Medulloblastomas (MBs) are the most common malignant brain tumors in children. Current therapeutic approaches combine surgery, radiotherapy, and chemotherapy. Although, there has been significant improvement in long-term survival rates, the tumor remains incurable in about a third of patients while cognitive deficits and other sequelae of therapy are common among long-term survivors. Hence a major challenge remains to differentiate high-from low-risk patients and to tailor therapy based on the degree of biological aggressiveness. A clinical risk-stratification system has been widely used in MBs based on age, extent of resection and the Chang staging system. However, recent reports indicate that these clinical variables are inadequate methods of defining disease risk. This has prompted search for new markers for MB stratification. Recent studies indicate that the classification of MBs according to profiles of histopathology and molecular abnormalities possibly help better risk-stratification of patients, thereby rationalizing approaches to therapy, increasing cure rate, reducing long-term side effects and developing novel therapeutic strategies. The most accurate outcome prediction till date has been obtained through microarray gene expression profiling. In this article, the current histopathological classification and the recent advances in molecular genetics of MBs are reviewed. Global efforts to translate this knowledge of disease biology into clinical practice especially as outcome predictors are highlighted.

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Background: Homozygosity for MTHFR C677T polymorphism can lead to significantly high homocysteine levels and hyperhomocysteinemia is an important risk factor for thrombotic events. Aims: The aim was to determine role of MTHFR C677T polymorphism in North Indians with ischemic stroke. Settings and Design: In a prospective study, the subjects of stroke were recruited from the neurology clinic of the hospital. Controls were healthy individuals from the Hematology clinic without any history of stroke. Materials and Methods: Plasma homocysteine levels were measured by enzyme immuno assay method after 3 months of acute episode. Serum folate and Vitamin B12 levels were estimated by competitive inhibition radioassay. MTHFR polymorphism was detected by PCR-RFLP using Hinf I enzyme. Statistical analysis: The analysis of significance of results was done using SPSS software package. A pvalue < 0.05 was taken as significant. Results: Thirty-two acute ischemic stroke patients (aged 1-44 years) were studied. Fourteen (43.8%) had recurrent stroke. Nine (28%) had multiple infarcts. Four of 32 patients (12.5%) had high homocysteine levels. Three out of these 4 hyper-homocysteinemia patients were homozygous ( TT ) for MTHFR polymorphism (2 with recurrent stroke). Two of three homozygous cases with TT genotype had low serum folate. Five of 32 stroke cases (18.8%) were heterozygous ( CT ) genotype. Conclusions: Primary hyper-homocysteinemia appears to be an important risk factor for ischemic stroke in North Indians, most due to MTHFR C677T homozygosity. Folate levels may modify the presentation of the MTHFR TT genotype.

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Background and Purpose: The role of carotid artery stenting (CAS) as an alternative to carotid endarterectomy in the treatment of for symptomatic carotid artery stenosis is investigated. Materials and Methods: Forty-seven patients underwent CAS over 10-year period. Forty-nine vessels were treated. Stenosis quantification was done using North American symptomatic carotid endarterectomy trial method. The mean follow-up period by clinical and Duplex examination ranged is 5.6 years. Results: The technical success rate was 100%. There were four deaths (8.1%) and two (4.1%) minor strokes within thirty days of procedure. There was no major strokes. All patients with minor stroke achieved complete recovery at 1-month follow up. Two deaths occurred probably due to hyperperfusion syndrome (HS) and two due to cardiac arrest. Conclusion: CAS is an effective treatment modality of symptomatic carotid artery disease but should be carefully done in high-risk groups having severe medical ailments and those having severe bilateral stenosis of the carotid arteries.

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Context: Neuropsychiatric systemic lupus erythematosus (NPSLE) has varied manifestations. Aims: To study the pattern of neuropsychiatric (NP) involvement in systemic lupus erythematosus (SLE). Settings and Design: Hospital based cross sectional and retrospective study. Patients and Methods: Patients admitted with a diagnosis of SLE, during a period of 16 months, were evaluated and any NP syndrome present classified as per the American College of Rheumatology (ACR) nomenclature. Statistical analysis: SPSS software Version 10 was used for descriptive analysis and correlative study. Results: Out of 50 patients with SLE, all the patients with NPSLE [39 (78%)] were females, mean age 25.66 years (range: 11-44). The commonest manifestation was headache [20 (55.6%)]. Seizures were seen in 8 (20.51%) and psychosis in 6 (16.2%). Fine distal tremor was seen in 8 (20.51%) of patients. Conclusions: Headache is a frequent NP syndrome. Fine distal tremor is a syndrome not included in ACR classification but seen is 20% of our patients with NPSLE.

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Selective neuronal loss following status epilepticus(SE) was first described just under 100 years ago. The acute pathology following SE was shown to be 'ischemic cell change' and was assumed to arise through hypoxia/ischemia. Recently, erythropoietin (Epo) has been shown to have potent anti-apoptosis activity in central nervous system neurons in animal models of ischaemic injury. Aims: In this report, in order to determine Epo preconditioning on hippocampus neuronal apoptosis, we examined caspase-3 expression following SE caused by Li-pilocarpine in rats. Settings and Design: Animals were classified into three groups: EP group (pilocarpine group), rhEpo-pilocarpine group and control group. Four hours after preconditioning with Epo intraperitoneally, pilocarpine hydrochloride was administered intraperitoneally and observed for behavioral manifestations of SE. The animals were sacrificed at one hour after SE onset. Materials and Methods: At the above-mentioned time point, animals were deeply anesthetized and were perfused through the left ventricle. Detection of hippocampus neuronal apoptosis was performed with caspase-3 immunohistochemical technique on three groups. To further confirm which cell population upregulates caspase-3, brain sections were stained for NeuN (green) and caspase-3 (red).Statistical analysis ANOVA and Fisher's post hoc test was used. Results Quatification of hippocampus neurons revealed that the number of caspase-3-positive cells in the CA1/CA3 area and dentate gyrus(DG)of three groups had a significant difference. In comparison with control group, there was an increase by 74% and 534%, 42% and 272% in the CA1/CA3 area and DG of EP group and rhEpo-treated group respectively. There was a decrease by 18% and 26% in the CA1/CA3 area and DG of rhEpo-treated group compared with those in EP group. In addition, colocalization of caspase-3 with NeuN was shown. Conclusions Systemic rhEpo therapy reduced caspase-3 expression in SE induced by Li-pilocarpine.

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Objective: To evaluates the physical growth and psychomotor development of infants born to women with epilepsy on regular Anti Epileptic Drugs (AEDs). Setting: Govt. Stanley Medical College and Hospital, Tertiary care referral centre, Chennai. Design: Open prospective cohort study with a control group. Materials and Methods: Consecutive women with epilepsy who were on regular anticonvulsants were followed up from their first trimester. Their babies were examined at birth and anthropometric measurements including anterior fontanelle size were noted. They were followed up till one year and periodically evaluated at 1st, 6th and 12th month of age. Development testing using Griffith scale was done at 2nd, 6th and 12th month. An equal number of control babies were also studied using the same scale for one year at the specified intervals. The results in both the groups were compared. Results : 30 babies were enrolled in the case and control group. The AEDs received by the mothers with epilepsy were Phenytoin, Carbamazepine, and Sodium valproate. At birth and 1st month the weight, head circumference and length of case and control babies were equal. At 6th and 12th month reduction in the above 3 parameters were noted in the case babies ( P <0.01). Area of anterior fontanelle (AF) was larger in the study group particularly in those exposed to phenytoin in utero( P <0.001). In the case babies reduction in the sitting, prone and erect progression of the locomotor scores was observed at 2nd month ( P <0.001). Prone progression alone improved by 12th month and other two remained less than the control ( P <0.001). No difference was observed in reaching behaviour and personal/social scores in both groups. Infants exposed to Phenytoin monotherapy had a negative impact on sitting progression. Conclusion : Among infants exposed to AEDs in utero physical growth was equal to that of control at birth but reduced at 6th and 12th month probably due to extraneous factors. The Locomotor scores showed reduction in all areas in 2nd, 6th and 12th month except prone progression which alone improved by 12th month. Phenytoin exposure in utero resulted in large AF and it had a negative impact on sitting progression in comparison with Carbamazepine and Sodium valproate.

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Intracerebral hemorrhage (ICH) is the most devastating form of stroke with a high morbidity and mortality. ICH constitutes about 20-30% of all strokes, with the prevalence being higher in Asian population. Treatment of ICH is predominantly conservative, which includes control of blood pressure, use of anti-cerebral edema measures such as mannitol and mechanical ventilation. The benefit of early surgery in ICH is debatable. Initial hematoma volume and subsequent growth in its size are important predictors of a poor outcome in ICH. This means that therapies aimed at preventing hematoma enlargement in the earliest possible window period could lead to a better outcome in ICH. Recombinant factor VIIa (rFVIIa) is one such agent, which has been shown to prevent hematoma expansion and improve outcome in acute ICH. The purpose of the current review is to focus on the evidence regarding the usefulness of rFVIIa in acute ICH.

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Aim: To explore the methods for achieving pain relief in patients with atypical trigeminal neuralgia (TN) using microvascular decompression (MVD). Study Design and Settings: Retrospective study of 26 patients treated during the years 2000 to 2004. Materials and Methods: Twenty-six patients in whom vascular compression of the trigeminal nerve was identified by high definition magnetic resonance tomographic angiography (MRTA) were treated with MVD for atypical TN in our department. Clinical presentations, surgical findings and clinical outcomes were analyzed retrospectively. Results: In this study, single trigeminal division was involved in only 2 patients (8%) and two or three divisions in the other 24 patients (92%). Of prime importance is the fact that in 46.2% of the patients, several conflicting vessels were found in association. Location of the conflicts around the circumference of the trigeminal root was supero-medial to the root in 53.5%, supero-lateral in 30.8% and inferior in 15.7%. MVD for atypical TN resulted in complete pain relief in 50% of the patients with complete decompression, partial pain relief in 30.8% and poor pain relief or pain recurrence in 19.2% of the patients without complete decompression postoperatively. Conclusions: Complete decompression of the entire trigeminal root plays an important role in achieving pain relief in patients with atypical TN with MVD.

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Background: We report the clinical presentation, radiographic studies, intraoperative findings, histopathological analysis, and post-treatment outcome in 26 patients diagnosed with spinal synovial cysts (SSCs). Aims: To describe the clinical presentation, radiographic studies, operative findings, and postoperative follow-up in 26 patients with SSCs. Settings and Design: The study was retrospective in design, involving chart review. Individual patient data was tabulated and patterns were recognized. Materials and Methods: The charts for 26 patients who underwent surgical extirpation of SSC between April 1993 and October 2002 were retrospectively reviewed. Specifically, initial clinical presentation, pertinent radiographs (X-rays, magnetic resonance imaging, computed tomography), intraoperative findings, histopathology, and postoperative follow-up were noted. Statistical Analysis Used: Patient data was tabulated and analyzed for patterns in demographics, symptoms and histopathology. Results: SSCs were more common in females than males (17:9 ratio). Presenting symptoms were back pain with radiculopathy in 13 (50%), radicular pain in the absence of back pain in 10 (38%), and back pain without radicular pain in three (11%). In addition, 17 patients (65%) had sensory deficit, and 9 (35%) had motor deficit. Most SSCs occurred at the lumbar (19/26) or lumbosacral (5/26) regions, with only 2 (2/26) in the thoracic region. One patient had bilateral SSC at the L4-5 level. Intraoperatively, each cyst was located adjacent to a degenerated facet joint. These lesions could grossly be identified intraoperatively and histopathological confirmation was achieved in all the cases. Conclusions: SSCs are important lesions to consider in the differential diagnosis of lumbar epidural masses and surgical resection leads to significant improvement in the majority of cases.

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Craniometaphyseal dysplasia (CMD) is a rare congenital bone dysplasia with abnormal bony overgrowth leading to characteristic facial features and cranial nerve compression. We present a 10-year-old child with bony swelling at the nasal root since birth along with decreased hearing in both ears. She had normal developmental milestones and intelligence. On examination, she had bossing of forehead with very broad nasal root, short septum, hypertelorism and epicanthic folds. CT scan with 3D reconstruction revealed grossly thickened calvarium and hyperostosis and sclerosis of the cranial base. As the major concern of the parents was cosmetic, craniofacial reconstruction was performed with good cosmetic outcome

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Five Indian cases of Aicardi syndrome, aged 1-13 years and all with the classic triad of infantile spasm, corpus callosal agenesis and chorioretinal lacunae, were presented. The purpose of this report was to demonstrate the clinical, electroencephalographic (EEG) and radiological spectrum and the treatment outcome of this disorder among the Indians. None of the patient had any family history of similar illness. All had profound psychomotor impairment with no meaningful speech development. There were microphthalmia in 2, optic disc coloboma in 1, interhemispheric cyst in 1, periventricular heterotopia in 2 and thoracolumbar kyphoscoliosis in 2 cases. They all had early onset epilepsy and there were multiple types of seizures. Two cases (40%) had the characteristic dissociated burst-suppression pattern in EEG. Two cases whose antiepileptic medications included vigabatrin had complete control of seizure.

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An abdominal pseudocyst is a rare, but important complication in patients with a ventriculo-peritoneal (VP) shunt insertion. Several predisposing factors for this complication have been suggested, including infection, obstruction or dislodgement, but the pathophysiology is still unknown. However, the abdominal inflammatory process is accepted widely as a hypothesis for the formation of an abdominal pseudocyst. In this study, we report the case of a 21-year-old male that presented with a high-grade fever, poor appetite, shortness of breath and unconsciousness 1 week after receiving a VP shunt insertion for obstructive hydrocephalus. Ultrasonography and computed tomographic scans of the abdomen revealed a well-defined large hepatic cyst surrounding the peritoneal tube of the VP shunt. A hepatic cerebrospinal fluid (CSF) cyst was diagnosed and Staphylococcus epidermis was cultured via CSF. After externalization of the VP shunt and adequate antibiotic treatment, the hepatic cyst was resolved. There was no recurrence observed in the regular follow up.

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Summary: Head injuries account for significant proportion of neurosurgical admissions and bed occupancy. Patients with head injuries also consume significant proportions of neurosurgical resources. A prospective 6-month study has been carried out to evaluate the expenditure incurred on head injury patients in a modern neurosurgical center equipped with state of the art infrastructure. Costing areas included wages / salaries of health care personnel, cost of medicines / surgical items / crystalloids, general store items, stationary, all investigation charges, equipment cost, overhead building cost, maintenance cost, electricity and water charges and cost of medical gases, air conditioning and operation theatre expenses. Expenditure in each area was calculated and apportioned to each bed. The statistical analysis was done using X2 test. The cost of stay in ward was found to be Rs. 1062 / bed / day and in neurosurgical ICU Rs. 3082 / bed / day. The operation theatre cost for each surgery was Rs. 11948. The cost of hospital stay per day for minor, moderate and severe head injury group was found to be Rs. 1921, Rs. 2569 and Rs. 2713 respectively. The patients who developed complications, the cost of stay per day in the hospital were Rs. 2867. In the operative group, the cost of hospital stay per day was Rs. 3804. The total expenditure in minor head injury was Rs. 7800 per patient, in moderate head injury was Rs. 22172 per patient, whereas in severe head injury, it was found to be Rs. 32852 per patient. Patients who underwent surgery, the total cost incurred was Rs. 33100 per operated patient.

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Background: The effect of initiation of levodopa therapy on the memory functions in patients with Parkinson's disease remains poorly understood. Aims: To evaluate the effect of initiation of levodopa therapy on memory, in patients with early Parkinson's disease. Settings and Design: Prospective case control study. Materials and Methods: Seventeen patients with early Parkinson's disease were evaluated for verbal memory using Rey's auditory verbal learning test, and visual memory using the Benton's visual retention test and Form sequence learning test. UPDRS scores, Hoehn and Yahr's Staging and Schwab and England scores of Activities of daily living. Hamilton's depression rating scale and MMSE were also evaluated. Six controls were also evaluated according to similar study protocol. Levodopa was then prescribed to the cases. Same tests were repeated on all the subjects after 12 weeks. Results: The mean age of the patients was 59.8 ( + 12.9 yrs); mean disease duration of 3.26 ( + 2.06 yrs). The mean UPDRS scores of patients were 36.52 ( + 15.84). Controls were of a similar age and sex distribution. A statistically significant improvement in the scores on the UPDRS, Hamilton's depression scale, Schwab and England scale, and a statistically significant deterioration in the scores of visual memory was observed in patients with PD after starting levodopa, as compared to their baseline scores. There was no correlation between degree of deterioration and the dose of levodopa. Conclusion: Initiation of levodopa therapy in patients with early and stable Parkinson's disease is associated with deterioration in visual memory functions, with relative preservation of the verbal memory.

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Subclavian steal syndrom (SSS) is a clinical entity characterized by brachial and basilar insufficiency as a result of critical proximal subclavian artery stenosis or occlusion. We report a patient of giant hypervascular thyroid nodule presenting with features of SSS. The left hand ischemia and symptoms of vertebro-basilar artery in our patient were probably related to stealing of blood by the hypervascular thyroid nodule from the subclavian artery. The patient was relieved of the symptoms upon percutaneous subclavian stent placement

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Context: A large number of patients are admitted to hospitals in large cities without any identification. These "unknown" patients represent a unique problem in developing countries. There is no systematic study in world literature on this subgroup of patients. Aims: To elucidate the natural history and outcome of traumatic brain injury patients admitted in the department of neurosurgery as "unknowns". Settings and Design: Retrospective analysis of all traumatic brain injury patients admitted to the department of neurosurgery as "unknown", between January 2002 and March 2005. Results: Three hundred and twenty five patients were admitted as unknowns over a 3 year and 3 months period. Most of the patients were young males and became known during their hospital stay. Overall, 33 patients stayed for longer than one month, with 4 of them staying for longer than 6 months. Mortality in mild, moderate and severe head injury group was 1%, 6% and 46% respectively. Conclusions: Unknown patients represent a unique subgroup in metropolitan cities like Delhi. Managing them raises several medico-legal issues. Many of them occupy hospital beds for longer duration than required. There is an acute shortage of rehabilitation homes in Delhi for long term care and rehabilitation of such patients.

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Background: Patients who undergo carpal tunnel surgery sometimes complain of the restriction of the grip and pinch function, palmar tenderness, cosmetic problems, and scar formation at the site of the incision. Aims: We used a modified mini uni-skin incision with appropriate hand position for microscopic view in the surgical treatment of carpal tunnel syndrome to prevent cosmetic problems related with scar formation after surgery. Settings and Design: In this study we used two different skin incision techniques; mini uni-skin incision and standard incision. In mini uni-skin incision technique the hands were positioned in a way that the wrist are hyperextended. A small skin incision one cm long was done from the inferior flexion crease towards the point between the thirth and fourth fingers. Materials and Methods: Standard incision and mini uni-skin incision were compared according to their cosmetic result, grip and pinch function, palmar tenderness, and painful scar formation. Statistical analysis: Student -t test was used for this study. Results: 56 (43%) patients were operated with mini uni-skin incision, and 73 (57%) cases were operated with standard incision. The scores of grip, pinch and cosmetic results were better in the patients who were operated with mini uni-skin incision technique from those of standard incision. Conclusion: In this clinical study we used a modified skin incision (mini uni-skin incision) technique in the surgical treatment of carpal tunnel surgery. Our results revealed that mini uni-skin incision is superior from the standard incision.

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We described a 69-year-old woman with neurological manifestations due to mixed connective tissue disease (MCTD). The patient demonstrated subacute cognitive decline, seizure and gait disturbance with no connective tissue manifestation. She had been diagnosed with dementia at another hospital, later in our hospital, serological examinations disclosed high titers of anti-RNP antibody. Cognitive dysfunction in this patient was dramatically ameliorated by steroid therapy. Three months later, she developed edema of the hands, synovitis and acrosclerosis. The patient was finally diagnosed as having MCTD. We emphasized MCTD as a rare cause of "treatable dementia".

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A rare case of hemangioblastoma located in the region of hippocampus is reported. A 27-year-old female presented with a single episode of generalized convulsion. The vascular and cherry red color hemangioblastoma was resected by a temporo-zygomatic approach. There has been no recurrence of tumor at a follow-up of 11 years

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Background: Virtually all the literature on head injury has focused on the outcome prediction of severe and mild head injuries and very few studies have been dedicated to patients sustaining moderate head injuries. Aim: To identify the patient following moderate head injury who may die, develop severe disability or significant cognitive and behavioral problems on the first day of injury itself. Setting: Tertiary teaching hospital. Design: Prospective study divided into two groups. Materials and Methods: The study included 85 patients whose Glasgow coma scale score were 9-12 and who had isolated moderate head injury. Among the above patients a preliminary prospective study was conducted in first group of 64 patients using 7 clinical factors, 18 neuro-behavioral sequel and CT brain data in prediction of outcome with moderate head injury. From the results obtained in the above study three statistically significant factors were identified and a mathematical model was developed and used prospectively in the next 21 patients and its accuracy was evaluated. Statistical Methods Used: Multiple regression analysis and Kendall's tau non- parametric test using statistical package for social sciences (SPSS 11-5-version) were used to find out the predictive factors. Results: Results of these patients showed combination of CT scan brain data, verbal response and neurological signs could provide a reliable prediction in moderate head injury. Conclusion: Based upon the above results a mathematical model was developed giving a value for the above-mentioned factors. The mathematical model was "CT brain data x (Verbal response + Neurological Signs)". Its overall accuracy when used on the day of admission was around 80%.

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Carotid dissection is an uncommon complication of trauma. They can present with immediate or delayed complications. We describe the case of a young patient with bilateral carotid dissections and acute infarcts. Brief review of literature and treatment options are discussed.

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