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Objective: To determine the feasibility, reliability, and acceptability of video teleconference (VTC)-based neuropsychological assessment using Addenbrooke's cognitive examination-III (ACE-III). Methods: This study was performed from January 2022 to April 2022, during the third wave of the COVID-19 pandemic in India. We administered ACE-III using video-teleconferencing and compared the scores to face-to-face (FTF) testing for the eligible participants. We also conducted a participant's satisfaction survey of VTC-administered ACE-III compared to FTF-administered ACE-III, using a 7-point Likert scale. Results: We screened 37 participants and 24 (64.9%) successfully underwent ACE-III testing through VTC. We included 20 patients (mean age: 62.7 ± 10 years, mean education: 12.0 ± 4.6 years, 85% men) for final analysis, (who completed both VTC and FTF-administered ACE-III). Nine patients had major neurocognitive disorder (dementia), eight had mild neurocognitive disorder (MCI), and three had subjective cognitive decline (SCD). The two tests were administered at a median gap of 36 (18,74.5) days. The Intraclass correlation coefficients (ICC) of ACE-3 total scores (0.97) and the subdomain scores was high (>0.8). There was “very low” to “no” bias on the Bland–Altman plots, across all domains. The mean overall satisfaction score was 4.1, indicating that VTC is “as good as” FTF. Conclusions: Results support the feasibility and acceptability of remote administration of ACE-III via VTC. There is a good agreement between the ACE-III scores across VTC and in-person conditions.

Background: Parkinson's disease (PD) is a slowly progressive and disabling disorder, so the cost of illness may change with time. We aimed this study to know the annual cost of care of PD in India. Methods: After ethics approval, a prospective cohort study was conducted at the movement disorder clinic of tertiary care hospital for 2 years (2014–2016). The outcomes were a description of the total annual direct cost of Parkinson's disease including health care as well as non-health care cost. We also did correlation analysis to know the determinants of the total cost. Results: A total of 200 consecutive patients of PD with 141 (70.50%) males and 59 (29.50%) females with a mean age of 56.84 ± 10.51 years were enrolled. The annual Median Direct cost of care was INR 27,315.0 (IQR 13636.6–44908.4), whereas the Indirect cost was INR 21,400 (IQR 9800 – 96800). Cost on drugs (Direct health care) formed 68.50% (Median) of the total Direct cost. Total direct cost formed 11.38% of the Median total yearly income of our patients. Of the direct cost, the Median expenditure on drugs was INR 18,712.8 (8064.0 -30696.0). Only 5% of patients had health care insurance. The total direct cost was determined by the stage of Parkinson's disease and duration of disease (P = < 0.01) but not predicted by age, gender, age at onset, and the yearly income of patients. Conclusion: Annual cost of care of Parkinson's disease is high and increases with the duration of the disease as well as the progression of the disease.

Background: Spinocerebellar ataxia is a neurodegenerative disease. Information on comparative assessment of quality of life (QoL) among SCAs, particularly SCA 12, is scarce. We aimed to compare health-related QoL in SCA 1, 2 and 12. Methods: We conducted a cross-sectional study among individuals with genetically-confirmed SCAs. Ataxia severity was assessed using Brief Ataxia Rating Scale (BARS), independence in activities of daily living (ADL) using Katz index (Katz ADL) and depression using Beck's Depression Inventory-II (BDI-II). QoL was assessed via Short Form Health Survey version 2.0 (SF-36). Results: We enrolled 89 individuals (SCA1 = 17, SCA2 = 43, SCA12 = 29; 56% males). Mean age at onset (41.0 ± 11.6 for SCA12 versus 24.9 + 7.0 for SCA1 and 28.8 ± 9.8 years for SCA2) was significantly higher among SCA12. SCA12 had lower BARS (mean score 4.1 ± 4.5 versus 10.6 ± 4.6 for SCA1 and 12.5 ± 4.5 for SCA2). SCA12 scored better on all SF-36 subdomains including Physical (PCS) and Mental Component Summary (MCS) scores. PCS score amongst SCA12 was 44.4 ± 9.0 versus 30.4 ± 9.1 for SCA1 and 33.3 ± 8.9 for SCA2. MCS score for SCA12 was 51.4 ± 11.4 versus 41.8 ± 11.5 for SCA1 and 41.8 ± 11.2 for SCA2. SCA12 had lower mean BDI scores (5.0 ± 6.0) versus SCA1 (9.5 ± 11.6) and SCA2 (10.9 ± 10.3). BARS and BDI emerged as significant predictors of most SF-36 subdomains. Conclusions: Our study suggests that despite older age and comparable disease duration, SCA12 patients experience better QoL, less severe depression and ataxia versus SCA1 and SCA2. Severity of ataxia and depression are significant predictors of QoL among the three SCA types.

Parkinson's disease (PD) is the second most common progressive neuro-degenerative disorder. Research in PD is gradually increasing in India due to increased clinical cases, which could double by 2030 worldwide. Although its prevalence is low in India as compared to other countries, the total burden is much higher due to the large population size. PD is progressively debilitating, with pronounced motor and nonmotor symptoms (NMSs) that severely affect the quality of life (QoL) of patients and their caregivers. The progressive nature of the disease lays great emphasis on doctors to focus on the patients' QoL. As a consequence, Health-related QoL (HRQoL) has gradually become one of the main indicators for assessing health-related outcome. There is a growing need to pay attention to the NMSs and a pressing need to look at the QoL of Indian patients with PD through a culture and value specific lens. Research into the holistic QoL assessment with emphasis on psychological domains may allow for the early evaluation and intervention of depressive and cognitive symptoms in PD. This could result into increased productivity, reduced morbidity, and healthcare cost, which would in turn result into better QoL of Indian PD patients.

The COVID-19 crisis has worsened the pre-existing barriers to accessing neurological specialist care in Low and middle income countries. Telemedicine has been available for well over 2 decades but has not been widely adopted in LMIC’s due to issues around cost, feasibility, infrastructure and regulation. Virtual care is an offshoot of traditional telemedicine leveraging the widely available internet enabled devices to connect patients with their healthcare providers. In this manuscript, we provide an overview of the virtual care, relevance to neurology and some guidance on implementing virtual care in an Indian context.

Context: Parkinson's disease (PD) has devastating effects on quality of life (QoL), but there is no instrument that has been developed for Hindi-speaking persons with Parkinson's disease (PWP). Objective: The objective of this study was to develop and validate an instrument in Hindi language to measure health-related QoL (HRQoL) in PWP. Subjects and Methods: Literature review and interviews of stakeholders were done to create a pool of 68 items to develop a questionnaire. Self-rated global QoL item was also included in the questionnaire. Questionnaire was tested on 300 Hindi-speaking PWP. Item reduction was achieved through factor analysis and clinimetrics to finalize the QoL in PD (QLPD) instrument. Validity and reliability of the QLPD were tested. Results: “QLPD” is a 45-item instrument with nine subscales, namely, activities of daily living, mobility, psychological, fear, social, family, treatment, finance, and nonmotor symptom subscales. Internal consistency of QLPD's summary score and all subscales except treatment subscale was high (α = 0.74–0.94). Intraclass correlation coefficient between summary score and global QoL was 0.79. Summary score and subscale scores were significantly different (P < 0.0001) for predefined five categories on global QoL (very good to very bad). QLPD subscales exhibited good convergent and divergent validity with subscales of 39-item PD questionnaire and short form-36 scale. Higher Hoehn and Yahr stage, lower monthly per capita income, and higher levodopa equivalent daily dosage were found to be independently associated with poor HRQoL. Conclusion: QLPD is a valid and reliable instrument to measure HRQoL in Hindi-speaking PWP.

Introduction: Tubercular meningitis (TBM) is a common cause of chronic meningitis in India; however, there is a paucity of literature on optimum duration and choice of drug therapy. Materials and Methods: This was an ambispective cohort study. Results: Two hundred and forty-four patients of central nervous system tuberculosis (CNS TB) who were seronegative for HIV were studied of whom 198 had TBM and 46 patients had tuberculoma without meningitis. Before completion of treatment, 84% of TBM patients underwent imaging. There was no difference in disability or mortality in patients, who were treated with various drug regimens in terms of duration of therapy or number of drugs at initiation of treatment. However when patients developed new complications, adding more drugs improved survival. Prolonging corticosteroid administration in patients with nonsatisfactory improvement at 8 weeks was not associated with prevention of disability. Conclusions: CNS TB is treated by neurologists and physicians in India, as per their experience due to different recommendations in various guidelines. There is a tendency to decide when to stop treatment based on neuroimaging given the fear of poor outcomes associated with recurrence of the disease. The duration of treatment or choice of drugs at the start of treatment did not affect disability.

Introduction: A large proportion of the population suffers from disturbed sleep and a majority of these present to clinicians with the complaint of insomnia. Many of these people possibly suffer from other primary sleep disorders such as sleep apnea; however, insomnia disorder also remains an extremely common condition directly impacting the quality of life and work efficiency of affected people. While a number of approaches are used for treatment by many clinicians, a much smaller percentage can seek help from specialty sleep clinics. Since very little data are available regarding the effectiveness of treatment offered, this retrospective study aims at examining the outcome of chronic insomnia patients attending to a quaternary care specialty sleep center. Methods: Consecutive patients with the presenting complaint of and diagnosis of insomnia, during a study period of 2 years between 2013 and 2015, were identified and analyzed. Patients were classified based on the comorbidity and types of treatment received. Life stressors were also identified and analyzed. Outcomes were reviewed for those who had follow-up data available. Results: Based on the defined inclusion criteria, 102 patients could be analyzed. Among these, at least 3-month follow-up was available for 48 patients. It was observed that among patients for whom at least 3-month follow-up was available, 91.4% (43/48) showed a good response to treatment with physician-administered cognitive behavioral therapy for insomnia along with treatment for comorbid conditions. Conclusion: With correct diagnostic classification, as well as appropriate and easily available treatment strategies, excellent treatment outcomes are observed at specialty sleep clinics.

Background: Movement disorders are one of the prominent nonataxic symptoms in patients of spinocerebellar ataxia (SCA). The type of movement disorder may provide clinical clue to the type of SCA. Objective: The objective of this study is to evaluate various movement disorders in patients of genetically proven SCAs and to establish a probable clinico-genetic correlation. Methods: Ninety-Five patients of genetically proven SCAs were assessed for the presence of various movement disorders. Results: Patients with SCA (75.8% males) with at least one movement disorder contributed 43.16%. Age for onset of movement disorder was 43.39 ± 13.43 years. SCA-12 (38.95%) was the most common subtype. Among the patients with at least one movement disorder, action tremor of hands contributed majority (90.2%). Dystonia and parkinsonism were present in 17.07% and 12.2% of patients (with movement disorder), respectively. Action tremor of hands was present in 34 patients with SCA-12 (91.89%), and 20 patients (54.05%) had onset of hand tremor preceding the onset of ataxia. Majority of patients with SCA-12 (81%) were of the same ethnic origin belonging to Agrawal community. Patients with movement disorder had a later onset (45 ± 13.88 years) of ataxic symptoms compared to those without a movement disorder (32.8 ± 11.92) (P =

Creutzfeldt–Jakob disease (CJD) belongs to a group of prion disease that is caused by abnormally folded proteins and is clinically characterized by rapidly progressive cognitive decline, gait abnormalities, and myoclonus. Familial CJD is very rare and is described only in few families around the world. We report a case with rapidly progressive cognitive decline, ataxia, and myoclonus, with a history of several members of his family developing similar symptoms and succumbing to it. Clinical presentation and neuroimaging were suggestive of CJD. On genetic analysis, our index case and two of his family members (younger brother and younger son) were found to have D178N mutation in PRNP gene. The polymorphism of the 129th amino acid was V/V. We report the first kindred familial CJD from South-East Asia with genetically proven D178N-129V haplotype.

Objective: The aim is to compare the retinal nerve fiber layer (RNFL) thickness of longitudinally extensive transverse myelitis (LETM) eyes without previous optic neuritis with that of healthy control subjects. Methods: Over 20 LETM eyes and 20 normal control eyes were included in the study and subjected to optical coherence tomography to evaluate and compare the RNFL thickness. Result: Significant RNFL thinning was observed at 8 o'clock position in LETM eyes as compared to the control eyes (P = 0.038). No significant differences were seen in other RNFL measurements. Conclusion: Even in the absence of previous optic neuritis LETM can lead to subclinical axonal damage leading to focal RNFL thinning.

Background: Cognitive impairment is increasingly being recognized as a major cause of morbidity and increased dependence over the caregivers in Parkinson's disease (PD) patients. Objective: The present study aimed to compare the cognition testing in young- and late-onset PD patient. Methods: Sixty PD patients (20 young onset and 40 late onset) fulfilling UKPDS Brain Bank diagnostic criteria were enrolled in the study. Patients were assessed clinically and using scales for cognition testing such as Scales for Outcomes in PDCognition (SCOPA-COG), Unified Parkinson's Disease Rating scale (motor part), and Hoehn and Yahr staging. Results: Young-onset group comprised 20 (33.3%) and late-onset group comprised 40 (66.7%) patients. Most of the young- and late-onset patients, 15 (75%) and 21 (52.5%), had SCOPA-COG score in the range of 30–39, respectively. On comparison between young- and late-onset groups, SCOPA-COG score's mean ± standard deviation (SD) for young and late onset was 32.60 ± 2.52 and 30.30 ± 3.65, respectively, with statistical significance (P = 0.01). SCOPA-COG score's mean ± SD for mild, moderate, and severely impaired PD patients was 31.48 ± 3.19, 30.60 ± 3.24, and 23.50 ± 3.53, respectively, which on group comparisons (ANOVA) were statistically significant (P = 0.004). However, the SCOPA-COG score was statistically insignificant with respect to disease duration. Conclusion: There was statistically significant difference in SCOPA-COG score between young- and late-onset PD patients and in patients with more severe motor impairment.

Surgery is an established and increasingly utilized treatment option in medically refractory temporal lobe epilepsy. Many psychiatric problems are known to complicate in the postoperative period. Most studies have a follow-up period of less than 24 months. We report the cases of three patients who developed severe psychiatric problems in the late postoperative period after successful temporal lobectomy for refractory epilepsy - Psychosis, major depression with psychosis, and severe anxiety disorder, respectively. None of the patients had past or family history of psychiatric disease. All three patients had undergone anterior temporal lobectomy on the right side for intractable epilepsy. They remained absolutely seizure-free after surgery. We conclude that psychiatric morbidity may arise de novo long after temporal lobectomy. This association between temporal lobectomy for epilepsy and late onset psychiatric morbidity should be carefully studied. Mechanisms underlying this late complication require deeper understanding of the effects of epilepsy surgery.

Background: Since a majority of population in India does not drive automobiles, one item on the Epworth Sleepiness Scale (ESS) requires modification and validation. In addition, data collected by us indicated that a majority of rural and urban Indians regularly spend time in prayer/spiritual activity. The main purpose of this study was to develop a cross-cultural adaptation of the ESS for a North Indian population, in Hindi language (ESS-I). The study also provides evidence of reliability and validity of the modified version. Methodology: The subjects included were normal volunteers aged 18-75 years (Group 1) (n = 70), compared with patients with complaints of excessive daytime sleepiness, who had undergone polysomnography (Group 2) (n = 22) and patients who had undergone multiple sleep latency test (Group 3) (n = 10). The study was carried out in four phases: Translation and retranslation of the original scale with modification of item 8 (mainly addition of option of question on "while offering prayers or in spiritual activity"); reliability (test-retest) (n = 30); internal consistency (using Cronbach′s alpha index) (n = 102); and sensitivity to change (n = 8). Results: Group 1 showed spiritual activity as a significantly more commonly practiced activity than driving. The Cronbach′s alpha for the modified version was 0.892 (excellent), and this was not improved by removing the modified item. The alpha value for Group 1 versus Groups 2 and 3 was 0.667 and 0.892, respectively. The scale was reliable over time (test-retest), and it was sensitive to sleepiness change in patients with obstructive sleep apnea during treatment. Conclusion: The ESS-I, is comparable to the original scale. It is reliable, valid, and change-sensitive. It is proposed that the modified version can be very useful for detecting sleepiness among Indian population, especially those who do not drive their own vehicles.

Objective: To evaluate neuropsychiatric co-morbidities (depression, psychosis and anxiety) in non-demented patients with Parkinson′s disease (PD). Background: Non-motor symptoms like neuropsychiatric co-morbidities are common in Parkinson′s disease and may predate motor symptoms. Currently there is scarcity of data regarding neuropsychiatry manifestations in Indian patients with PD. Methods: In this cross-sectional study consecutive 126 non-demented patients with PD (MMSE ≥25) were enrolled. They were assessed using Unified Parkinson′s disease rating scale (UPDRS), Hoehn & Yahr (H&Y) stage, Schwab and England (S&E) scale of activity of daily life. Mini-international neuropsychiatric interview (MINI) was used for diagnosis of depression, psychosis and anxiety. Beck′s depression inventory (BDI), Brief psychiatric rating scale (BSRS) and Hamilton rating scale for anxiety (HAM-A) scales were used for assessment of severity of depression, psychosis and anxiety respectively. Results: Mean age and duration of disease was 57.9 ± 10.9 years and 7.3 ± 3.6 years respectively. At least one of the neuropsychiatric co-morbidity was present in 64% patients. Depression, suicidal risk, psychosis and anxiety were present in 43.7%, 31%, 23.8% and 35.7% respectively. Visual hallucinations (20.6%) were most frequent, followed by tactile (13.5%), auditory (7.2%) and olfactory hallucinations (1.6%). Patients with depression had higher motor disability (UPDRS-motor score 33.1 ± 14.0 vs 27.3 ± 13.3; and UPDRS-total 50.7 ± 21.8 vs 41.0 ± 20.3, all p values

Background: There is limited data on the prevalence of impulse control disorder and related behaviors (ICD-RBs) in Indian patients with Parkinson′s Disease (PD). In the context of potential genetic and environmental factors affecting the expression of ICD-RBs, studying other multiethnic populations may bring in-sights into the mechanisms of these disorders. Objectives: To ascertain point prevalence estimate of ICD-RBs in Indian PD patients, using the validated “Questionnaire for Impulsive-Compulsive Disorders in Parkinson′s disease (QUIP)” and to examine their association with Dopamine replacement therapy (DRT). Materials and Methods: This was a hospital based observational cross-sectional study. After taking informed consent, patients and their informants (spouse, or primary caregiver) were made to complete the QUIP, and were instructed to answer questions based on behaviors that occurred anytime during PD that lasted at least four consecutive weeks. Results: Total of 299 patients participated in the study. At least one ICD-RB was present in 128 (42.8%), at least one Impulse control disorder (ICD) was present in 74 (24.75%) and at least one Impulse control related compulsive behaviour (ICRB) was present in 93 (31.1%) patients. Punding was the most frequent (12.4%) followed by hyper sexuality (11.04%), compulsive hobbyism (9.4%), compulsive shopping (8.4%), compulsive medication use (7.7%), compulsive eating (5.35%), walkabout (4%) and pathological gambling (3.3%). ≥ 2 ICD-RBs were observed in 15.7% of patients. After multivariate analysis, younger age of onset, being unmarried were specifically associated with presence of ICD. Longer disease duration was specifically associated with presence of ICRB. Whereas smoking and higher dopamine levodopa equivalent daily doses (DA LEDD) were associated with both presence of ICD and ICRB. Higher LD LEDD was specifically associated with presence of ICD-RB. Conclusions: Our study revealed a relatively higher frequency of ICD-RBs, probably because of the use of screening instrument and because we combined both ICDs and ICRBs. Also high proportion of DA use (81.6%) among our patients might be responsible. The role of genetic factors that might increase the risk of developing ICD-RBs in this population needs further exploration.

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.

Patients with restless leg syndrome present with sensory symptoms similar to peripheral neuropathy. While there is evidence of abnormalities of dopaminergic pathways, the peripheral nervous system has been studied infrequently. We studied conventional nerve conduction studies, quantitative thermal sensory testing and sympathetic skin response in 57 patients with primary restless leg syndrome. Almost two third patients demonstrated abnormalities in the detailed testing of the peripheral nervous system. Sbtle abnormalities of the peripheral nervous system may be more common than previously believed.

Context: Few studies have been done to see the level of knowledge among patients and caregivers about Parkinson′s disease (PD). Aims: The aim of the current study was to determine the knowledge of PD among patients and caregivers at a movement disorder clinic in India. Settings and Design: A tertiary care neurology facility in north India. Materials and Methods: We conducted a questionnaire based interview among the subjects collected on the annual PD day in 2006. Results: Out of 200 questionnaires that were distributed 172 subjects responded. Of these, there were 103 (59.8%) patients with PD and 69 (40.11%) caregivers. Mean age of the patients and caregivers was 55.4 ± 13.3 years and 49.4 ± 15.9 years respectively. Mean duration of PD was 6.8 ± 4.7 years (range-21). Ninety nine patients out of the 103 (96.1%) and 57 caregivers out of 69 (82.6%) had previously attended the PD education program. High scores (>90%) were obtained to questions on body parts affected, pathology in PD, main drug treatment, epidemiology and effect of exercise. Low scores were seen to questions on surgery in PD and biochemical abnormality in PD. No significant difference was noted in correct answers among patients and caregivers on duration of PD. Conclusion: Patients and caregivers had adequate knowledge about PD. Patient and caregiver education programs may be useful in imparting knowledge about PD.

We report a rare case of neurodegeneration with brain iron accumulation (NBIA) presented with episodic inspiratory stridor. A 10-year-old boy presented with 3-year history of gradually progressive spastic gait and generalized dystonia (involving all four limbs, neck, jaw, and speech). MRI brain showed "Eye of Tiger" sign. He recently developed severe inspiratory stridor associated with almost gasping respiration. Direct video laryngoscopy showed paradoxical vocal cord closure during inspiration. He was treated with EMG-guided botulinum toxin-A injection given into bilateral thyroarytenoid muscles, resulting in dramatic response with complete disappearance of the stridor within a week. The effect lasted 18 months.