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Objective:To determine the demographic pattern of juvenile-onset parkinsonism (JP, Materials and Methods:We conducted a 2-year, pan-India, multicenter collaborative study to analyze clinical patterns of JP, YOPD, and EOPD. All patients under follow-up of movement disorders specialists and meeting United Kingdom (UK) Brain Bank criteria for PD were included.Results:A total of 668 subjects (M:F 455:213) were recruited with a mean age at onset of 38.7 ± 8.1 years. The mean duration of symptoms at the time of study was 8 ± 6 years. Fifteen percent had a family history of PD and 13% had consanguinity. JP had the highest consanguinity rate (53%). YOPD and JP cases had a higher prevalence of consanguinity, dystonia, and gait and balance issues compared to those with EOPD. In relation to nonmotor symptoms, panic attacks and depression were more common in YOPD and sleep-related issues more common in EOPD subjects. Overall, dyskinesias were documented in 32.8%. YOPD subjects had a higher frequency of dyskinesia than EOPD subjects (39.9% vs. 25.5%), but they were first noted later in the disease course (5.7 vs. 4.4 years).Conclusion:This large cohort shows differing clinical patterns in JP, YOPD, and EOPD cases. We propose that cutoffs of

Clinical practice guidelines for Wilson's disease (WD) have been published by the American Association for the Study of Liver Diseases and European Association for the Study of the Liver in 2008 and 2012, respectively. Their focus was on the hepatic aspects of the disease. Recently, a position paper on pediatric WD was published by the European Society of Pediatric Gastroenterology Hepatology and Nutrition. A need was felt to harmonize guidelines for the hepatic, pediatric, and neurological aspects of the disease and contextualize them to the resource-constrained settings. Therefore, experts from national societies from India representing 3 disciplines, hepatology (Indian National Association for Study of the Liver), pediatric hepatology (Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition), and neurology (Movement Disorders Society of India) got together to evolve fresh guidelines. A literature search on retrospective and prospective studies of WD using MEDLINE (PubMed) was performed. Members voted on each recommendation, using the nominal voting technique. The Grades of Recommendation, Assessment, Development and Evaluation system was used to determine the quality of evidence. Questions related to diagnostic tests, scoring system, and its modification to a version suitable for resource-constrained settings were posed. While ceruloplasmin and 24-h urine copper continue to be important, there is little role of serum copper and penicillamine challenge test in the diagnostic algorithm. A new scoring system – Modified Leipzig score has been suggested with extra points being added for family history and serum ceruloplasmin lower than 5 mg/dl. Liver dry copper estimation and penicillamine challenge test have been removed from the scoring system. Differences in pharmacological approach to neurological and hepatic disease and global monitoring scales have been included. Rising bilirubin and worsening encephalopathy are suggested as indicators predicting need for liver transplant but need to be validated. The clinical practice guidelines provide recommendations for a comprehensive management of WD which will be of value to all specialties.

Clinical practice guidelines for Wilson's disease (WD) have been published by the American Association for the Study of Liver Diseases and European Association for the Study of the Liver in 2008 and 2012, respectively. Their focus was on the hepatic aspects of the disease. Recently, a position paper on pediatric WD was published by the European Society of Pediatric Gastroenterology Hepatology and Nutrition. A need was felt to harmonize guidelines for the hepatic, pediatric, and neurological aspects of the disease and contextualize them to the resource-constrained settings. Therefore, experts from national societies from India representing 3 disciplines, hepatology (Indian National Association for Study of the Liver), pediatric hepatology (Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition), and neurology (Movement Disorders Society of India) got together to evolve fresh guidelines. A literature search on retrospective and prospective studies of WD using MEDLINE (PubMed) was performed. Members voted on each recommendation, using the nominal voting technique. The Grades of Recommendation, Assessment, Development and Evaluation system was used to determine the quality of evidence. Questions related to diagnostic tests, scoring system, and its modification to a version suitable for resource-constrained settings were posed. While ceruloplasmin and 24-h urine copper continue to be important, there is little role of serum copper and penicillamine challenge test in the diagnostic algorithm. A new scoring system - Modified Leipzig score has been suggested with extra points being added for family history and serum ceruloplasmin lower than 5 mg/dl. Liver dry copper estimation and penicillamine challenge test have been removed from the scoring system. Differences in pharmacological approach to neurological and hepatic disease and global monitoring scales have been included. Rising bilirubin and worsening encephalopathy are suggested as indicators predicting need for liver transplant but need to be validated. The clinical practice guidelines provide recommendations for a comprehensive management of WD which will be of value to all specialties.

Although subcutaneously administered apomorphine is widely used as an effective adjunct therapy for Parkinson’s disease (PD), it was approved for use in India only in 2019. This review summarizes the history, pharmacology, clinical spectrum, indications, efficacy, and side effects of subcutaneous apomorphine use in patients with PD along with clinical recommendations for its use in Indian context. Intermittent subcutaneous apomorphine injection or continuous subcutaneous apomorphine infusion is effective adjunctive treatments for patients with advanced PD with levodopa-related refractory motor complications and some specific nonmotor symptoms (NMS) as growing evidence shows apomorphine also improves aspects of NMS of PD. Common side effects of subcutaneous apomorphine are skin nodules, nausea, and somnolence with incidence being higher with infusion than that with injection. Impulse control disorders and neuropsychiatric complications common to most dopamine agonists can also occur. As per National Institute for Health and Care Excellence (NICE), United Kingdom, apomorphine, as intermittent injection or continuous subcutaneous infusion, is one of the best medical therapies and may be considered before using deep brain stimulation (DBS) or levodopa/carbidopa intestinal gel (LCIG). Head-to-head open-label comparative multicenter data suggest that apomorphine is at least as effective as DBS or LCIG in relation to nonmotor and motor benefit. More studies are needed to reduce the paucity of apomorphine data in Indian population. We also discuss criteria to select one device therapy over another and newer apomorphine delivery strategies in the pipeline.

Background: Multiple system atrophy (MSA) is a neurodegenerative disease leading to severe physical impairment, with a disease duration from onset to death of 6–9 years. Methods: The clinical and neuropathological features of 4 MSA cases with disease duration of 15 years or more were analyzed. Results: All patients presented with parkinsonism and had a mean latency of 11 years before the development of dysautonomia. Mean duration from onset of first symptoms to anterocollis, inspiratory stridor, and dysphagia was 9 years. Despite the limited levodopa response, all patients developed levodopa-induced dyskinesia. Conclusions: Late appearance of dysautonomia is a favorable prognostic factor in MSA-P. Greater awareness of this uncommon “benign” subgroup of MSA will improve diagnostic accuracy and help to more accurately inform treatment options. © 2012 Movement Disorder Society

Movement Disorders are currently growing to be one of the major subspecialties of neurology worldwide, primarily by developments of research and therapeutics in this field. As any specialty grows, it leads to the formation of its society and the publication of journal to disseminate the knowledge. The International Parkinson’s Disease and Movement Disorders Society (IPMDS, formerly known as Movement Disorders Society) played its role with inception since 1980s. Further development of Movement Disorders subspecialty leads each region and country to have their own national societies and publications. Similarly, the seeds of Movement Disorders were sown in India in mid-1980s but it took a major stride in last few years with the formation of Movement Disorders Society of India (MDSI) in 2014 and following this, it is now at the crux of starting its journal—Annals of Movement Disorders (AOMD). This would be a right time to look back into the history of Indian Neurology with specific reference to the Movement Disorders and pen down these chronicles since the inception of modern neurology in India. The current chronicles of Indian Movement Disorders is penned based on personal interviews with various senior Movement Disorder specialists of India. However, it should also be remembered that many of the chronicles are limited by the memories of people and their biases with whom the interviews are conducted.

In advanced Parkinson´s disease (PD), axial symptoms such as speech, gait, and balance impairment often become levodopa-unresponsive and they are difficult to manage, even in patients with subthalamic nucleus deep brain stimulation (STN-DBS). We anecdotally observed that oral administration of amantadine was very effective in treating both residual and stimulation-induced axial symptoms after bilateral STN-DBS in one PD patient. Therefore, we conducted a prospective multicenter observational study to evaluate the effects of amantadine on speech, gait and balance in PD patients with STN-DBS and incomplete axial benefit. Primary outcomes were changes in speech (UPDRS III, item 18), gait (item 29) and postural stability (item 30) with amantadine treatment compared to baseline. Secondary outcome was the patients´ subjective scoring of axial symptoms with amantadine compared to baseline. Forty-six PD patients with STN-DBS were enrolled in the study and followed for 10.35 ± 8.21 months (median: 9.00; range: 1-31). The mean daily dose of amantadine was 273.44 ± 47.49 mg. Gait scores significantly improved (from 1.51 ± 0.89 to 1.11 ± 0.92, P = 0.015) with amantadine treatment, whereas postural stability and speech scores were similar before and after treatment. Thirty-five (76.1%) patients reported subjective improvement in speech, gait or balance with amantadine, whereas thirty (65.2%) patients reported improvement in gait and balance. In conclusion, our data suggest that amantadine may have new beneficial effects on axial symptoms in PD patients with STN-DBS. Fil: Chan, Hiu Fai. University of Toronto; Canadá. Queen Elizabeth Hospital; Hong Kong Fil: Kukkle, Prashanth L.. University of Toronto; Canadá Fil: Merello, Marcelo Jorge. Fundación para la Lucha contra las Enfermedades Neurológicas de la Infancia; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina Fil: Lim, Shen Yang. University of Malaya; Malasia Fil: Poon, Yu Yan. University of Toronto; Canadá Fil: Moro, Elena. Universite Joseph Fourier; Francia

Multiple system atrophy (MSA) is a neurodegenerative disease leading to severe physical impairment, with a disease duration from onset to death of 6-9 years.The clinical and neuropathological features of 4 MSA cases with disease duration of 15 years or more were analyzed.All patients presented with parkinsonism and had a mean latency of 11 years before the development of dysautonomia. Mean duration from onset of first symptoms to anterocollis, inspiratory stridor, and dysphagia was 9 years. Despite the limited levodopa response, all patients developed levodopa-induced dyskinesia.Late appearance of dysautonomia is a favorable prognostic factor in MSA-P. Greater awareness of this uncommon "benign" subgroup of MSA will improve diagnostic accuracy and help to more accurately inform treatment options.