Objective: To derive population norms on the Malayalam adaptation of Addenbrooke's Cognitive Examination (M-ACE) and the inclusive Malayalam mini mental state examination (M-MMSE). Materials and Methods: Education-stratified norms were obtained on randomly selected cognitively unimpaired community elders (n = 519). Results: Valid data on norms was available on 488 subjects (age 68.5 ± 7.1 and education 7.9 ± 5.4). Education and age, but not gender had a significant effect on both M-ACE and M-MMSE. When compared to the effect of age, the effect of education was sevenfold more on the M-ACE and ninefold more on the M-MMSE. The mean composite score on the M-ACE (and the M-MMSE) was 42.8 ± 9.8 (14.9 ± 3.1) for those with 0 (n = 72), 55.9 ± 12.5 (19.7 ± 4.1) with 1-4 (n = 96), 62.6 ± 11.4 (21.9 ± 3.7) with 5-8 (n = 81), 77 ± 10.2 (25.7 ± 2.4) with 9-12 (n = 136) and 83.4 ± 7.2 (26.7 ± 1.6) with >12 (n = 103) years of formal education. Conclusions: Education has the most potent effect on performance on both M-ACE and M-MMSE in the Indian cohort. Education-stratified scores on the M-ACE and the M-MMSE, will provide a more appropriate means of establishing the cognitive status of patients. It is also our feeling that these cut-off scores will be useful across India.

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Background: Stroke and myocardial infarction (MI) are both life-threatening diseases of vascular origin with a tendency to recur. In both conditions, risk of recurrence is reduced through similar drug regimens. Aim: To determine if compliance with prescribed medication after stroke or MI was similar in the two populations. Setting and Design: Retrospective data collection and cross-sectional telephonic survey of patients discharged from a single academic medical center. Materials and Methods: Adult patients consecutively discharged over a two-year period with a diagnosis of first-ever stroke (ischemic or hemorrhagic) or first-ever MI (ST-elevation) were identified through ICD-9 codes. Clinical details were abstracted from hospital records. Medication compliance was assessed through a structured telephone interview. Statistical Analysis: Bivariate analysis using Chi-square and Fisher exact tests, to determine the prevalence of noncompliance in stroke versus MI patients and differences in baseline characteristics, and multivariate analysis with logistic regression to determine independent predictors of noncompliance. Results: Follow-up data was collected for 298 stroke and 275 MI patients. Compliance was lower in stroke patients (68% stroke patients compliant with at least half their discharge prescriptions versus 90% MI patients; P <0.001). Literacy and post-discharge follow-up were associated with greater compliance ( P <0.05 for both). Compliance was highest with anti-hypertensive drugs (98% after MI, 78% after stroke), followed by anti-platelet agents (94% after MI, 75% after stroke) and anti-lipid agents (70% after MI, 59% after stroke). Patients reported simply not feeling the need, acquiring fresh medical advice or a perceived lack of benefit, as reasons for not complying with their discharge prescriptions. Conclusions: Although similar drugs are involved, compliance with prescribed regimens is appreciably lower after stroke than after MI. Our findings underscore the need for better patient education regarding secondary prevention after stroke.

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Background : Limb girdle muscular dystrophy (LGMD) is a heterogeneous group of disorders characterized by limb girdle weakness. There are no clear clinical features that distinguish various types of LGMD. Materials and Methods : We studied 26 patients with chronic progressive weakness in limb girdle distribution without early facial involvement with muscle biopsies suggestive of dystrophy/myopathy and positive for dystrophin antibodies. Immunohistochemistry studies of muscle biopsies were done on all patients to classify different types of sarcoglycanopathies. Results : The mean age of presentation was in the third decade. There were 14 male and 12 female patients. The common pattern of inheritance was autosomal recessive, seen in 53.8%. The more frequent type of LGMD was sarcoglycanopathy (SGP) (53.8%). Amongst the SGPs, alpha-SGP (26.9%) was the most common followed by beta-SGP (15.3%), gamma-SGP (3.8%) and delta-SGP (7.6%). Calf hypertrophy was noted in 53.5% of LGMD and 57.1% of SGPs, extensor digitorum brevis hypertrophy in 42% of LGMD and 35.7% of SGPs, winging of scapula in 39.2% of the LGMD group and 35.7% of the SGPs, valley sign in 28.5% of the LGMD group and 21.4% of the SGPs. Hip abductor sign was positive in 71.4% of LGMD and 64.2% of SGPs. Differential weakness of knee flexors was more common in SGP (57.1%). The mean creatine phosphokinase (CK) value was 2519IU/L and was elevated in 92.8% patients. Muscle biopsy showed a dystrophic pattern in 75% of LGMD and a myopathic pattern in the remaining. Symptomatic cardiac involvement was seen in one patient. ECG changes were seen in 44% of LGMD patients and 50% of the SGP. The common changes noted were T wave inversion in V1, V2 (16%), left ventricular hypertrophy LVH (12%) and right bundle branch block (RBBB) in 12% of the LGMD group. Conclusion: Sarcoglycanopathy is a more frequent form of LGMD whereas alpha type is the most common among the SGP. The four types of SGP do not differ in the pattern of muscle involvement. A relatively earlier onset, selective weakness of knee flexors and a very high CK may help differentiate SGP from other forms of LGMD. Immunohistochemistry is very useful in classifying the different types of LGMD prior to genetic analysis.

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Neuroleptic malignant syndrome (NMS) is a rare, life-threatening but potentially treatable condition. This study was performed to investigate the clinical spectrum, antecedent events and outcome of NMS patients admitted in the Neurology department of a large teaching hospital of North India. Fourteen cases of NMS were taken after a thorough search during a three-year period (May 2000 to April 2003). The Incidence of NMS was 1.40/ 1000 patients treated with neuroleptics and mortality rate was 14.28%. Amongst the neuroleptics Haloperidol (parenteral) was implicated as a most common drug for NMS in 57% of patients. An association with coexisting precipitating illness was clearly recorded in 71.4% patients. All the recorded patients of NMS received 500-700 mg CPZ equivalent/day of neuroleptics. NMS as an indiosyncratic phenomenon was noticed in 28% patients. 85.7% responded to dopaminergic drugs along with supportive treatment and showed partial or complete recovery within 7-14 days. In those with partial recovery residual deficits included Parkinsonian features, depression and diaphoresis in a small percentage of patients.

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Objective: New-onset acute symptomatic seizures can be the presenting feature of acute neurological diseases. The etiological spectrum of new-onset acute symptomatic seizures and outcome may be different in developing countries when compared to developed countries. Aim: To study the clinical profile of new-onset acute symptomatic seizures as the first presenting event in patients with acute neurological illness in a neurological intensive care unit (NICU) in a developing country. Settings and Design: Prospective study in a NICU in a tertiary care hospital. Materials and Methods: Consecutive patients with acute symptomatic new-onset seizure admitted to NICU in a tertiary care hospital over a period of 28 months. The etiology was determined by neuroimaging and appropriate investigations including cerebrospinal fluid examination. Results: Of the 3,151 admissions, 66 (2.1%) were related to new-onset acute symptomatic seizures as the first presentation. The mean age was 49.07 + 20.20 years. Tonic-clonic, generalized tonic-clonic or partial seizure with or without secondary generalization were the seizure type. At presentation 52 (79%) patients had single seizure, 10 (15%) patients had seizure clusters and four (6%) patients presented with status epilepticus (SE). The major etiological risk factors were central nervous system (CNS) infections (32%), metabolic disorders (32%) and cerebrovascular diseases (21%). In the NICU 10 (15%) patients had early seizure recurrence and three (4.5%) developed SE. Of these 13 patients, in nine (69%) patients the pathology was infection-related and the other commonality was involvement of cortical gray matter. Factors associated with seizure recurrence or SE were focal cerebral lesions involving cortical gray matter or diffuse cerebral pathology, meningoencephalitis. In 69% of these patients the pathology was infection-related. There were only two deaths, both in patients with SE and related to the underlying etiology. Conclusion: The risk of seizure recurrence and SE after the first acute symptomatic seizure is likely to be high in patients with acute focal cerebral lesions and diffuse CNS infections. The commonality in both the pathologies is cortical gray matter involvement.

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We report an unusual case of neurobrucellosis who presented with headache, vision loss, confusional state, retrobulbar neuritis, and left hemiparesis. Neuroimaging studies showed features of ischemic infarct and white matter abnormalities. Pulmonary involvement in form of lobar pneumonia of left basal lobe was another unusual manifestation. Diagnosis was based on the rising titers of antibrucella antibody. Patient showed remarkable improvement on triple drug therapy in form of doxycycline, rifampicin and streptomycin.

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Objective: To investigate the association between the pathological features of carotid plaque and ipsilateral symptoms of cerebral ischemia. Materials and Methods: We sought to identify clinical observational studies comparing the incidence of pathological features between symptomatic and asymptomatic carotid plaques in terms of ulceration, thrombosis and intraplaque hemorrhage (IPH), published between 1996 and 2006. A search on, PubMed was supplemented by a review of bibliographies of relevant articles and lists of references in it. Odds ratios (OR) for the presence of each feature as a role in the pathogenesis of neurological events were calculated and combined by a meta-analysis. Results: We integrated 16 clinical studies totaling 2839 plaques harvested at endarterectomy procedures. The reported incidence for each feature was highly variable. The methods in defining ulceration, thrombosis and IPH were very heterogeneous. The time intervals between the latest onset of ischemic symptoms and pathological examinations varied greatly. Overall, the incidence of ulcerated plaques in the symptomatic group were significantly higher than that in the asymptomatic group (study number, n=10; OR, 2.32; 95% CI, 1.90-2.83. A random-effect model was performed among studies regarding surface thrombosis because an apparent trend for heterogeneity was observed (n=6; OR, 1.57; 95% CI, 0.68-3.64). There was no difference with respect to IPH between the two groups (n=11; OR, 1.09; 95% CI, 0.91-1.32). Conclusions: Ulcerated carotid plaque is significantly correlated with the production of neurological events, whereas thrombosis fails to correlate with ischemic symptoms because of the presence of substantial heterogeneity. The association between IPH and clinical presentations is less clear.

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Parry Romberg's syndrome is an uncommon disorder characterized by atrophy of skin and subcutaneous tissue of one side of face. It has neurologic sequel. The commonest of which is epilepsy. Here, we present a 17-year old girl with features of Parry Romberg's disease with intractable epilepsy. Her seizures have stopped with systemic corticosteroids. This treatment response, together with previous reports is suggestive of an autoimmune basis to this disorder. Thus the epilepsy in some such cases may be steroid responsive.

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The authors report a rare case of acute ipsilateral blindness that occurred after a standard fronto-temporal craniotomy for aneurysm in supine position. Posterior ischemic optic neuropathy caused by external pressure on the ipsilateral eye, its differentials and subsequent medico-legal implications are discussed.

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Context: Neuronavigation provides a patient-specific, three-dimensional (3-D) anatomy for preoperative planning and intraoperative navigation. However, the initial and maintenance costs are quite prohibitive, especially in the Indian scenario. Aims : To study the efficacy and limitations of neuronavigation, especially in the Indian scenario. Settings and Design: A prospective nonrandomized study. Materials and Methods : A total of 121 patients underwent intracranial surgery from 2002-2006, in which neuronavigation was used. In this, the initial part, we studied the efficacy and limitations of neuronavigation in the initial 37 patients. The efficacy of the image guidance was graded according to a point's scale in which points were awarded ranging from 0 to 3. Cranial image guided score (IGS) was calculated by the summation of grading during designing the flap/burr hole, delineation of the intraoperative anatomy, navigation and access to the lesion and resection / biopsy of the lesion or completion of the procedure. The scoring ranged from 0-12 and the utility of IGS in cranial neurosurgical procedures was calculated based on the total points for each surgery. Results and Conclusion : Cranial image guidance was useful in a variety of operative steps. Intraoperative approach and navigation was relatively easier with an increase in perception of safety. Limitations of IGS include learning curve, cost and the phenomenon of brain shift. Drawbacks of the study included that this was a subjective rather than a truly objective study and the relatively lesser number of patients. We hope to conduct a larger study with randomization but the question of ethical approval would be a primary concern.

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Recurrence of coiled aneurysm usually due to coil compaction is a known phenomenon. Extent of recurrence and its relation to re-bleeding is not known. The authors report a case of spontaneous asymptomatic extrusion of guglielmi detachable coils from the dome of a previously ruptured anterior communicating artery aneurysm two years after the initial endovascular obliteration. The initial aneurysm had a suitable neck-aspect ratio for endovascular obliteration with uncomplicated coiling procedure. Extreme degree of coil compaction with subsequent expulsion of the coils from the dome due to water-hammer effect of blood flow is thought to be the main pathogenic mechanism of this rare but worrying complication. Surgical management includes clipping at the neck ensuring complete obliteration of the aneurysm. Postoperative angiogram to confirm its satisfactory obliteration is advised.

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We present our initial experience with the high field (1.5T) intra-operative magnetic resonance imaging, the operating room set-up, our initial cases, the difficulties we faced and how this tool affected a change in the surgical strategy intra-operatively and finally our results. 11 patients were operated on from June 1st to August 1st 2006 of which there were astrocytomas (7), pituitary adenoma (1), craniopharyngioma (1) and meningiomas (2) Localization and lesion targeting were accurate, intra-operative imaging helped to assess the resection volumes, enable corrections for brain shift, perform further tumor resection at the same sitting and help preserve eloquent cortical areas. Gliomas formed 63.6% of the tumors operated on and in 71.4% of these, our surgical strategy changed intra-operatively. Meningiomas formed 9.1% of the tumors operated and image guidance enabled a minimally invasive approach, although no change in our surgical plan was required. One pituitary adenoma and a craniopharyngioma were also operated on with good outcome.

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Background: Although the risk reduction of recurrent stroke with angiotensin converting enzyme-inhibitors with or without a diuretic has been demonstrated under randomized double blind conditions of the PROGRESS study, it is unclear whether the benefit is reflected in primary care practice and in populations with different demographic and clinical characteristics. Aim: To assess the effect and acceptability of perindopril with or without indapamide prevention of recurrent stroke, as reflected by its incidence. Setting and Design: Multicentre, prospective, observational study in the setting of primary care throughout India. Materials and Methods: Patients with a stable stroke or transient ischaemic attack (TIA) received a 12-month perindopril ± indapamide-based regimen, similar to that used in PROGRESS. The principal outcome was the annual incidence of recurrent stroke. Statistical Analysis: Summary statistics and the Kaplan-Meier procedure. Results: The mean age of 298 patients was 58.3 years (SD=12.6). 229 (77.5%) had an ischaemic stroke; 231 (77.5%) were hypertensive; 200 (85.5%) were receiving aspirin and 81 (27.2%) statins. During the 12-month perindopril-based treatment, there were 8 (2.7%) recurrent strokes, with a Kaplan-Meier estimate of strokes plus TIA of 3.3% (95% CI, 1.0-5.6). Conclusions: The incidence of recurrent stroke is similar to that observed under double blind randomized conditions in the treatment arm of the PROGRESS study. This suggests that perindopril ± indapamide-based prevention may be effective in reducing risk of recurrent stroke, (although the uncontrolled study design does not actually demonstrate this), in the setting of day-to-day clinical practice and among patients with different demographic and clinical characteristics than the PROGRESS population.

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Background: Electrodiagnostically, localization of ulnar nerve lesions, which commonly occurs at the elbow, is sometimes problematic. Measurement of motor ulnar nerve conduction velocity (NCV) at the elbow is amongst the most popular techniques to diagnose ulnar neuropathy. In this method, recording from the first dorsal interosseous muscle (FDI) is suggested to be more sensitive than the abductor digiti minimi (ADM). However, the criterion for abnormality is based on the normal values recorded from ADM. Aims: To determine the normal values of Ulnar motor NCV using FDI and ADM and the difference between the values obtained from FDI and ADM. Additionally, to measure the amount of reduction of NCV across the elbow for each recording site. Materials and Methods: This was a cross-sectional study performed on 50 healthy volunteers (100 nerves). All subjects were in the same condition regarding joint position and surface hand temperature. We recorded ulnar NCV at forearm and across the elbow with recording electrode on both FDI and ADM, simultaneously. Results and Conclusions: The mean NCV at the elbow recorded from ADM and FDI were 62.65 ± 7.62 m/s and 60.49 ± 7.42 m/s respectively, showing significant difference. The ulnar minimum normal NCVs recorded from ADM and FDI were 47.4 m/s and 45.6 m/s, respectively. If the normal values of ADM are used as the basis for recording from FDI, it could lead to false-positive diagnosis of cases suspicious of ulnar neuropathy. Therefore it is preferred to use the normal values of FDI itself while recording.

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Extraneural metastases of intracranial germinoma are rarely reported. The authors describe the first case of metastatic lung germinoma of the thoracic spine. A 27-year-old man presented with right shoulder pain and right upper limb weakness. He had a history of repetitive radiation therapy - nine (whole-abdomen; 15Gy), 12 (whole brain; 30Gy, whole spine 42Gy) and 14 years ago (local; 32Gy) - for abdominal metastasis, temporal and fourth ventricle metastasis and spinal dissemination and metastatic pineal germinoma, respectively. Magnetic resonance imaging revealed a lung mass invading the thoracic spine that was diagnosed as a germinoma by tumor biopsy. He was treated by irradiation with 54Gy and two cycles of chemotherapy with cisplatin and etoposide. He did not have any sign of tumor eight years later.

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An 80-year-old man with a history of Type 2 diabetes mellitus was hospitalized due to generalized convulsive status epilepticus. Initially, hyperglycemia and ketoacidosis were diagnosed, but his seizures were refractory to the medical treatment. Additionally, a high level of serum theophylline (29.1 mg/mL) was detected. Following detoxification of theophylline by oral activated charcoal, the patient regained consciousness and was free from seizures without antiepileptic drug treatment. Brain magnetic resonance imaging revealed subacute subdural hematomas at the bilateral occipital hemispheres. This case suggests that theophylline toxicity may be a predisposing factor for seizures in patients with a history of traumatic brain injury in spite of the presence of diabetic ketoacidosis that may have an anticonvulsant action.

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