Kallmann syndrome (KS) is a neuronal migration disorder characterised by hypogonadotrophic hypogonadism and anosmia or hyposmia. Five patients with clinical findings suggestive of KS were evaluated with MRI. All patients had abnormalities of olfactory system. Olfactory bulbs were absent in all patients. Olfactory sulci were absent in 3 patients and hypoplastic in 2 patients. Anterior pituitary was hypoplastic in two patients. The MRI findings in KS are characteristic and MRI is a useful adjunct to the diagnosis of KS.

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Vitamin B12 deficiency usually presents with pernicious anemia or various neuropsychiatric manifestations. Commonly seen neuropsychiatric manifestations include large fiber neuropathy, myelopathy (subacute combined degeneration of the spinal cord), dementia, cerebellar ataxia, optic atrophy, psychosis and mood disorders. The present report highlights an unusual presentation of vitamin B12 deficiency- acute onset extrapyramidal syndrome in a 55-year-old man. The patient presented with a 10-day history of slowness of all activities including a slow gait, mild tremors of hands and low volume speech. On examination, he had features of mask-like facies, reduced blink rate and cogwheel rigidity. He was investigated for the possible causes and was found to have laboratory features of vitamin B12 deficiency. Other causes for acute onset parkinsonism were excluded by appropriate investigations. He showed a dramatic improvement following treatment with intramuscular vitamin B12 injections. At a five-year follow up, he was found to be functionally independent with no neurological deficits.

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BACKGROUND AND OBJECTIVE: Myasthenic crisis (MC) is one of the important and common complications in the natural history of myasthenia gravis (MG). MC can be precipitated by multiple factors including deficiency or excess of the acetylcholinesterase inhibitors (AChEI). Any episode of MC is an emergency requiring aggressive therapy. We studied the demographic, clinical and treatment-related characteristics of patients who developed MC. MATERIALS AND METHODS: A retrospective study was conducted in patients with MC admitted during a 31-month period from February 1999 to August 2001, at a tertiary care center in India. RESULTS: Eleven patients (9.69% of the total 114 patients with MG) were admitted with 12 episodes of MC. Mean age at presentation was 39.83 + 13.18 years with male predominance. Seven patients had undergone thymectomy previously; of which 2 had postoperative MC. Six patients had thymoma. Steroid or cholinesterase inhibitor withdrawal and infections were the commonest precipitating factors for MC. Patients required ventilatory support for median 14 days. They responded to low volume of plasma exchange (PE) (mean 854 ml / day with mean 6.5 cycles per patient). CONCLUSIONS: This report highlights that the subset of Indian patients with MG who are at risk to develop MC belong to the 3rd and 4th decade, have bulbar symptoms at presentation and are associated with thymoma. Patients with MC should have judicious drug adjustments under supervision and should be treated aggressively during impending MC.

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AIMS: We discuss our experience with the surgical management of scalp vascular malformation and review the literature on the subject. SETTINGS AND DESIGN: A prospective case-control study of eight patients with scalp vascular malformations admitted to our hospital between 1997 and 2002. METHODS AND MATERIALS: All the patients were investigated with selective internal and external carotid angiography. Depending upon the origin of feeding arteries, the scalp vascular malformations were classified into two categories: Group I: the primary scalp arteriovenous malformations and Group II: secondary venous dilatations. Six patients belonged to Group I and two patients were in Group II. RESULTS: Five patients belonging to Group I underwent successful excision of the arteriovenous malformation. There was no recurrence in this group. Of the two patients in Group II, one patient who had scalp vascular dilatation simulating a primary scalp vascular malformation underwent excision of the lesion. This patient developed severe postoperative brain edema and died. CONCLUSIONS: Primary scalp vascular malformation can be excised safely. However, excision of secondary scalp venous dilatation without treatment of the intracranial component can be dangerous.

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A case of vertebral osteomyelitis is presented where initial presumptive diagnosis of tuberculous infection was made on clinico-radiological grounds but eventually turned out to be Salmonella infection upon exploration, biopsy and culture. Patient recovered completely following debridement and appropriate antibiotics (fluoro-quinolones) for a period of six weeks. Internal fixation allowed early ambulation.

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BACKGROUND: Sural nerve biopsy (SNBx) is associated with multiple complications such as paresthesia, pain, or numbness in the sural nerve distribution at the site of biopsy and wound infection. An accurate idea of these adverse events would be useful while taking informed consent from patients. AIMS: We conducted a prospective study to determine the extent of sensory deficits after SNBx. SETTINGS AND DESIGN: It is a prospective, hospital-based (tertiary teaching hospital) study. MATERIALS AND METHODS: All the patients who had SNBx between May 2003 and March 2004 were eligible for inclusion. However, patients with sensory impairment in sural nerve territory or abnormal sural nerve conduction studies prior to the procedure were excluded. SNBx was performed in the ankle region under local anesthesia, and a 3 cm nerve segment was excised. Touch, pain, temperature, vibration and joint position were tested after the nerve biopsy. The extent of sensory deficit was determined. Any other complications, when present were also noted. Follow-up assessment was performed at three months or later. RESULTS: Fifty patients (26 women) fulfilled the inclusion and exclusion criteria. The mean age was 37.4 (16-63) years. One-two weeks after the SNBx, 46 (92%) patients had sensory deficit along the lateral aspect of the foot at the site of the biopsy, and 48 (96%) patients had sensory impairment extending beyond the outer aspect of the fifth toe. At follow-up, sensory deficit was present in 89% patients and paresthesia in 39%. CONCLUSIONS: The majority of the patients undergoing SNBx develop persistent sensory deficits, which often extend beyond the typical sural nerve territory.

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AIMS: To evaluate the management strategy of extradural hematomas (EDH), particularly with respect to its volume. METHODS AND MATERIALS: Two hundred and three patients with EDH and no other significant intracranial injuries were treated over a period of three years. RESULTS: The factors influencing management strategy and outcome were the Glasgow coma scale (GCS), volume of extradural hematoma (EDHV) and its location. The ultimate clinical outcome was significantly better in patients having EDHV of less than 30 ml. CONCLUSIONS: The key factors influencing the management strategy and clinical outcome are EDHV and GCS at the time of admission. A patient with EDH should not be considered for conservative management if EDHV is more than 30 ml and GCS is 13 or less.

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BACKGROUND: Limb girdle muscular dystrophy (LGMD) is a phenotypic expression of a heterogeneous group of diseases and sarcoglycanopathy is one of the causes of LGMD. There is only one study on sarcoglycanopathies in the Indian literature. No data is available from northern India. MATERIALS AND METHODS: All cases of muscular dystrophies, which were diagnosed in our laboratory in the last six years, were reviewed. Immunohistochemistry for various sarcoglycan proteins was done. Clinical features and pathological findings of the cases that were diagnosed as sarcoglycanopathies were reviewed. RESULTS: In the last 6 ½ years (1998-June 2004), we received 1435 muscle biopsies, of which 498 cases were of muscular dystrophies, and 13 cases were of sarcoglycanopathies (8 of gamma, 3 of alpha, 1 of both alpha and gamma, and 1 with absence of all four sarcoglycans). Sarcoglycanopathies comprised 2.6% of all muscular dystrophies, 11.8% of LGMD and 0.90% of all muscle diseases diagnosed in our laboratory. The mean age of onset was 7.2 years and the M:F ratio was 1.1:1. Most of them presented with difficulty in getting up, climbing stairs, calf hypertrophy and markedly raised CPK levels. Histological features were like dystrophinopathies. CONCLUSION: Sarcoglycanopathies are a relatively rare cause of LGMD and should be confirmed by immunohistochemistry as it will facilitate counseling and also prognostification. Although rare, in patients with muscle weakness, calves hypertrophy and raised CPK levels this possibility should be considered and needs to be differentiated from dystrophinopathies.

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AIMS: A series of cases with chronic subdural hematoma operated upon by residents in neurosurgery is analysed. MATERIALS AND METHODS: 159 patients treated between 1998 and 2001 were included in the study. Mean age was 76.4 years and male/female ratio was 1.7/1. The patients were classified both on admission and at discharge according to the Markwalder scale. The standard operative procedure consisted of an enlarged single burr-hole, rinsing the subdural space with iso-osmotic saline solution and insertion of a subdural drain. CONCLUSION: In CSDH, operation by the residents is safe and the results are comparable to those of the major series of the literature as the surgical procedure is standardized.

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Non-convulsive seizures (NCSzs) and non-convulsive status epilepticus (NCSE) occur in a substantial proportion of patients with acute brain injury. These acute seizure disorders are often unrecognized and under-diagnosed. Seizure semiology of NCSz is too subtle clinically to be noticed. Most often, mental status impairment is the presenting feature. Changes in the functions of the thalamo-cortical system in patients with impaired consciousness can be detected by continuous EEG (cEEG) monitoring. cEEG monitoring allows detection of the changes at a reversible stage, often when there are no clinical indications of such phenomena. In addition EEG provides reasonable spatial resolution and excellent temporal resolution. This makes cEEG an excellent method for supplementing single or serial recordings in the detection of NCSzs and NCSE. Recent advances in digital EEG have made cEEG monitoring in the neurological intensive care unit (NICU) technically feasible. Current evidence suggests that the common clinical denominator associated with electrographic seizures or NCSzs is mental status impairment. In NCSE, the duration of ictal activity and the time of delay to diagnosis are independent predictors of poor outcome. It will be prudent to do cEEG monitoring in any patient with impaired consciousness either in the setting of acute brain injury or with no clear explanation to detect NCSzs/NCSE. Early recognition and timely intervention is likely to be associated with good outcomes.

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BACKGROUND: To study the clinico-radiological determinants of outcome in patients with Chiari I malformation (CIM). MATERIALS AND METHODS: The disability assessment of 48 patients with C I M who underwent posterior decompression was done by modified Klekamp and Samii scoring system. The outcome was regarded as good when the patient was ambulant without any aid with an improvement in the disability score; and, poor when (a) there was postoperative deterioration or lack of improvement; (b) the patient was non-ambulant without aid, irrespective of the improvement in the clinical score; or, (c) there was a perioperative mortality. Patients with hydrocephalus; those who underwent syringo-subarachnoid or syringo-peritoneal shunt as the primary procedure; and, patients with atlanto-axial dislocation were excluded from the study. STATISTICAL METHODS USED: Categorical data were expressed in proportions and analyzed with Chi square test. Analysis of factors predicting clinical outcome at 6 months was done utilizing logistic regression analysis. RESULTS: The outcome assessed at six months showed that 30 patients (62.5%) had good outcome while 18 patients (37.5%; including two perioperative mortalities) had a poor outcome. CONCLUSIONS: Significant predictors of outcome in patients with CIM include the duration of symptoms (P value=0.006), respiratory distress (P value=0.001), and basilar invagination (P value=0.048). The effect of syringomyelia in predicting the clinical outcome could not be determined due to the differences in the number of patients in the groups with or without syringomyelia.

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A 45-year-old lady underwent right fronto-parietal craniotomy and subtotal excision of a parasagittal meningioma. Bone flap was not replaced as it was infiltrated by the tumor. In the postoperative period she developed episodes of altered sensorium associated with worsening of left hemiparesis and a sunken scalp at the site of bone defect. Computed tomography (CT) of brain showed sunken scalp flap in the right fronto-parietal region with compression of the underlying brain. A diagnosis of syndrome of the trephined was considered and her symptoms improved with cranioplasty. Pathophysiology of the syndrome of the trephined is discussed.

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The craniovertebral junction has a predilection for a variety of congenital anomalies due to its complex development. The association of atlantoaxial dislocation (AAD) with the maldevelopment of the posterior arch of axis is extremely rare. We report two such cases and present the management strategy.

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Brain metastasis from esophageal carcinoma is rare. In our center, among 301 cases of esophageal cancer referred for radiotherapy during a 14-year period, brain metastasis from esophageal carcinoma was detected in one case. An unusual case of esophageal carcinoma that presented with brain metastasis is reported.

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AIMS: The authors analyze epidemiology, location, clinical profile and outcome of double extradural hematoma (EDH) or EDH at more than one site. DESIGN: A retrospective clinical study. MATERIALS AND METHODS: 46 cases of double EDH were studied. All the cases were evaluated for their mode of injury, clinical presentation, level of sensorium at the time of injury, CT scan finding and outcome after surgery, and were compared with single EDH cases. RESULTS: Out of 1025 cases 46 cases had 'double' EDH. There was bilateral EDH in 39 cases; multiple EDH in 3 cases and ipsilateral double EDH was present in 4 cases. The most common site was frontal (70%). The majority of the patients (80.3%) were in altered sensorium from the time of injury, similar situation was seen in 52.2% of cases with single EDH. The number of patients having a low GCS score was higher when first examined in the double EDH group and the mortality rate was 34.8% as compared to 9% in the single EDH group. CONCLUSION: Majority of the double EDH cases presented with a low GCS and there was a relatively quick neurological deterioration in these cases.

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Brain irradiation is commonly used for many primary brain malignancies. We will present two cases of post-radiation vasculopathy of large arteries of patients who received radiation therapy to the brain as part of their tumor management. We will underscore the significance of this condition and suggest the overall management of patients receiving brain radiation at an early age.

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BACKGROUND: 11C-flumazenil (FMZ) positron emission tomography (PET) is a new entrant into the armamentarium for pre-surgical evaluation of patients with intractable temporal lobe epilepsy (TLE). AIMS: To analyze the clinical utility of FMZ PET to detect lesional and remote cortical areas of abnormal benzodiazepine receptor binding in relation to magnetic resonance imaging (MRI), 2-Deoxy-2 [18F] fluoro-D-glucose, (18F FDG) PET, electrophysiological findings and semiology of epilepsy in patients with intractable TLE. MATERIALS AND METHODS: Patients underwent a high resolution MRI, prolonged Video-EEG monitoring before 18F FDG and 11C FMZ PET studies. Regional cortical FMZ PET abnormalities were defined on co-registered PET images using an objective method based on definition of areas of abnormal asymmetry (asymmetry index {AI}>10%). SETTINGS AND DESIGN: Prospective. STATISTICAL ANALYSIS: Student's "t" test. RESULTS: Twenty patients (Mean age: 35.2 years [20-51]; M:F=12:8) completed the study. Mean age at seizure onset was 10.3 years (birth-38 years); mean duration, 23.9 years (6-50 years). Concordance with the MRI lesion was seen in 10 patients (nine with hippocampal sclerosis and one with tuberous sclerosis). In the other 10, with either normal or ambiguous MRI findings, FMZ and FDG uptake were abnormal in all, concordant with the electrophysiological localization of the epileptic foci. Remote FMZ PET abnormalities (n=18) were associated with early age of seizure onset (P=0.005) and long duration of epilepsy (P=0.01). CONCLUSIONS: FMZ-binding asymmetry is a sensitive method to detect regions of epileptic foci in patients with intractable TLE.

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Rett syndrome (RS), a neurological developmental disorder, is one of the commonest causes of cognitive impairment in girls and women. These patients are often initially misdiagnosed as idiopathic mental retardation, cerebral palsy, or autism. Despite several reports from the West, there are very few reports from the Indian population. We present four female children with RS and emphasize the importance of early diagnosis.

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Cerebral amyloid angiopathy (CAA) is well known to present with lobar intracerebral hemorrhage, dementia or transient neurological events. White matter changes with CAA have only been recently described and can be seen with either sporadic or familial CAA. We present a 50-year-old man with rapidly progressive dementia in whom MRI brain showed symmetrical white matter changes in the parieto-occipital regions. Brain biopsy revealed changes of CAA along with features of Alzheimer's disease. Immunohistochemistry revealed amyloid beta protein. The subcortical lesions were thought to occur from hypoperfusion of the distal white matter. The role of amyloid in the pathogenesis of CAA and the mechanism of leukoencephalopathy are discussed.

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BACKGROUND: Children with febrile seizures (FS) are at higher risk of developing epilepsy. There is robust literature on epilepsy with onset in childhood following FS but very little on the same issue in adults. AIMS: We intended to assess the association between adult-onset epilepsy and history of childhood FS. SETTINGS: The neurology clinic of a university hospital. DESIGN: A retrospective study. MATERIALS AND METHODS: Records of 101 consecutive adults (>14 years old) who were referred to our hospital with adult-onset seizures were reviewed and the patients and their families were interviewed to assess the medical history. STATISTICAL ANALYSIS: Chi-square test and Mantel-Haenszel method. RESULTS: Of the 101 patients, 9 were excluded for reasons of bacterial meningitis, recent head trauma, brain tumor, tricyclic antidepressants' overdose and missing reliable data of the childhood FS event. Thirty-one (33.7%) of the remaining 92 patients had history of FS in the childhood (71% men). Localization-related epilepsies were significantly associated with history of FS [Odds ratio: 3.29; (95% CI, 1.30-8.06)] ( ²= 5.49, df = 1, P=0.012) when compared to other epilepsies and epilepsy syndromes. An initial unprovoked simple partial seizure was also significantly associated with a positive history of FS [Odds ratio: 8.05; (95% CI 2.88-22.45)] ( ²= 15.86, df = 1, P<0.001). CONCLUSIONS: Localization-related epilepsies and partial seizures seem to be associated with a history of FS in childhood. This warrants more investigation to understand the mechanism as well as a possible pathology common in both localization-related epilepsies and FS in the affected probands.

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AIMS: Thirty-seven patients with intracranial cavernomas managed in our department are retrospectively analyzed. MATERIALS AND METHODS: The data of 37 patients with cavernoma who were admitted to our department between 1995 and 2003 were reviewed retrospectively. There were 30 male and 7 female patients with a median age of 26 years (range, 9-57 years). Four cases were treated surgically, 13 were treated by stereotactic radiosurgery (SRS) and the remainder were managed conservatively. RESULTS: New hemorrhage or additional neurological deficits were not observed in the surgically treated cases, 12 patients who underwent SRS and the other patients who were followed up. One of the 13 patients treated by SRS, underwent microsurgery due to increased seizure frequency. One of the patients treated surgically died on the 11th postoperative day. CONCLUSION: Clinical observation should be the choice of management for patients without new or progressive neurological deficits, without two or more hemorrhages and in patients where the seizures are controlled with drugs. Surgery is the first choice for the cavernomas located in the non-eloquent locations. Radiosurgery may be an alternative for patients having deep-seated and eloquent area located cavernomas and for patients not willing or suitable for surgery.

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We report a case of cerebral abscess, which ruptured into the lateral ventricle. The radiological features on conventional MRI, appearance on both diffusion weighted images (DWI) and apparent diffusion coefficient (ADC) maps are described.

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Fukuyama congenital muscular dystrophy (FCMD) is the most common congenital muscular dystrophy in Japan and there are isolated reports of non-Japanese patients with FCMD. We report an Indian patient with congenital muscular dystrophy and characteristic radiological findings similar to those with FCMD.

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AIMS: To look for changes in nerve conduction velocity (NCV) in early stages of glucose intolerance, i.e. in impaired glucose tolerance (IGT) and in asymptomatic newly diagnosed Type 2 diabetic subjects (NDD). MATERIALS AND METHODS: A total of 225 subjects were categorized as: Group 1: Subjects with normal glucose tolerance (NGT), Group 2: IGT subjects and Group 3: NDD subjects. Motor (MCV) and Sensory nerve Conduction Velocity (SCV) measurements were done. RESULTS: The mean MCV was significantly lower in the NDD group (47 ± 5 m/s) when compared with the other two groups (IGT=50 ±4.5 m/s; NGT= 53 ± 4 m/s; P=0.0001). The IGT group of subjects also exhibited a significantly lower mean MCV when compared with the NGT subjects (P=0.0001). The mean SCV in the NDD group (42+10 m/s) was also significantly lower (P<0.0007) than the NGT (46+6 m/s) and the IGT (48+10 m/s) groups. No significant difference in the mean SCV between the NGT and IGT groups was noted. In the multiple linear regression analysis both age and male gender were the risk factors for abnormal MCV and SCV. Abnormal MCV was found to be associated with 2-hr post glucose levels (R2 = 14.5%), while HbA1c (R2 = 4.9%) contributed towards abnormal SCV. CONCLUSION: Abnormal NCV is a common finding in NDD subjects. Slower mean MCV demonstrated by IGT subjects, calls for early screening of these subjects for complications.

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