7%), who had distinct clinical features and the deletion was inherited from the unaffected father. The second twin suffered more severe clinical manifestations including autistic features, behavioral problems, mild-moderate mental retardation and seizures, which were under control with multidrug regimen when compared with the first twin. Conclusion PCDH19 is a major causative gene in patients with epilepsy and further data is required to gain a better understanding of phenotype-genotype correlation. In addition to gene sequencing, deletion/duplication analysis will improve the molecular diagnosis in patients with clinical findings. Copyright © 2020 Annals of Indian Academy of Neurology.Background Tolosa-Hunt Syndrome (THS) is one of the causes of cavernous sinus syndrome causing painful ophthalmoplegia. Literature on long-term outcome of this rare condition is scarce. Aims and Objectives The aim is to study the recurrence and role of steroid-sparing agents in THS. Methodology All cases of THS treated at a tertiary-level teaching hospital during a 10-year period were studied. Clinical and radiological profile, response to treatment and recurrences were noted. Results A total of 44 cases were studied. The mean age was 49.5 years, Males constituted 23/44 (52%). The first symptom was pain in 90%. Ptosis with ophthalmoplegia was the most common deficit 29/44 (66%). Lesions confined to cavernous sinus 27/44 (61%) was the most frequent magnetic resonance imaging finding. All patients received steroids as the initial treatment and 15/44 (34%) received steroid-sparing agents. Follow-up ranged from 6 to 120 months (Mean 39 months). Two patients had alternative diagnosis of leptomeningeal malignancy and hypertrophic pachymeningitis on follow-up. Recurrences occurred in 18/37 (48.6%). Time for recurrence varied from 8 months to 7 years. (Mean 18 months). No clinical or radiological predictors for recurrence were identified. https://www.selleckchem.com/products/fht-1015.html Patients who received steroid-sparing agents had a significantly lower recurrence 3/15 (20%) versus 14/26 (53.8%)P less then 0.034. Conclusions Around 50% of patients with THS can have recurrence. Steroid-sparing agents appear to prevent recurrence. A prospective multicenter randomized controlled trial may help to evaluate the risk and benefits of steroid-sparing therapy and to identify any possible predictors for recurrence. Copyright © 2020 Annals of Indian Academy of Neurology.Background and Aims To study the clinical profile of genetically proven Huntington's disease (HD) patients from eastern India. Methods This cross sectional study selected patients of HD after genetic confirmation of expanded CAG repeats in Huntingtin (HTT) gene. We performed detail clinical evaluation including cognitive and neuropsychological assessment, and imaging of brain. Results This study included 75 patients (male 57.3%; female 42.7%). Mean age at onset was 37.12 (range 16-62) years; juvenile variety (onset below 20 years) was detected in 5.3%. Paternal transmission was commoner. Manifestations at onset were motor in 81.3% patients, behavioral in 10.7% and cognitive impairment in 8%. After chorea, next common movement disorder was dystonia. Frontal lobe dysfunction was found in 77.3% patients. Behavioral disturbances were observed in 77.3% patients and commonly manifested as depression, irritable behavior and anxiety. Among the three onset groups (motor/behavioral/cognitive), there was no significant difference regarding age at onset, gender distribution, pattern of inheritance (paternal/maternal), and at the time of evaluation, all groups had essentially similar pattern of clinical features. Mean CAG repeat of the patients was 48.25 (range 40-79). Our study showed some differing clinical characteristics compared to previous studies from the Indian subcontinent. Conclusion Clinical features in our study showed differences from previous studies from the Indian subcontinent. We had more cognitive-onset patients. However, behavioral onset was lower in our study. Motor, behavioral and cognitive onset groups of HD were comparable regarding demographics, family history, CAG repeat lengths and major clinical features at the time of evaluation. Copyright © 2020 Annals of Indian Academy of Neurology.Background and Aims Despite the evidence from randomized clinical trials, the effectiveness of intravenous tissue recombinant plasminogen activator (IV-tPA) for elderly patients (≥80 yrs) with acute ischemic stroke (AIS) is often an important consideration in clinical practice. We evaluated the effect of older age on arterial recanalization, timing of recanalization and outcome in thrombolysed AIS patients. Methods Consecutive AIS patients treated with IV-tPA and transcranial Doppler (TCD) examination within 3 hours of symptom-onset were included. Thrombolysis in Brain Ischemia (TIBI) flow-grading system was used to interpret TCD findings of persistent occlusion, re-occlusion and complete recanalization within 2 hours of IV-tPA bolus. Poor functional outcome was defined by modified Rankin score of 3 or more. Univariate and multiple logistic regression analyses were performed to assess the effect of age on clinical and TCD outcome measures. Results The study included 361 patients (elderly = 85, less then 80 yis no increase in the hemorrhagic risk. Perhaps, decision for IV thrombolysis in elderly patients should be made cautiously. Copyright © 2020 Annals of Indian Academy of Neurology.Background Although first described more than two decades ago, posterior reversible encephalopathy syndrome (PRES) continues to be enigmatic. We prospectively followed consecutive patients of PRES both clinically and radiologically for a better understanding of natural history, symptomatology, and prognosis of this not so uncommon entity. Patients and Methods The current study included 22 consecutive patients of PRES who were followed both clinically as well as radiologically at a tertiary care institute in Northern India from December 2014 to June 2016. Results Mean age was 30.68 ± 12.68 years. The most common symptoms included altered sensorium (77.3%), headache (72.7%), seizures (63.6%), vomiting (36.4%), and visual disturbances (22.7%). About 94.5% of patients had parieto-occipital signal changes on neuroimaging. Magnetic resonance imaging (MRI) (n = 20) revealed involvement of sites considered atypical for PRES in 95% (frontal [55%], temporal [40%], cerebellum [40%], basal ganglia [15%], deep white matter [10%] and brainstem [10%]).