The spectrum of chronic dysimmune neuropathies has widened well beyond chronic demyelinating polyradiculoneuropathy (CIDP). Pure motor (multifocal motor neuropathy), sensorimotor with asymmetrical involvement (multifocal acquired demylinating sensory and motor neuropathy), exclusively distal sensory (distal acquired demyelinating sensory neuropathy) and very proximal sensory (chronic immune sensory polyradiculopathy) constitute the variants of CIDP. Correct diagnosis of these entities is of importance in terms of initiation of appropriate therapy as well as prognostication of these patients. The rates of detection of immune-mediated neuropathies with monoclonal cell proliferation (monoclonal gammopathy of unknown significance, multiple myeloma, etc.) have been facilitated as better diagnostic tools such as serum immunofixation electrophoresis are being used more often. Immune neuropathies associated with malignancies and systemic vasculitic disorders are being defined further and treated early with better understanding of the disease processes. As this field of dysimmune neuropathies will evolve in the future, some of the curious aspects of the clinical presentations and response patterns to different immunosuppressants or immunomodulators will be further elucidated. This review also discusses representative case studies.

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Background: Guillain-Barré syndrome (GBS) is an acute inflammatory polyneuropathy commonly characterized by rapidly progressive, symmetric weakness and areflexia. Materials and Methods: We retrospectively assessed the clinical manifestations, results of electrodiagnostic tests, functional status and prognosis of 36 children diagnosed with GBS. Results: Based on clinical and electrophysiological findings, the patients were classified as having acute inflammatory demyelinating polyradiculoneuropathy (AIDP) (n = 25), acute motor axonal neuropathy (AMAN) (n = 10) and acute motor-sensory axonal neuropathy (AMSAN) (n = 1). Twenty (55.5%) patients were males and 16 (44.5%) patients were females. The mean age of the 36 patients was 68.1 ± 45.01 months (range, 6-180 months). Five (13.8%) patients were younger than 2 years. The most common initial symptoms were limb weakness, which was documented in 34 (94.4%) patients. In our study, 18 patients (51.4%) showed albuminocytological dissociation (raised protein concentration without pleocytosis) on cerebrospinal fluid (CSF) examination. Three patients (8.3%) required mechanical ventilation therapy during hospitalization. Unfortunately, three (8.3%) patients died; one patient had AIDP and two patients had axonal involvement (one case was AMAN and another case was AMSAN). When we compared the cases of residual sequel/dead and cases of complete recovery for neural involvement type including AIDP, AMAN and AMSAN, we did not find a statistically significant difference between the groups (P > 0.05). Conclusion: Our findings showed that cases of GBS was not uncommon in children younger than 2 years of age, and CSF protein level might be found high in the first week of the disease in about one half of the patients, with a higher rate of morbidity and mortality in patients with axonal involvement than in those with AIDP.

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Background: Neurological affection in Sjogren's syndrome (SS) can occur in the central and peripheral nervous system. Literature describing the neurological involvement in SS among Indian patients is lacking. Materials and Methods: Six patients of SS fulfilling the histological or serological criteria of the American European Consensus Group for SS were studied prospectively. The patients underwent clinical examination and laboratory investigations. Their clinical and investigation features are described. Results: The age of the patients ranged from 26 to 48 years, with a male to female ratio of 2:4. In our series, peripheral sensori-motor neuropathy and sensory ataxic neuropathy was seen in 3/6, mononeuritis multiplex in 2/6, cranial neuropathy in 2/6, autonomic neuropathy in 1/6, myelopathy in 4/6, optic neuropathy in 2/6, with presence of classical sicca features in 5/6 patients. Positive lip biopsy was seen in three, altitudinal field defect in one and positive Schirmer's test in five patients. Nerve conduction study abnormalities were seen in three and evidence of vasculitis was seen in nerve biopsy of one patient and chronic nonuniform axonopathy was seen in another. Antibody to Ro (SSA) or La (SSB) was positive in five patients. Conclusions: SS involves different parts of the nervous system with varied presentations. Clinical suspicion and adequate laboratory testing helps to diagnose and manage this disorder that is relatively rare in Indian patients.

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A 35-year-old businessman with a history of migraine with aura developed new neck pain while lying on a sofa in his home. He was given neck massage and physical therapy for a day, and subsequently after two days developed severe generalized headache when sitting or standing. He was hospitalized in his hometown. Investigations revealed venous sinus thrombosis and bilateral thin subdural collections for which he was anticoagulated. Headache improved and then worsened and became severe in all positions. He was then admitted under our care. MRI scan of the brain at our hospital showed left subdural hematoma with midline shift. It required urgent evacuation. His previous first brain MRI was re-evaluated. It showed characteristic features of spontaneous intracranial hypotension (SIH). If not recognized early, SIH results in various complications, some of which require immediate intervention. Any change in the pattern of headache in SIH one must alert the clinician due to the possibility of one of its complications.

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Simultaneous or sequential involvement of lungs is frequently encountered with neurological syndromes like meningoencephalitis, cerebellitis, aseptic meningitis, transverse myelitis, or multiple cranial nerve palsies. However, pulmonary involvement is frequently overlooked when all the attention of physician is diverted to neurological disorder. Prompt and early recognition of such potentially treatable association is required to improve diagnostic and therapeutic outcome. We report six patients presenting with various neurological manifestations like meningitis, meningoencephalitis, and myelits associated with atypical pneumonia. With proper clinical correlation and relevant investigations, all of them were diagnosed in time and had remarkable recovery with appropriate treatment.

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Background: Causative role of Chlamydia pneumoniae infection in patients with acute ischemic stroke (AIS) remains unresolved till date. Aim: To investigate the role of C. pneumoniae antibodies in AIS. Materials and Methods: Patients with AIS and sex- and environment-matched controls were enrolled. Antibodies to C. pneumoniae (IgA, IgG and IgM) were measured using enzyme-linked immunosorbent assay (ELISA). Results: A total of 51 patients and 48 controls were enrolled. The IgA seropositivity was significantly associated with AIS (unadjusted odds ratio 3.1; 95% CI 1.38, 6.96; P = 0.005), whereas IgG (unadjusted OR 0.44; 95% CI 0.18, 1.09; P = 0.07) and IgM (unadjusted OR 1.1; 95% CI 0.36, 3.3; P = 0.88) were not. There was no difference in IgA or IgG positivity in different stroke subtypes. On multivariate analysis after adjusting for sex, hypertension, diabetes mellitus, smoking and alcohol, the IgA seropositivity yielded an adjusted OR for stroke (4.72; 95% CI 1.61, 13.83; P = 0.005), while IgG seropositivity did not (OR 0.25; 95% CI 0.08, 0.83; P = 0.23). Conclusions: An increased risk of AIS was demonstrated in patients seropositive for C. pneumoniae for IgA antibodies.

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Epilepsy is a common neurological disorder with diverse etiologies. Neuroimaging plays an important role in workup of patients with epilepsy. It helps to identify brain pathologies that require specific treatment; and also in formulating syndromic and etiological diagnoses so as to give patients and their relatives an accurate prognosis. Magnetic resonance imaging, specially the 3 tesla MRI is the imaging of choice because of its ability to detect small lesions like mesial temporal sclerosis, cortical dysplasias, small tumors, etc that are not detected by conventional MR or CT scan of brain. Identification of these lesions often helps in managing refractory epilepsies more effectively. However, cost and non-availability of MR in large part of the country necessitate the use of CT as an alternative. CT is often the initial investigation and also useful in acute situations. Functional imagings are used for pre-surgical work-up of refractory epilepsy cases with an aim to identify the epileptogenic focus and to delineate functional areas nearing the focus.

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Objectives: This study was conducted to find out knowledge, attitude and practice (KAP) of epilepsy among 12 ^th -class students in Uttarakhand state. Secondly data of Uttarakhand was compared with KAP study from other parts of the country. Materials and Methods: All 12 ^th - class students studying in six schools of randomly selected 36 villages in Chakrata block of Dehradun district of Uttarakhand state were provided a printed questionnaire having answer as "yes or no". This questionnaire used was used previously by various authors and validated for KAP analysis. These filled questionnaires were collected by village health workers and medical officer. Results: This study conducted on 219, 12 ^th -class students revealed that epilepsy was heard by 98%, 74.9% thought epilepsy a mental disease and 4.8% believed that it is contagious. Negative attitude showed as nearly 2/3 ^rd students stated that epilepsy is hindrance in marriage and occupation. Nearly 41% would use onion or shoe for terminating seizure attack. Ayurvedic treatment was preferred over allopathic drugs. Conclusions: Study on 12 ^th -class students of Uttarakhand revealed poor knowledge, attitude and practice for epilepsy and needs special education program to dispel these misconceptions.

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Background and Objective: Edaravone has potent antioxidant and free radical scavenger properties. Few Japanese studies had demonstrated its neuroprotective role in acute ischemic stroke (AIS). This study aims to evaluate the efficacy of edaravone in terms of functional outcome in a group of Indian patients of AIS. Materials and Methods: Fifty patients of AIS were randomly divided into two groups. The study group received 30 mg of edaravone twice daily for 14 days by infusion, while control group received normal saline infusion as placebo. Outcome assessment was done by the Modified Rankin Scale (MRS). MRS score ≤2 at 90 days was considered as a favorable outcome. Results: Of 25 patients, 18 (72%) had favorable outcomes (MRS ≤2) at 90 days in edaravone group, while 10 (40%) of 25 patients in placebo group had favorable outcome (P < 0.005). Two patients expired (one in each group) during treatment. The mean Barthel index increased from 41.20 ± 32.70 at baseline to 82.40 ± 18.32 at day 90 in edaravone group as compared with placebo group in which scores were 44.20 ± 22.76 at baseline and 68.20 ± 21.30 at day 90 (P < 0.005). Conclusions: We therefore conclude that edaravone effectively improves functional outcome in AIS.

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Hemichorea-hemiballism syndrome (HCHB) is a relatively rare cause of unilateral chorea in diabetic patients and is due to non ketotoic hyperglycaemia. Characteristic magnetic resonance (MR) findings include T1 hyperintensity in the contralateral putamen without any significant signal alteration on other conventional MR sequences. We report susceptibility weighted imaging (SWI) findings in a case of HCHB syndrome.

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Background: After malaria, schistosomiasis is the second most prevalent tropical disease. The prevalence of oviposition in CNS of infected persons varies from 0.3 to 30%. The conus medullaris is a primary site of schistosomiasis, either granulomatous or acute necrotizing myelitis. Objective: To report the clinical, radiological, and laboratory results of spinal cord schistosomiasis (SCS) and to design proper therapeutic regimens. Materials and Methods: Seventeen patients (13 males and four females) with SCS were enrolled between 1994 and 2009 at Mansoura University Hospitals. Their median age at diagnosis was 19 years (13-30 years). Independent neurological, radiological, and laboratory assessments were performed for both groups, excluding pathological confirmation that was done earlier in eight patients (Group 1). In the group 2 (nine patients), indirect hemagglutination (IHA) test for bilharziasis in blood and cerebrospinal fluid (CSF) was performed. Higher positive titer in CSF than serum indicated SCS plus induction of antibilharzial and corticosteroid protocols for 12 months with a three-year follow-up. Results: Rate of neurological symptoms of granulomatous intramedullary cord lesion was assessed independently in 16 cases and acute paraparesis in one case. All patients in group 2 had positive IHA against Schistosoma mansoni with median CSF and serum ranges 1/640 and 1/320, respectively. Seven patients (41.18%) had complete recovery, eight patients (47.06%) showed partial recovery, and no response was reported in two patients (11.76%) (P = 0.005). There was no recorded mortality in the current registry. Conclusions: Rapid diagnosis of SCS with early medical therapies for 12 months is a crucial tool to complete recovery.

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Here we present the case of a 17-year-old girl belonging to an ethnic tribe (Bodo tribe) of Assam, presenting with bilateral cerebellar signs and with history suggestive of an autosomal dominant pattern of inheritance, who was found to have spinocerebellar ataxia 7 on genetic testing. This case throws light on the probability of more such cases in the multi-ethnic society of the North-Eastern Indian states, which are not studied or reported till date.

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Meige's syndrome is characterized by blepharospasm and oromandibular dystonia. It has been reported as a complication of typical antipsychotics. To the best of our knowledge, case of olanzapine-induced tardive Meige's syndrome has not been reported in the literature. We we are reporting report a case of Meige's syndrome developing after long term therapy with olanzapine.

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Subacute sclerosing panencephalitis (SSPE) is a rare, invariably fatal degenerative disease of the central nervous system developing after measles infection. Besides neurological symptoms as initial presenting symptoms, rare reports of its presentation with pure psychiatric symptoms have been reported. We here report a case of 14 year old male who initially presented with manic symptoms and then subsequently diagnosed to be suffering from SSPE. Improtance of ruling our organic conditions is emphasized.

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