Human rabies still continues to be a significant health problem in India and other developing countries where dogs are the major vectors of transmission. Rabies in humans can present in two clinical forms, i.e., furious and paralytic. While diagnosis of furious rabies can be made based on the typical symptoms and signs, paralytic rabies poses a diagnostic dilemma to the neurologists who may encounter these cases in their practice. Although there are certain clinical features that distinguish this disease from other forms of Guillain-Barre syndromes, confirmation of diagnosis may require laboratory assistance. Conventional techniques such as antigen detection, antibody assays and virus isolation have limited success. The recently introduced molecular techniques show more promise in confirming the cases of paralytic rabies. There has not been much success in the treatment of confirmed rabies cases and recovery from rabies is extremely rare. Therefore, preventive measures of this dreaded disease after an exposure become extremely important. The present article reviews the current status of human rabies with regard to antemortem diagnosis, disease management and post-exposure prophylaxis.

This paper discusses the effects of curcumin on patients with Alzheimer's disease (AD). Curcumin (Turmeric), an ancient Indian herb used in curry powder, has been extensively studied in modern medicine and Indian systems of medicine for the treatment of various medical conditions, including cystic fibrosis, haemorrhoids, gastric ulcer, colon cancer, breast cancer, atherosclerosis, liver diseases and arthritis. It has been used in various types of treatments for dementia and traumatic brain injury. Curcumin also has a potential role in the prevention and treatment of AD. Curcumin as an antioxidant, anti-inflammatory and lipophilic action improves the cognitive functions in patients with AD. A growing body of evidence indicates that oxidative stress, free radicals, beta amyloid, cerebral deregulation caused by bio-metal toxicity and abnormal inflammatory reactions contribute to the key event in Alzheimer's disease pathology. Due to various effects of curcumin, such as decreased Beta-amyloid plaques, delayed degradation of neurons, metal-chelation, anti-inflammatory, antioxidant and decreased microglia formation, the overall memory in patients with AD has improved. This paper reviews the various mechanisms of actions of curcumin in AD and pathology.

Myotonic syndromes and periodic paralyses are rare disorders of skeletal muscle characterized mainly by muscle stiffness or episodic attacks of weakness. Familial forms are caused by mutation in genes coding for skeletal muscle voltage ionic channels. Familial periodic paralysis and nondystrophic myotonias are disorders of skeletal muscle excitability caused by mutations in genes coding for voltage-gated ion channels. These diseases are characterized by episodic failure of motor activity due to muscle weakness (paralysis) or stiffness (myotonia). Clinical studies have identified two forms of periodic paralyses: hypokalemic periodic paralysis (hypoKPP) and hyperkalemic periodic paralysis (hyperKPP), based on changes in serum potassium levels during the attacks, and three distinct forms of myotonias: paramyotonia congenita (PC), potassium-aggravated myotonia (PAM), and myotonia congenita (MC). PC and PAM have been linked to missense mutations in the SCN4A gene, which encodes α subunit of the voltage-gated sodium channel, whereas MC is caused by mutations in the chloride channel gene (CLCN1). Exercise is known to trigger, aggravate, or relieve symptoms. Therefore, exercise can be used as a functional test in electromyography to improve the diagnosis of these muscle disorders. Abnormal changes in the compound muscle action potential can be disclosed using different exercise tests. Five electromyographic (EMG) patterns (I-V) that may be used in clinical practice as guides for molecular diagnosis are discussed.

Assessment of Quality of Life in Epilepsy has currently been emphasized to provide comprehensive care to patients. Aim: To develop and standardize and assess the psychometric properties of Bengali version of QOLIE-10 and to assess the relationship of quality of life with seizure variables and presence of psychiatric morbidity. Design: English QOLIE-10 was translated into Bengali by a translation committee using translation-re-translation technique. Inter-rater reliability between the English and Bengali version was assessed during initial practice session held amongst 20 bilingual patients. It was found that item 3 (related to driving) was reported to have difficulty in answering by all the patients as none drove any vehicle. Thus, this item was dropped. The inter-rater reliability of the resultant 9 item scale was found to be high (kappa = 0.9). One hundred and seven epilepsy patients attending the Epilepsy clinic were selected for the study if they met the following criteria: age >15 years, duration of seizure >1 year, regular intake of antiepileptic drugs, presence of informant and ability to read Bengali. For each patient, demographic and clinical data (seizure frequency, last seizure date, seizure type as per record, medicine intake history and records of past investigations such as EEG) was collected. Each patient were administered QOLIE-9 (Bengali) and SRQ-24 Bengali version to screen for psychiatric morbidity. Results: The Cronbach's Alpha coefficient for QOLIE-9 was 0.81, which did not improve if any item was dropped. All items showed strong correlation with the total score. The instrument showed stable factor structure with three factors (Limitation, Depression, Illness effects). However, the item with regard to memory problem did not fit into any of the factors. The QOLIE-9 total showed a significant correlation with the seizure frequency (r = 0.76**). SRQ positive (i.e., suspected psychiatric morbidity) cases had higher QOLIE-9 score (thus, poorer quality of life) in comparison to non-psychiatric cases. Conclusion: Bengali QOLIE-9 is a valid and reliable instrument to assess the quality of life in patients suffering from epilepsy.

Objective: To optimize the use of phenobarbital and/or phenytoin as frontline drugs for treatment of childhood epilepsy. Design: Before-and -after study. Setting: Epilepsy clinic at paediatric OPD, Sassoon General Hospital, Pune. Materials and Methods: Epilepsy is a condition in which seizures are triggered recurrently from within the brain. For epidemiological classification purpose epilepsy is considered to be present when two or more unprovoked seizures occur at an interval greater than twenty four hours apart. Seizures were classified as generalized and partial seizures, with underlying etiology investigated with EEG, CT scan in majority of the patients. Follow - up rate, seizure - control and antiepileptic drugs used among 151 children enrolled as on 31 March 2005 were compared with 106 children with new onset epilepsy enrolled as on February 2006. Eight children with breakthrough convulsion after a seizure free period of five to eighteen months were followed up after injection vitamin D. Nineteen children with poor control of seizures receiving polytherapy with newer antiepileptic drugs were assessed with frontline antiepileptic medication of phenobarbital and/or phenytoin. Serum calcium, phosphorus, alkaline phosphatase were done in seventy two consecutive children with seizure disorder. Results: During post protocol period good seizure control was achieved in 84.8% as against 80.7% and use of phenobarbital and/or phenytoin increased to 65.11% from 22.87%. Of the 8 cases with breakthrough seizures seven remained seizure free after vitamin D administration and with no dose enhancement of AED medications of the nineteen. Children receiving polytherapy thirteen children could be successfully switched to phenobarbital and/or phenytoin. Forty four (61%) children had hypocalcemia (less than 9 mg%), fifty seven (79%) children had raised alkaline phosphatase levels (more than 270 IU). Comments: Phenobarbital and/or phenytoin have been found to be effective frontline AED. Periodic administration of vitamin D plays a supportive role.

Background: Assessment of Quality of life (QoL) is fast assuming significance as the measure of health in many disorders. Aim: To correlate clinical severity and QoL in patients with Wilson's disease (WD). Materials and Methods: We evaluated patients of WD on regular follow up for at least two years and aged over 18 years using Neurological Symptom Score (NSS) for clinical severity and WHO-BREF for QoL at a university teaching hospital. Patients with inability to respond to the questionnaire due to behavioral problems, low IQ or other disease related factors were excluded. These 30 patients (M:F:: 23:7) had a mean age of 27.97 ± 11.16 years at evaluation and the mean duration of treatment of 9.2 ± 6.4 years. Results: All four domains of WHO-QoL-BREF viz., Physical, Psychological, Social and Environmental correlated well with each other ( p < 0.01). The NSS correlated inversely with the physical domain ( p < 0.02), while the duration of treatment had a positive correlation with the physical domain ( p < 0.01). None of the other features of QoL showed any significant correlation with age, NSS or duration of treatment. Conclusion: QoL is complementary to formal neurological assessment and should be routinely incorporated in the evaluation of outcome of patients with WD and other chronic neurological disorders.

Background: Lipid peroxidation is an indicator of free radical metabolism and oxidative stress in human beings and other organisms. Malondialdehyde (MDA), an end product of lipid peroxidation, is a metabolite that can be readily estimated in serum samples. Excess oxidative stress may be a final common pathway through which anti epileptic drugs may exert their teratogenic potential in pregnant women with epilepsy. Our objective in this study was to ascertain the variations in malondialdehyde (MDA) in women with epilepsy. Material and Methods: This study was carried out in the Kerala Registry of Epilepsy and pregnancy after obtaining clearance from the Institutional Ethics Committee. Informed consent was obtained from all the subjects. The quantitative examination of MDA was performed according to standard procedures. The ideal plasma level of MDA is below 2 nmol/ml. Results: Fifteen women with confirmed epilepsy (mean age 26.9 ± 3.5) were included in the study. Two women were pregnant. MDA levels ranged from 1.7 to 2.8 nmol/ml (mean level = 2.13 ± 0.37 nmol/ml). Eight women (53 %) had MDA levels above the upper limit of normal. Three patients had levels above 2.5 nmol/ml, which corresponded to the 75 centile. Conclusions: This study had shown that the estimation of MDA levels in plasma is a convenient method to study lipid peroxidation and thereby oxidative stress in women with epilepsy. Over half of Women With Epilepsy (WWE) have excess oxidative stress as indicated by high levels of MDA in the plasma. Correlations between MDA level and characteristics of epilepsy, AED therapy, nutritional status and other medical conditions need to be observed in a larger cohort.

The syndrome of central diabetes insipidus (cDI) and spastic cerebellar ataxia is rare with only a few reports in the literature. We report the case of a 21-year-old patient who was diagnosed to have central diabetes insipidus at the age of 7 years and presented to us at the age of 21 years with progressive spastic cerebellar ataxia that evolved over four years. His MRI showed normal hyperintense signal from the posterior pituitary. The persistence of posterior pituitary signal in patients with cDI is unusual and is observed in the familial variety of cDI, the possible etiology in our patient. A brief review of the literature on the rare syndromic association of cerebellar ataxia and cDI has been discussed.

The authors report a family from Punjab (India) with 10 members having benign familial neonatal convulsions (also known as benign familial neonatal seizures) in two generations. This disorder is quite rare. The clinical presentation of index case along with the findings of computed tomography of the brain and electroencephalograph is described. Important features of all the family members along with a brief review of the literature are also given.

Baller Gerold Syndrome (BGS) is a rare autosomal recessive disorder that is apparent at birth. The disorder is characterized by distinctive malformations of the skull and facial area and bones of the forearms and hands. We are reporting a new case of BGS in a 10-month-old female child born of an epileptic mother who was on sodium valproate during the initial months of pregnancy. The baby was born with premature closure of the metopic suture, unilateral radial aplasia with limb malformation and other congenital anomalies that conformed with the description of BGS. The parents and other family members were unaffected, karyotyping was normal and there was no history of consanguinity. Fetal valproate exposure has been previously reported as the cause of this fetal malformation syndrome, which is generally inherited as an autosomal recessive trait. The peculiar pregnancy history and the supporting literature on the effects of valproic acid on the fetus exposed in utero to it with numerous case reports in the literature referring to BGS as a result of fetal exposure to valproate made us conclude that this is indeed a case of BGS secondary to valproate-induced teratogenesis.