Refractory status epilepticus (RSE) is a common problem in intensive care units and emergency departments. The important risk factor predisposing patients with SE to RSE is delay in receiving treatment. Self-sustaining SE is associated with progressive, time-dependent development of pharmacoresistance. Early termination of convulsive SE by aggressive treatment is the best way to prevent RSE. RSE once develop, requires more aggressive treatment as it is associated with higher mortality and morbidity. To date, no randomized controlled trials have been done for RSE. The most experience exists with coma inducing agents like pentobarbital, midazolam and propofol. New evidence suggests for the possible role of newer AEDs.

Objective: Genetic counseling for individuals undergoing presymptomatic testing is lacking in India although testing is easily available. This has an impact on family members of Huntington's disease (HD), an autosomal dominant disease, wherein the age at onset of symptoms varies. Aim: We examine if attitudes differ towards presymptomatic testing for HD amongst HD family members, physicians and laypersons. Materials and Methods: A modified questionnaire enquiring about opinions on various personal, family, social and future health care with regards to presymptomatic testing of HD was designed. A physician explained briefly about HD and presymptomatic testing of HD and recorded responses of unaffected family members of HD (n=25) and laypersons (n=50). Medical doctors (n=50) answered the questionnaire based on their knowledge of HD. Results: HD family members, Medical doctors and laypersons were similar in their opinion to undergo the testing. Majority (60%) of HD family members did not wish to communicate test results with their friends when compared to the other two groups. Medical doctors and HD family members were more concerned about certainty of developing disease when the test results are positive. Majority (80%) of Medical doctors and less than half in the other groups felt that their decision to have a child would strongly depend on test results. Large proportion (80%) of HD family members did not wish to report their test results to their employers. Conclusions: Individuals with knowledge about HD and the test differ in their decision of sharing test results and reproductive choices.

Aims: To determine the usefulness of fibrinolytic markers as early prognostic indicators in patients with isolated head trauma. Materials and Methods: Sixty-two consecutive patients (26 women and 36 men; mean age 61 years, range 2-76 years) with isolated head trauma seen within the first three hours of the trauma were included in the study. The Glasgow Coma score (GCS), platelet counts (Plt), prothrombin time (PT), partial thromboplastin time (PTT), fibrinogen, fibrin degradation products (FDP) and D-dimer levels were measured. Head computerized tomography (CT) findings were categorized as brain edema, linear fracture, depressed fracture, contusion and bleeding. Plt counts, PT, PTT, fibrinogen, FDP, D-dimer levels and CT findings were compared with both GCS and mortality in the first week. Statistical significance was accepted at P£0.05. Results: A marked negative relationship was found between GCS and PT, PTT, FDP and D-dimer levels ( P <0.001). Plt levels did not correlate with GCS. Mortality was most strongly related to GCS, PT, FDP and D-dimer levels ( P <0.001, P <0.001, P <0.001 and P <0.001, respectively). We found no relationship between mortality and CT findings, nor was there any significant relationship between Plt, PTT and fibrinogen levels. Conclusion: GCS and fibrinolytic markers measured within the first three hours were useful in determining the prognosis of patients with isolated head trauma.

Background and Aims: Chronic inflammatory process plays an important role in atherothrombosis. Interleukin-1 (IL-1) is one of the key modulators of the inflammatory response and its activity is critically regulated by its receptor antagonist (IL-1Ra). A variable number tandem repeat polymorphism in intron 2 of IL-1Ra gene and a C to T single base polymorphism in the promoter of IL-1β gene (C-511®T) have been reported to affect the levels of IL-1 as well as its antagonist, IL-1Ra. It is also reported in several studies that these polymorphisms are associated with the susceptibility to cardio-cerebral vascular disease. However, data are limited in China. In this article, we studied the relationships between these polymorphisms and the risk of ischemic stroke in China. Materials and Methods: One hundred and twelve patients committed ischemic stroke were compared with 95 demographically matched healthy volunteers. Results: The frequencies of the IL-1Ra 1/1 genotype and IL-1Ra allele 1 (Ra*1 allele) in stroke patients were significantly higher than those in healthy volunteers [93.7% vs. 82.1%, P =0.014; 0.964 vs. 0.905, P =0.007]. No significant differences were found in the IL-1β -511 genotype and the allele distribution between the two groups. Conclusions: Our results implicated that IL-1 gene polymorphism might be associated with the susceptibility to ischemic stroke.

Background: Memory dysfunction is common in multiple sclerosis (MS). A retrieval failure has been reported as the primary cause for the memory deficits, although some studies also described a faulty acquisition. Aims: The aim of the study was to examine memory function in relapsing remitting (RR) and secondary progressive (SP) MS patients, analyze the patterns of performance and to investigate whether disease course influences this performance. Design and settings: Case-control prospective study conducted in a clinical setting. Materials and Methods: Fifty-five RR, 23 SP MS patients and 80 normal subjects were evaluated with a comprehensive neuropsychological battery. Memory was assessed with tasks from the Signoret memory battery. Attention and executive function were also assessed. Statistical Analysis : Univariate analysis of variance, Mann-Whitney U-test, multivariate logistic regression and Chi-square test were used as appropriate. Results: MS patients performed significantly worse than controls on almost all measures of memory ( P <0,001). MS subgroups differed in tasks of delayed recall (logical memory- P =0,019; wordlist delayed recall, P <0,001), semantic cued recall ( P <0,001), recognition trials ( P =0,006) rate of forgetting ( P <0,001) and confabulation and intrusion errors ( P =0,004). Conclusions: Memory is consistently impaired in MS patients and disease course differentially affects the pattern of performance. SP patients show greater difficulties and a more pervasive pattern of dysfunction than RR patients. Delayed recall was the most affected memory measure and performance on this task discriminates between RR and SP MS patients. Relapsing remitting patients performed within the mildly impaired range while SP patients showed a moderate to severe impairment.

Background and Aims: Delayed traumatic hematomas and expansion of already detected hematomas are not uncommon. Only few studies are available on risk factors of expanding hematomas. A prospective study was aimed to find out risk factors associated with such traumatic lesions. Materials and Methods: Present study is based on 262 cases of intracerebral hematomas / contusions out of which 43 (16.4%) hematomas expanded in size. computerized tomography (CT) scan was done in all the patients at the time of admission and within 24 hours of injury. Repeat CT scan was done within 24 hours, 4 days and 7 days. Midline shift if any, prothrombin time, activated partial thromboplastin time, bleeding time, clotting time and platelet counts, Glasgow coma scale at admission and discharge and Glasgow outcome score at 6 months follow up were recorded. Results: Twenty six percent, 11.3 and 0% patients developed expanding hematoma in Glasgow Coma scale (GCS) of 8 and below, 9-12 and 13-15 respectively. The chances of expanding hematomas were higher in patients with other associated hematomas (17.4%) as compared to isolated hematoma (4.8%) (Fisher's exact results P =0.216). All the cases of expanding hematoma had some degree of midline shift and considerably higher proportion had presence of coagulopathy. The results of logistic regression analysis showed GCS, midline shift and coagulopathy as significant predictors for the expanding hematoma. Thirty nine patients (90.7%) of the total expanding hematomas developed within 24 hours of injury. Conclusions: Enlargement of intracerebral hematomas is quite common and majority of them expand early after the injury. These lesions were common in patients with poor GCS, associated hematomas, associated coagulopathy and midline shift.

Objectives: We are presenting 20 cases of the intriguing clinico-electromyographic entity, now considered a potassium channel disorder, Neuromyotonia. Our experience with the clinical manifestations, underlying abnormalities and response to various therapies is documented. Materials and Methods: Patients with diffuse pain or undulating muscle movements, with or without stiffness were sent for electromyographic and further studies. Patients with "neuromyotonic discharges" were included after exclusion of hypocalcaemia. Results: Our cases included 19 males and one female of age group 15 to 52 years, the majority being between 30 to 45 years. Undulating movements were seen in 19, of which two had focal twitching. Muscle stiffness was a complaint in five; pain was the chief presenting complaint of 19, which started in the calf in all. Irritability, insomnia and a peculiar worried pinched face were present in 12 patients. CSF was abnormal with mildly raised protein in eight. Curiously, 11 of these patients had taken ayurvedic treatment for various complaints in the preceding one month. Bell's palsy was associated in four, peripheral neuropathy in two and residual poliomyelitis in two. Electromyographic evidence of spontaneous activity in the form of "neuromyotonic discharges" was seen in all. Antibodies to voltage gated potassium channels was tested in one patient and was positive (titer was 1028 pM). Membrane stabilizers (e.g., phenytoin sodium) in our experience did not provide adequate rapid relief; we tried high-dose intravenous Methylprednisolone in 19 with significant amelioration of complaints. One patient was offered intravenous immunoglobulin, to which he responded. Conclusions: Neuromyotonia is a heterogeneous condition and can present in varied ways including diffuse nonspecific pain. This uncommon condition is potentially treatable and can be picked up with high index of suspicion.

Background: Antiepileptic drug (AED) therapy following first unprovoked seizure is controversial. Aim: To study the patients' preferences towards AED therapy following first unprovoked generalized tonic clonic seizure (GTCS). Design: Prospective cohorts with one year follow-up study. Setting: Government teaching hospital, a tertiary care center. Materials and Methods: Patient cohort included patients with first attack of unprovoked GTCS within 30 days of onset, aged between 18-60 years and with normal brain CT scan. Counseling was done for all the patients and the relatives regarding seizure recurrence, duration and adverse effects of AED therapy if preferred. Patients were encouraged to make their own decision in preferring or deferring AED with reasons. They were followed up for one year. Results: Of the 73 enrolled (54 males and 19 females) 39 (53%) preferred to go on AED therapy. The reasons for preferring AED therapy were; (a) fear of seizure recurrence, 21 (54%); (b) risky occupation, 14 (36%); and (c) fear of injury, 4 (10%). The reasons for deferring were: (a) fear of adverse effects of long-term AED therapy, 19 (56%) and (b) preferring to wait for the second attack, 15 (44%). All the patients were happy about being involved in the decision-making. Conclusion: Following first attack of unprovoked GTCS the decision regarding AED therapy may be taken by the patients and their family members after adequate counseling and such decisions have more relevance from their perspective.

Background: Evidence suggests that mitochondrial dysfunction stimulates the production of reactive oxygen species (ROS) that promote neural cell death in stroke and in Parkinson's disease. The sites of mitochondrial ROS production are not established but are generally believed to be located within the electron transport chain. Aims: We studied the mitochondrial respiratory chain enzymes function from human circulating lymphocytes. Setting and Design: Open study. Materials and Methods: Forty patients with Parkinson's disease (PD) with 30 age-matched control subjects were selected in this study. The patients had received no treatment before the study was conducted. Statistical Analysis: The data from patients and controls were compared using two-tailed student's t-test and values were expressed as means ± standard deviation (SD). Results: Respiratory complex I + III and IV activities were significantly lower (P <0.001) in patients than in control subjects. Conclusions: The use of lymphocytes for investigating the respiratory chain enzymes provides an easy, noninvasive method to assess mitochondrial function in patients with PD. Furthermore, our study supports the hypothesis that a biochemical defect in the respiratory chain may be involved in the pathogenesis of PD.

Background: Image-guided procedures such as computed tomography (CT) guided, neuronavigator-guided and ultrasound-guided methods can assist neurosurgeons in localizing the intraparenchymal lesion of the brain. However, despite improvements in the imaging techniques, an accurate diagnosis of intrinsic lesion requires tissue sampling and histological verification. Aims: The present study was carried out to examine the reliability of the diagnoses made on tumor sample obtained via different stereotactic and ultrasound-guided brain biopsy procedures. Materials and Methods: A retrospective analysis was conducted of all brain biopsies (frame-based and frameless stereotactic and ultrasound-guided) performed in a single tertiary care neurosciences center between 1995 and 2005. The overall diagnostic accuracy achieved on histopathology and correlation with type of biopsy technique was evaluated. Results: A total of 130 cases were included, which consisted of 82 males and 48 females. Age ranged from 4 to 75 years (mean age 39.5 years). Twenty per cent (27 patients) were in the pediatric age group, while 12% (16 patients) were ³ 60-years of age. A definitive histological diagnosis was established in 109 cases (diagnostic yield 80.2%), which encompassed 101 neoplastic and eight nonneoplastic lesions. Frame-based, frameless stereotactic and ultrasound-guided biopsies were done in 95, 15 and 20 patients respectively. Although the numbers of cases were small there was trend for better yield with frameless image-guided stereotactic biopsy and maximum diagnostic yield was obtained i.e., 87% (13/15) in comparison to conventional frame-based CT-guided stereotactic biopsy and ultrasound-guided biopsy. Conclusions: Overall, a trend of higher diagnostic yield was seen in cases with frameless image-guided stereotactic biopsy. Thus, this small series confirms that frameless neuronavigator-guided stereotactic procedures represent the lesion sufficiently in order to make histopathologic diagnosis.

Background: Monomelic amyotrophy (MMA) is a benign motor neuron disorder, which particularly affects young people and the etiology is still unknown. Gangliosides are located on the outer surface of motor neurons. Anti-GM1 antibodies have been found to be elevated in multi-focal motor neuropathy with conduction block and other neurological diseases, which may have therapeutic implication. Aim: To evaluate IgM anti-GM1 antibody titers in patients of monomelic amyotrophy. Setting and Design: prospective controlled study. Materials and Methods: Forty-six clinically and electrophysiologically diagnosed cases of MMA were assessed for IgM anti-GM1 antibody titers by enzyme-linked immunosorbent assay (ELISA) method and compared with titers in healthy controls, cases of amyotrophic lateral sclerosis (ALS) and acute inflammatory demyelinating polyneuropathy (AIDP). Titer of 800 units was taken as upper limit of normal (Buhlmann Laboratories AG, Switzerland). Statistical Analysis Used: one-way ANOVA. Results: The mean age of 46 patients with MMA was 24.5 (± 7.3) years, with male female ratio of 44:2. The mean age of 19 healthy controls was 24.1 (± 3) years with male: female ratio of 18:1. Five (26%) individuals in the healthy control group, 22 (48%) patients of MMA, four (30%) of ALS and five (50%) of AIDP had high titers of IgM anti-GM1 antibody ( P >0.05). Conclusions: Although larger number of patients with MMA had higher IgM anti-GM1 antibody titers, the difference was not statistically significant from titers of healthy individuals, and of patients in the ALS and AIDP group.

Background: Intracerebral hemorrhage (ICH) results in secondary brain edema and injury that may lead to death and disability. ICH also causes inflammation. It is unclear whether inflammation contributes to brain edema and neuron injury or functions in repairing the brain tissue. Aims: To understand the effect of inflammation in ICH, we have carried out an investigation on the various aspects and the dynamic changes of inflammation. Settings and Design: An ICH model was generated by injecting 50 ml autologous tail artery blood stereotactically into the right caudate nucleus of 30 rats, which were randomly divided into five ICH groups. Similarly, five Sham control groups were generated by inserting the needle to the right caudate nucleus of rats. Materials and Methods: Rat behavior was evaluated over the time course (6 h, 24 h, 48 h, 72 h and 7 d) in each group. The rats were then killed by administering an overdose of pentobarbital. Following the euthanasia, the brain water content, neuronal loss, glia proliferation, inflammatory infiltration and brain morphology of the rats were measured. Additionally, the expression of TNF-a,IL-6, ICAM-1, VEGF, NF-kB, C3 and CR2 was analyzed by immunohistochemistry. Statistical Analysis: The data were analyzed by student's t test. Results: Rat brain water content increased progressively over the time course and reached its peak at 48h followed ICH. The maximum of inflammatory infiltrate (especially neutrophils) and immunopositive cells of TNF-a, IL-6 and NF-kB, were at 48h. The expression of C3 and CR2 reached their peaks at 48-72h, while the expression ICAM-1 and VEGF were at maximum at 72h followed ICH. Conclusions: The results suggested that the inflammatory cytokines, complement system and VEGF may have a function in the development of the brain edema and neuron injury followed ICH.

Context: Behηet's disease (BD) is a multisystem inflammatory disorder with unknown etiology characterized by recurrent oral and genital aphthous ulcers and uveitis. Behηet's disease can affect the central nervous system. Aims: We aimed to investigate subclinical neurological involvement in patients who were suffering from BD and who had no neurological symptoms. Settings and Design: A total of 49 patients were included in the study. For the investigation of subclinical neurological involvement, the patients received imaging and/or neurophysiologic evaluations. Materials and Methods: The evaluation techniques were as follows: single photon emission computed tomography, 33 patients; cranial magnetic resonance imaging (MRI), 25 patients; brainstem auditory evoked potential examination, 36 patients; and electroencephalography (EEG), 30 patients. Statistical Analysis Used: The Mann-Whitney U test and Wilcoxon Rank-Sum W test were used. Results: Patients in the MRI and EEG groups showed significantly more abnormalities than did age- and gender-matched controls. Conclusions: Early diagnosis of neurological involvement in BD is important in reducing or preventing complications. Cranial MRI and EEG were found to be useful for detecting subclinical neurological abnormalities in patients with Behηet's disease.

We report here a case of 18 year old male with tremors of hands, deafness, tendency to fall while walking, drowsiness and double vision of total duration 1½ years. He had internuclear ophthalmoplegia, broken saccades, hypertonia and hyperreflexia of all four limbs, intention tremors, signs of gait and limb ataxia. Pupillary reactions and fundus examination were normal and signs of meningeal irritation or sensory neurological deficit were absent. MRI head and cervical spine with gadolinium enhancement revealed demyelination as evident from multiple oblong foci isointense on T1-weighted images and hyperintense on T2-weighted and fluid attenuated inversion recovery sequences in corpus callosum, sub-cortical white matter, right thalamus, pons and periaqueductal region of midbrain. Ill-defined linear hyperintense signals were observed in cervical spinal cord. No skeletal abnormality was noted in the skull or cervical spine. Oligoclonal bands were present in the cerebrospinal fluid. Brainstem auditory evoked potentials were abnormal, although visual evoked potentials were in normal range. A diagnosis of primary progressive multiple sclerosis (PPMS) was made fulfilling the revised criteria as laid down. In view of its presentation, it is a unique case of PPMS from India.

Global aphasia is an acquired language disorder characterized by severe impairments in all modalities of language. The specific sites of injury commonly include Wernike's and Broca's areas and result from large strokes - particularly those involving the internal carotid or middle cerebral arteries. Rarely, deep subcortical lesions may cause global aphasia. We present three cases with global aphasia due to a more rare cause: left thalamic hemorrhage. Their common feature was the large size of the hemorrhage and its extension to the third ventricule. HMPAO-SPECT in one of the cases revealed ipsilateral subcortical, frontotemporal cortical and right frontal cortical hypoperfusion. Left thalamic hemorrhage should be considered in the differential diagnosis of global aphasia.

We describe a 60-year-old woman with "probable" sporadic Creutzfeldt-Jacob disease (CJD) who manifested with two months history of rapidly progressive dementia and abnormal behavior, speech and gait abnormality, excessive sleepiness and myoclonic jerks. Scalp EEG showed diffuse slowing of background activity to delta range and triphasic sharp wave complexes occurring periodically twice in one-second interval. Magnetic resonance Imaging (MRI) of brain revealed high signal intensity on T2 weighted image (T2WI) and fluid attenuated inversion recovery sequences in the caudate and putamen bilaterally. Diffusion weighted images showed bilateral symmetric hyperintense signals in the caudate and putamen. The role of MRI in the diagnosis of CJD is discussed.

Multiple simultaneous intracerebral hemorrhages (ICH) are uncommon. We report the case of an 80-year-old woman with previous diagnosis of normal pressure hydrocephalus and who was brought to our hospital with altered mental status and urinary incontinence. Medical history of hypertension, hematological disorders or severe head trauma was absent. Platelet count and coagulation profile were unremarkable. An initial head computed tomography (CT) showed sulcal enlargement and ventricular dilatation, but no evidence of ICH. A tap test indicated as a guide to case selection for shunt surgery accidentally resulted in cerebrospinal fluid (CSF) overdrainage. The patient presented sudden neurological deterioration, with sluggishly responsive pupils and generalized tonic-clonic seizures. A new head CT demonstrated multiple supra and infratentorial ICH. The patient became comatose and had a fatal course. Hence, CSF overdrainage may either cause or precipitate multiple simultaneous ICHs, affecting both the infratentorial and supratentorial regions.

Spinal anesthesia is widely used during surgical procedures. It is generally safe and the frequency of severe, permanent neurological complications associated with it has been reported to be extremely low. We report a patient, who developed paraplegia following spinal anesthesia. A 29-year-old male was referred with acute, flaccid, sensory motor paraplegia, with bladder and bowel involvement. He developed this immediately after an operation for inguinal hernia under spinal anesthesia. Spinal magnetic resonance imaging revealed hemorrhagic myelitis in the conus at D12. He was referred after he did not respond to intravenous methylprednisolone for 10 days. This case brings up the difficulty encountered in determination of the interspace used for spinal anesthesia and the potential for traumatic injury to the spinal cord. It also demonstrates the tragic outcome after a clinician violates some important, standard and established guidelines.

Cases of glioblastoma multiforme (GBM) metastasizing to the leptomeninx or the intramedullary spine are quite rare and prognoses are relatively poor. We present three cases of GBM with spinal metastasis, one of which also had leptomeningeal dissemination. Three patients with GBM were admitted to our clinic for postoperative radiotherapy after surgery. Leptomeningeal metastasis and dissemination were diagnosed with magnetic resonance imaging. Radiotherapy provided only temporary relief from pain with small improvement in neurological deficit but no survival advantage.

Symptomatic hemorrhagic transformation is common in supratentorial and cerebellar infarction, but is rare in brainstem infarction. It is seldom reported in basilar artery occlusion. Although early arterial recanalization by thrombolytic agent has became the new trend of treatment, for some neurologists anticoagulant is still a conventional alternative treatment of basilar artery occlusion, especially in longer-existing ischemic deficits. We report a case of massive pontine hemorrhage associated with enoxaparin (low-molecular-weight heparin) treatment for basilar artery occlusion. On the basis of the clinical information and neuroimaging, an embolism was the most likely cause of stroke. The case presented herein adds massive pontine hemorrhagic transformation to the list of possible complications of anticoagulants for basilar artery occlusion. Apart from no evidence-based benefit in treatment of basilar artery occlusion, anticoagulant may contribute to devastating hemorrhagic transformation.

A seven-month-old infant presented with a generalized seizure. The radiological evaluation revealed a large arteriovenous fistula in the left sylvian region. The fistula connected the left middle cerebral artery and the vein of Trolard. A giant varix was present at the venous end. The child underwent craniotomy, complete disconnection of the fistula and excision of the varix. Except for recurrent seizures, which were eventually controlled on anticonvulsants, the child's neurological development has been good on long-term follow-up of three years. Pial arteriovenous fistulae are rare intracranial vascular malformations. Though significant operative risks exist, they can be successfully managed surgically with good long-term prognosis.

A 63-year-old woman presented with an extremely rare oculomotor schwannoma not associated with neurofibromatosis, manifesting as a transient diplopia and ptosis. Magnetic resonance images showed a well-enhanced mass extending from the cavernous sinus to the intraorbital region. Surgical exposure confirmed the tumor originating from the oculomotor nerve in the cavernous sinus. The intraorbital cystic part of the tumor was partially resected to preserve the oculomotor function. This is the first case of oculomotor schwannoma in the cavernous sinus with intraorbital component.

We report two patients of diabetic nonketotic hyperosmolar state presenting acutely with "self-limiting hemichorea - hemiballismus" and "generalized convulsive status epilepticus". CT scan in both the patienta revealed a hyperdense nonenhancing basal ganglia. Magnetic resonance imaging brain of patient 1 showed it to be hyperintense on T1W image and iso-hyper intense on T2W image, minimally enhancing with contrast injection