnosis could be made. Five months later, the headache and blurred vision recurred, and the patient was referred to our outpatient clinic. Laboratory analysis at that moment revealed only mild eosinophilia (11%; 600 × 106/L). Renal function was normal, and anti-neutrophilic cytoplasmatic antibodies and anti-nuclear antibodies were not present. Cranial magnetic resonance imaging showed a unilateral thickening of the choroidal cerebral plexus, and analysis of the cerebrospinal fluid revealed eosinophilic meningitis. Another 3 months later, the patient developed a persistent fever and a pulmonary infiltrate that did not respond to antibiotics. Broncho-alveolar lavage revealed profound eosinophilia (30%). During admission, eosinophilic skin infiltrates and generalized edema developed. Now, we considered the diagnosis of Churg–Strauss syndrome, and therapy with prednisone at a dose of 1 mg/kg was initiated. Subsequently, the patient developed a painful injected pharynx and chest pain, with diffuse ST segment elevation on electrocardiography. Echocardiopgraphy revealed a mild pericardial effusion, without wall motion abnormalities. Intravenous gammaglobulin (400 mg/kg per day for 5 days) was now administered. As the chest pain recurred and there was a rise in the plasma troponin-I concentration, a coronary angiography was performed. This showed multiple aneurysms and stenotic lesions in all coronary arteries (Fig. ). MRI scanning excluded involvement of the aorta and its major branches. In view of the potentially fatal coronary lesions, a more powerful immune-suppressive regimen consisting of methylprednisolone and cyclophosphamide (750 mg and 1,250 mg i.v. per day during 3 days, followed by oral doses of 1 mg/kg/day prednisone, and 2 mg/kg/day of cyclophosphamide) was chosen. The prednisone was tapered gradually and stopped after 1 year. The cyclophosphamide was tapered and stopped after 16 months. A control coronary angiography 1 year after initiation of therapy showed a complete regression of all lesions (Fig. ).
Our patient fulfilled the classification criteria of the Churg–Strauss syndrome as established by the American College of Rheumatology (asthma, eosinophilia, pulmonary infiltrates, and a biopsy containing accumulation of eosinophils), although he also had signs and symptoms compatible with other vasculitis syndromes, such as Kawasaki disease (bilateral conjunctival injection, injected pharynx).
Endocardial thickening due to local infiltration of eosinophils, which can trigger intracardial thrombus formation, is the most common presentation of heart involvement in patients with the Churg–Strauss syndrome [, ]. Pericardial involvement, presenting either as an acute pericarditis with pericardial effusion, as in our patient, or as chronic constrictive pericarditis, has been reported in 8–32% of patients []. In contrast, clinically significant vasculitis of the coronary arteries has been reported only anecdotically [–]. In general, symptomatic cardiac involvement is associated with poor prognosis in patients with medium or small vessel vasculitis and justifies a strong and immediate immunosuppressive treatment. Indeed, the present case report shows that therapy with prednisone and cyclophosphamide may lead to a complete and uneventful recovery of potentially fatal coronary artery lesions.


A 63-year-old man who had suffered the left middle cerebral artery (MCA) territory ischemic stroke made a good clinical recovery. Pre-procedure neurologic examination showed minimal right upper extremity motor weakness with mild speech and cognitive dysfunction. The Magnetic Resonance Imaging and MR angiography showed about 90% luminal stenosis on the left proximal internal carotid artery (ICA). The patient agreed to be enrolled into the Carotid Revascularization Endarterectomy and Stent Trial (CREST) and he was randomized to Stenting arm. The CAS was performed under the CREST Stenting protocol by a CREST certified interventionist under neuroleptic anesthesia.
Diagnostic angiography showed approximately 70% of luminal stenosis by NASCET criteria (). A 6 Fr Shuttle® Guiding catheter (Boston Scientific, USA) was placed at the proximal left common carotid artery. Subsequently a 4.5 mm sized Accunet® protection device (Guidant Co. USA) was introduced thru the guiding catheter and placed at the distal cervical segment of the left ICA. A 4 × 15 mm sized Voyager angioplasty balloon (Abbott Vascular, USA) was used to perform pre-stent angioplasty and the balloon was inflated upto 8 atm. The post-angioplasty angiography which showed dissection of atheromatous plaque with contrast filling (). A 7 × 40 mm sized RX-Acculink® Carotid stent (Guidant Co. USA) was then introduced by coaxial manner and deployed across the stenotic segment. Immediate after the stent deployment, the patient's showed acute expressive dysphasia, disorientation and decreased verbal comprehension. He also developed right lower extremity weakness. Immediate post stenting angiography of the left ICA showed about 4 × 10 mm sized spindle shaped filling defect (), which was captured by the protection device. The intracranial circulation showed significantly decreased blood flow (). Assuming the lesion could be a mixture of multiple fragmented plaque debris associated with platelet aggregation, we started Reopro® (Lilly, USA) infusion (0.25 ug/kg bolus IV) to prevent further potential progression of platelet aggregation. After 10 minutes of Reopro® infusion, the patient's intracranial flow was reestablished. However, even after 25 minutes of Reopro infusion, the size of filling defect did not show interval change (). Thus, we thought the filling defect would more likely to be an organized embolus from the plaque than platelet aggregation, we decided removing the lesion with the protection device to prevent fracturing the lesion during the retrieval process. To facilitate the removal of the filling defect, we angioplastied the stented left ICA with 6 × 20 mm sized balloon catheter. Then, we carefully advanced Rx-Accunet retrieval device2® (Guidant, USA) and tried to pull the EPD and retrieval device down thru the stent without squeezing the filling defect. However, since the retrieval device could not completely capture the EPD, a couple of the EPD wires were always catching stent struts during the removal process. Thus, we used the 6 Fr Shuttle® guiding catheter as a supporting retrieval device. We carefully advanced the 6 Fr Shuttle® catheter up to the proximal 1/3 of the retrieval device () without squeezing the filling defect and successfully pulled the whole system through the stent toward the descending aorta. At this point, we removed protection device and retrieval device thru the guiding catheter but left shuttle at the descending thoracic aorta. Post-embolus removal angiography showed no evidence of residual emboli on both extracranial and intracranial circulations (). The retrieved embolus was 12 × 3 mm in size (). The histopathologic examination showed relative acellular matrix with frequent cholesterol clefts, focal calcifications and focal fibrous strands which are consistent with typical pathologic findings of organized atherosclerotic plaque (). The patient was discharged from the hospital post-CAS 2nd day without additional neurologic deficit to his pre-procedure deficits. Post-CAS 30th day clinical follow up showed improved speech and cognitive dysfunction than pre-CAS status.


Baby R K, 3-year-old girl, presented with progressive bilateral breast enlargement over 1 year, irregular vaginal bleeding for 10 months and pubic hair development for 2 months. There was no clitoral enlargement, neck swelling, pigmented skin marks, bone pain/fractures. Parents could not comment on any recent height gain. She had no significant medical illness or radiation exposure in the past. Her birth and neonatal period were normal. Her milestones were normal for her age.
On examination, her vitals were normal. There was no thyroid swelling, café au lait spots or any bony abnormality. Tanners staging was B3P3. There was no axillary hair or genital ambiguity. Cardiac, thoracic and neurological examinations were normal. A solitary well defined pelvic mass firm to hard, mobile was palpable in right lower abdomen.
Her investigations showed normal hemogram, liver and renal functions. Her bone age, height age and chorological age were respectively 8 year, 10 months, 4 year (100 cm) and 3 year. Thyroid functions were normal. Her hormonal profile was as follows: LH <0.1 mIU/ml, FSH <0.1 mIU/ml, Testosterone 0.334 ng/ml, DHEAS 17.97 μg/dl, Estradiol >1000 pg/ml.
Computed Tomography (CT) pelvis showed a heterogeneously enhancing abdomino-pelvic mass.
She underwent resection of right tubo-ovarian mass, which was suggestive of juvenile granulose cell tumor. Post-operatively, child had withdrawal vaginal bleeding on day 2 of surgery lasting 48 h. Repeat hormonal profile done on 6th post-operative day showed markedly reduced Estradiol levels 17 pg/ml, LH <0.1 mIU/ml, FSH 0.1 mIU/ml, testosterone 0.025 ng/ml, DHEAS 0.947 μg/dl. She is now 6 months post-surgery. There was no vaginal bleeding so far.


Baby S P, a 6-year-old girl, presented with progressive bilateral breast enlargement for 7 months, cyclical vaginal bleeding (3/25-30days) for 4 months and pubic hair development was noted for 2 months. There was no clitoral enlargement, neck swelling, pigmented skin marks, bone pain/fractures. There was h/o recent increase in height in the last 1 year. She had no significant medical illness or radiation exposure in the past. Her birth and neonatal period were normal. Her milestones were normal for her age.
On examination, her vitals were normal. There was no thyroid swelling, café au lait spots or any bony abnormality noted. Tanners staging was B3P3. There was no axillary hair or genital ambiguity. Cardiac, thoracic and neurological examinations were normal. Examination of the abdomen was normal.
Her investigations showed normal hemogram, liver and renal functions. Her bone age, height age and chorological age were respectively 7.8 year, 7 year (119 cm) and 6 year. Thyroid functions were normal. Her hormonal profile was as follows: LH < 0.1 mIU/ml, FSH < 0.1 mIU/ml, Testosterone 0.334 ng/ml, DHEAS 17.9 μg/dl, Estradiol > 162.8 pg/ml.
CT pelvis showed a bulky left ovary of size 5.4 cm predominantly solid with follicles seen at periphery and pubertal uterus.
She underwent laparascopic resection of right tuboovarian mass which on histopathology was mixed germ cell sex cord stromal tumor. Post-operatively, child had withdrawal vaginal bleeding on day 4 of surgery lasting 72 h. Repeat hormonal profile done on 6th post-operative day showed markedly reduced Estradiol levels 14.37 pg/ml, LH <0.184 mIU/ml, FSH 0.25 mIU/ml, testosterone 0.025 ng/ml, DHEAS 9.87 μg/dl. At 6 months follow-up, there was no vaginal bleeding so far.


16-year-old female presented with an anterior mid-line swelling in front of the neck since childhood. The swelling was gradually increasing in size and moves with deglutition.
She was investigated and Ultrasonography (USG) neck was performed, which suggested right paramedian neck mass measuring 26 × 16 × 13 mm. Dermoid Thyroglossal cyst. Fine needle aspiration cytology (FNAC) was performed suggestive of cystic changes.
Patient was referred to Nuclear Medicine Department for further evaluation of the patient.
Patient was injected 5 m Ci of Tc 99 m Pertechnetate and static images were acquired after 10 min. Initial static images showed avid tracer uptake at the base of tongue and in clinically palpable nodule at the level of hyoid and another small focus of tracer uptake in mid-line just above the clinically palpable nodule [Figures –].
Marker images were acquired. Lateral images were acquired to localize another focus of tracer uptake, which showed the tracer uptake along the line of descent. Patient was asked to drink water and images were acquired to exclude possibility of esophageal tracer uptake. Post-water drinking images showed persistent tracer uptake in suprahyoid location.
Computerized tomography (CT) scan of the patient was performed.
Thyroid function test dated: 5/08/10
T3: 1.28 ng/ml
T4: 7.65 ug/dl
TSH: 3.30 u IU/ml
Antibodies: Thyroid peroxidase (TPO) anti-body: Positive 11.4, ANA-Anti Nuclear Ab: N: negative.


A 52-year-old woman was admitted to our hospital with progressive onset of lower extremity weakness for two months. Neurological examination revealed spastic paraparesis (grade: 4/5), sensory deficit below the T7 level, mild hyperreflexia on lower limbs, extensor plantar responses, and clonus on the right side. In her medical history, she received RT and chemotherapy for pulmonary mucinous cystadenocarcinoma on right upper lobe two years prior to her admission. A review of the radiation treatment chart revealed the RT conducted in two phases. In the first, she had received 20 fractions of 2 Gy per month.
Two months later, a second phase RT was given in 11 fractions 2 Gy each. Total tumor dose of 62 Gy was given in three months. The 18 months subsequent to RT were uneventful.
Magnetic resonance imaging (MRI) of the spinal cord demonstrated a long segment of intramedullary lesion extending from T3 to T6 levels. The spinal cord appeared diffusely enlarged over the involved segment and a partially enhancing lesion was observed after Gadolinium administration []. Suspected diagnoses according to MRI findings were primary intramedullary tumor, metastatic tumor, or radiation myelitis. The spinal tap revealed a normal cerebrospinal fluid (CSF) chemistry and microscopy. Somatosensorial evoked potential responses were prolonged on right lower limb and absent on the left.
For positron emission tomography (PET)/computed tomography (CT) examination, the patient was intravenously injected 402.93 MBq (10.89 mCi) of F-18 fluorodeoxyglucose (F-18 FDG). After 90 minutes of uptake period, the patient was imaged using an integrated PET/CT camera (GE Discovery STE 8, USA). In the PET/CT images, there was no pathological FDG uptake suggesting malignancy at the spinal cord in the thoracic region []. On the basis of patient history, clinical and radiological findings, the patient was diagnosed as DRM.
The patient was treated with high dose methylprednisolone, 1 g/day for 5 days (pulse therapy) followed by oral methylprednisolone 80 mg day-1 for a week, tapered over 3 weeks. Simultaneously, rehabilitation therapy was also applied. Despite these therapies no objective improvement was detected on neurological examination in six months. Moreover, spasticity deteriorated slightly.
The control MRI findings were not parallel to neurological examination results. Follow-up MRI of spinal cord showed complete resolution of the hyperintense lesion on spinal cord [].


An 18-year-old male patient presented with the signs and symptoms of continuous, high-grade fever without chills and rigors since one week. He also had associated vomiting, altered sensorium, and was unconscious with delirium of one-day duration. On examination, the patient was unconscious, pulse: 82/minute, respiratory rate: 14/minute, blood pressure (BP): 110/60 mm of Hg. On auscultation, S1 and S2 were heard, and lungs were clear. There was no organomegaly, but tenderness in the right hypochondrium was present. Plantars were downgoing; he was started on antimalarial therapy. As per altered sensorium and deterioration, mechanical ventilation and intubation were done.
Basic laboratory investigations were done. Hemoglobin was 12.9g/dL, total leukocyte count was normal with normal differential count, and there was severe thrombocytopenia. Erythrocyte sedimentation rate (ESR) was normal. Plasmodium falciparum ring forms: 1/1000 RBC were seen. Complete urine examination showed trace amount of albumin. Biochemical analysis showed elevated serum bilirubin (2.4mg/dL), alkaline phosphatase, and transaminases. Disseminated intravascular coagulation (DIC) profile showed prolonged prothrombin time (PT), activated partial thromboplastin time (APTT), fibrinogen -64mg/dL, and markedly reduced platelets. Lactate dehydrogenase was raised significantly.
The patient received blood components and platelets, and requested for fresh frozen plasma in view of DIC. Transfusion therapy received by the patient from day one of admission until death was reviewed [] and the description of the anaphylactic reaction [] was taken into consideration for investigating the adverse reaction. During the same period, plasma units of the same implicated platelet components were transfused to three different patients with bleeding, without any adverse transfusion reactions.
Investigation of transfusion reaction included clerical check of patient identification; labels of blood components, and grouping and typing, along with requisition forms revealed no misidentification. Visual inspection of the postreaction sample of the patient was negative for hemolysis. Pretransfusion testing done on day one of admission revealed ‘O’, Rh-positive blood group and negative antibody screening. On day five, the patient was transfused with ‘O’, Rh-positive platelets and no pretransfusion sample was available. Direct Coomb's test and IgG monospecific antibody were positive in the post-transfusion sample. In view of the positivity of the direct Coomb's test, antibody screening for any irregular or clinically significant red cell antibody was done to rule out immune-mediated hemolytic transfusion reaction. Indirect Coomb's test was negative. Antibody screening by commercial three red cell panel was also negative. Based on this workup, the possibility of immune-mediated hemolytic transfusion reaction was ruled out.
Blood components as well as the sample of the patient were submitted for culture, and the result was negative for both. The activity of anti-IgA, IgG, and IgM was tested by gel column hemagglutination method.[] Gel column hemagglutination was positive for IgG antibodies and negative for IgA []. Serum imunoglobulins were measured by capture sandwich immunoassay using direct chemiluminescent technology and the results showed raised IgE antibody levels [].
Signs and symptoms of transfusion reaction such as rashes all over the body, bronchospasm, endotracheal secretions, hypoxemia, tachycardia, and breathlessness developed immediately after the platelet transfusions. As the gel hemagglutination assay was positive for IgG and serum IgE levels were raised, we considered non-IgA-mediated or IgE- and IgG-mediated severe anaphylactic transfusion (alternate pathway) reaction.[]
Based on some of the pulmonary signs and symptoms like breathlessness, hypoxia, and chest radiograph with lung infiltrates, transfusion-related acute lung injury (TRALI) might have been considered as the important differential diagnosis. However, the underlying critical illness of the patient with ventilator-associated pneumonia may explain the cause for acute lung injury. Subsequent transfusion of the plasma units of the implicated platelet components was uneventful.[]


A 32-year-old Polish Caucasian man was referred to the otolaryngology clinic by his general practitioner with a two-month history of right-sided nasal obstruction and epistaxis. The patient was an amateur boxer and had sustained many blows to the nose and face, but could not recall any major recent injuries. He had no symptoms of rhinorrhoea, postnasal drip, hyposmia or facial pain. The patient denied suffering from dysphagia, dysphonia or weight loss.
The patient was hypertensive (blood pressure of 167/95 when checked in the preassessment clinic) but was not on medication for this and was noted to have a high body mass index (35.9 kg/m2). He had no previous surgical history, was on no regular medications and had no drug allergies. There was no relevant family history. The patient was an ex-smoker.
Flexible nasendoscopic examination revealed a large right-sided nasal mass arising from the right middle meatus with some contact bleeding. Examination of the left nasal cavity, postnasal space and larynx was normal. There was no significant septal deviation. Systemic examination was unremarkable. Blood tests revealed a normal haemoglobin, platelet count, clotting and renal function.
A non-contrast-enhanced computerized tomography (CT) scan was performed and demonstrated a bulky mass lesion extending from the right maxillary antrum into the right nasal airway (Figure ). The mass was filling the entire right maxillary antrum (which was expanded) and thinning the lateral nasal wall. No abnormalities were seen on the left side.
The patient was subsequently taken to theatre for functional endoscopic sinus surgery. A large polypoid lesion was seen filling all of the maxillary antrum with extension into the right nasal cavity. The lesion was suspicious for a malignancy as the mass had produced a partial auto-maxillectomy and a very large middle meatal antrostomy. The entire lesion was excised piecemeal (Figure ) and sent for histology. The left nasal cavity was normal.
Histological examination revealed polypoid, inflamed and congested respiratory mucosa together with organizing blood clot. There were associated foamy and haemosiderin laden macrophages but no evidence of malignancy. The conclusion was of an inflammatory nasal polyp with organizing haematoma.
The patient was seen in the outpatients department two weeks postsurgery and was well with complete resolution of his symptoms. He was advised to have his blood pressure re-assessed by his general practitioner.