There is an increasing awareness for recognition of sleep disorders in India; however, there is still a huge gap in the number of people suffering from various sleep disorders, in the community versus those visiting hospital clinics for the same. Ours is a neurology services-based sleep disorders clinic, which has evolved successfully over the last decade. In this study, we aimed to evaluate the changes in referral patterns and distribution of various sleep disorders in the patients presenting to the clinic. Materials and Methods: This is a retrospective chart review-based study on all patients seen over an 8-year period, divided into 2 groups comprising of patients seen during the first 4 years versus those seen over the next 4 years. Only those patients who had the sleep disorder as their presenting manifestation and those who had been formally interviewed with a pre-structured questionnaire detailing about the main features of the common sleep disorders according to the ICSD-R were included. Patients, in whom the sleep disorder could be clearly attributable to another neurological or systemic disorder, were excluded. Statistical analysis was carried out to identify the differences between the two groups as regards the distribution of various sleep disorders and other clinical data. Results: Among 710 patients registered in the clinic, 469 were included for analysis and 222 patients formed group 1 while 247 formed group 2. The main differences observed were in the form of a clear increase in the percentage of patients with sleep-related breathing disorders, sleep-related movement disorder, and the hypersomnias on comparison of distribution over the first 4 years versus the last 4 years; while a clear decline was seen in the number of patients with insomnia and parasomnias. A 3-fold increase was observed in the number of patients in whom polysomnography was obtained. Conclusion: The distribution of various sleep disorders as seen in a neurology service-based sleep clinic is demonstrated in this study. Increasing referrals for sleep-disordered breathing, restless legs syndrome, and fewer referrals for insomnia and parasomnias might reflect on changing physician and patient awareness in our community.

Background: Obstructive sleep apnoea (OSA) syndrome is an established and modifiable but under recognized risk factor for common disorders like stroke and hypertension. Objective: To assess awareness level of health care practitioners and medical students about OSA as a risk factor for stroke and hypertension. Methods: Questionnaire based survey with multiple response type and fill in the blanks type questions. The data was compiled and analyzed using SPSS version 19. Results: 180 participants completed the survey questionnaire. Only 24 (13.3%) identified OSA as a reversible risk factor for ischemic stroke. 11 (6%) participants only could answer OSA as an identified risk factor for hypertension as per Seventh Joint National Committee report. Poor awareness extended over all categories of participants (medical students, trained doctors and nursing staff) . Conclusion: This study reveals dismal level of awareness, among health professionals and medical students, about OSA being an established and modifiable risk factor for hypertension and ischemic stroke.

Central sleep apnea is a period of at least 10 s without airflow, during which no ventilatory effort is present. Most of the central apneas occur in Non-Rapid eye movement (NREM) sleep. Central apnea occuring in Rapid eye movement (REM) sleep is extremely rare. We present our patient who had a diagnosis of obstructive sleep apnea in another sleep center since 2003. His Auto Continuous Positive Airway Pressure (CPAP) machine was disrupted so he admitted to our center to renew his machine and for daytime sleepiness while using his machine. The polysomnography revealed central apneas ending with respiratory arousals and periodic leg movements in rapid eye movement (REM) stage. We found no cause for central apneas. The patient benefited from servo ventilator therapy. We present this case as an unusual form of central apnea with the review of the literatures. Even the patients diagnosed as obstructive sleep apnea should be analyzed carefully. The diagnosis and the therapeutic approach may change in the favor of the patient.

Aims: To evaluate clinical profile and short-term outcome of psychogenic non-epileptic seizures (PNES) in Indian adult population. Setting and Design: A prospective observational study, conducted at tertiary teaching institute at New Delhi. Materials and Methods: Sixty-three patients with confirmed PNES were enrolled. The diagnosis was based on witnessing the event during video-electroencephalography (Video-EEG) monitoring. A detailed clinical evaluation was done including evaluation for coexistent anxiety or depressive disorders. Patients were divided into two groups on the basis of excessive or paucity of movements during PNES attacks. Patients were followed-up to 12 months for their PNES frequency. Statistical Analysis: Means and standard deviations were calculated for continuous variables. Chi-square and Students t-test were used to compare categorical and continuous variables respectively. Results: The mean age at onset of PNES was 25.44 years; with F:M ratio of 9.5:1. Coexistent epilepsy was present in 13 (20.63%) cases. Twenty-two patients (44%) with only PNES ( n = 50) had received antiepileptic drugs. Out of 63 patients of PNES 24 (38.1%) had predominant motor phenomenon, whereas 39 (61.9%) had limp attacks. The common features observed were pre-ictal headache, ictal eye closure, jaw clenching, resistant behavior, ictal weeping, ictal vocalization, and unresponsiveness during episodes. Comorbid anxiety and depressive disorders was seen in 62.3% and 90.16% patients, respectively. Short-term (6-12 months) outcome of 45 patients was good (seizure freedom in 46.66% and >50% improvement in 24.44% cases). Conclusion: PNES is common, but frequently misdiagnosed and treated as epileptic seizures. A high index of suspicion is required for an early diagnosis. Proper disclosure of diagnosis and management of the psychiatric comorbidities can improve their outcome. Limitation: Limited sample size and change in seizures frequency as the only parameter for the assessment of the outcome are the two major limitations of our study.

Purpose: We studied the phenotype and electroencephalographic (EEG) features, and therapeutic aspects of idiopathic generalized epilepsies (IGEs) in South Indian population. Patients and Methods: This prospective cross-sectional hospital-based study was carried out on non-consecutive 287 patients (age 22.2 ± 7.7 years; M:F = 139:148) with IGE syndrome. Their clinical and EEG observations were analyzed. Results: Majority of the patients had onset of seizures <20 years of age (n = 178; 62%). Thirty one patients (10.8%) had family history of epilepsy. Nearly half of them (49.9%) had <5 years of duration of seizures. The type of IGEs included Juvenile myoclonic epilepsy (JME): 115 (40.1%); IGE with generalized tonic-clonic seizures (GTCS) only: 102 (39.02%); childhood absence epilepsy (CAE): 35 (12.2%); GTCS on awakening: 15 (5.2%); Juvenile absence epilepsy (JAE): 11 (3.8%); and unclassified seizures: 9 (3.1%). The triggering factors noted in 45% were sleep deprivation (20%), non-compliance and stress in 5% each. The EEG (n = 280) showed epileptiform discharges in about 50% of patients. Epileptiform discharges during activation was observed in 40/249 patients (16.1%): Hyperventilation in 32 (12.8%) and photic stimulation in 19 (7.6%). The seizures were well controlled with anti-epileptic drugs (AEDs) in 232 (80.8%) patients and among them, 225 (78.4%) patients were on monotherapy. Valproate (n = 131) was the most frequently prescribed as monotherapy. Conclusions: This is one of the largest cohort of patients with IGE. This study reiterates the importance of segregating IGE syndrome and such analysis will aid to the current understanding and management.

Anti N Methyl D Aspartate receptor immune encephalitis (Anti NMDARE) is a recently defined, under-recognized and often misdiagnosed disease, which typically occurs in young females and may be associated with an underlying tumor, usually ovarian teratoma. If diagnosed early, initiation of immunotherapy and tumor removal (if present) may result in recovery. We report a case of a 17 years old girl with Anti NMDARE who was initially misdiagnosed as Functional psychosis, Neuroleptic Malignant Syndrome and Sepsis syndrome. To the best of our knowledge, this is only the second case of anti NMDARE being reported from India. This case report underscores the need for a greater awareness of this entity across multiple specialties, e.g., general medicine, psychiatry and neurology, to ensure a heightened diagnostic suspicion, which can lead to timely diagnosis and adequate therapy of this treatable disease.

Fast micrographia is a rare clinical sign, which is reported in patients with pallidal pathology. A 68-year-old male presented with hypophonia and short shuffling gait with decreased arm swing. About 3 weeks before, he had an acute myocardial infarction and a period of hemodynamic and respiratory distress during which he required mechanical ventilatory support. He was found to have a fast handwriting with micrographia from the outset. His rapid alternating hand and finger movements were normal. Magnetic resonance imaging (MRI) of the brain showed features of hypoxic ischemic encephalopathy including hyperintensities on T1 and T2 weighted images in the globus pallidus, and putamen bilaterally.

Transcranial Doppler (TCD) can be aptly called as the doctor's stethoscope of the brain. Since its introduction in 1982, by Rune Aaslid, TCD has evolved as a diagnostic, monitoring, and therapeutic tool. During evaluation of patients with acute ischemic stroke, TCD combined with cervical duplex ultrasonography provides physiological information on the cerebral hemodynamics, which is often complementary to structural imaging. Currently, TCD is the only diagnostic tool that can provide real time information about cerebral hemodynamics and can detect embolization to the cerebral vessels. TCD is a noninvasive, cost-effective, and bedside tool for obtaining information regarding the collateral flow across various branches of the circle of Willis in patients with cerebrovascular disorders. Advanced applications of TCD help in the detection of right-to-left shunts, vasomotor reactivity, diagnosis, and monitoring of vasospasm in subarachnoid hemorrhage and as a supplementary test for confirmation of brain death. This article describes the basic ultrasound physics pertaining to TCD insonation methods, for detecting the flow in intracranial vessels in addition to the normal and abnormal spectral flow patterns.

Background and Purpose: The purpose of this study was to assess the effectiveness of modified constraint induced movement therapy (m-CIMT) in stroke subjects. Materials and Methods: A total of forty sub-acute stroke subjects were randomly assigned to either a m-CIMT (n = 20) or in a control group (n = 20). The m-CIMT group (14 men, 6 women; mean age = 55.2 years) consisted of structured 2 h therapy sessions emphasizing affected arm use, occurring 5 times/week for 2 weeks. A mitt was used to restrain the unaffected arm for 10 h/day for 2 week. The control group (11 men, 9 women; mean age = 56.4 years) consisted of conventional rehabilitation for time-matched exercise program. The outcome measures were evaluated at pre- and post-intervention by using the Wolf Motor Function Test (WMFT) and Fugl-Meyer assessment (FMA) of motor recovery after stroke. Results: After intervention significant effects were observed in m-CIMT group on WMFT (pre-test and post-test score was 28.04 ± 6.58, 13.59 ± 2.86; P =0.003). Similarly on FMA (pre- and post-test score was 31.15 ± 6.37, 55.7 ± 6.4; P = 0.00). Conclusion: There is a significant improvem ent in upper extremity function so it indicates that m-CIMT is effective in improving the motor function of the affected arm in stroke subjects. However, its long-term effect has not proved since there was no follow-up after intervention.

Context: Glycated hemoglobin A ₁ c (HbA ₁ c) indicates long-term uncontrolled hyperglycemia in the body, which in diabetic patients leads to various vascular complications as a part of generalized atherosclerosis culminating ultimately into ischemic stroke. Aims: Study aims to show the association between marker of uncontrolled long-term hyperglycemia HbA ₁ c and marker of atherosclerosis (Carotid intima media thickness [CIMT] and carotid plaque) in ischemic stroke patients. Subjects and Methods: Carotid sonography using high resolution 7.5 MHz sonography technique was done in each patient to find the occurrence of increased CIMT and presence of plaque according to Mannheim CIMT Consensus (2004-2006). Levels of HbA ₁ c measured in blood in both diabetic and non-diabetic patients and a comparison made between them. Finally an association sought between HbA ₁ c levels with CIMT and plaque. Results: The average value of HbA ₁ c of this cohort was 7.51 ± 1.75% with higher values in diabetic patients (9.29 ± 1.73%). The patients with high CIMT (>0.8 mm) had higher values of HbA ₁ c then that of normal CIMT patients and this was nearly significantly (P = 0.06). However, HbA ₁ c levels of blood were significantly associated with stroke patients with presence of carotid arteries plaque (P = 0.008). Conclusions: Prediction of future risk and prevention strategies for ischemic stroke could be formulated by utilizing HbA ₁ c levels in both diabetic and non-diabetic population.

Spinal cord infarction (SCI) often remains undiagnosed due to infrequent occurrence and lack of established diagnostic procedures. The unique pattern of blood supply explains the heterogeneity of clinical presentation. We present three cases of SCI to highlight the varied spectrum of clinicoradiological findings. The first patient had posterior spinal artery infarction, and spine imaging showed infarction of adjacent vertebral body, which is usually rare. The second patient had anterior spinal artery infarction and the cANCA titers were elevated. The third patient had a pure motor quadriparesis. Initial imaging did not show any cord infarction, but signal changes were noted on serial imaging. Fibrocartilagenous embolism (FCE) seems the most likely etiology in the first and third cases. A high index of clinical suspicion is necessary for prompt diagnosis. Sensitivity of the initial magnetic resonance imaging remains limited, necessitating serial follow-up scans. Infarction of the adjacent vertebral body is a useful confirmatory sign. Fat suppression images can delineate the marrow signal changes better. Elderly patients with vascular risk factors and degenerative discs need to avoid mechanical triggers that predispose to FCE. Younger patients with SCI will need evaluation for cardioembolic source and vasculitis.

Analgesia and sedation has been widely used in intensive care units where iatrogenic discomfort often complicates patient management. In neurological patients maximal comfort without diminishing patient responsiveness is desirable. In these patients successful management of sedation and analgesia incorporates a patient based approach that includes detection and management of predisposing and causative factors, including delirium, monitoring using sedation scales, proper medication selection, emphasis on analgesia based drugs and incorporation of protocols or algorithms. So, to optimize care clinician should be familiar with the pharmacokinetic and pharmacodynamic variables that can affect the safety and efficacy of analgesics and sedatives.

Background: The present understanding of the clinical course, complications, and outcome of myasthenic crisis (MC) is based chiefly on observational studies and retrospective case series. Aim: To study the baseline demographic and clinical variables, risk factors, complications, outcome, and mortality in patients of MC. Materials and Methods: All patients of myasthenia gravis (MG) who presented with myasthenic crisis between July 2009 and December 2010 were included. Results: Ten patients of MC were included in this study. The median age of the patients was 40.5 years (range 14-71 years). Seven were females and three were males. Nine had generalized MG and one patient had oculobulbar involvement only. Median duration of disease was 3 years (range 1 month to17 years). Two patients had thymoma. Two patients had history of thymectomy in the past. Infection was the most common triggering factor accounting for five cases (50%) followed by inadequate treatment/drug withdrawal in three (30%) and steroid initiation and hypokalemia in the remaining two patients (20%). Median duration of MC was 12 days (range 3-28 days). Mortality was in 3 out of 10 (30%) during MC. Management in the intensive care unit (ICU) and treatment with plasma exchange/intravenous immunoglobulins were associated with good outcome. Conclusions: Ventilator support and management in intensive care unit are the most important components in the management of MC. The high mortality rate seen in present study may be more reflective of the actual ground reality in resource constrained developing countries, however, larger prospective studies are needed to confirm these findings.

Congenital myasthenic syndromes (CMS) are frequently misdiagnosed due to their wide clinical heterogeneity. Molecular defects in various end-plate associated proteins are being identified. Better understanding of the molecular pathogenesis and genotype-phenotype correlations can help evolve newer therapeutic targets. We present a report of two siblings with familial limb girdle myasthenia who showed significant objective clinical improvement after initiation of terbutaline. The possible mechanism of action and utility of terbutaline in the setting of CMS are described. Terbutaline is a potential treatment option in certain subtypes of CMS refractory to conventional medicines. However, long-term follow-up is required to determine the overall efficacy and safety profile.

Background: Acute hypokalemic paralysis, characterized by acute flaccid paralysis is primarily a calcium channelopathy, but secondary causes like renal tubular acidosis (RTA), thyrotoxic periodic paralysis (TPP), primary hyperaldosteronism, Gitelman's syndrome are also frequent. Objective: To study the etiology, varied presentations, and outcome after therapy of patients with hypokalemic paralysis. Materials And Methods: All patients who presented with acute flaccid paralysis with hypokalemia from October 2009 to September 2011 were included in the study. A detailed physical examination and laboratory tests including serum electrolytes, serum creatine phosphokinase (CPK), urine analysis, arterial blood gas analysis, thyroid hormones estimation, and electrocardiogram were carried out. Patients were further investigated for any secondary causes and treated with potassium supplementation. Result: The study included 56 patients aged 15-92 years (mean 36.76 ± 13.72), including 15 female patients. Twenty-four patients had hypokalemic paralysis due to secondary cause, which included 4 with distal RTA, 4 with Gitelman syndrome, 3 with TPP, 2 each with hypothyroidism, gastroenteritis, and Liddle's syndrome, 1 primary hyperaldosteronism, 3 with alcoholism, and 1 with dengue fever. Two female patients were antinuclear antibody-positive. Eleven patient had atypical presentation (neck muscle weakness in 4, bladder involvement in 3, 1 each with finger drop and foot drop, tetany in 1, and calf hypertrophy in 1), and 2 patient had respiratory paralysis. Five patients had positive family history of similar illness. All patients improved dramatically with potassium supplementation. Conclusion: A high percentage (42.9%) of secondary cause for hypokalemic paralysis warrants that the underlying cause must be adequately addressed to prevent the persistence or recurrence of paralysis.

Silent thyroiditis is a rare cause of thyrotoxic periodic paralysis. The objective was to present a case of silent thyroiditis presenting as periodic paralysis. A 23-year-old man presented with recurrent acute flaccid predominantly proximal weakness of all four limbs. He had a similar episode 3 weeks back. On examination he was found to have hypokalemia secondary to thyrotoxicosis. Clinically there were no features of thyrotoxicosis or thyroiditis. He was initially treated with intravenous and later oral potassium supplementation and propranolol. At 8 weeks of follow-up his thyroid profile became normal and his propranolol was stopped. He had no further recurrence of paralysis. He was diagnosed as a case silent thyroiditis presenting as thyrotoxic periodic paralysis. In cases of recurrent or acute flaccid muscle paralysis, it is important to suspect thyrotoxicosis, even if asymptomatic. Definitive treatment of thyrotoxicosis prevents recurrence.

Background: Migraine is a common neurological disorder with significant impact on quality of life. The aim of this study was to investigate the prevalence and characteristics of migraine headaches in medical students, to measure its impact on their life, and to assess their knowledge about the ailment. Information about lifestyle variables was also collected. Materials and Methods: All medical students who confirmed of having headache for more than 1 year formed the study group. Students filled a detailed questionnaire focusing on demographics, pain characteristics, accompanying factors, triggers, and family history of migraine. Lifestyle variables were enquired and migraine associated disability was assessed by MIDAS (Migraine Disability Assessment). The diagnosis of migraine was made according to the International Headache Society criteria. Results are expressed in n = numbers and percentage. Results: Sixty-eight percent of medical students had headache. The prevalence of migraine in the whole cohort was 28%; however, of the headache group, migraine constituted 42%. There was a female preponderance. One-fourth of the students had weekly or daily attacks with 31% students reporting increase in their headache intensity and frequency. Forty-four percent of students had severe headaches. Dizziness, allodynia, and neck stiffness were reported as accompanying symptoms. Trigger factors were identified in 99% students, predominant of which were poor sleep hygiene, environmental changes, head movements, and mental stress. Only 4% of students did regular exercise. Twenty-seven percent of students reported self-medication use of analgesics. One-fourth of the students had migraine-associated disability but only 6% realized that they had migraine. Conclusion: Our study found a high prevalence of headache with migraine in medical students. The students' awareness of the disease was very low with one-fourth of the students resorting to self-medication. Our study identified previously less-recognized triggers like head movement and accompanying symptoms like neck stiffness. Migraine-attributed burden was high in medical students.

Nummular headache (NH) is a newly categorized headache disorder characterized by a consistent clinicographics in each attack. Currently, it is considered as a primary headache disorder due to epicranial neuralgia but the pathomechanism is still unknown. We report a woman, whose recurrent NH subsided after trans-sphenoidal surgery for her pituitary oncocytoma. The recovery of NH in this patient encourages the central mechanism for NH occurrence. After a review of literature concerning, NH and intracranial secondaries we propose that central NH is a referral pain from pain-sensitive structures, such as meninges, superimposing by pre-existing lower pain threshold or pain modulation.

Background: The purpose of this study was to evaluate the difference in the occurrence of the various "traditional" imaging signs of intracranial hypertension (IIH) on magnetic resonance imaging (MRI) in patients with idiopathic (IIH) and secondary intracranial hypertension. Materials and Methods: In a retrospective analysis, the MRI findings of 21 patients with IIH and 60 patients with secondary intracranial hypertension (41 with tumors; 19 with intracranial venous hypertension) were evaluated for the presence or absence of various "traditional" imaging signs of IIH (perioptic nerve sheath distention, vertical buckling of optic nerve, globe flattening, optic nerve head protrusion and empty sella) using the Fisher's exact test. Odds ratios were also calculated. Statistical Package for the Social Sciences version 17.0 was used for statistical analysis. Subgroup analysis of the IIH versus tumors and IIH versus venous hypertension were performed. Results: Optic nerve head protrusion and globe flattening were significantly associated with IIH. There was no statistically significant difference in the occurrence of rest of the findings. On subgroup analysis, globe flattening and optic nerve head protrusion occurred significantly more often in IIH than in tumors. However, there was no statistically significant difference in the occurrence of any of these findings in patients with IIH and venous hypertension. Conclusions: IIH is a diagnosis of exclusion. While secondary causes of raised intracranial pressure (ICP) have obvious clinical findings on MRI, some conditions like cerebral venous thrombosis may have subtle signs and differentiating between primary and secondary causes may be difficult. In the absence of any evident cause of raised ICP, presence of optic nerve head protrusion or globe flattening can suggest the diagnosis of IIH.

Objectives: Sympathetic skin response (SSR) is a test for evaluation of the sympathetic sweat gland pathways, and it has been used to study the central sympathetic pathways in spinal cord injury (SCI). This study aimed to assess the autonomic pathways according to normal or abnormal SSR in urinary incontinence patients due to incomplete spinal cord injury. Materials and Methods: Suprapubic, palmar, and plantar SSR to the peripheral nerve electrical stimulation were recorded in 16 urinary incontinence patients with incomplete spinal cord injury at various neurological levels and in 30 healthy control subjects. Results: All the recordings of SSR from the incomplete SCI patients with urinary incontinence as compared with their counterparts in the control group showed significantly reduced amplitudes with more prominent reduction in the suprapubic area recording site (P value < 0.0004). SSR with significantly prolonged latencies were recorded from palm and plantar areas in response to suprapubic area and tibial N stimuli, respectively (P value < 0.02). In this study, a significantly higher stimulus intensity (P value < 0.01) was needed to elicit SSR in the cases compared with the control group. Conclusion: This study showed abnormal SSR in urinary incontinence patients due to incomplete SCI. In addition, for the first time we have described recording of abnormal SSR from the suprapubic area as another way to show bladder sympathetic system involvement.

Background: Joubert Syndrome (JS) is a rare genetic developmental disorder, first identified in 1969. In patients with JS, certain regions of the brain (mainly cerebellar vermis and brainstem) are underdeveloped or malformed. This can lead to impaired attention, visual, spatial, motor, language and social functional skills. JS is characterized by a host of features, many of which do not occur in every patient. Aim of the Study: To spotlight and increase awareness of clinical profile and neuroimaging findings of children with Joubert syndrome. Methods: This is a retrospective case series study of patients with JS who attended the Pediatric Neurology Clinic in Aladan and Alfarawanya Hospitals in Kuwait, from September 2007 to September 2012. Clinical and radiological data were obtained from the patient medical records. Results: Cerebellar vermis hypoplasia/aplasia and apnea were present in all patients, polydactly in 3 of 16, renal problems with cysts in 5 patients and 11 of 16 had abnormal electroretinograms (ERGs). Blood investigations of organic acids, amino acids and very-long-chain fatty acid, were normal in the all the nine patients. Conclusion: JS is a rare genetic brain malformation with association of retinal dystrophy and renal abnormalities. The retinal dystrophy may be progressive. The prognosis of patients depends mainly on the degree of brain malformation.

To bring to light the greatly hazardous effects of the use of flue less gas geysers in the domestic setting. Over a period of two years (2008 to 2010) twenty six cases were documented as presenting with unexplained neurological events while bathing in an ill ventilated bathroom with a functional flue less gas geyser. The cases were mainly of three distinct prototypes namely seizure like episodes seen in 11 patients, carbon monoxide intoxication in 13 patients with near cardiac arrest in 4, and as a precipitating factor for epilepsy as seen in 2 cases. Out of the 13 cases presenting as carbon monoxide intoxication 4 had subtle cognitive defects and 2 developed early Parkinsonian features on follow up. To increase awareness regarding gas geyser induced epilepsy and associated carbon monoxide intoxication, both of which are entirely preventable conditions. We also wish to emphasize the importance of stringent and universal implementation of gas geyser usage and installation laws.

Methanol is a highly toxic alcohol resembling ethanol in smell and taste. Methanol poisoning is a lethal form of poisoning that can cause severe metabolic acidosis, visual disturbances, and neurological deficit. Brain lesions typically described in methanol toxicity are in the form of hemorrhagic and non-hemorrhagic necrosis of the basal ganglia and sub-cortical white matter. To our knowledge, lesions in the parietal, temporal, or frontal areas of cerebrum and cerebellar hemispheres have been rarely reported so far. We herewith report this rare presentation.

This is a case report of suicidal ingestion of chlorfenapyr, presenting with neurological complications after a latent period of more than a week, and rapidly progressing to death within days of symptoms. Chlorfenapyr is a moderately hazardous pesticide according to World Health Organization toxicity classification, and kills target organism by depriving it of energy through interference with oxidative phosphorylation at mitochondrial level. A pro-pesticide, chlorfenapyr takes time to convert to its active form and either this active form or a toxic metabolite causes delayed neurological symptoms. It causes significant neurotoxicity in rat models. This case report provides for the first time from India (second worldwide), clinical and "radiological evidence" (magnetic resonance imaging showing demyelinating/oedematous changes) of "chlorfenapyr neurotoxicity in humans." It also highlights the "latent period" between ingestion and onset of fatal manifestations. Earlier, similar case reports of human deaths with delayed onset neurological symptoms, due to chlorfenapyr poisoning have been reported, from Japan, Columbia, and Korea.

We report three cases of Vitamin B12 deficiency with symmetrical posterior spinal cord lesions and discuss the differential diagnosis, some of which are not well known. Because the degree of resolution of the clinical symptoms in subacute combined degeneration depends on early detection, MRI findings should not be missed.

Lemierre's Syndrome (LS) is a potentially life-threatening condition, characterized by clinical or radiologic evidence of internal jugular vein thrombosis following an oropharyngeal infection, most commonly by Fusobacterium necrophorum. A high index of suspicion and early recognition is important for successful management and to prevent systemic complications like multiorgan failure with extremely high morbidity, prolonged hospitalization and, not uncommonly, death. We are reporting a rare case of LS that was complicated with internal jugular vein and cavernous sinus thrombosis along with lung metastatic lesions, which was diagnosed and treated at our institute.

VogtKoyanagi-Harada (VKH) is a rare syndrome affecting tissues with melanocytes. The possibility that VKH syndrome has an autoimmune pathogenesis is supported by the high frequency of human leukocyte antigen-DR4 commonly associated with other autoimmune diseases. Eyes are the main affected organ, resulting in blindness. Brain disease as a late onset event is extremely rare. Here, we are reporting a 57-year-old woman with previously diagnosed VKH syndrome, presenting with a late-onset brain encephalopathy. She was treated with corticosteroids and discharged from hospital with good general condition.

Allgrove Syndrome or triple A syndrome is a rare familial multisystem disorder characterized by achalasia, alacrima and adrenal insufficiency. The objective was to describe a case of 4A syndrome where autonomic dysfunction was the presenting feature. A 22-year-old male presented with erectile dysfunction and loss of spontaneous morning erections for six months. He was having nocturnal diarrhea and recurrent postural dizziness for three months. He was found to have hyperpigmentation at pressure points, postural hypotension and other features of autonomic dysfunction. Laboratory investigations and imaging studies revealed hypoadrenalism, achalasia, alacrima and peripheral neuropathy. Autonomic neuropathy-related features persisted even after correction of hypoadrenalism. Based on clinical features and investigation he was diagnosed as a case of 4A syndrome presenting with autonomic dysfunction. Allgrove or 4A syndrome should be considered as a rare differential diagnosis of someone presenting with features of autonomic neuropathy.

Ataxia, although rare, can be a symptom of many debilitating movement disorders. Hereditary ataxias are one subset of this condition and manifest when there is a genetic abnormality involved. Ataxia oculomotor apraxia type 1 (AOA1), an autosomal recessive ataxia, results from a mutation on the aprataxin gene (APTX). We characterized a novel homozygous deletion mutation (IVS4-12delT) on the APTX gene in a 14-year-old male born to consanguineous parents. This case report emphasizes the importance of investigating and increasing awareness of novel genetic mutations in order to help diagnose and further classify hereditary ataxias.

Recurrent bacterial meningitis (RBM) in many instances is associated with identifiable anatomical defects. Presence of congenital deafness with recurrent meningitis should alert clinician for presence of middle and inner ear malformation. These defects can be demonstrated by various neuro imaging techniques and can be surgically corrected. In this case report we describe a child seen at our institute with congenital deafness and recurrent meningitis, discuss the approach to RBM and briefly describe inner ear malformation associated with the same and how to differentiate them.

Orthostatic tremor (OT), is usually a disease of old age and is characterized by quivering movements of the legs during quiet standing or in the state of isometric contraction in the lower limbs. This is relieved on walking or on lying down. It is diagnosed by surface electromyography, particularly over the quadriceps femoris muscles which shows a distinctive frequency of 13 to 18 Hz on standing. Some investigators consider it as a variant of essential tremor (ET) and the two conditions often co-exist. The disease is usually non-familial. Two brothers presented with tremor in the lower limbs on standing and on the outstretched hands without any family history. Subsequently, they were proved to be suffering from OT and ET by clinical examination and surface EMG. Simultaneous occurrence of OT and ET in two young brothers without any family history in the previous generation has not been described before and they also appeared at a much earlier age than what is described in the literature.

Niemann-Pick Type C disease (NPC) is a rare inherited metabolic disorder characterized by lipid accumulation and systemic manifestations due to multiple organ involvement. Only a few cases of NPC have been reported so far from India. Varying presentations and often lack of access to complex diagnostic tests have leaded to initial misdiagnosis on few occasions. We here report a provisionally diagnosed case of NPC with prominent horizontal gaze palsy along with characteristic vertical gaze palsy and normal findings on microscopic examination of skin biopsy specimen.

Neuronal ceroid lipofuscinosis (NCL) are a group of genetically mediated neurodegenerative disorders affecting children and young adults. They are characterized by global mental and motor deterioration, vision loss, and epilepsy ultimately resulting in death. Of the various types, late infantile variety is the 2 ^nd most common form of NCL. Here the authors report a case of a 9-year-old boy who presented with progressive mental and social deterioration since the age of 2½ years. As the disease progressed, he developed progressive vision loss, gait ataxia, action myoclonus, and epilepsy. Electroencephalogram revealed generalized sharp and slow wave discharges with background slowing. Magnetic resonance imaging of the brain revealed diffuse cerebral and cerebellar atrophy markedly affecting the cerebellum along with periventricular T2 hyperintensities. Skin biopsy from axilla revealed characteristic intracytoplasmic eosinophilic inclusions and periodic acid Schiff positive bodies within the eccrine ducts suggestive of NCL.

Intracranial neurenteric cysts are exceedingly rare congenital intracranial lesions that result from disorder of gastrulation. Still, more rarely, the cyst contents may leak into the CSF and give rise to recurrent episodes of chemical meningitis. We present a case of chemical meningitis due to a leaking posterior fossa neurenteric cyst in a young female, with emphasis on its imaging features. The final diagnosis was achieved by sufficiently characteristic imaging features; histopathologic documentation could not be achieved as the patient denied surgery.

Varadi Papp syndrome or oral-facial-digital syndrome type VI (OFDS VI) is a rare autosomal-recessive disorder distinguished from other OFDSs by metacarpal abnormalities with central polydactyly and by cerebellar abnormalities. Our patient had a broad forehead, arched eyebrows, left-sided squint, hypertelorism, epicanthic folds, fleshy nodular tongue, midline upper lip cleft, high arched palate, both pre-axial and post-axial polydactyly of limbs, hypotonia and cerebellar hypoplasia with molar tooth sign consistent with the diagnosis of Varadi Papp syndrome.