Of the 70 million persons with epilepsy (PWE) worldwide, nearly 12 million PWE are expected to reside in India; which contributes to nearly one-sixth of the global burden. This paper (first of the two part series) provides an in-depth understanding of the epidemiological aspects of epilepsy in India for developing effective public health prevention and control programs. The overall prevalence (3.0-11.9 per 1,000 population) and incidence (0.2-0.6 per 1,000 population per year) data from recent studies in India on general population are comparable to the rates of high-income countries (HICs) despite marked variations in population characteristics and study methodologies. There is a differential distribution of epilepsy among various sociodemographic and economic groups with higher rates reported for the male gender, rural population, and low socioeconomic status. A changing pattern in the age-specific occurrence of epilepsy with preponderance towards the older age group is noticed due to sociodemographic and epidemiological transition. Neuroinfections, neurocysticercosis (NCC), and neurotrauma along with birth injuries have emerged as major risk factors for secondary epilepsy. Despite its varied etiology (unknown and known), majority of the epilepsy are manageable in nature. This paper emphasizes the need for focused and targeted programs based on a life-course perspective and calls for a stronger public health approach based on equity for prevention, control, and management of epilepsy in India.

Contraception is an important choice that offers autonomy to women with regard to prevention of unintended pregnancies. There is wide variation in the contraceptive practices between continents, countries, and societies. The medical eligibility for contraception for sexually active women with epilepsy (WWE) is determined by the type of anti-epileptic drugs (AEDs) that they use. Enzyme inducing AEDs such as phenobarbitone, phenytoin, carbamazepine, and oxcarbazepine increase the metabolism of orally administered estrogen (and progesterone to a lesser extent). Estrogen can increase the metabolism of certain AEDs, such as lamotrigine, leading to cyclical variation in its blood level with resultant adverse effect profile or seizure dyscontrol. AEDs and sex hormones can increase the risk of osteoporosis and fracture in WWE. The potential interactions between AEDs and hormonal contraception need to be discussed with all women in reproductive age-group. The alternate options of oral contraception such as intrauterine copper device, intrauterine levonorgestrel release system, and supplementary protection with barriers need to be presented to them. World Health Organization has recommended to avoid combination contraceptive pills containing estrogen and progesteron in women who desire contraception and in breastfeeding mothers. Care providers need to consider the option of non-enzyme-inducing AEDs while initiating long-term treatment in adolescent and young WWE.

Ipsipulsion is a clinical sign specifically seen in lateral medullary syndrome. It is characterized by two involuntary phenomenons. One is static eye deviation ipsilateral to the side of lesion especially in the absence of visual fixation. Second is the saccadic lateropulsion whereby voluntary saccades towards the side of lesion are hypermetric and saccades towards opposite side are hypometric. The vertical saccades may also appear oblique. Ipsipulsion is produced due to damage to the contralateral olivocerebellar pathways that crosses midline in medulla and pass through the ipsilateral inferior cerebellar peduncle to supply ipsilateral cerebellar hemisphere.

Single small enhancing CT lesions (SSECTL) have been very commonly encountered in clinical practice.These lesions typically are small (often < 20 mm), enhancing as a ring lesion or a disc and with varying amounts of surrounding edema. Most SSECTL present as focal seizures. Once the diagnosis of SSECTL and likely to be a solitary cysticercus lesion is made, the patient is given appropriate AED therapy. Depending on the resolution pattern on repeat imaging performed at intervals not less than six months if patient remains asymptomatic, cysticidal therapy is instituted along with AEDs. Any deviation from the classical clinical or radiological patterns needs further evaluation and other etiologies described for the SSECTL will need to be ruled out, including that of tuberculosis. Largely these lesions resolve and the clinical condition remains benign and in most patients AEDs can be withdrawn in two to three years period.

The systematic study of neuropathology was initiated by Godwin Greenfield in the early part of the 20 ^th century. He worked at the National Hospital, Queen Square, London for the major period of his life and worked on various subjects like cerebrospinal fluid, intracranial tumours, cerebellar ataxias, dystrophia myotonica, disseminated sclerosis, subacute combined degeneration of the spinal cord, and the like. After his retirement he visited the National Institute of Neurological Diseases and Blindness in Bethesda, Maryland, USA, from time to time and there he died suddenly from myocardial infarction.

Background and Purpose: Demonstration of lepra bacilli is essential for definite or unequivocal diagnosis of pure neuritic leprosy (PNL) on nerve biopsy. However, nerves always do not show bacilli owing to the changes of previous therapy or due to low bacillary load in tuberculoid forms. In absence of granuloma or lepra bacilli, other morphologic changes in endoneurium and perineurium can be of help in making a probable diagnosis of PNL and treating the patient with multidrug therapy. Materials and Methods: Forty-six biopsies of PNL were retrospectively reviewed and histologic findings were compared with 25 biopsies of non leprosy neuropathies (NLN) including vasculitic neuropathy and chronic inflammatory demyelinating polyneuropathy (CIDP). The distribution of endoneurial infiltrate and fibrosis, perineurial thickening, and myelin abnormalities were compared between PNL and NLN biopsies and analyzed by Chi-square test. Results: Out of 46 PNL casses, 24 (52.17 %) biopsies were negative for acid fast bacilli (AFB). In these cases, the features which favor a diagnosis of AFB-negative PNL were endoneurial infiltrate (51.1%), endoneurial fibrosis (54.2%), perineurial thickening (70.8%), and reduced number of myelinated nerve fibers (75%). Interpretation and Conclusion: Nerve biopsy is an efficient tool to diagnose PNL and differentiate it from other causes of NLN. In absence of AFB, the diagnosis of PNL is challenging. In this article, we have satisfactorily evaluated the various hisopthological features and found that endoneurial inflammation, dense fibrosis, and reduction in the number of myelinated nerve fibers are strong supportive indicators of PNL regardless of AFB positivity.

Aims: To assess sleep quality in patients with primary headaches before and after prophylactic treatment using a validated sleep-screening instrument. Materials and Methods: A total of 147 patients, including 63 tension type headache (TTH) and 84 migraine patients were included. Patients were examined in terms of frequency and severity of headaches and sleep quality before and 12 weeks after prophylactic treatment with either propranolol or amitriptyline. Results: Baseline Visual Analogue Score (VAS) in migraine patients was 7.99 ± 1.39 compared with 6.86 ± 1.50 in TTH group (P < 0.001). VAS score after the first month of treatment was 6.08 ± 1.88 in migraine patients and 5.40 ± 1.61 in TTH (P = 0.023). VAS scores decreased after the third month of treatment to 4.32 ± 2.29 in migraine patients and 4.11 ± 1.66 in TTH patients (P = 0.344). The decrease was significant for patients treated with amitriptyline but not for those with propranolol. Baseline Pittsburgh Sleep Quality (PSQI) scores were 5.93 ± 2.43 in migraine patients and 6.71 ± 2.39 in TTH patients. Poor quality of sleep (PSQI ≥ 6) prior to prophylactic treatment was observed in 61.4% of migraine patients and in 77.7% of TTH patients. Comparison of PSQI scores before and 3 months following treatment showed significantly improved quality of sleep in all treatment groups; the greatest significance was detected in migraine patients with initial PSQI scores of ≥6 and treated with amitriptyline (P < 0.001). Conclusions: Increased understanding of routine objective sleep measures in migraine patients is needed to clarify the nature of sleep disturbances associated with primary headaches. This may in turn lead to improvements in headache treatments.

Background: Restless legs syndrome also known as Willis-Ekbom's Disease (RLS/WED) is a common illness. Cambridge-Hopkins diagnostic questionnaire for RLS (CHRLSq) is a good diagnostic tool and can be used in the epidemiological studies. However, its Hindi version is not available. Thus, this study was conducted to translate and validate it in the Hindi speaking population. Materials and Methods: After obtaining the permission from the author of the CHRLSq, it was translated into Hindi language by two independent translators. After a series of forward and back translations, the finalized Hindi version was administered to two groups by one of the authors, who were blinded to the clinical diagnosis. First group consisted of RLS/WED patients, where diagnosis was made upon face to face interview and the other group - the control group included subjects with somatic symptoms disorders or exertional myalgia or chronic insomnia. Each group had 30 subjects. Diagnosis made on CHRLSq was compared with the clinical diagnosis. Statistical Analysis: Analysis was done using Statistical Package for Social Sciences (SPSS) v 21.0. Descriptive statistics was calculated. Proportions were compared using chi-square test; whereas, categorical variables were compared using independent sample t-test. Sensitivity, specificity, and positive predictive value of the translated version of questionnaire were calculated. Results: Average age was comparable between the cases and control group (RLS/WED = 39.1 ± 10.1 years vs 36.2 ± 11.4 years in controls; P = 0.29). Women outnumbered men in the RLS/WED group (87% in RLS/WED group vs 57% among controls; χ² = 6.64; P = 0.01). Both the sensitivity and specificity of the translated version was 83.3%. It had the positive predictive value of 86.6%. Conclusion: Hindi version of CHRLSq has positive predictive value of 87% and it can be used to diagnose RLS in Hindi speaking population.

Purpose: We studied the sleep macroarchitecture with polysomnography (PSG) in drug naïve patients with Parkinson's disease (PD) and reassessed them following treatment with levodopa. Materials and Methods: This prospective hospital-based study included 15 patients with PD (age: 59 ± 11.2 years, duration of PD: 11.8 ± 12.3 months; and male: female (M:F) = 11:4). They were assessed for demography, phenotype, modified Hoehn and Yahr staging (H & Y); Schwab and England and Activities of Daily Living (S and E ADL) Scale; and Unified PDRating Scale (UPDRS). Sleep was assessed using Epworth Sleepiness Scale (ESS), Pittsburgh Sleep Quality Index (PSQI), and National Institute of Mental Health and Neurosciences (NIMHANS) comprehensive sleep disorder questionnaire. They underwent overnight PSG at baseline and after13.3 ± 5.7 months of levodopa (440 mg/day). Results: Patients with PD had responded to levodopa as indicated by the significant improvement in UPDRS motor score in ON state compared to OFF state. Nocturnal sleep quality indices did not vary significantly, but the excessive daytime somnolence improved (P = 0.04) with levodopa. Sleep efficiency (P = 0.65), latency to sleep onset (P = 0.19), latency to stage 1 (P = 0.12), and duration of stage 1 (P = 0.55) had increased. Duration of 'awake in bed' (P = 0.24), slow wave sleep (P = 0.29), and rapid eye movement (REM) sleep (P = 0.24) decreased with treatment. Periodic leg movements (PLMs) had reduced (P = 0.68) and mean oxygen saturation during sleep improved (P = 0.002). Surprisingly, snore index (P < 0.03) during sleep had increased with levodopa. Conclusions: Sleep alterations in PD occur even in early stages due to the disease process. There was improvement in most of the parameters of sleep macroarchitecture with levodopa.

Objectives: Very few studies in India have addressed the role of vitamin D in cognitive function. The present study was conducted to assess the serum levels of 25-hydroxyvitamin D (25(OH)D) and its association with markers of cognitive impairment and homocysteine levels in the elderly Indian population. Materials and Methods: The study population consisted of patients with dementia (Group A, n = 32), mild cognitive impairment (MCI; Group B, n = 24), and elderly age-matched controls (Group C, n = 30). Measurement of serum levels of 25(OH)D and total homocysteine were done. Results: Significant decreased concentration of 25(OH)D and increased concentration of homocysteine was observed. Association of serum levels of vitamin D with markers of cognitive decline as well as serum homocysteine levels was observed in patients with dementia and MCI when compared to controls. Conclusion: Correlation of vitamin D with markers of cognitive decline and homocysteine opens a new door for early diagnosis of cognitive impairment.

Background: Leptin, a 16 kDa peptide hormone synthesized and secreted specifically from white adipose cells protects neurons against amyloid β-induced toxicity, by increasing Apolipoprotein E (APO E)-dependent uptake of β amyloid into the cells, thereby, protect individuals from developing Alzheimer's disease (AD). The APO E ε4 allele is a known genetic risk factor for AD by accelerating onset. It is estimated that the lifetime risk of developing AD increases to 29% for carriers with one ε4 allele and 9% for those with no ε4 allele. Objectives: To determine the levels of serum leptin, cholesterol, low density lipoprotein (LDL-C), and high density lipoprotein (HDL-C) in the diagnosed cases of AD and the association of them with cognitive decline and Apolipoprotein E (APO E) genotypes in AD. Materials and Methods: Serum levels of serum leptin, cholesterol, LDL-C, and HDL-C along with APO E polymorphism were studied in 39 subjects with probable AD and 42 cognitive normal individuals. Results: AD group showed significantly lower levels of leptin (P = 0.00) as compared to control group. However, there was no significant difference in cholesterol, triglycerides, LDL-C, and HDL-C levels in AD and control groups. The frequency of ε4 allele in AD (38.5%) was found to be significantly higher than in control (10.3%). ε3 allele was more frequent than ε4 allele in AD and control group.

Pediatric chronic inflammatory demyelinating polyneuropathy (CIDP) is an uncommon acquired disorder of unknown cause, presumed to have an immunological basis. We report 20 patients seen at Children's Hospital Los Angeles over a period of 10 years. The outcome of our patients was favorable in a vast majority with good response to various treatments instituted. However, residual neurologic deficit was common. The choice of treatment modality was empirical and selected by the treating neurologist. Intravenous immunoglobulin (IVIG) and corticosteroids were most commonly utilized for treatment. Plasmapheresis, mycophenolate mofetil, rituximab, cyclophosphamide, azathioprine, and abatacept were added if the patients were refractory to IVIG or became corticosteroid dependent. The spectrum of disease severity ranged from a single monophasic episode, to multiphasic with infrequent relapses with good response to IVIG, to progressive disease refractory to multiple therapies.

Muscle involvement is a common manifestation of both clinical and subclinical hypothyroidism, with serum creatine kinase (CK) elevation being probably the most common manifestation, and is seen in up to 90% of patients, but is usually mild (less than 10 times the upper limit of normal). Rhabdomyolysis is a distinctively uncommon presentation of hypothyroidism described usually in the setting of precipitating events such as strenuous exercise, alcohol, or statin use. Rarely rhabdomyolysis and myoedema seen in hypothyroidism can be complicated by the development of anterior compartment syndrome leading to neurovascular compression. We describe a case of a patient with hypothyroidism who developed acute onset bilateral foot drop on initiation of statins. This case highlights the need for cautious use of statins in patients at risk for rhabdomyolysis.

"Eight-and-a-half" syndrome is a rare condition involving the ipsilateral abducens nucleus or paramedian pontine reticular formation (PPRF), the ipsilateral medial longitudinal fasciculus (MLF), and the adjacent facial colliculus/facial nerve fascicle. The condition is often caused by a lesion (vascular or demyelinating) in the dorsal tegmentum of the caudal pons. There are new variants of this syndrome caused by extension of lesion to involve new adjacent structures in pontine tegmentum. We report two patients with different etiology presenting with clinical features suggestive of eight-and-a-half syndrome associated with hemiataxia representing "nine" syndrome (8– + – = 9) adding new dimension to "eight-and-a-half" syndrome.

Erdheim Chester disease (ECD) is a rare non-Langerhans cell histiocytosis, commonly involving the musculoskeletal system. Other tissue can also be involved, including the central nervous system with wide spectrum of clinical features, at times being nonspecific. This can cause diagnostic dilemmas with delay in diagnosis and initiation of therapy. Here we describe a 63-year-old man who had presented with ataxia and behavioral changes, bony pains, weight loss, and fatigue. His computed tomography (CT), 99Tc scintigraphy and histopathological features on bone biopsy were consistent with ECD. Thus, ECD should be considered as a differential diagnosis in patients presenting with bony pain and nonspecific features of multiorgan involvement.

Congenital adrenal hyperplasia (CAH) is characterized by adrenal steroid biosynthesis defect. Steroid replacement therapy should be performed regularly in these patients. Adrenal crisis may be present in acute stress due to increased cortisol requirements or in steroid deficiency due to stopping steroid medication abruptly. In patients with acute adrenal insufficiency, severe hypotension or hypovolemic shock occurs typically. Acute encephalopathy can be seen due to hypoxia, hypervolemia, or hypoglycemia. Diffusion restriction can be seen in cortical-subcortical regions of frontal and parieto-occipital lobes and in splenium of corpus callosum. In CAH patients with neurologic symptoms, Diffusion weighted images (DWI) is very important in the diagnosis and follow-up of acute encephalopathy.

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described inflammatory disease of central nervous system with distinct clinical and radiological features. The etiopathogenesis of this rare entity remains to be understood. The histopathological findings closely resemble chronic inflammatory diseases like sarcoidosis and malignancies like lymphoma. With advancements in serology, immunopathology and radiology CLIPPERS is identified as a distinct entity that differs considerably in its clinical presentation, immunopathology, radiological findings and response to steroids. We describe a case that presented to us with progressive quadriparesis and lower cranial nerve deficits whose radiological and pathological findings were consistent with CLIPPERS. The patient had a good outcome with long term immunosuppression.

Snake bites are very common in India, particularly in West Bengal. Snake bite can cause various hematological, neuromyopathical complications. It can be very fatal if not detected and treated early. Timely intervention can save the patient. We are reporting a case of hematotoxic Russell viper snake bite presented with subarachnoid hemorrhage. Patient was successfully treated with antivenom serum (AVS) along with other conservative management. Subarachnoid hemorrhage as an initial presentation in viper bite is very rare and we discuss the case with proper literature review.

Conjunctival bleeding although occurs in many pathological conditions enlisting diseases like purulent conjunctivitis, infection with epstian bar virus, and accidental damage to conjunctiva. We report here a rare case of bloody tears which was accompanied with severe headache in a12-years school-going girl.

Subclinical rhythmic electrographic discharge of adults (SREDA) is an EEG pattern seen in normal individuals and others with different diseases. we report a case of healthy young woman with alleged epilepsy but normal responsiveness during sustained SREDA. SREDA is a rare EEG variant with variable clinical significance. This is the first report of atypical SREDA in a 25 year-old woman.