Interplay between susceptibility genes and environmental factors is considered important player in the genesis of multiple sclerosis (MS). Among environmental factors, a role for an infectious pathogen has long been considered central to the disease process. This opinion has support both from epidemiological data and the findings of immunological abnormalities in spinal fluid that reflect an immune response to an as yet undetermined antigen, possibly a pathogen, in the cerebrospinal fluid. Our review will outline the current understanding of the role of infection in the causation and progression of MS. We will review the data that point to an infectious cause of MS and consider the specific agents Chlamydophila (Chlamydia) pneumoniae, Human Herpes Virus 6, and Epstein-Barr Virus, that are implicated in either the development or progression of MS.

The following article recommends guidelines for epilepsy surgery for India. This article reviews the indications, the various surgical options available and the outcome of surgery for drug resistant epilepsy based on current evidence. Epilepsy surgery is a well-established option for patients who have been diagnosed to have drug resistant epilepsy (DRE) (on at least two appropriate, adequate anti-epileptic drugs (AEDs) (either in monotherapy or in combination) with continuing seizures), where the presurgical work-up has shown concordance of structural imaging (magnetic resonance imaging) and electrical mapping data (electroencephalography (EEG), video EEG). There may be a requirement of functional imaging techniques in a certain number of DRE like positron emission tomography (PET), single photon emission tomography, (SPECT)). Invasive monitoring should be restricted to a few when all noninvasive investigations are inconclusive, there is a dual pathology or there is a discordance of noninvasive data. The types of surgery could be curative (resective surgeries: amygdalo hippocampectomy, lesionectomy and multilobar resections; functional surgeries: hemispherotomy) and palliative (multiple subpial transaction, corpus callosotomy, vagal nerve stimulation). Epilepsy surgery in indicated cases has a success range from 50 to 86% in achieving seizure freedom as compared with <5% success rate with AEDs only in persons with DRE. Centers performing surgery should be categorized into Level I and Level II.

Insomnia is a disorder characterized by inability to sleep or a total lack of sleep, prevalence of which ranges from 10 to 15% among the general population with increased rates seen among older ages, female gender, White population and presence of medical or psychiatric illness. Yet this condition is still under-recognized, under-diagnosed, and under-treated. This article aims to review the operational definitions and management of chronic insomnia. A computerized search on PubMed carried from 1980 to January 2009 led to the summarization of the results. There are several strategies to manage chronic insomnia. To initiate treatment, it is necessary to define it and differentiate it from other co-morbid psychiatric disorders. Non-pharmacologic strategies such as stimulus control therapy and relaxation and cognitive therapies have the best effect sizes followed by sleep restriction, paradoxical intention and sleep hygiene education which have modest to less than modest effect sizes. Among pharmacotherapeutic agents, non-benzodiazepine hypnotics are the first line of management followed by benzodiazepines, amitryptiline and antihistaminics. However, adequate trials of combined behavior therapy and pharmacotherapy are the best course of management.

Psychomotor seizures, referred to as limbic or partial complex seizures, have had an interesting evolution in diagnosis and treatment. Hughlings Jackson was the first to clearly relate the clinical syndrome and likely etiology to lesions in the uncinate region of the medial temporal lobe. With the application of electroencephalography (EEG) to the study of human epilepsy as early as 1934 by Gibbs, Lennox, and Davis in Boston, electrical recordings have significantly advanced the study of epilepsy. In 1937, Gibbs and Lennox proposed the term "psychomotor epilepsy" to describe a characteristic EEG pattern of seizures accompanied by mental, emotional, motor, and autonomic phenomena. Concurrently, typical psychomotor auras and dreamy states were produced by electrical stimulation of medial temporal structures during epilepsy surgery by Penfield in Montreal. In 1937, Jasper joined Penfield, EEG was introduced and negative surgical explorations became less frequent. Nevertheless, Penfield preferred to operate only on space occupying lesions. A milestone in psychomotor seizure diagnosis was in the year 1946 when Gibbs, at the Illinois Neuropsychiatric Institute, Chicago, reported that the patient falling asleep during EEG was a major activator of the psychomotor discharges and electrographic ictal episodes becoming more prominently recorded. Working with Percival Bailey, Gibbs was proactive in applying EEG to define surgical excision of the focus in patients with intractable psychomotor seizures. By early 1950s, the Montreal group began to clearly delineate causative medial temporal lesions such as hippocampal sclerosis and tumors in the production of psychomotor seizures.

Aims: To evaluate the prevalence and characteristics of different neurological and psychiatric presentations in patients admitted to hospital with systemic lupus erythematosus (SLE). Materials and Methods: In this retrospective hospital-based study, we examined the medical records of patients with SLE who were referred to the hospitals affiliated to Shiraz University of Medical Sciences from 1995 to 2005. All patients of SLE who had clinical neurological or psychiatric features were included in this study. The patients' demographic data, findings on general examination, neuropsychiatric manifestations, and the results of laboratory investigations and imaging studies were recorded. Clinical manifestations were classified according to the American College of Rheumatology (ACR) case definitions. Results: Of the 407 study patients, 11.3% had neuropsychiatric complications. The most frequent findings were seizure (63%), headache (60%), and decreased level of consciousness (50%). Cerebrovascular disease (28.3%), seizure disorder (26.5%), and acute confusional state (19.6%) were the most prevalent syndromes. Conclusion: The prevalence and nature of different neurological presentations of SLE in Iranian patients has some similarities to that seen in other populations, as well as some differences. Ethnic and environmental factors may contribute to these differences.

Objective: To describe the clinical, radiological, and cerebrovascular fluid (CSF) findings and the outcome of microbiologically or histopathologically proven fungal infections of the central nervous system (CNS) in HIV-negative patients. Methodology and Results: We identified definite cases of CNS mycosis by screening the medical records of our institute for the period 2000-2008. The clinical and imaging details and the outcome were abstracted from the medical records and entered in a structured proforma. There were 12 patients with CNS mycosis (i.e., 2.7% of all CNS infections treated in this hospital); six (50%) had cryptococcal infection, three (25%) had mucormycosis, and two had unclassified fungal infection. Four (33%) of them had diabetes as a predisposing factor. The common presentations were meningoencephalitis (58%) and polycranial neuritis (41%). Magnetic resonance imaging revealed hydrocephalus in 41% and meningeal enhancement in 25%, as well as some unusual findings such as subdural hematoma in the bulbocervical region, carpeting lesion of the base of the skull, and enhancing lesion in the cerebellopontine angle. The CSF showed pleocytosis (66%), hypoglycorrhachia (83%), and elevated protein levels (100%). The diagnosis was confirmed by meningocortical biopsy (in three cases), paranasal sinus biopsy (in four cases), CSF culture (in three cases), India ink preparation (in four cases), or by cryptococcal polysaccharide antigen test (in three cases). Out of the ten patients for whom follow-up details were available, six patients recovered with antifungal medications (amphotericin B, 1 mg/kg/day for the minimum period of 6 weeks) and/or surgical treatment. Four patients expired (only one of them had received antifungal therapy). Conclusions: Most patients with CNS mycosis recover with appropriate therapy, but the diagnosis and management of these rare infections remains a challenge to clinicians.

Objective: To determine whether learning and serial position effect (SPE) differs qualitatively and quantitatively among different types of dementia and between dementia patients and controls; we also wished to find out whether interference affects it. Materials and Methods: We administered the Malayalam version of the Rey Auditory Verbal Learning Test (RAVLT) to 30 cognitively unimpaired controls and 80 dementia patients [30 with Alzheimer's disease (AD), 30 with vascular dementia (VaD), and 20 with frontotemporal dementia (FTD)] with mild severity on the Clinical Dementia Rating Scale. Results: All groups were comparable on education and age, except the FTD group, who were younger. Qualitatively, the learning pattern and SPE (with primacy and recency being superior to intermediate) was retained in the AD, VaD, and control groups. On SPE in free recall, recency was superior to intermediate in the FTD group (P < 0.01 using Bonferroni correction). On recognition, the AD and VaD groups had more misses (P < 0.01), while the FTD group had more false positives (P < 0.01). Conclusion: Quantitative learning is affected by dementia. The pattern of qualitative learning remains unaltered in dementia in the early stages.

Objectives: To evaluate (1) the prevalence of operationally defined depressive disorder (ICD-10) in chronic stroke subjects and (2) the relationship of post-stroke depression (PSD) with disability. Design: Cross-sectional, descriptive study. Setting: Neurological rehabilitation unit of a tertiary care university research center. Materials and Methods: Participants were those with first episode of supratentorial stroke of more than 3 months' duration with impaired balance and gait who had been referred for rehabilitation. Data were collected on demographic data, stroke data (side and type of lesion and post-stroke duration), cognition (mini mental state examination), depressive ideation (Hamilton Depression Rating Scale - HRDS), impairment (Scandinavian Stroke Scale), balance (Berg Balance Scale), ambulatory status (Functional Ambulation Category), walking ability (speed), and independence in activities of daily living (Barthel Index). Statistical analysis was done using SPSS 13.0. We carried out the chi-square test for ordinal variables and the independent t test for continuous variables. Results: Fifty-one patients (M:F: 41:10) of mean age 46.06 ± 11.19 years and mean post-stroke duration of 467.33 ± 436.39 days) were included in the study. Eighteen of the 51 participants (35.29%) met the criteria for depression. Demographic variables like male gender, being married, living in a nuclear family, urban background, and higher HRDS score were significantly correlated with PSD (P < 0.05). Depression was related to functional disability after stroke but to a statistically insignificant level (P > 0.05) and was unrelated to lesion-related parameters. Conclusion: Depression occurs in one-third of chronic stroke survivors and is prevalent in subjects referred for rehabilitation. PSD is related primarily to demographic variables and only to a lesser extent to functional disability following stroke.

Background: Confirmation of tuberculous meningitis (TBM) and initiation of treatment are often delayed due to limitations in isolating Mycobacterium tuberculosis from cerebrospinal fluid (CSF). Objectives: To evaluate the role of the BACTEC radiometric method in a clinical setting for the early diagnosis of TBM. Materials and Methods: Patients meeting criteria for clinically probable TBM over a 3 year period were included. Clinical features, results of CSF investigations (protein, glucose, cell count, Ziehl-Neelsen staining, culture in Lowenstein-Jensen (LJ) medium and BACTEC) and brain CT imaging were reviewed. Drug sensitivity was tested using BACTEC. Patients were started on standard treatment and functional outcome, and response at discharge and follow-up were assessed. Patients were divided according to whether or not M. tuberculosis was isolated by BACTEC and the clinical, radiological, and laboratory features compared. Results: Sixty patients were evaluated. The mean age was 30 years ΁ 11.7 years. Headache and fever were the most common symptoms and the mean duration was 26 days. CT findings were hydrocephalus (n=21), basal exudates (n=16), and tuberculoma (n=14). In 40 patients, M. tuberculosis was isolated by BACTEC and average 15 days was required for detection, whereas it was 30 days in LJ medium. Results of drug-sensitivity testing (n=32) were obtained average 7 days after isolation. Patients from whom M. tuberculosis had been isolated by BACTEC more often had tuberculomas in CT imaging (P=0.018). Conclusion: Use of the BACTEC method allows early confirmation in patients with clinically probable TBM. It can guide clinicians in the rational use of anti-tuberculosis treatment by confirming diagnosis and identifying drug- sensitivity.

Background: Indian-Asian multiple sclerosis behaves somewhat differently from Western disease. It is not known if the response to β-interferon is also different. Aim: To demonstrate the decrease in relapses with β-interferon in Indian patients with multiple sclerosis. Patients and Methods: Patients with relapsing-remitting or secondary progressive multiple sclerosis with at least two relapses were started on β-interferon. Results: Sixteen patients were followed up for a period of 1-3 years. Fifteen had relapsing-remitting multiple sclerosis (MS). The mean number of relapses in these patients before interferons were started was 3.4. The mean yearly relapse rate was 1.3. The mean Kurtzke Expanded Disability Status Scale (EDSS) at the start of β-interferon therapy in relapsing-remitting MS was 1.7. Ten of these patients were on Avonex^® (interferon β1a) and six (including the patient with secondary progressive MS) were on Betaferon^® (interferon β1b). On follow-up, three patients (two on Avonex^® and one on Betaferon^® ) had relapses. The respective β-interferon being received by these patients was continued, with no further relapses. The remaining patients had no relapse or clinical or MRI progression after starting the drug. The side effect profile of the drug in these patients was favorable; although nearly all developed fever on the first day of the injection, only 50% of the patients continued to have fever after 3 months. Two patients developed psychiatric symptoms, requiring discontinuation of the drug. Conclusion: Our prospective follow-up study shows that β-interferons are safe and effective in Indian patients with relapsing-remitting or secondary progressive MS.

Idiopathic CD4 T-lymphocytopenia (ICL) is a syndrome characterized by depletion of CD4 T-cells without evidence of human immunodeficiency virus (HIV) infection. There are a few reported cases of ICL associated with different diseases and clinical conditions, most commonly the opportunistic infections like Tuberculosis, fungal and parasitic diseases which are also seen in HIV-positive patients. We report a case without risk factors or laboratory evidence of HIV infection who presented with refractory cryptococcal meningitis and was found to have ICL.

Congenital myasthenic syndromes (CMSs) are a heterogeneous group of disorders, characterized by dysfunction of neuromuscular junction (NMJ) transmission. These syndromes are genetically inherited and are present since birth. Some have characteristic clinical or electrodiagnostic features but in many cases determination of the specific form requires genetic studies or specialized morphological and electrophysiological studies on muscle tissue. We report a case of a 4-year-old boy with progressive ptosis and limitation of ocular movements who was diagnosed as slow-channel CMS based on the characteristic electrodiagnostic features. Repetitive compound muscle action potentials (R-CMAPs) were recorded after single nerve stimulus, with decremental response after repetitive trains performed at 3 Hz. CMSs are at times clinically difficult to distinguish from acquired myasthenia. The characteristic clinical and electrodiagnostic features help in the diagnosis and enable rational therapy. In this article we discuss the characteristics of synaptic R-CMAPs.

Familial amyloidotic polyneuropathy (FAN type 1) is a rare systemic disease that causes severe and disabling peripheral neuropathy. We describe the phenotypic, radiological, and pathological characteristics of a patient with familial amyloid polyneuropathy type 1 who had evidence of motor-sensory-autonomic neuropathy, ocular vitreous deposits, diffuse leptomeningeal involvement, and hypertrophic cardiomyopathy. Muscle involvement, an infrequently reported feature, was also observed. Early recognition of the disease has significant therapeutic implications.

Although sodium valproate (VPA)-induced hepatic encephalopathy is a well-recognized entity, VPA can occasionally produce encephalopathy secondary to hyperammonemia in the presence of normal hepatic function, namely valproate-induced non-hepatic hyperammonemic encephalopathy (VNHE). Known risk factors include therapy with multiple antiepileptic drugs, especially when topiramate is one of the drugs; presence of underlying inborn errors of metabolism; febrile states; and insufficient nutritional intake. We describe a 5-year-old male child who developed VNHE while on polypharmacy with topiramate and phenobarbitone; the child also had poor nutritional intake. The encephalopathy reversed with withdrawal of VPA and treatment with L-carnitine. We emphasize the need for early recognition, investigation, and treatment of this potentially life-threatening condition. We also recommend that VPA, topiramate, and phenobarbitone should not be given in combination.