Background: Surgical management of intractable epilepsies is currently an established mode of therapy in various clinical settings. Aims: To retrospectively evaluate the neuropathological findings in both temporal and extratemporal lobe resections in such patients. Materials and Methods: The study included resected specimens from patients with intractable epilepsy managed at a tertiary care hospital of India, during a 10-year period (1995-2004). Results: A total of 153 patients, with mean age of 19.4 years and male predominance (73.2%) were included in the study. Overall, there was a predilection for the temporal lobe (73.2%), while 41cases were extratemporal in location. On histopathology, mesial temporal sclerosis (MTS) (24.8%) was the commonest lesion, followed by tumors (19.6%) and isolated focal cortical dysplasia (FCD - 15.11%). Other less common findings included Rasmussen encephalitis, non-specific gliosis and vascular malformations. In addition, 20.9% (32 cases) had dual lesions, majority of which included FCD with ganglioglioma (15 cases) or with dysembryoplastic neuroepithelial tumor (12 cases). In the temporal lobe, neoplasms and dual lesions formed the majority (apart from MTS), unlike dual lesions followed by neoplasms and FCD, in the extratemporal location. Conclusion: This series demonstrates that most patients with chronic intractable epilepsy have significant histopathological findings and highlights the neuropathological spectrum of such lesions, in the Indian context. This was similar to that reported from the West, but different from the single Indian series available in the literature. Further, the overall profile of temporal lobe lesions was not different from the extratemporal ones.

Background: The knowledge of warning symptoms and risk factors for stroke has not been studied among patients with stroke in developing countries. Aims: We aimed to assess the knowledge of stroke among patients with stroke and their relatives. Settings and Design: Prospective tertiary referral hospital-based study in Northwest India. Materials and Methods: Trained nurses and medical interns interviewed patients with stroke and transient ischemic attack and their relatives about their knowledge of stroke symptoms and risk factors. Statistical Analysis: Univariable and mulivariable logistic regression were used. Results: Of the 147 subjects interviewed, 102 (69%) were patients and 45 (31%) were relatives. There were 99 (67%) men and 48 (33%) women and the mean age was 59.7±14.1 years. Sixty-two percent of repondents recognized paralysis of one side as a warning symptom and 54% recognized hypertension as a risk factor for stroke. In the multivariable logistic regression analysis, higher education was associated with the knowledge of correct organ involvement in stroke (OR 2.6, CI 1.1- 6.1, P =0.02), whereas younger age (OR 2.7, CI 1.1-7.0, P =0.04) and higher education (OR 4.1, CI 1.5-10.9, P =0.005) correlated with a better knowledge regarding warning symptoms of stroke. Conclusions: In this study cohort, in general, there is lack of awareness of major warning symptoms, risk factors, organ involvement and self-recognition of stroke. However younger age and education status were associated with better knowledge. There is an urgent need for awareness programs about stroke in this study cohort.

Background: It is now agreed that the prognosis of seizure disorder due to solitary cysticercus granuloma (SCG) is generally good. However, the choice antiepileptic drugs (AEDs) remain empirical, with no comparative trials of different AEDs being available. Aims: To determine the safety and efficacy (measured by the incidence of 'treatment failure') of clobazam in comparison to standard treatment with phenytoin-sodium for prevention of seizures in persons with solitary cysticercus granulomas (SCGs). Settings and Design: This pilot study was conducted in a neurology department of a medical college hospital in the form of a prospective, randomized, open-labeled trial. Materials and Methods: Forty-eight patients with seizures due to SCG were randomized in an open-labeled trial to either, clobazam (1 mg/kg oral loading followed by 0.5 mg/kg/d) (n=21) or phenytoin (15 mg/kg, oral loading in 3 divided doses over 24 h, followed by 5 mg/kg/d) (n=27). They were followed over 6 months with the primary outcome measure being treatment failure (either discontinuation or modification of AEDs) due to either adverse effects or breakthrough seizures. Results: Treatment failures were noted to be significantly less common ( P =0.03) in the clobazam-treated group (n=1; 4.7%) than in phenytoin-treated group (n=9; 33.3%). These included one patient (4.7%) in the clobazam-group who had breakthrough seizures and 3 (11.1%) who had breakthrough seizures and 6 (22.2%) in the phenytoin-treated group who had adverse effects requiring treatment discontinuation. Conclusions: Clobazam was well tolerated, safe and more effective than phenytoin in the AED treatment of patients with SCG.

Background: Endoscopic third Ventriculostomy (ETV) is one of the surgical options for obstructive hydrocephalus. There are varying opinions about results of ETV in infants. We are therefore presenting the results of ETV in 54 infants. Materials and Methods: A prospective study of 54 infants undergoing ETV in our institution in the last 2 years was carried out. There were 48 cases of congenital hydrocephalus with aqueductal stenosis, 6 of post tubercular meningitis hydrocephalus. Average follow up was 18 months. Results: There was 83.3% (45 cases) clinical success rate in our study. Infection, persistent cerebro-spinal fluid (CSF) leak and bleeding occurred in 4 (8%) cases each while blockage of stoma was observed in 8 (14.8%) patients. Majority of ETV stoma closure (6 out of total 8) occurred following infection (4) or bleeding during surgery (2). One patient (2%) had transient diabetes insipidus. Overall failure rate in our study was 16.7% (8 stoma blocks and 1 procedure abandoned). Low birth weight pre mature infants had higher failure rate (3 out of 5 infants 60%) compared to full term infants with normal birth weight (12.3%). Age did not have any impact on the success rate ( P >0.05). Success rates were not significanlty different in patients with aqueductal stenosis (85.4%) and TBM (66.6%) (Fisher's exact test, P =0.3). Conclusion: ETV was fairly safe and effective in full term normal birth weight infants while the results in low birth weight pre mature infants were poor.

Background: Low backache (LBA) is now increasing in younger population due to misdirected spinal kinetics secondary to improper posture, heavy load lifting and motorbike driving. Hence minimally invasive procedures are increasingly sought after. Among these, PLDD is currently popular and in use. We present our long term follow-up in the use of Nd:YAG laser for PLDD. Aim: To evaluate the efficacy of PLDD in treatment of contained herniation of lumbar discs & long term follow up results. Materials and Methods: Forty patients with contained lumbar disc herniation on MRI and who did not respond to 6 weeks conservative treatment were subjected to PLDD. L4-5 disc was treated in 31, L5-S1 in 12 and L1-2 and L3-4 in one each. Nd:YAG laser at 1064 nm was used for the procedure. Total laser energy of 1500-2000 Joules was delivered at the disc space depending upon the size. Results: There was immediate pain relief in 32/40 (80%). According to MacNab criteria good to fair response was seen in 37/40 (92%) and 3 patients (7.5%) responded poorly to this treatment. On follow up which ranged from 1 to 7 years, 34/40 (85%) had pain relief with no need for further treatment. Complications: Significant pain at local puncture site was experienced by 8 (20%), pain during lasing was experienced by one. One patient developed muscular spasm. Conclusion: Percutaneous laser disc decompression is a safe, relatively noninvasive and effective treatment modality for contained, nonsequestered, herniated lumbar disc disease in carefully selected patients.

Background: The chronic nature of idiopathic intracranial hypertension (IIH) represents a risk factor for progressive optic nerve damage and structural abnormalities of the retina. Aim: We measured the retinal nerve fiber layer (RNFL) thickness in patients followed with the diagnosis of IIH who had no or mild visual impairment to search for possible structural alterations in the retina for diagnostic and prognostic purposes. Settings and Design: Case-control prospective study. Materials and Methods: The study group consisted of 12 women followed and treated with the diagnosis of IIH in our clinic. The selection criteria were the, normal optic nerve, normal visual fields or mild visual field defects (Grade 1-3) by Humphrey perimeter. Randomly assigned, age-matched 12 healthy women were taken as the control group. Retinal nerve fiber layer thickness was evaluated with scanning laser polarimetry and both eyes were studied for each case in both groups. Statistical Analysis Used: Mann-Whitney U test. Results: The mean ages of the patient and the control groups were 34.58±4.2 and 34.42±5.7 years respectively ( P =0.87). The mean duration of disease was 5.5±3 years. Some parameters related to RNFL thickness were found to differ significantly between patients with IIH and control subjects. Namely superior ratio ( P =0.007), inferior ratio ( P =0.039), superior-nasal ratio ( P =0.025), maximum modulation ( P =0.01) and symmetry ( P =0.006) were lower in the patient group than controls. Conclusion: Scanning laser polarimetry might be a good adjunct for determining possible structural affects of IIH on the retina in patients with no or mild visual impairment.

Background: Both basic and clinical research has demonstrated that antiepileptic drugs can be effective in alleviating neuropathic pain. It was hypothesized that oxcarbazepine might be effective in reducing the symptoms of painful diabetic neuropathy. Aims: To investigate the long-term efficacy and safety of oxcarbazepine in symptoms of painful diabetic neuropathy. Materials and Methods: This study included thirty-eight painful diabetic neuropathy patients, which were screened with clinical assessment and electrophysiological studies. The efficacy and safety of oxcarbazepine were evaluated according to the changes in pain intensity and social interference subitems scores of Short-form Brief Pain Inventory besides electrophysiological studies at the end of six months of the treatment. Statistical Analysis: The Students t, Mann-Whitney U and Rank Sum test and Chi-square tests were applied to examine variables differences. The level of statistical significance was chosen to be P <0.05. Results: A significant difference was found in all of subitems of pain intensity and social interference at the end of the study according to the baseline scores. Improvement was observed in 52.7%, 63.1%, 55.3% and 63.2% of patients for worst, least, average and pain right now at the end of six months, respectively. Improvement was observed as 60.6%, 63.2%, 52.6%, 60.5%, 68.4% and 63.2% for general activity, mood, walk, work, people relations, sleep and life enjoyment subitems, respectively. None of these patients had any prominent side effect leading to discontinue the treatment. Conclusion: Long-term oxcarbazepine treatment was found to be effective and safe in the symptoms of painful diabetic neuropathy.

Background: Although folic acid deficiency is known to be one of the factors in the development of spina bifida and other neural tube defects (NTD) the exact pathophysiology still remains unclear. Progesterone is an endogenous hormone which increases significantly during pregnancy. Aims: We aimed to study the possible negative effects of high dose progesterone on neural tube development in early chick embryos. In order to test our hypothesis, early chick embryos were exposed to physiological saline, normal and high doses of progesterone. Settings and Design: 160 fertile, specific pathogen free white leghorn eggs (Gallus gallus), all at stage eight of development were divided into four equal groups. Materials and Methods: The first group was incubated without any operation. The second group was injected with physiological saline. The third and fourth groups were injected with two and twenty times more than physiologic doses of progesterone respectively. After 48 hours of incubation, all embryos were analyzed for the presence of NTDs under light microscopy. Statistical Analysis Used: None. Results: At 48 hours of incubation, 84% (135/160) of the embryos passed characteristics of Stage 12 development and were included to the study. None of the eggs in the first three groups showed NTDs, whereas 81.8% (27/33) of the eggs in the fourth group showed NTDs. Conclusions: Our study showed that progesterone at levels twenty times more than its physiologic level might cause NTDs. Further studies are needed to explain the mechanisms of this teratogenic effect.

Background: Chronic progressive external ophthalmoplagia (CPEO) is a phenotypic mitochondrial disorder that affects external ocular and skeletal muscles and is associated with a single or multiple mitochondrial DNA (mtDNA) deletions and also nuclear gene mutations. There are also some reports about the relationship between CPEO and the nuclear Twinkle gene which encodes a kind of mitochondrial protein called Twinkle. Aims: To study the mtDNA deletions and Twinkle gene G1423C point mutation in Iranian patients with CPEO. Materials and Methods: We collected 23 muscle samples from patients with CPEO, 9 women (mean age 34.3 years) and 14 men (36.7 years). Multiplex polymerase chain reaction (PCR) method was used to find the presence of single or multiple deletions in mtDNA. Single stranded conformational polymorphism (SSCP) and restriction fragment length polymorphism (PCR-RFLP) methods were carried out to investigate point mutation (G1423C) in the Twinkle gene in all DNA samples. Results: Different sizes of mtDNA deletions were detected in 16 patients (69.6%). Each of the 5.5, 7, 7.5 and 9 kb deletions existed only in 1 patient. Common deletion (4977bp) and 8 kb deletion were detected in 5 and 3 patients respectively. Multiple deletions were also present in 4 patients. Out of 23 patients included in our study, two cases (8.7%) had Twinkle gene mutation (G1423C) and 5 patients (21.7%) did not show any deletions in mtDNA or the Twinkle gene mutation. Conclusion: Our study provides evidence that the investigation of mtDNA and Twinkle gene mutations in CPEO may help with early diagnosis and prevention of the disease. Patients who did not show deletions in the mtDNA or G1423C mutation in the Twinkle gene may have other mtDNA, Twinkle or nuclear gene mutations.

Background: Juvenile myoclonic epilepsy is a heterogeneous syndrome, both in genetic and clinical aspects. Aims: This study was conducted to compare the efficacy of valproic acid in familial versus sporadic cases of this syndrome. Settings and Design: Seventy patients with JME were identified; 24 patients (34.3%) had positive history of JME in their first degree relatives (group I) and 46 patients (65.7%) were sporadic (group II). Materials and Methods: Valproic acid was started for the patients with upward titration. The cases were followed for one year after final titration of the drug with regular blood monitoring. Patients, who had no myoclonic, absence and grand mal seizures within one year, were considered excellent responders. Statistical Analysis: We used Student T-test and Fisher's exact test for quantitative and qualitative variables respectively. Logistic Regression test was used to evaluate the predictive factors for final treatment outcomes. Results: Mean dosage of valproic acid was 800 mg/d in both groups (13 mg/kg and 12.4 mg/kg respectively). Mean therapeutic levels of the drug in group I and II were 74 µg/ml and 78.4 µg/ml respectively. Excellent responders' rate was 66.7% in group I and 76.1% in group II. History of absences and older age at the onset of grand mal seizures decreased excellent responders' rate in both groups. Conclusions: Considering response to valproic acid, there is no significant difference in familial versus sporadic cases of JME, whereas history of absences and older age at the onset of grandmal seizures, decrease the probability of being excellent responders in this syndrome.

Background: Lumbar disc prolapse is a common problem and the current surgical standard for its treatment is a microsurgical discectomy. Microendoscopic discectomy (MED) is a minimally invasive spinal procedure being done successfully for prolapsed intervertebral disc disease. Aims: We report the technique, outcome and complications seen in 107 cases of prolapsed lumbar intervertebral disc who underwent MED. Setting and Design: The study was carried out at the Department of Neurosurgery, at a tertiary hospital in South India and the data was collected prospectively. Materials and Methods: 107 patients with prolapsed lumbar intervertebral disc who were seen at our institution between November 2002 and January 2006 were included in the study. Data was collected prospectively. The METRx system (Medtronic Sofamor Danek, Memphis,TN) was used to perform MED. Outcome assessment was done by the modified Macnab criteria. Results: 107 patients (67 males, 40 females) underwent MED for prolapsed lumbar intervertebral disc. Follow up ranged from 2 to 40 months with a mean follow up 12.9 months. Seventy six patients had an excellent outcome, 22 patients had a good outcome, 5 patients had a fair outcome and 3 patients had a poor outcome. One patient with a long dural tear required conversion to a standard microdiscectomy and was excluded from outcome assessment. Complications included dural puncture with K-wire (1), dural tear (2), superficial wound infection (1), discitis (1) and recurrent disc prolapse (2). Conclusions: Microendoscopic Discectomy (MED) is a safe and effective procedure for the treatment of prolapsed lumbar intervertebral disc.

X-linked adrenoleukodystrophy (XALD) is an inherited disorder of peroxisomal metabolism. Atypical presentations have been occasionally reported in literature. However, extrapyramidal and cerebellar manifestations are distinctly rare. We report a patient of X-linked adrenoleukodystrophy with cranial and cervical dystonia and neurological presentation resembling spinocerebellar degeneration followed by a brief review of relevant literature.

Emery-Dreifuss muscular dystrophy (EDMD) is a rare and genetically heterogeneous disorder. We report two patients with emerin deficient X-linked EDMD and two probable patients with EDMD with typical early contractures, progressive muscle weakness and cardiac involvement. Family history was noted in one case. Muscle biopsy revealed features of dystrophy in all.

Headaches are commonly associated with various psychiatric disorders. The comorbidity of migraine and psychiatric disorders has been well documented. Here we present a case of schizophrenia with comorbid headache treated with clozapine. The patient's headache fulfilled the diagnostic criteria for cluster headache (CH). To our knowledge this is the first report of CH responding to clozapine therapy. The relationship of headache and psychiatric disorders is a matter of debate and there has been very little research on the aspect of causality or direction of causation. The response of both the conditions to a serotonin-dopamine antagonist such as clozapine might be important in giving newer insights into the pathogenesis of these disorders. It also has the clinical implication of being useful in patients with dual diagnosis.

The histological features of arterialized medullary vein (MV) in spinal dural arteriovenous fistulas (SDAVF) were studied in five consecutive patients who presented with progressive congestive myelopathy. Retrograde venous filling on preoperative angiography was recognized as being severe in 3 cases and moderate in 2 cases. Direct intradural interruption of the arterialized MV was performed in all patients. The arterialized MV was sampled and examined histologically to determine the percentage of the hyperplasia of venous wall (hypertrophic ratio). Histological examination of arterialized MV showed that hypertrophic alteration of venous wall structure was due to hyperplasia of elastic fibers, ranging from 41 to 82%. Patients with angiographically severe venous hypertension tended to have a higher hypertrophic ratio than patients with moderate venous hypertension. Our observations support the clinical concept that long-standing arterial stress in the spinal venous circulation causes histological alterations of spinal vascular structure associated with the progression of venous hypertension. We suggested that possibly the histological parameter can be used for predicting neurological recovery after occlusion of the fistulas.

We report a 19-year-old man presenting to the department of Psychiatry for the evaluation of prominent behavioral symptoms associated with episodic headaches, with normal inter-episodic periods. A diagnosis of classic migraine with hypomanic aura was made. Other possible co-morbid or causative illnesses were excluded and preventive therapy with valproate was started due to the prominent affective symptoms as a part of the migranous aura. With this the frequency of headaches gradually decreased over the next four months. He was followed up for 2 years when he was found to be symptom-free. Recent research into the mechanisms of migraine has identified that the cortical hyperexcitability and an imbalance between neuronal inhibition and excitement mediated by gamma-aminobutyric acid and excitatory amino acids respectively may be the underlying mechanism. The high rate of affective disorders in patients with migraine, association of migraine with an aura comprising of mood symptoms and good response to treatment with mood-stabilisers might give newer insights into the pathophysiology of mood disorder as well.

Distal symmetrical polyneuropathy and neuromuscular weakness is common neurological problem in recovery phase of acute organophosphate (OP) poisoning. Various types of extra pyramidal syndromes are uncommon sequel after OP poisoning. These are reported to be reversible within few weeks and characteristically associated with normal magnetic resonance imaging (MRI). In this report we are presenting a case with extra pyramidal syndrome after acute OP poisoning with few interesting MRI changes in striatum.

We report a patient with an uncommon presentation in the form of massive bilateral calvarial hyperostosis with bi-parasagittal en plaque meningioma. The tumour was removed by bilateral fronto-parieto-occipital craniotomies. The patient was subjected to post operative radiotherapy to reduce the chances of recurrance. The management of such a case is a surgical challenge.