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Oculomotor abnormalities are one of the cardinal clinical features of progressive supranuclear palsy (PSP). Vertical saccadic slowing is an early sign of PSP. The association between oculomotor abnormalities and sleep architecture has not been studied so far.To study the association of oculomotor abnormalities of PSP with the sleep stages by using video polysomnography (vPSG).This was a cross-sectional single-center study. Twenty-two patients with PSP and 15 age and gender-matched controls were recruited. Saccades, vestibulo-ocular reflex, and optokinetic nystagmus were assessed and graded clinically in all patients and one overnight vPSG was done in all cases.Vertical saccades, upward more than downwards, were affected in all cases. While horizontal saccades were normal only in 41% of cases. Vertical optokinetic nystagmus (OKN) was affected in all cases. Horizontal OKN was normal in 36% of patients. The vertical upward saccades had a negative correlation with N1% and duration (r = -0.418; p = 0.05, r = -0.457; p = 0.03), N3% and duration (r = -0.486; p = 0.02, r = -0.510; p = 0.01), REM% (r = -0.449; p = 0.04), total sleep time (r = -0.487; p = 0.02) and sleep efficiency (r = -0.444; p = 0.04). There was a positive correlation between horizontal OKN and sleep onset latency (r = 0.432; p = 0.05).Vertical saccadic restriction in PSP has significant negative correlation with total sleep time and sleep efficiency. The oculomotor and sleep abnormalities in PSP are probably interlinked and their assessment is useful in determining the characteristics of the disease.

Pediatric neurobrucellosis represents a common anthropozoonosis in endemic areas but only anecdotal reports are available till date. Using appropriate search terms in the database platforms of MEDLINE, SCOPUS and Web of Sciences, we performed a systematic review of all the cases of pediatric neurobrucellosis published in the medical literature till date, in the light of a case report. The protocol was registered under PROSPERO (CRD42022333907). Our search strategy yielded 187 citations of which 51 citations were included. A total of 119 cases were reviewed. Of these cases, eight of them had insufficient data. The most common presentation was meningitis with or without encephalitis (n = 79, 71.2%). A high prevalence of cranial neuropathies (n = 22, 20.7%) was observed in the pediatric population in which abducens palsy was the most common (n = 9, 8.1%). Diagnosis was based on multimodal investigations including standard agglutination test (n = 44, 39.6%), Rose Bengal test (n = 37, 33.3%), blood culture (n = 23, 20.7%), serology (n = 20, 18.0%) and cerebrospinal fluid (CSF) culture (n = 11, 9.9%). Rifampicin-based triple drug regimen was the most commonly employed (83/102, 81.4%). Pediatric neurobrucellosis was associated with greater frequency of sequalae (5.4%), deafness (2.7%) and mortality (2.7%), when compared to that of general population. Neurobrucellosis mimics neuro-tuberculosis in various aspects. The review highlights several unique aspects of this entity in children. A high index of suspicion can ensure prompt diagnosis, timely initiation of management and favorable outcomes.

Background Habituation deficit is considered as a neurophysiological abnormality among migraineurs in the interictal period. For clear comprehension and clarity about the mechanism underlying habituation in migraine, a sophisticated method, i.e., high frequency oscillations (HFOs) evoked potentials, have been utilized. However, studies pertaining to this in the Indian context are rare. Objective The aim of the study is to determine the utility of HFO of somatosensory evoked potential (SSEP) in deciphering the pathophysiology of migraine. Materials and Methods Sixty subjects including 30 migraineurs in the interictal period and 30 healthy controls were considered for the study. Median nerve SSEP was recorded in patients and controls by standard protocols. HFO was extracted offline using the Digital zero-phase shift band-pass filtering at 450 and 750 Hz. The early and late HFOs were determined with respect to the N20 peak and were compared between the groups. Results Of total 30 migraineurs, 18 had hemicranial headache and 12 had holocranial headache. N20 latency, P25 latency, N20 onset to peak amplitude, and N20 onset to P25 amplitude were comparable in migraineurs and controls. The intraburst frequency of early HFOs in migraineurs was significantly higher (p = 0.04), whereas the peak-to-peak amplitude was significantly lower (p = 0.001). Conclusion Early HFOs on SSEP represent the thalamocortical excitatory drive in migraineurs. Overall, the study reports that reduced amplitude of early HFOs in the interictal period suggest reduced thalamocortical drive in migraineurs.

Background: Disorders of tetrahydrobiopterin metabolism represent a rare group of inherited neurotransmitter disorders which manifest mainly in infancy or childhood with developmental delay, neuroregression, epilepsy, movement disorders and autonomic symptoms. Methodology: A retrospective review of genetically confirmed cases of disorders of tetrahydrobiopterin metabolism over a period of three years (Jan 2018 to Jan 2021) was performed across two paediatric neurology centres from South India.Results: A total of nine patients(M:F=4:5) fulfilled the eligibility criteria. The genetic variants detected include homozygous mutations in the QDPR(n=6), GCH1(n=2) and PTS(n=1) genes. The median age at onset of symptoms was 6-months(range 3-78 months), while that at diagnosis was 15-months (8-120 months) resulting in a median delay in diagnosis of 9-months. The main clinical manifestations included neuroregression (89%), developmental delay(78%), dystonia(78%) and seizures(55%). Management strategies included phenylalanine restricted diet, levodopa/carbidopa, 5-Hydroxytryphtophan, and folinic acid. Only, Patient-2 afforded and received BH4 supplementation at a sub-optimal dose later in the disease course. We had a median duration of follow up of 15 months (range 2-48 months). Though biochemical response has been marked, except for patients with GTPCH deficiency, only mild clinical improvement was noted with regards to developmental milestones, seizures or dystonia in others. Conclusion: Tetrahydrobiopterin deficiencies represent an rare yet potentially treatable cause for non-phenylketonuria hyperphenylalaninemia when diagnosed and treated early in life. Screening for disorders of biopterin metabolism in patients with hyperphenylalaninemia prevents delayed diagnosis. This study expands the genotype phenotype spectrum of patients with disorders of tetrahydrobiopterin metabolism from South India.

Background Out of several neuroprotective drugs (NPDs) studied in animals and humans, four NPDs (citicoline, edaravone, cerebrolysin, and minocycline) have been found to have beneficial effects in acute ischemic stroke (AIS). Objective The purpose is to evaluate the efficacy of citicoline, edaravone, minocycline, and cerebrolysin compared with placebo in patients with middle cerebral artery (MCA) territory AIS. Materials and Methods This was a prospective, single center, single-blinded, and hospital-based study. One hundred patients with MCA territory AIS with 20 patients in each group including control group were included. Barthel index (BI), National Institute of Health Stroke Scale (NIHSS) score, and modified Rankin Scale score were recorded at admission, at day 11 and after 90 days. Results The mean NIHSS score was significantly lesser at day 11 and after 90 days in citicoline, edaravone, and cerebrolysin group in comparison with placebo. Similarly, the mean BI score was significantly higher at day 11 and after 90 days in citicoline, edaravone, and cerebrolysin group in comparison with placebo. In minocycline group, there was no significant change in the NIHSS score and BI score at day 11 and after 90 days. Conclusion There was significant improvement in the functional outcome of patients with AIS involving MCA territory at 90 days receiving citicoline, edaravone, and cerebrolysin. However, minocycline did not offer the same efficacy as compared with other neuroprotective agents.

Objectives: The objective of this study is to describe the clinical, biochemical, radiological, and genetic profile of patients presenting with progressive spastic paraparesis due to homocysteine remethylation pathway defect. Methods: This was a retrospective study conducted by reviewing the medical records of patients with serum homocysteine levels >50 μmol/L between January 2015 and January 2019 at our hospital. We included patients presenting with progressive spastic paraparesis, having serum homocysteine >50 μmol/L with low or normal blood methionine suggesting disorders of homocysteine remethylation. Demographic details, clinical manifestations, biochemical abnormalities, neuroimaging findings, and genetic profile were analyzed. Results: A total of seven patients (M: F = 5:2) fulfilled the study eligibility criteria. The mean age at onset of the disease was 13.4 ± 2.4 years (range: 9–17 years). Spastic paraparesis was the presenting manifestation in 4/7 (57.1%) patients. Other manifestations included cognitive decline, poor scholastic performance, behavioral disturbances, seizures, and spastic bladder. Severe hyperhomocysteinemia (>100 μmol/L) was noted in 6/7 (85.7%) patients with median levels of serum homocysteine being 185.7 μmol/L (range: 85.78–338.5 μmol/L). Neuroimaging showed parieto-occipital predominant leukoencephalopathy in 5/7 (71.4%) and diffuse cerebral atrophy in 1/7 (14.2%). Genetic analysis in three patients revealed pathogenic missense variants c.459C >G (p.Ile153Met), c.973C >T (p.Arg325Cys), and c.1031G >T (p.Arg344Met) in MTHFR gene. All the patients received vitamin B12 (injection and oral), folic acid, and pyridoxine and two patients received betaine. At the last follow-up of a median duration of 12 months, there was a good clinical and biochemical response with reduction in the median value of serum homocysteine by 77.5 μmol/L. Conclusion: Evaluation of serum homocysteine and blood methionine in adolescents presenting with progressive spastic paraparesis gives clue to a treatable homocysteine remethylation disorders.

Sleep disturbances in Parkinson's disease (PD) are common. Non -motor symptoms including sleep disturbance have gender difference in PD. The objective is to determine gender differences in PD in relation to the sleep quality.156 patients with PD with males (n = 119) and females (n = 37) were included. The sleep quality and sleep disturbances were assessed using Pittsburgh sleep quality index (PSQI), Parkinson's disease Sleep Scale (PDSS) and Epworth Sleep Scale (ESS).Females with PD had higher frequency with insomnia (56.7%) as compared to males with PD (44.5%) (p = 0.05), nightmares (32.4%) as compared to males with PD (16.8%) (p = 0.04), greater PSQI component score of habitual sleep efficiency (p = 0.008), sleep disturbance (p = 0.03) and daytime dysfunction (p = 0.02), greater global PSQI score (p = 0.03), higher percentage of patients with global PSQI score 5 (64.8%) than males with PD (41.1%) (p = 0.01), suggesting poor quality of sleep in females with PD. Higher total ESS score (p = 0.03), higher frequency of females with PD with total ESS score 10 (45.9%) than in males with PD (26.9%) (p = 0.02), and lower total PDSS score (p = 0.009). On correlation analysis, the global PSQI score in females with PD had strong positive correlation with duration of PD, HAM-A and HAM-D scores and negative correlation with MMSE scores.Females with PD had a poor sleep quality with higher frequency of insomnia, nightmares, and daytime sleepiness. The longer duration of PD, higher scores of anxiety and depression scale and lower MMSE scores are associated with poor sleep quality in females with PD.

Introduction: Review of literature revealed very limited studies considering a combination of serum prolactin (PRL) and serum creatine kinase (CK) as markers for differentiating epileptic and psychogenic nonepileptic seizures (PNES). Therefore, in the present study, we analyzed the role of serum PRL and serum CK, individually and in combination. Methodology: This prospective study was conducted in a tertiary care medical teaching hospital over a period of 18 months. Patients aged over 15 years suspected to have new-onset seizures presenting within 5 h of ictus were included in this study. CK, serum PRL was measured at 0–1, 1–3, and 3–5 h after seizures. Results: Hundred subjects were studied for the role of serum PRL and serum CK in differentiating epileptic and PNES. The mean age was 42.24 years with a male:female ratio of 1.27:1. All patients of generalized tonic–clonic seizures (GTCS), who presented within 1 h, had elevated PRL, whereas 75% of patients with partial seizures had elevated PRL within 1 h of presentation. Nearly 91.66% of patients with GTCS who presented within 1 h had elevated CPK, whereas 70% of patients with partial seizures had elevated CPK. None of the patients diagnosed with PNES showed rise in either of the markers. Conclusion: In the present study, none of the patients with PNES showed raise in either serum PRL or CK. However, there was no correlation between the types of seizure and PRL or serum CK levels.

Objectives CNS infections like meningitis and encephalitis pose enormous healthcare challenges due to mortality, sequelae and socioeconomic burden. In tertiary setting, clinical, microbiological, cytological and radiological investigations are not distinctive enough for diagnosing microbial etiology. Molecular diagnostics is filling this gap. We evaluated the clinical impact of a commercially available multiplex molecular diagnostic system − SES for diagnosing suspected CNS infections. Patients and methods This study was conducted in our tertiary level Neurology ICU. 110 patients admitted during Nov-2010 to April-2014 were included. CSF samples of patients clinically suspected of having CNS infections were subjected to routine investigation in our laboratory and SES test at XCyton Diagnostics. We studied the impact of SES in diagnosis of CNS infections and its efficacy in helping therapeutic management. Results SES showed detection rate of 42.18% and clinical specificity of 100%. It had 10 times higher detection rate than conventional tests. Streptococcus pneumoniae and Mycobacterium tuberculosis were two top bacterial pathogens. VZV was most detected viral pathogen. SES results elicited changes in therapy in both positive and negative cases. We observed superior patient outcomes as measured by GCS scale. 75% and 82.14% of the patients positive and negative on SES respectively, recovered fully. Conclusion Detecting causative organism and ruling out infectious etiology remain the most critical aspect for management and prognosis of patients with suspected CNS infections. In this study, we observed higher detection rate of pathogens, target specific escalation and evidence based de-escalation of antimicrobials using SES. Institution of appropriate therapy helped reduce unnecessary use of antimicrobials.

Cerebral venous sinus thrombosis (CVST) is common in Asians, accounting for 15% of all strokes in the young. CVST causing malignant cerebral oedema with brain herniation and death are referred as malignant CVST. This study was aimed at evaluating the outcome of patients and factors predicting the outcome with malignant CVST after decompressive surgery. It was a retrospective, observational, single centre, hospital-based and cross-sectional study. Records of patients with malignant CVST who had decompressive surgery were analysed. Over 5 years (2010–2015), 30 patients (15 men and 15 women) underwent decompressive surgery. In univariate analysis, age more than 50 years (p = 0.05); presence of midline shift of more than 10 mm (p = 0.03) and total effacement of basal cisterns (p = 0.01) had significant correlation with poor outcome. On multivariate analysis, presence of midline shift of more than 10 mm (p = 0.01) was a significant predictor of poor outcome. Decompressive surgery is a life saving therapeutic intervention in patients with malignant CVST and more than two-thirds of patient shows favourable outcome. Age more than 50 years, midline shift >10 mm and total effacement of basal cisterns determine poor outcome following decompressive surgery.

Background: Intravenous thrombolysis (IVT) has now become a standard treatment in eligible patients with acute ischemic stroke (AIS) who present within 4.5 h of symptom onset. Objective: To determine the usefulness of IVT and the subset of patients who will benefit from IVT in AIS within 4.5 h. Materials and Methods: Patients with AIS within 4.5 h of symptom onset who underwent IVT were studied prospectively. The study period was from October 2011 to October 2015. Results: A total of 97 patients were thrombolysed intravenously. The mean onset to needle time in all patients was 177.2 ± 62 min (range: 60–360). At 3 months follow-up, favorable outcome was seen in 65 patients (67.1%) and poor outcome including death in the remaining 32 patients (32.9%). Factors predicting favorable outcome was age 15 (P = 0.03), RBS >250 mg/dl (P = 0.01), Dense cerebral artery sign, age, glucose level on admission, onset-to-treatment time, NIHSS on admission score >5 (P = 0.03), and occlusion of large artery (P = 0.02). Conclusion: Milder baseline stroke severity, blood glucose

Rapid eye movement sleep behavior disorder (RBD) is common in Parkinson's disease (PD). Little information exists about RBD in women with PD. The aim of this study was to determine the clinical expression of RBD in women with PD and note any differences in women with PD with and without RBD. One hundred fifty-six patients with PD were recruited. There were 37 women with PD and probable RBD was diagnosed using the RBD Screening Questionnaire. Other scales included Pittsburgh Sleep Quality Index, Parkinson's Disease Sleep Scale, Epworth Sleep Scale, Hamilton Anxiety Rating Scale and Hamilton Depression Rating Scale. Probable RBD was diagnosed in 10 women with PD (27%). Most often (70%) RBD occurred after the onset of parkinsonian symptoms. Women with probable RBD were older, had shorter duration of PD symptoms, lower tremor score, and higher axial signs score. They had insomnia (80% versus non-probable RBD patients 44%, p=0.019), and poor sleep quality with excessive daytime sleepiness. Anxiety and depression were common in women with probable RBD. Episodes were brief and confined to vocalization and simple limb movements. No injury to self or bed partners was noted. Women with PD have fewer fights and less aggressive dream enacting behaviour than men, but suffer from significant disturbed sleep, and levels of anxiety and depression.

Background: Liquefied petroleum gas geysers are used very frequently for heating water in developing countries such as India. However, these gas geysers emit various toxic gases; one among them is colorless, odorless carbon monoxide (CO). In the past few years, there were reports of unexplained loss of consciousness in the bathroom. However, the exact cause for these episodes has been recognized as toxic encephalopathy due to toxic gases inhalation mainly CO. Objective: To analyze the clinical profile and outcome of patients brought with loss of consciousness in the bathroom while bathing using gas geyser. Materials and Methods: Case records of patients with the diagnosis of gas geyser syndrome from 2013 to 2015 were retrieved and analyzed. Twenty-four cases were identified and included in the study. This was a retrospective, descriptive study. Results: Twenty-four patients were brought to our Emergency Department with loss of consciousness in the bathroom while bathing. Twenty-one cases had loss of consciousness during bathing and recovered spontaneously. Two cases were found dead in the bathroom and were brought to the Department of Forensic Medicine for postmortem. One case was brought in deep altered state of consciousness and succumbed to illness within 1 week.Conclusion: Awareness regarding CO intoxication due to usage of ill-fitted, ill-ventilated gas geyser is necessary as they are entirely preventable conditions.

Introduction Infarctions of the corpus callosum are rare vascular events. It is relatively immune to vascular insult because of its rich vascular supply from anterior and posterior circulations of brain. Objective Report of 3 patients with largely diffuse acute corpus callosum infarction. Methods 3 patients with largely diffuse acute corpus callosum infarction were studied and each of these 3 patients had 3 different aetiologies. Results The 3 different aetiologies of largely diffuse acute corpus callosum infarction were cardioembolism, tuberculous arteritis and takayasu arteritis. Conclusion Diffuse corpus callosum infarcts are rare events. This case series narrates the three different aetiologies of diffuse acute corpus callosum infarction which is a rare vascular event.

Antiplatelet therapy has established clinical benefit on cardiovascular outcome and has reduced the rates of re-infarction/in stent thrombosis following percutaneous coronary intervention in acute coronary syndromes. Major bleeding episodes can occur with antiplatelet therapy and intracranial hemorrhage (ICH) is one of the most feared complications resulting in significant morbidity and mortality. Identification of high risk groups and judicious use of antiplatelet therapy reduces the bleeding risk. Ticagrelor is a newer P2Y12 receptor antagonist with established clinical benefit. However, risks of having an ICH with these newer molecules cannot be ignored. Here, we report a case of spontaneous acute subdural hematoma developing in a patient on antiplatelet therapy with aspirin and ticagrelor. Early recognition, discontinuation of the medication and appropriate management resulted in resolution of hematoma and good clinical outcome. Authors have reviewed the antithrombotic drugs and their tendencies in causing intracranial bleeds from a neurophysicians perspective.