Annals of Indian Academy of Neurology最新文献

{"title":"Comparison of Clinical and Electrophysiologic Characteristics of Peripheral Neuropathy in Progressive Supranuclear Palsy and Parkinson's Disease: An Observational Study.","authors":"Yumkham M Devi, Mritunjai Kumar, Ashutosh Tiwari, Minakshi Dhar, Sweety Kumari, Niraj Kumar","doi":"10.4103/aian.aian_512_24","DOIUrl":"https://doi.org/10.4103/aian.aian_512_24","url":null,"abstract":"<p><strong>Background and objectives: </strong>Although widely described in Parkinson's disease (PD), peripheral neuropathy (PNP) is scarcely reported in progressive supranuclear palsy (PSP). We aimed to compare the frequency, clinical and electrophysiologic characteristics of PNP in PSP and PD patients.</p><p><strong>Methods: </strong>This cross-sectional study included 23 PSP and 93 PD patients. Demographic data, Movement Disorders Society-Unified Parkinson's Disease Rating Scale-III (MDS-UPDRS-III), Hoehn-Yahr staging, Toronto Clinical Neuropathy Score, nerve conduction study (NCS), and sympathetic skin response (SSR) were recorded. Diagnosing isolated large fiber neuropathy required abnormal NCS. Isolated small fiber neuropathy required clinical findings of pinprick and thermal sensory loss and/or allodynia and/or hyperalgesia with/without impaired SSR, along with normal NCS.</p><p><strong>Results: </strong>PNP was commoner in PSP than PD (65.2% vs. 50.5%, P = 0.21). While a comparable proportion in both groups had clinical neuropathy, NCS abnormalities predominated in PSP (65.2% vs. 39.8%, P = 0.03). All patients had distal symmetrical axonal polyneuropathy. A significantly higher proportion of PSP patients had large fiber involvement (65.21% vs. 39.78%, χ2 = 4.82; P = 0.03) and mixed fiber PNP (60.9% vs. 33.3%, P = 0.01). PSP patients with neuropathy had a significantly shorter disease duration [median (interquartile range {IQR} = 2 (2-3) years vs. 6 (3-9) years, P < 0.001], higher MDS-UPDRS-III score [median (IQR) = 47 (36-54) vs. 34 (28-49), P = 0.049], higher Hoehn-Yahr stage [median (IQR) = 4 (2-5) vs. 3 (1-5), P < 0.001], and shorter duration of levodopa use [median (IQR) = 2 (1-2) years vs. 3.5 (2-5) years, P = 0.006]. NCS parameters were comparable between PSP and PD patients with neuropathy. While PNP in PSP was not associated with any of the clinical variables, a longer disease duration was independently associated with PNP in PD.</p><p><strong>Conclusions: </strong>PNP affected two-thirds of PSP patients and was more prevalent than PD. Both groups had distal symmetrical axonal polyneuropathy, with mixed fiber PNP predominating in PSP. A longer disease duration in PD was associated with PNP.</p>","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2024-12-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142783510","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neimann-Pick Disease Presenting as a Case of Severe Dementia and Seizures.","authors":"Greeshmendu Rajan, Mary Iype, Mini Sreedharan, Shahanaz Ahamed, Sankar V Hariharan, Reshmi Raveendran, Roshni R Lal, Priyanka Sahu, Shama Perween, Neeraja Beena","doi":"10.4103/aian.aian_601_24","DOIUrl":"https://doi.org/10.4103/aian.aian_601_24","url":null,"abstract":"","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2024-12-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142778934","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"MRI-Negative Central Nervous System SLE: A Unique Case Report.","authors":"Deepti Grover, Himanshu Shakya, Ashwin Kumar Panda, Sourav Hazra, Aldrin Anthony Dung Dung, Suman Kushwaha, Rajinder K Dhamija","doi":"10.4103/aian.aian_654_24","DOIUrl":"https://doi.org/10.4103/aian.aian_654_24","url":null,"abstract":"","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2024-12-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142778932","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Novel Presentation of Type 1 Wolfram Syndrome as Intracranial Hemorrhage and Longitudinally Extensive Transverse Myelitis: Neuroimaging and Angiographic Findings.","authors":"Ravi P Singh, S S Jayanth, K T Seetam, Vikram V Holla, H R Arvinda, M Netravathi","doi":"10.4103/aian.aian_613_24","DOIUrl":"https://doi.org/10.4103/aian.aian_613_24","url":null,"abstract":"<p><strong>Abstract: </strong>Wolfram syndrome (WS), a rare genetic neurodegenerative condition, is primarily characterized by diabetes mellitus, optic atrophy, diabetes insipidus, and sensorineural hearing loss, along with diverse systemic manifestations. Late-stage cases exhibit widespread brain atrophy. While bleeding tendencies are not commonly observed in Type 2 WS (WS2), there is a distinctive bleeding tendency that is exclusive to WS2, with no documented occurrences in Type 1 WS (WS1). Extensive documentation exists regarding various neurologic manifestations of this syndrome; however, as of now, there is no reported mention of intracranial hemorrhage, a rarity within this condition.</p>","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2024-12-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142778936","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Rituximab-Induced Hypersensitivity Pneumonitis in Multiple Sclerosis: A Rare Phenomenon.","authors":"S S Jayanth, Kshiteeja Jain, Bv Murali Mohan, M Netravathi","doi":"10.4103/aian.aian_699_24","DOIUrl":"https://doi.org/10.4103/aian.aian_699_24","url":null,"abstract":"","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142765788","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Progressive Supranuclear Palsy in India: Past, Present, and Future.","authors":"Srinivas Raju, Kuldeep Shetty, Lulup Sahoo, Vijayashankar Paramanandam, Jay M Iyer, Suvorit Bowmick, Soaham Desai, Deepika Joshi, Niraj Kumar, Sahil Mehta, Rukmini Mridula Kandadai, Pettarusp Wadia, Atanu Biswas, Divyani Garg, Pankaj Agarwal, Syam Krishnan, Jacky Ganguly, Heli Shah, Mitesh Chandarana, Hrishikesh Kumar, Rupam Borgohain, V L Ramprasad, Prashanth Lingappa Kukkle","doi":"10.4103/aian.aian_515_24","DOIUrl":"https://doi.org/10.4103/aian.aian_515_24","url":null,"abstract":"<p><strong>Abstract: </strong>Progressive supranuclear palsy (PSP) has emerged as a key area of interest among researchers worldwide, including those in India, who have actively studied the disorder over the past several decades. This review meticulously explores the extensive range of Indian research on PSP up to the present and offers insights into both current initiatives and potential future directions for managing PSP within the region. Historical research contributions have spanned 80 publications from 1974 to 2023, encompassing diverse themes from clinical phenotyping and historical analysis to isolated investigative studies and therapeutic trials. Traditionally, these studies have been conducted in single centers or specific departments, involving a broad range of recruitment numbers. The most frequently encountered phenotype among these studies is PSP-Richardson's syndrome, with patients typically presenting at an average age of 64 years, alongside various other subtypes. Recently, there has been a significant shift toward more collaborative research models, moving from isolated, center-based studies to expansive, multicentric, and pan India projects. A prime example of this new approach is the PAn India Registry for PSP (PAIR-PSP) project, which represents a comprehensive effort to uniformly examine the demographic, clinical, and genetic facets of PSP across India. Looking ahead, there is a critical need for focused research on unraveling genetic insights, identifying risk factors, and developing effective treatment interventions and preventive models. Given its vast population, India's role in advancing our understanding of PSP and other tauopathies could be pivotal, and this work reflects the work on PSP in India till now.</p>","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142765733","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Symptomatic and Prophylactic Dantrolene Treatment in a Case of RYR1-Related Congenital Myopathy.","authors":"Hüseyin B Şenol, Ayşe I Polat, Adem Aydın, Ayşe S Hız, Uluç Yiş","doi":"10.4103/aian.aian_560_24","DOIUrl":"https://doi.org/10.4103/aian.aian_560_24","url":null,"abstract":"","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2024-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142613601","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Surface Electromyography for the Diagnosis of Tremor Syndrome: A Study of 97 Patients.","authors":"Kartika Gulati, Sanjay Pandey","doi":"10.4103/aian.aian_553_24","DOIUrl":"https://doi.org/10.4103/aian.aian_553_24","url":null,"abstract":"<p><strong>Background and objectives: </strong>Tremor is one of the most frequent movement disorders encountered in clinical practice with heterogeneous phenomenology and etiology. Surface electromyography (SEMG) is a noninvasive and reproducible test that can diagnose tremor syndromes.</p><p><strong>Methods: </strong>In this retrospective study, the clinical and electrophysiologic records of 97 consecutive patients with tremor syndromes who visited our movement disorder clinic between January 2023 and March 2024 were examined.</p><p><strong>Results: </strong>In our study, 28.8% (n = 28) of patients were of essential tremor (ET) syndrome. SEMG of ET syndrome patients showed synchronous bursts in 71.4% (n = 20), alternating bursts in 10.7% (n = 3), synchronous bursts with co-contraction of agonist and antagonist muscles in 10.7% (n = 3), and both synchronous and alternating bursts in 3.6% (n = 1) of patients. Tremor-ataxia syndrome formed 21.6% (n = 21) of our study population, with 71.4% (n = 15) of patients showing synchronous bursts and co-contraction and 28.6% (n = 6) patients having alternating bursts. Moreover, 12.3% (n = 12) patients were of parkinsonian tremor, of whom alternating bursts were present in 75% (n = 9) and synchronous bursts with co-contraction were present in 25% (n = 3) of patients. In addition, 11.3% (n = 11) of patients had dystonic tremor (DT), of whom 81.8% (n = 9) had synchronous bursts and co-contraction and 18.2% (n = 2) had alternating bursts.</p><p><strong>Conclusions: </strong>Synchronous bursts with co-contraction suggestive of DT were observed in most patients with tremor-ataxia syndrome and a small number of patients with ET syndrome. Our data suggests that SEMG helps differentiate these two clinical syndromes, which is difficult in a clinical setting.</p>","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":"27 6","pages":"690-694"},"PeriodicalIF":1.9,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142871000","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Starry Sky Appearance on Diffusion Imaging in Chikungunya Encephalitis.","authors":"Nirmal Pandey, Saket Nigam, Sachin Khanna, Niti Luthra","doi":"10.4103/aian.aian_363_24","DOIUrl":"10.4103/aian.aian_363_24","url":null,"abstract":"","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":"717-719"},"PeriodicalIF":1.9,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142339804","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Case of Facial-Onset Sensory Motor Neuronopathy - A Rare Variant of Motor Neuron Disease.","authors":"S Pramod Kumar, S Haritha, P R Sowmini, S Lakshmanan, S Sakthi Velayutham, K Malcolm Jeyaraj, V Kannan, R Viveka Saravanan, K Mugundhan","doi":"10.4103/aian.aian_280_24","DOIUrl":"10.4103/aian.aian_280_24","url":null,"abstract":"","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":"754-757"},"PeriodicalIF":1.9,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142139096","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}