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Background and Objectives: Working memory (WM) impairment is a common and progressive abnormality in Parkinson’s disease (PD), which ultimately leads to dementia. Individuals with PD exhibit altered electroencephalogram (EEG) patterns while engaging in tasks that require WM. The study examined the EEG patterns associated with WM maintenance in PD patients. Methods: The study consisted of two groups of PD patients with a sign of dementia (PDD) who had a Mini Mental State Examination (MMSE) score ≤24, PD patients with no sign of dementia (PDND) with an MMSE score >24, and a group of healthy individuals serving as controls (CON). We obtained EEG during a word memorization task using a 128-channel system. The spectral power of the EEG was computed using the fast Fourier transformation algorithm. Results: Compared to both PDND and CON groups, PDD group exhibited higher power in the delta and beta frequency bands. Conversely, there was a notable decrease in power in the gamma band in both the PDD and PDND groups compared to the CON group. In addition, the PDD group exhibited higher power in the gamma band compared to the PDND group. Power in the alpha (LA1, LA2, and UA) and theta bands was greater in PDND and PDD compared to CON, but was similar between PDND and PDD groups. Conclusion: While performing WM tasks, PDD exhibits increased delta and beta power. These findings can be used as a quantitative biomarker to evaluate the severity of initial WM impairments, which could potentially develop into dementia in the future.

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.

Abstract Growing evidence suggests that non-motor symptoms (NMS) in Parkinson’s disease (PD) have differential progression patterns that have a different natural history from motor progression and may be geographically influenced. We conducted a cross-sectional analysis of 1607 PD patients of whom 1327 were from Europe, 208 from the Americas, and 72 from Asia. The primary objective was to assess baseline non-motor burden, defined by Non-Motor Symptoms Scale (NMSS) total scores. Other aims included identifying the factors predicting quality of life, differences in non-motor burden between drug-naïve and non-drug-naïve treated patients, and non-motor phenotypes across different geographical locations. Mean age was 65.9 ± 10.8 years, mean disease duration 6.3 ± 5.6 years, median Hoehn and Yahr stage was 2 (2–3), and 64.2% were male. In this cohort, mean NMSS scores were 46.7 ± 37.2. Differences in non-motor burden and patterns differed significantly between drug-naïve participants, those with a disease duration of less than five years, and those with a duration of five years or over (p ≤ 0.018). Significant differences were observed in geographical distribution (NMSS Europe: 46.4 ± 36.3; Americas: 55.3 ± 42.8; Asia: 26.6 ± 25.1; p

Context: Appropriate mitochondrial function and oxidative balance are critical to neuronal survival. Accumulation of reactive oxygen species leads to oxidative stress that can cause free radical damage to biomolecules of the cell components and the molecules in the cellular milieu that eventually lead to a variety of chronic diseases including neurodegenerative disorders. Mitochondrial dysfunction initiates neuronal apoptosis thereby leading to neurodegenerative diseases including Parkinson's disease (PD). Aim: To evaluate oxidative stress vis-a-vis mitochondrial function (Cytochrome C oxidase activity) in PD patients, Parkinson plus syndrome (PPS) patients in comparison with healthy controls (HCs). Settings and Design: Cross-sectional Study Methods: We assessed oxidative stress by chemiluminescence using luminol, and cytochrome c oxidase activity (CCO) by CCO kit using spectrophotometry in PD patients (n = 80), PPS patients (n = 40), and HCs (n = 40). Statistical Analysis: Data were presented as number (%) or mean ± SD/median as approximate. Quantitative baseline variables were compared among the groups using one-way ANOVA and qualitative variables were compared using Chi-square test. The difference in median was compared using Kruskal–Wallis test followed by Post-hoc Bonferronni correction. Results: Compared to HCs (Median 7.53 ± 15.58 RLU/sec/cell), ROS level in PD (14.13 ± 29.5), and PPS (17.43 ± 15.91) patients was significantly higher (P = 0.0029: HC vs, PD & P = 0.0500: HC vs. PPS). Also, ROS in PD patients (14.13 ± 29.5) was higher that PPS patients (17. 43 ± 15.91) but the difference was not statistically significant (P = 0.84). The CCO activity was found to be diminished in PD (Median: 0.025 ± 0.013 units/ml) and PPS patients (0.027 ± 0.008) in comparison to HCs (0.117 ± 0.049). Conclusion: Mitochondrial dysfunction and oxidative stress is associated with PD and PPS and may play an important role in etiopathogenesis. Though the cause–effect conundrum has not been comprehensively probed but addressing oxidative stress and mitochondrial damage may serve as an adjunctive therapy for PD and PPS. Iron metabolism as reflected in the red cell indices may aid in differentiating PD from PPS.

Background: Pain is a common symptom in Parkinson's disease (PD) patients. Scales to rate pain in PD are marred by several flaws, either not being available in other languages or not specific for PD. Objectives: To assess the frequency of pain among bilingual Indian PD patients using “King's Parkinson's disease pain scale” (KPPS) and to validate it. Methods: We randomly administered KPPS in Hindi/English to all consecutive bilingual persons with PD. The results were appropriately analyzed. Results: A total of 119 PD patients were enrolled with a mean age of 64.34 (± 9.57) years. Median Hoehn and Yahr stage was 2 (42.85%). Pain was present in 62 (52.1%) PD patients. The most common type was musculoskeletal (74.19%). The mean total KPPS score was 16.02 ± 10.57. KPPS score was significantly higher in women and correlated positively with unified Parkinson's disease rating scale (UPDRS) part 2 and 4 scores (r = 0.27 and r = 0.25). Risk factors for pain were female gender, higher H and Y stage, total UPDRS score, and individual UPDRS part 3 and 4 scores. Difficulty falling asleep (P = 0.01), frequent awakenings (P = 0.01), diminished smell sensation (P = 0.003), diminished speech volume (P = 0.02), gait freezing (P = 0.03), and falls (P = 0.001) correlated with the presence of pain. The interclass correlation coefficient between the Hindi and English versions of KPPS was 0.835, while Bland–Altman analysis showed 96.7% agreement suggesting excellent correlation and validation. Conclusions: KPPS is an easy tool for characterization, scoring, and follow-up of pain in PD patients. The Hindi version has good agreement with the original English version.

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.

Background: Parkinson’s disease (PD) is a chronic neurodegenerative disease with appearance of dementia as the disease progresses. Dementia in PD worsens the quality of life and poses a burden on caregivers. Objective markers of the presence and as well as onset of dementia needs to be explored to improve the quality of life.We attempted to identify EEG abnormalities that differentiate PD patients with or without dementia using high density EEG. The differential changes in the EEG spectral activity could mark the early stage as well as onset of dementia in Parkinson’s disease (PD). Methods: The present study was designed to find out the resting eyes-closed (EC) EEG characteristics in PD with dementia (PDD) and without dementia (PDND) compared to healthy controls (CON) of both gender using high density EEG. Absolute power of seven frequency bands based on individual alpha frequency was estimated by Fast Fourier transform algorithm in 58 PD patients (30 PDND and 28 PDD) and 26 CON. Results: Compared to CON, PDND had higher power in theta and lower alpha1 bands while PDD had higher power in delta, theta, lower alpha1 and beta bands. Higher delta and gamma power with no difference in theta and lower alpha 1 power was the characteristic feature of PD patients with dementia compared to non-dementia. Conclusions: This cross-sectional study proposes to use these differential changes in power as EEG signatures of appearance of dementia in PD. Absence of high delta power, but presence of high theta and lower alpha 1 power defined PDND group compared to CON.

OBJECTIVE: To elucidate the effect of step-synchronized vibration training using PDShoe on balance, gait, and quality of life in patients of Parkinson’s disease (PD) with freezing of gait (FOG).MATERIALS AND METHODS: In a pilot study, 17 patients of PD with FOG were recruited for step-synchronized vibration training. The training involved 10 sessions of gait training over 2 weeks. Each session included three 6-min bouts of walking with step-synchronized vibration applied to the second metatarsal head and medial surface of calcaneus. Participants were assessed with the Unified Parkinson’s Disease Rating Scale-III (UPDRS III), Timed Up and Go (TUG) test, Berg Balance Scale (BBS), Timed 10-Meter Walk Test, Freezing of Gait Questionnaire (FOG-Q), Falls Efficacy Scale-International (FES-I), and Parkinson’s Disease Questionnaire-39 (PDQ-39). Measurements were collected pre-intervention, post-intervention, and at a 2-week follow-up. Friedman test followed by Wilcoxon signed-rank test were used for statistical analysis.RESULTS: All participants completed the intervention without any adverse effects. Fifteen participants reported for a follow-up evaluation 2 weeks after intervention. There was statistically significant improvement in UPDRS III (P = 0.044) and significant improvement in TUG test (P = 0.005), BBS (P = 0.026), FES-I (P = 0.041), and PDQ-39 (P = 0.021) scores between pre and follow-up evaluations. No significant changes were seen in FOG-Q and Timed 10-Meter Walk Test.CONCLUSION: Step-synchronized vibration is a novel intervention to improve balance, gait, motor features, and quality of life in patients of PD with FOG. Further research is warranted to confirm the results found in this pilot study.

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.

Objectives: Stress, anxiety, and depression are known to be associated with the development of neurodegenerative disorders through interactions with the underlying pathophysiology. We hypothesized that the presence of these symptoms contributes to cognitive disturbances and dementia in Parkinson's disease (PD). The present study aimed to investigate the levels of stress, anxiety, and depression in PD patients relative to healthy individuals. Materials and Methods: Anxiety, stress, and depression levels were assessed using standardized questionnaires in PD without dementia (PDND, n = 30), PD with dementia (PDD, n = 28), and healthy controls (HC, n = 26). Arithmetic subtraction task was used as a stressor. Galvanic skin response, heart rate and salivary cortisol, and alpha-amylase were measured during baseline and after induced stress (arithmetic task). Results: Acute anxiety, acute stress, and depression levels were significantly higher in PDND compared to HC, whereas both acute and chronic anxiety, stress, and depression levels were significantly higher in PDD compared to PDND and HC. Cortisol and alpha-amylase levels were significantly higher in PDND compared to HC during both baseline and postarithmetic task. Posttask levels of cortisol were lower in PDD compared to PDND. Conclusion: This study concludes that higher levels of salivary cortisol and alpha-amylase at baseline and poststress task with normal levels of chronic stress and anxiety were associated with no dementia in PD. Presence of higher levels of acute, chronic anxiety, and stress along with depression with lower cortisol reactivity to stressor suggests onset of dementia in Parkinson's patients.

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 =

Atypical Parkinson syndromes (APSs) often have symptoms that overlap with those of Parkinson’s disease (PD), especially early in the disease, making these disorders difficult to diagnose. Previous studies have demonstrated an association of oligomeric α-synuclein (α-Syn), a key element in the pathogenesis of PD, with Sirtuin (SIRT)2 proteins for modulating PD. We aimed to evaluate SIRT protein expression in serum of PD patients and compare it with APSs and normal elderly control (GC) and to correlate this with α-Syn. SIRT protein expression was evaluated in sera of 68 PD; 34 APS and 68 GC without any neuro-psychiatric illness as controls by surface plasmon resonance (SPR). SIRT2 expression was correlated with α-Syn in PD and GC. Significant (p < 0.0001) differences were observed between serum SIRT2 concentration in PD and APS and GC as well as between APS and GC. Receiver operating characteristic (ROC) analysis revealed the strong cut-off value to differentiate PD from APS and GC and also APS from GC. Significant correlation was observed among SIRT2 levels in early PD patients with Unified Parkinson’s Disease Rating Scale (UPDRS), Hoehn & Yahr (H & Y) and increased duration of disease. In addition, a strong positive correlation of SIRT2 with α-Syn (p < 0.0001) was observed. However, no such difference was detected for serum SIRT1 in cases of PD and APS or for GC. The present study is the first to report elevated serum SIRT2 in PD. The study also provided a simple test to distinguish PD from APS and may have translational utility for diagnosis.

This study aimed to determine whether there is an influence of interleukin 6 ( IL-6 ) gene promoter polymorphisms on IL-6 plasma levels and its role in the development of ischemic stroke in young Indians. One hundred young patients with ischemic stroke (age ≥ 45 years) and equal number of age- and sex-matched controls were genotyped for 174G>C, −572G>C, and −597G>A promoter polymorphisms by polymerase chain reaction–restriction fragment length polymorphism. Plasma IL-6 levels were measured by enzyme-linked immunosorbent assay. Plasma IL-6 levels were significantly higher in patients as compared to controls (patients: 28.61 ± 8.61 pg/mL, controls: 7.60 ± 4.10 pg/mL, P = .001). Both −174G>C (allelic χ 2 / P value: 4.79/.028, genotypic χ 2 / P value: 5.3/.021) and −572G>C (allelic χ 2 / P value: 9.63/.00113 Genotypic χ 2 / P value: 74/.0002) polymorphisms exhibited genotypic as well as allelic significant association with the disease phenotype. Comparison was made between patients and controls for all 3 polymorphisms using a recessive model with respect to plasma IL-6 levels; no polymorphism showed any significant correlative association with the increased IL-6 levels ( P = .31, .51, .32). Interleukin 6 is an inflammatory marker that is considerably influenced by nongenetic factors and is not a good candidate gene for studying genetic components associated with ischemic stroke. It seems that the variability in IL-6 levels is an integrated effect of nongenetic influences and the inflammatory events that follow ischemic stroke instead of being its cause. It is suggested that there is no direct association between −174G>C, −572G>C, and −597G>A polymorphisms and elevated IL-6 levels in the development of ischemic stroke.

Use of complementary and alternative medicine (CAM; 補充與替代醫學 bǔ chōng yǔ tì dài yī xué) in Parkinson disease (PD) ranged 40–70%. The objective of this study was to determine the frequency, types and factors associated with the use of CAM in Indian PD patients. PD patients, fulfilling UKPD-Society brain-bank diagnostic-criteria, attending Movement-disorders clinic of a tertiary-care teaching hospital in India from 1st May to 15th December 2012 were enrolled. Information on socio-demographic, clinical data and treatment along with factors (source of information, benefits, harms, reason for use and cost) associated with CAM use were recorded. Out of 233 consecutive PD patients, 106 (46%) used CAM. Mean ± SD age of CAM users was 56 ± 11.2 years. Among CAM users, 72% were males, with mean age-onset 49 ± 11.16 years (P = 0.042) and 73% receiving levodopa therapy (p = 0.006). Longer duration PD, higher education (graduates and above), urban residence, and fairly good perceived health were other factors seen among CAM users. Reasons for using CAM were ‘feel good factor’ (73%), 9% took CAM due to side effects from allopathic-medicines. Commonly used CAM were Ayurvedic, homeopathic medicines, and acupuncture (針灸 zhēn jiǔ) [74/106 (70%)]. Median CAM cost in Indian Rupees (INR) was 1000/month (USD16, range: 0-400USD/month in year 2012). Almost half of PD patients use CAM. Three-quarters of Indian CAM using PD patients believe that CAM is harmless, using it at a substantial cost. CAM-users are educated, young, urban dwellers, longer duration PD and receiving levodopa. Commonly used CAM was Ayurvedic, Homeopathic medicines and acupuncture.

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.

D-penicillamine interferes with elastin and collagen metabolism and produces several cutaneous and multi-systemic side-effects. We present two cases of Wilson′s disease who on long-term penicillamine therapy developed drug-induced degenerative dermopathy manifesting as skin fragility over pressure sites and cutis laxa-like changes.

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.

Introduction: Health Related Quality of Life (HRQoL) in Parkinson’s Disease (PD) lacks universally agreed definition and its components. A conceptual framework helps in understanding the essential domains and their inter-relationship while developing patient reported outcome measure. Aim: To construct a conceptual framework for developing HRQoL scale in PD. Materials and Methods: A panel of 7 experts extracted 6 major domains for measuring HRQoL in PD from literature review including 8 disease specific scales for PD, 2 books on quality of life, 5 websites, relevant articles; and content analysis of semistructured interviews of stakeholders (28 persons with PD, 6 caregivers and 9 clinicians). Extracted domains were subjected to consensus of stakeholders (7 persons with PD, 7 caregivers and 7 clinicians) on 7 point Likert scale. The panel constructed a conceptual framework and a definition of HRQoL in PD in context of available guidelines for developing patient reported outcome measures. Results: The extracted domains were physical, non motor symptom, psychological, family/social, finance and treatment domains. Median of all six domains on 7 point Likert scale was 7 and inter-quartile distance was

Introduction: Using 125Iα-Bgtx as a ligand, postsynaptic cholinergic nicotinic receptors has been identified in species. Presynaptic receptors can be defined as receptors at or near the nerve terminal that can positively or negatively modulate transmitter release, that directly or indirectly influence the probability of an action potential resulting in exocytosis. These receptors are identified by utilizing b-Bgtx. Our study involves the identification of these receptors in different regions of brain. Material and methods: The activity of postsynaptic AChR and presynaptic membrane receptor in membrane preparation and triton extract of muscle and brain tissue was determined by virtue of its high affinity for a and b-Bgtx by rapid filtration method in case of membrane preparations and ammonium sulphate precipitation method in triton extracts i.e; solubilized receptor. Results: Specific α-Bgtx binding as fmoles/mg tissue was observed in occipital lobe (6.1±0.1) parietal lobe (6±0.6), temporal lobe (5.2±1.4), cerebral cortex (6.2±0.3), hippocampus(5.6±0.2), hypothalamus (4.3±0.4) and brain stem (4.8±0.2). No specific binding of α-Bgtx was found in cerebellum and frontal lobe. β-Bgtx binding activity was present in all the above regions of brain and was in range of 4.2-4.9 fmoles/mg tissue. Presynaptic receptors also shows immunoreactivity with myasthenic sera. Conclusions: In addition to muscle, β-Bgtx binding proteins are present in each regions of the brain, while as a-Bgtx binding proteins are absent in some regions. β-Bgtx binding sites could play an important role in the pathogenesis of myasthenia gravis.

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.

X-linked adrenoleukodystrophy (X-ALD) affects the nervous system white matter and adrenal cortex secondary to mutations in the ABCD1 gene that encode the peroxisomal membrane protein. We conducted a genomic and protein expression study of susceptibility gene with its clinical and biochemical analysis. To the best of our knowledge this is the first preliminary comprehensive study in Indian population that identified novel mutations and SNPs in a relatively large group. We screened 17 Indian indigenous X-linked adrenoleukodystrophy cases and 70 controls for mutations and SNPs in the exonic regions (including flanking regions) of ABCD1 gene by direct sequencing with ABI automated sequencer along with Western blot analysis of its endogenous protein, ALDP, levels in peripheral blood mononuclear cells. Single germ line mutation was identified in each index case in ABCD1 gene. We detected 4 novel mutations (2 missense and 2 deletion/insertion) and 3 novel single nucleotide polymorphisms. We observed a variable protein expression in different patients. These findings were further extended to biochemical and clinical observations as it occurs with great clinical expression variability. This is the first major study in this population that presents a different molecular genetic spectrum as compared to Caucasian population due to geographical distributions of ethnicity of patients. It enhances our knowledge of the causative mutations of X-ALD that grants holistic base to develop effective medicine against X-ALD.

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.