Dengue viral infections are common worldwide. Clinical manifestations form a broad spectrum, and include uncomplicated dengue fever, dengue hemorrhagic fever, and dengue shock syndrome. Encephalopathy has been well reported and has classically been thought to result from the multisystem derangement that occurs in severe dengue infection; with liver failure, shock, and coagulopathy causing cerebral insult. However, there is increasing evidence for dengue viral neurotropism, suggesting that, in a proportion of cases, there may be an element of direct viral encephalitis. Understanding the pathophysiology of dengue encephalopathy is crucial toward developing a more effective management strategy. This review provides an overview of the clinical spectrum of dengue infection, and examines evidence supporting the existence of dengue encephalitis.

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Progressive myoclonic epilepsy (PME) is a disease complex and is characterized by the development of relentlessly progressive myoclonus, cognitive impairment, ataxia, and other neurologic deficits. It encompasses different diagnostic entities and the common causes include Lafora body disease, neuronal ceroid lipofuscinoses, Unverricht-Lundborg disease, myoclonic epilepsy with ragged-red fiber (MERRF) syndrome, sialidoses, dentato-rubro-pallidal atrophy, storage diseases, and some of the inborn errors of metabolism, among others. Recent advances in this area have clarified molecular genetic basis, biological basis, and natural history, and also provided a rational approach to the diagnosis. Most of the large studies related to PME are from south India from a single center, National Institute of Mental Health and Neurological Sciences (NIMHANS), Bangalore. However, there are a few case reports and small series about Lafora body disease, neuronal ceroid lipofuscinoses and MERRF from India. We review the clinical and research experience of a cohort of PME patients evaluated at NIMHANS over the last two decades, especially the phenotypic, electrophysiologic, pathologic, and genetic aspects.

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Susceptibility-weighted imaging (SWI) is a rapidly evolving technique that utilizes both the magnitude and phase information to obtain valuable information about susceptibility changes between tissues. SWI is very sensitive to the paramagnetic effects of deoxyhemoglobin. SWI plays an important role in the diagnostic evaluation and management of acute stroke. In addition, it also plays an important role in the imaging of patients with chronic arterial occlusion and in understanding the effects of chronic infarction, like incomplete infarction and cortical laminar necrosis. The hemodynamic status and oxygen extraction fraction can also be evaluated. SWI is useful in evaluating cerebral venous sinus thrombosis by demonstrating the hemorrhagic venous infarction and thrombus in the sinus and the cortical veins, as well as secondary phenomena like venous stasis in the form of engorged cortical and transmedullary veins and collateral slow flow. Low-flow vascular malformations that are not visualized well on conventional sequences are depicted in exquisite detail along with the venous components on SWI. SWI is used for evaluating cavernomas, developmental venous anomalies, telangiactasias, dural arteriovenous fistulas and the various components of arteriovenous malformations. It has also evolved as a noninvasive technique for evaluating various anomalies of the venous system without administering contrast. Vasculopathies and vasculitis are associated with cerebral microbleeds which are detected on SWI. On the basis of the additional information provided by SWI, it can be included in the routine brain imaging protocol.

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Dengue infection is endemic in more than 100 countries, mostly in the developing world. Recent observations indicate that the clinical profile of dengue is changing, and that neurological manifestations are being reported more frequently. The exact incidence of various neurological complications is uncertain. The pathogenesis of neurological manifestations is multiple and includes: neurotrophic effect of the dengue virus, related to the systemic effects of dengue infection, and immune mediated. In countries endemic to dengue, it will be prudent to investigate for dengue infection in patients with fever and acute neurological manifestations. There is need for understanding of the pathogenesis of various neurological manifestations.

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Background : According to American Academy of Neurology (AAN) criteria, demonstration of demyelination in the sural nerve by teased fiber or ultrastructure is considered mandatory for diagnosis of chronic inflammatory demyelinating polyneuropathies (CIDP). In resource-restricted settings where these techniques are not freely available, it is useful to determine the utility of 'supportive' pathologic criteria (subperineurial edema, inflammation, onion bulb formation, and demyelination) proposed by AAN for diagnosis of CIDP. Settings and Design : Tertiary care hospital, retrospective study. Patients and Methods : Forty-six patients with idiopathic CIDP (32 with progressive course and 14 with relapsing-remitting course) satisfying AAN clinical and electrophysiologic criteria evaluated between January 1991 and August 2004 were reviewed. Frequency of specific pathological alterations such as demyelination, inflammation, onion bulb formation, and axonal changes in sural nerve biopsies was evaluated. Statistical Analysis : SPSS statistical package was used to calculate mean, range, and standard deviation. Student's t test, chi-square test, and ANOVA were used for determining statistical significance. Results and Conclusion : Reduction in myelinated fiber density was most frequent (93.5%), followed by demyelination (82.8%), inflammation (58.7%), and onion bulb formation (28.3%). Endoneurial inflammation was frequent in the relapsing-remitting form and epineurial inflammation and axonal changes in those with progressive course. Greater disability at presentation, poor response to immunomodulation, and lower CSF protein levels was seen in those with axonal pathology. Pathological abnormalities were demonstrable in all (100%), whereas electrophysiological abnormalities were detected in 90.8%, suggesting that supportive histologic AAN criteria are helpful in diagnosis of CIDP.

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Insertion of a ventriculoperitoneal (VP) shunt is one of the most common surgical procedures in any neurosurgery unit worldwide. Distal catheter obstruction outside the peritoneum is a rare cause of shunt failure. We report the first case of distal obstruction in a 70-year old female by carcinoma breast engulfing the catheter and causing kinking. Intraoperatively, the catheter was intratumoral with no flow of cerebrospinal fluid distally. She underwent relocation of a new catheter to the opposite side of the abdomen and modified mastectomy with resolution of the hydrocephalus. The postoperative course has been uneventful.

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Background : Limb girdle muscular dystrophy (LGMD) type 2A is caused by mutation in the gene encoding for calpain-3 resulting in total or partial loss of protein. Diagnosis of LGMD2A, the most prevalent form of LGMD, is established by analyzing calpain-3 protein deficiency or CAPN3 gene mutation. Since there is no data from India regarding the incidence of LGMD2A, this study was undertaken. Aims : To study the frequency of LGMD2A in Indian population on the basis of protein analysis by immunoblotting and to correlate pathological and clinical features with protein analysis. Settings and Design : One hundred and seventy-one muscle biopsies of clinically suspected LGMD, unclassified muscular dystrophy or myopathy were analyzed in a tertiary national referral centre for neurosciences. Materials and Methods : Histopathological, immunohistochemical and enzyme histochemical analysis of muscle biopsies was performed followed by protein analysis for calpain-3 and dysferlin by immunoblotting. Results : Immunoblot identified 75 patients (43.8%) with calpain-3 deficiency, of which 36 (45%) had complete loss and 39 (55%) had partial loss of calpain-3 protein. In patients with LGMD phenotype alone, the incidence of LGMD2A was 47%. The biopsies of these patients displayed variety of morphological changes ranging from dystrophic pattern with presence of active fibre necrosis, regeneration and lobulated fibres to end stage muscle disease. The mean age of presentation and disease onset was 24 and 18 years respectively. Conclusions : This series of 75 patients is probably the first confirmed cases of LGMD2A (calpainopathy) from India. Our study suggests that LGMD2A is the most frequent form of LGMD in India, similar to the Western data, thus, highlighting the importance of immunoblotting for an accurate diagnosis.

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Cystic lesions of the brain may present with seizures or headache due to raised intracranial pressure. These cysts when associated with developmental brain anomalies may turn out to be pathologic surprises. In the present communication, two patients with glio-ependymal cysts were described with contrasting symptomatologies and surgical management. Non-enhancing cystic lesions of the brain, without mural nodule, may turn out to be glio-ependymal cysts, requiring total surgical excision or marsupilization.

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Dengue infection is endemic to India and an important public health problem. We report three confirmed cases of dengue infection with acute, pure motor, reversible quadriparesis due to hypokalemia. Clinicians should be aware of such an association and consider the clinical possibility in the differential diagnosis while evaluating acute quadriparesis in patients with dengue fever, especially in endemic areas.

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Background : Tourette syndrome (TS) is a neurobehavioral and neuropsychiatric disorder and its pathophysiology is not well understood. However, recent studies provide evidence implicating metabolic abnormalities of dopamine (DA) and serotonin (5-HT) of the basal ganglia both in TS patients and TS animal models. It is also well known that dopamine and serotonin transporters (DAT and SERT) are monoamine neurotransmitter transporters, which participate in the metabolism of DA and 5-HT, respectively. Objective : To evaluate whether expression of DAT and SERT in the striatum could lead to pathophysiological change in TS rat model. Materials and Methods : Twenty-four Wistar male rats were randomly allocated to: TS model group (n=12) and control group (n=12). The stereotypy counts were recorded during the 2-week period of inducing TS rat models. The levels of DA and 5-HT in striatum homogenate were measured by ELISA. The protein and mRNA expression of DAT and SERT in the striatum were tested respectively by Immunofluorescence, Western blot and quantitative real-time PCR. Results : ANOVA analysis indicated that the stereotypy scores were much higher in the TS model group than in the control group at different time points (P<0.01). By ELISA analysis, the DA concentration in striatum homogenate was higher in the TS model group (130.92 ± 25.60 ng/mL) than in the control group (101.00 ± 20.14 ng/mL) (P<0.01), but 5-HT concentration in striatum was found to be lower in the TS model group (59.79 ± 14.73 ng/mL) compared to the control group (77.01 ± 14.05 ng/mL) (P<0.05). Analysis of protein and mRNA levels revealed a lower expression of DAT, concomitant with a higher expression of SERT in striatum of the TS model group than in the control group. Conclusions : Lower expression in DAT, concomitant with higher expression in SERT could participate in the pathophysiology of TS.

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Background: Digital subtraction angiography (DSA) remains the gold standard in the evaluation of arteriovenous malformations (AVMs). Susceptibility-weighted imaging (SWI), a relatively new magnetic resonance imaging (MRI) technique, exploits the magnetic susceptibility differences of various tissues, such as blood, iron and calcification. Earlier studies have shown that the magnitude and phase information of SWI offers improved sensitivity, revealing low-flow vascular malformations that are invisible on conventional gradient-echo (GRE) sequences. Aim: To evaluate the imaging appearance of AVMs on SWI. Materials and Methods: In this retrospective study, the appearance of the various components (feeding artery, nidus, and draining veins) of AVMs on the phase, magnitude, and minimal intensity projection (minIP) images of SWI were analyzed in 14 patients with AVM and compared with conventional sequences. Results: Detection and delineation of various components of AVMs was best achieved in the magnitude images. Although minIP was most effective in detecting hemorrhage and calcification, it was the magnitude image that could separate the hemorrhagic and calcified component in the nidus from the remaining nidus. The minIP was less effective in detecting the AVM components, especially nidus and draining vein, whereas conspicuity was poor with the phase images. Conclusion: The magnitude images of the SWI help in differentiating the different components of AVM and also helps in differentiating nidus from hemorrhage and calcification.

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Acute spontaneous subdural hematoma of arterial origin, a neurosurgical emergency resulting from rupture of the perisylvian cortical artery, is a rare occurrence. We report four such patients who presented with progressive neurological deterioration. All the patients were operated and perisylvian cortical artery was identified as the source of bleeding in all the patients. Three of the patients had associated hypertension. We reviewed the clinical characteristics, etiology, and outcome of the reported cases in the literature. A high index of suspicion is necessary even in young patients in view of the phenomenon of re-rupture mimicking stroke. Early diagnosis and a wide craniotomy over the sylvian fissure to obtain hemostasis of bleeding points results in good outcome.

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Cervical ribs rarely become symptomatic. Cerebral ischemia or infarct due to cervical rib is extremely rare and, invariably, these patients have a history of upper limb symptoms before presenting with stroke. We report a young boy with cervical rib who presented with stroke. A right sided cervical rib was noted during angiogram, causing mild stenosis and post stenotic dilatation of right subclavian artery distal to the rib. An abduction angiogram showed complete occlusion of the right subclavian artery and visualization of collaterals. Right carotid angiogram also showed evidence of thromboembolic episodes in the right middle cerebral artery territory.

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Subfrontal extradural hematomas are uncommon, similar are orbital subperiosteal hematomas. Co-occurrence of both following head trauma is very rare. We describe co-occurrence of sub frontal extradural and orbital subperiosteal hematomas in four patients. The presenting symptoms were proptosis and visual complaints. Diagnosis was confirmed on computed tomography in three patients and magnetic resonance imaging in one patient. Frontal craniotomy and superior orbitotomy with evacuation of hematoma resulted in complete resolution of proptosis and visual symptoms. We emphasize on the early diagnosis of this rare condition and also emergency treatment to prevent permanent visual loss.

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The present study focuses on the incidence of epileptic seizures in 1000 patients (200 children and 800 adults) with migraine. Very few patients with migraine had history of epileptic seizures. No patient had migraine-induced seizures and none had seizure-induced migraine like headaches. Occurrence of psychogenic non-epileptic attacks during migraine headaches had not been highlighted in the past. In the present study, about a quarter of patients with migraine, especially adolescent and adult females, had history of psychogenic non-epileptic seizures during attacks of acute migraine. This appears to be the first report on these features in Indian subjects with migraine.

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Multicentric Gliomas, both supratentorial and infratentorial, with varying histopathological picture is extremely rare. We report a unique occurrence of such a combination in a 50-year-old man who presented with features of elevated intracranial pressure, ataxia and vertigo. Magnetic resonance imaging showed a diffuse non-enhancing lesion in the temporal lobe and insula and another non-contigous well defined enhancing lesion in the cerebellum. Both the lesions had mass effect. The lesions were decompressed; first the temporal lesion and then the cerebellar lesion. Histopathology revealed grade II astrocytoma in the temporal lobe and glioblastoma multiforme in the cerebellum. He recieved whole brain radiotherapy with which he showed symptomatic improvement and survived for 1.5 years.

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Background : Neural damage can be mitigated by calcium-channel blockers (CCBs). However, the mechanism of action of CCBs is not yet fully understood. Objective : To investigate the mechanism of action and efficacy of CCB, flunarizine in restoring neural function after crush injury to the nerves Materials and Methods : The sciatic nerves of rats were crushed using pincers to establish the model for crush injury. Two hundred and eighty-eight Sprague-Dawley (SD) rats were randomly divided into sham-operated, saline, and low-dose flunarizine and high-dose flunarizine (FI and FII) groups. The expression of the protein c-fos in the dorsal root ganglia (DRG) after crush injury to the sciatic nerves was investigated by using reverse transcription-polymerase chain reaction (RT-PCR) and Western blot. The effect of flunarizine on c-fos expression and its efficacy in restoring neural function was evaluated. Results : The c-fos messenger ribonucleic acid (mRNA) and protein expression in FI and FII groups was significantly lower than in the saline group and was the least in the FII group. Nerve-conduction velocity was increased in the order of: saline < FI< FII< sham-operated. There was no significant difference in the nerve-conduction velocity in the sham-operated and FII groups (P>.05). Conclusions : When administered after crush injury to peripheral nerves, flunarizine may protect neurons with lesions from further damage and improve neural function by downregulating c-fos expression.

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Background : Depression has significant negative impact on the quality of life in patients with epilepsy (PWE). Aim: This study assessed the prevalence of depression in PWE and the impact of seizure variables on the depression scores. Settings and Design : A case-control study of randomly selected PWE attending a tertiary hospital in a metropolitan, Nigeria. Materials and Methods : A total of 152 randomly selected subjects the Beck Depression Inventory (BDI) for quantitative assessment of depression, while the Hamilton Rating Scale for Depression (HRSD) was used by the investigators. Statistical Analysis : The Student t test assessed statistical significance of differences in the BDI and HRSD scores, whereas the scores were correlated with Pearson's correlation coefficient. Logistic regression analysis and Chi-square test for trend assessed the impact of seizure variables on the scores. The level of significance was taken as P < 0.05. Results : The prevalence of depressive symptoms was 42% and 45% using the HRSD and BDI, respectively, with significant differences in the scores of the patients and controls on the both scales (P < 0.001). The PWE scores on both scales yielded a correlation coefficient of 0.8 indicating their utility in detecting depressive symptoms. Seizure control was the most potent predictor of depression (HRSD: P = 0.004; BDI: P = 0.001). Conclusions : Depressive symptoms are common in epilepsy. Early detection and prompt management are recommended. Good seizure control with an appropriate antiepileptic drug, among other interventional measures, may contribute to the prevention of depression in epilepsy.

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Two cases of sporadic hemiplegic migraine, which fulfilled the diagnostic criteria as laid down in International Classification of Headache Disorders (ICHD)-2, are reported in children. In the first case, two unusual features were noted, namely, the occurrence of dysphsia in association with a left hemiparesis and the spread of sensory symptoms to the contralateral side during attacks. The second case is perhaps the youngest patient reported with this disorder.

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Background : Multiple sclerosis (MS) is mostly diagnosed clinically, but the diagnosis has significantly improved through the use of brain magnetic resonance imaging (MRI), testing of cerebrospinal fluid, and multimodal evoked potentials (MEPs). Even though MRI is the superior method in diagnosing this illness, MEPs remain important because they can detect clinically silent lesions in the sensory and motor pathways of the central nervous system (CNS). Aim : The aim of the study is to test the diagnostic sensitivity of MEPs and MRI and the ratio of their sensitivity in patients with MS. Materials and Methods : The study subjects included 293 patients with MS with disease duration of two to six years: 249 patients with relapsing-remitting (RR) MS and 44 with primary-progressive (PP) MS. All patients were subjected to an MRI brain scan, visual evoked potentials (VEPs), median somatosensory evoked potentials (SEPs), tibial somatosensory evoked potentials (SEPs), and auditory evoked potentials (AEPs). Abnormal Findings Included : changed wave morphology, interside difference in wave amplitude, absolute and interwave latency increased by 2.5 SD as compared with the control group. The control group comprised of 35 healthy subjects. Results : In this study the most abnormal findings were tibial SEPs, median SEPs, and VEPs. Our results suggest different sensitivity of MEPs in patients suffering from different forms of MS. In RR-MS the sensitivity of tibial SEPs was statically significant (Fischer's exact probability test) as compared to other evoked potential modalities. Similarly VEPs were more sensitive as compared to AEPs. In the PP-MS, median SEPs have been found to be more sensitive than VEPs, while tibial SEPs have been found to be more sensitive than AEPs. There was no significant difference in the sensitivity of MRI and MEPs both the forms of MS. Conclusion : Tibial SEPs produce the most abnormal results and the highest sensitivity in the RR-MS. We propose that this test as useful criterion for the diagnosis of MS.

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Bilateral medial thalamic infarcts may be due to thrombosis of internal cerebral veins or occlusion of artery of Percheron. Conventional MR imaging is often not helpful in differentiating the two. We discuss two cases in whom susceptibility-weighted imaging, including phase images contributed in demonstrating the thrombosed or patent internal cerebral veins.

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Background : The exact pathogenesis of pregnancy associated cerebral venous thrombois is still unsettled. Aims : To identify possible inherited and acquired prothrombotic risk factors and also identify the factors associated with mortality in pregnancy associated CVT. Settings and Design : Prospective cohort study to identify prothrombotic risk factors and case control study of influence of local traditional practice of puerperal water restriction on postpartum CVT. Materials and Methods : Consecutive patients with pregnancy associated CVT seen over a period of three years. Thrombotic workup included genetic markers, protein assays, and other factors. Statistical Analysis : Univariate and chi-square analysis. Results: Of the 41 patients studied during the study period, 71% of patient had a single and 34% had multiple prothrombotic risk factors. Methylene tetrahydro-folate reductase (MTHFR) heterozygosity (19.5%) and factor V Leiden heterozygous (7.3%) were the commonest genetic markers. Hyperhomocysteinemia (34%) and elevated factor VIII levels (14.6%) were the other important risk factors. In this cohort the mortality was 17%. Mortality increased by odds of 1.3 for every additional prothrombotic marker. The factors associated with increased mortality included: status epileptics (P = 0.05, OR 13.2, 95% CI 1.002 - 173), deep venous system involvement (P = 0.016, OR 9.64, 95% CI 1.53 - 60.6), presence of midline shift (P = 0.012, OR 24.7, 95% CI 2.05 - 29.8) and diffuse cerebral edema (P = 0.006, OR 14.5, 95% CI 2.18- 96.4). The traditional practice of decrease intake of water during puerperium was significant in woman with pregnancy associated CVT when compared to control subjects (P < 0.02). Conclusion : In patients with pregnancy associated CVT, prothrombotic markers can be multiple and are associated with increased odds of mortality. Deep venous system involvement, presence of midline shift and diffuse cerebral edema increased mortality. Peuperial water restriction may be a modifiable risk factor.

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In suboccipital craniectomy where the bone is not repositioned, there may be a significant cosmetic defect due to lack of skull bone in the suboccipital region. It may accompanied by sensory symptoms, including pain. To prevent any cosmetic defect and sensory symptoms we repositioned the bone chips at the craniectomy site in 42 suboccipital craniectomies before the closure of the scalp. At a mean follow-up of 22 months (range: 5-44 months), two patients complained of mild discomfort in the healed wound or of occasional local pain. One patient complained of mild itching at the site. In two patients, bone chips were accumulated at the lower part of the suboccipital craniectomy and failed to form a uniform bone cover at the operated site. In one patient, all bone chips were reabsorbed and there was no bone at the operated site. There was pseudomeningocele formation in one patient. In the rest of the cases there was satisfactory bone coverage at the operated site, both clinically and radiologically. The wound sites were aesthetically acceptable in 40 cases. Our study suggests that in the majority of cases where suboccipital craniotomy is not possible or not done, repositioning of the bone chips at the craniectomy site is associated with satisfactory aesthetic and functional outcome and formation of bone coverage at the operated site.

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Spinocerebellar ataxia 7 (SCA7) is a rare disease, and only few SCA7 families have been reported, especially from East Asia. Clinical features of a genetically confirmed SCA7 Chinese family were evaluated. The onset of the disease varied from 4 years to 48 years, and the initial presenting feature was cerebellar ataxia or visual impairment, or both. There were abnormal findings on fundus photography, electroretinogram, flash visual evoked potential and oscillatory potentials. Abnormal mitochondria were also found in skeletal muscle or liver biopsies. The number of cytosine adenine guanine (CAG) repeats ranged from 50 to 97, and the length of CAG repeat was inversely correlated with the age of onset (r=-0.867, P=0.025). Conclusion: The clinical manifestations and SCA7 gene of SCA7 patients were homogeneous in this study. Larger CAG repeats had not only resulted in earlier onset, but also related to the rapid progression and severity of the disease. Abnormal mitochondria may be a common finding in biopsy studies of various organs in SCA7 patients.

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Background : Intraoperative angiography (IOA) assumes an important role in the prevention of complications such as aneurysmal neck remnant or compromise of adjacent cerebral vasculature during surgery for cerebral aneurysms. Aims : To determine the feasibility, efficacy and safety of IOA in aneurysmal surgery Settings and Design : Prospective study of IOA in patients undergoing aneurysmal surgery. Materials and Methods : IOA was performed using digital subtraction angiography (DSA) compatible C-arm, radiolucent operating table and appropriate femoral sheath, guiding catheters and guide wires in 20 consecutive patients after surgical clipping of the cerebral aneurysm. The post-clipping IOA was compared with preoperative angiogram. Results : Complete aneurysmal obliteration was confirmed in all the patients. In two patients compromization of adjacent vessels was noted, which could be rectified by repositioning of the clip. Some degree of vasospasm was noted in all the patients. Intra-arterial nimodipine was administered in four patients with severe vasospasm. There was improvement in two patients. Time taken for performing IOA varied from 30 to 45 min. No complications attributable to IOA were encountered in this study. Conclusion : IOA is a safe and effective adjunctive tool for aneurysm clipping. Routine use of IOA in all cases of aneurysmal surgery is recommended.

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Background : The appropriate duration of albendazole therapy in neurocysticercosis is uncertain. The observation in small uncontrolled randomized trials in children that short-course therapy (1 week) is as effective as the conventional regimen (4weeks) must be tested. Objective : To compare the efficacy of 1 and 4 weeks of albendazole therapy in children with single small enhancing computed tomographic lesion (SSECTL). Study Design: An open-labeled, randomized, clinical trial. Materials and Methods : One hundred twenty children with SSECTLs presenting with seizure. Intervention: The subjects were assigned to two groups using random tables: group A (n=58) received albendazole for 1 week and group B (n=62) for 4 weeks. All the subjects were followed up for 6 months. Results : The proportions of subjects with complete resolution of lesion in the two groups were similar (group A 63.8% versus group B 51.6%). The proportion of subjects in the two groups in whom the lesion calcified on follow up (group A 19% versus group B 24.2%) also did not differ significantly. The incidence of seizure recurrence during the 6-month follow-up period was also similar in both the groups (group A 9.6% versus group B 3.4%, P > 0.05). Conclusion : One week of albendazole therapy is as effective as 4 weeks of therapy in children with SSECTLs.

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There is paucity of the studies on mutations in neurologic Wilson disease (WD) in India. We studied H1069Q, R778L, I1102T mutations in 26 patients with neurologic WD from 25 families in north India. The basis of diagnosis of neurologic WD was clinical, Kayser-Fleischer (KF) ring, and ceruloplasmin. Data collected included: family history, clinical characteristics, laboratory data, ultrasound findings, magnetic resonance imaging (MRI) findings, and severity of the disease. DNA was isolated from venous blood and subjected to H1069Q, R778L, and I1102T mutation study. The age range was 5-41 years. Family history was present in 8 patients. The H1069Q, R778L, and I1102T mutations were absent in all the patients and in 16 parents and siblings. Severity of the illness was related to the extent of MRI changes but not with age of onset and hepatic involvement. H1069Q, R778L, and I1102T mutations were absent in our patients, which may be due to genetic and ethnic heterogeneity and further studies are required.

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Background : Very small cerebral aneurysms are considered to be one of the challenges for endovascular treatment, with difficulty for catheterization and high risk for intraoperative rupture. We report the treatment of very small (< 3-mm) cerebral aneurysms by coil embolization. Materials and Methods : We performed a retrospective analysis of 11 consecutive patients with very small aneurysms treated by coil embolization in our institute between February 2007 and February 2009. Results : Three-dimensional rotational angiography (3DRA) was most accurate in the detection of these aneurysms; 3DRA revealed the aneurysms in two patients in whom conventional angiography failed to demonstrate the aneurysms. The Hunt-Hess (HH) grade was grade 0 (unruptured aneurysm) in one patient and grade I in ten patients. Coil embolization was successfully performed in 11 patients. Complete (n = 8) or near complete (n = 3) immediate occlusion was obtained. One or three soft coils were used in all the patients with the shortest available length. Balloon assistance was used in one patient and stent assistance was used in seven patients. Although coil migration into the MCA was seen in one patient and intraoperative aneurismal rupture occurred in one patient, no untoward clinical complication was seen. Follow-up DSA in 11 patients demonstrated persistent occlusion (n = 9) or progressive thrombosis (n = 2) of the aneurysms. All the patients with available follow-up had a modified Rankin Score (mRS) of 0. Conclusion : HH grade 0 and I very small cerebral aneurysms can be treated by endovascular coil embolization. Use of short, soft coils and balloon/stent assistance is useful.

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The coexistence of hemicrania continua with another primary headache disorder is a very rare event. We report a male patient with both hemicrania continua of 16- year duration and pre-orgasmic headache of three-year duration. Both headache disorders responded to indomethacin. The patient had also in addition persistent elevation of fasting serum insulin.

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