Annals of Indian Academy of Neurology最新文献

{"title":"Spectrum of Neurological Manifestations of HHV-6 Encephalitis in Immunocompetent Children - A Retrospective Study at a Tertiary Care Center in South India.","authors":"Dona T Thomas, Kalpana Devadathan, Greeshma Baby, Mohammed Pa Kunju, Prameela Joji","doi":"10.4103/aian.aian_717_24","DOIUrl":"https://doi.org/10.4103/aian.aian_717_24","url":null,"abstract":"<p><strong>Background: </strong>Human Herpes Virus 6 (HHV6) can cause severe neurological manifestations in immunocompromised individuals. However, there is sparse data regarding this in immunocompetent children.</p><p><strong>Objective: </strong>To describe the spectrum of neurological manifestations of HHV 6 infection and outcome in immunocompetent children admitted with suspected meningoencephalitis from January 2017 to December 2023.</p><p><strong>Methodology: </strong>We retrospectively analysed the electronic medical records of children admitted for suspected meningoencephalitis in a tertiary care Pediatric Neurology centre in South India. The children whose cerebrospinal fluid (CSF) was found to be positive for HHV6 DNA in film array meningoencephalitis (FA -ME) panel were included.</p><p><strong>Results: </strong>204 /416 children with suspected meningoencephalitis were included in the study. HHV 6 was detected in 12 children. The median age was 19 months (Range - 6 months to 16 years). 4 (33%) of them were classified as febrile status. 5 (42%) children had meningoencephalitis, and one had aseptic meningitis. One child each had features of probable acute demyelinating encephalo myelitis (ADEM) and anti NMDA receptor encephalitis. 9 (75%) children had uneventful clinical recovery, one child died, one child with probable ADEM had extrapyramidal signs at discharge, which improved completely at one year follow up and the baby with autoimmune encephalitis had significant neurological deficits.</p><p><strong>Conclusion: </strong>HHV 6 can cause significant neurological problems, with significant morbidity and mortality in immunocompetent children also.</p>","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143952421","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 Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS) with Associated Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) and a Review of Literature.","authors":"Shivam Mirg, Animesh Das, Akhil Parashar, Manjari Tripathi, Ajay Garg","doi":"10.4103/aian.aian_998_24","DOIUrl":"https://doi.org/10.4103/aian.aian_998_24","url":null,"abstract":"","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-05-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143966635","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":"In-Depth Understanding of Hirayama Disease: Dural Detachment Beyond Cervical Spine.","authors":"Seena Vengalil, Vijaykumar Boddu, Karthik Kulanthaivelu, Dipti Baskar, Saraswati Nashi, Nupur Pruthi, Hemant Bhargav, Alok M Uppar, Chandrajit Prasad, Madhulika Kotra, Kiran Polavarapu, Veeramani Preethish-Kumar, Atchayaram Nalini","doi":"10.4103/aian.aian_16_25","DOIUrl":"https://doi.org/10.4103/aian.aian_16_25","url":null,"abstract":"<p><strong>Background and objectives: </strong>Hirayama disease (HD) is a cervical flexion-induced compressive myelopathy. Typically, there is forward displacement and loss of attachment of dural sac to lamina at the cervical level during neck flexion. However, the extent of the dural detachment (DD) has not been studied carefully. We undertook this study to know the extent of DD in HD.</p><p><strong>Methods: </strong>We conducted a retrospective study of HD patients evaluated from 2015 to 2023. Patients with DD extending beyond the cervical spine were selected, and their clinical and radiological features were studied.</p><p><strong>Results: </strong>One hundred and thirty-two (62.8%) patients were identified to have DD beyond the cervical spine in a cohort of 210 HD patients. The mean age at onset and duration were 18.09 ± 2.3 years (13-26) and 38.63 ± 39.9 months, respectively. Proximo-distal involvement was noted in 50% of patients, while 33% and 17% of patients had isolated distal and proximal involvement, respectively. Wasted legs were observed in three patients. Cord atrophy was present in 96.9% of patients, extending from C5 to C7. Epidural detachment and engorgement of posterior epidural venous plexus were evident in all. DD extended from C2 to D10 vertebral level. The cranial extent of DD was from C2 to C4 in 87% of cases, and the caudal extent was D1-D5 in 84% of cases, extending up to D10 in two cases.</p><p><strong>Conclusions: </strong>The HD spectrum continues to evolve phenotypically and radiologically. The pathophysiological mechanisms and DD extend beyond the cervical spine in a large proportion of patients. This makes it important to cover a longer part of the spine during imaging. This may have implications on the management of patients, particularly those with isolated lower limb involvement.</p>","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143974764","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":"Phenotypic Variations in Clinical Presentations of Paramyotonia Congenita in Two Brothers.","authors":"Kamalesh Tayade, Vishakha Ghadi, Dynaneshwar Asole, Haresh Bharote, Anil Venkatachalam","doi":"10.4103/aian.aian_1070_24","DOIUrl":"https://doi.org/10.4103/aian.aian_1070_24","url":null,"abstract":"","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143957164","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":"The Expanding Spectrum of Anti-IgLON5 Disease: A Case Series from an Indian Cohort.","authors":"Avinash Ganapule, Divyani Garg, Ayush Agarwal, Anu Gupta, Roopa Rajan, Soaham Desai, Mitesh Chandarana, S Sidharth, Madhavi Tripathi, Ajay Garg, Divya M Radhakrishnan, Achal Kumar Srivastava","doi":"10.4103/aian.aian_1073_24","DOIUrl":"https://doi.org/10.4103/aian.aian_1073_24","url":null,"abstract":"<p><strong>Abstract: </strong>Anti-IgLON5 disease is an evolving entity that lies at the confluence of autoimmunity and neurodegeneration. Reports from India remain sparse. In this series, we describe seven Indian patients with anti-IgLON5-related disease. Patients presented across the fifth to eighth decades with a mean duration of illness of 16 months. All had movement disorders, which included gait ataxia, parkinsonism, and chorea. Six patients had sleep disturbances. Five had a frontal dysexecutive dementia phenotype. Two had epilepsy. Bulbar involvement was present in four, and one had amyotrophic lateral sclerosis (ALS)-like features. Magnetic resonance imaging was abnormal in two cases. Positron emission tomography of the brain also contributed to diagnosis. Combination immunotherapies were used in most of the patients, with three showing a sustained response and two deaths reported due to sepsis-related complications. It is important to recognize the increasing spectrum of IgLON5-related disease to enable timely initiation of immunotherapy before marked degeneration occurs.</p>","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143965679","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 Variant in C19orf12 Gene Causing Mitochondrial Membrane Protein-Associated Neurodegeneration (MPAN) - Case Report and a Brief Review of Indian Literature on MPAN.","authors":"Jayaram Saibaba, Sandeep Sibi, Deepak Amalnath, Dharanipragada Krishna Suri Subrahmanyam","doi":"10.4103/aian.aian_939_24","DOIUrl":"https://doi.org/10.4103/aian.aian_939_24","url":null,"abstract":"","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-04-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143967597","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":"Hyperammonemic Encephalopathy due to Underlying Ornithine Transcarbamylase Deficiency.","authors":"Boby V Maramattom, Priniya Saul","doi":"10.4103/aian.aian_1069_24","DOIUrl":"https://doi.org/10.4103/aian.aian_1069_24","url":null,"abstract":"","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-04-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143969289","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 Tribute to a Gentle Giant (Fondly Called CHIEF).","authors":"Meenakshisundaram Umaiorubahan","doi":"10.4103/aian.aian_260_25","DOIUrl":"https://doi.org/10.4103/aian.aian_260_25","url":null,"abstract":"","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-04-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12049211/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143960326","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Usefulness of 131I-MIBG Myocardial Scintigraphy in a Case of Pure Autonomic Failure - First Report in the Literature.","authors":"Ramkumar Sugumaran, Ragavendar Bhuvaneswaran, Jayaram Saibaba, Sunil K Narayan, Sai Kishore Sundarraj, Nandini Pandit","doi":"10.4103/aian.aian_990_24","DOIUrl":"https://doi.org/10.4103/aian.aian_990_24","url":null,"abstract":"","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143802267","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":"Efficacy of Subcutaneous and Intravenous Immunoglobulin in Patients with Multifocal Motor Neuropathy: A Systematic Review and Meta-analysis.","authors":"Indar Kumar Sharawat, Pragnya Panda, Lesa Dawman, Ananthanarayanan Kasinathan, Prateek Kumar Panda","doi":"10.4103/aian.aian_815_24","DOIUrl":"https://doi.org/10.4103/aian.aian_815_24","url":null,"abstract":"<p><strong>Background and objectives: </strong>Multifocal motor neuropathy (MMN) is an uncommon chronic autoimmune disorder in adults. Several randomized and non-randomized studies with small sample sizes have shown promising results with subcutaneous immunoglobulin (SCIg) in MMN patients who have a stable disease course with intravenous immunoglobulin (IVIg). This systematic review aimed to compare the efficacy of SCIg and IVIg treatment in MMN patients in terms of changes in muscle power, grip strength, motor function, disability, quality of life, as well as adverse effects.</p><p><strong>Methods: </strong>Random- or fixed-effect model was used, as appropriate, to determine the pooled weighted estimates for outcome variables, depending on the degree of heterogeneity. A total of 11 publications from nine studies (110 patients, mean age 54.3 ± 3.4 years, 57% males, duration of illness 9.7 ± 2.4 years) were included in the review.</p><p><strong>Results: </strong>Changes in the Medical Research Council sum score (MRC-SS), grip strength, dexterity, and disability due to neuropathy following IVIg and SCIg administration were comparable (P = 0.30, I² = 9%; P = 0.51, I² = 0%; P = 0.45, I² = 0%; and P = 0.57, I² = 0%, respectively). Changes in health-related quality of life were numerically better with SCIg, although the difference did not reach statistical significance (P = 0.17, I² = 72%,). Over the long term, there was a trend toward fewer disease fluctuations compared to IVIg (P = 0.43), and the efficacy comparable to IVIg was maintained in terms of muscle strength, disability, and quality of life parameters (P = 0.83, 0.85, and 0.59, respectively). The incidence of moderate to severe systemic adverse effects was significantly lower with SCIg infusion compared to previous years when patients received IVIg (P = 0.03).</p><p><strong>Conclusions: </strong>SCIg can be considered an acceptable alternative to IVIg in patients with MMN, with a better safety profile.</p>","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-04-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143966718","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}