Annals of Indian Academy of Neurology最新文献

Long-Term Efficacy and Tolerability of Cenobamate in Clinical Practice: A Retrospective Observational Study. Cenobamate在临床实践中的长期疗效和耐受性：一项回顾性观察研究。

IF 1.8 4区医学

Annals of Indian Academy of Neurology Pub Date : 2025-09-26 DOI: 10.4103/aian.aian_441_25

Shubhi Agrawal, Batool A Hussain, Kiran M Kanth

{"title":"Long-Term Efficacy and Tolerability of Cenobamate in Clinical Practice: A Retrospective Observational Study.","authors":"Shubhi Agrawal, Batool A Hussain, Kiran M Kanth","doi":"10.4103/aian.aian_441_25","DOIUrl":"https://doi.org/10.4103/aian.aian_441_25","url":null,"abstract":"Background and objectives: Cenobamate (CNB) was approved in the US in 2020 and showed 12% seizure freedom in drug-resistant epilepsy (DRE) in pivotal trials. The open-label extension (OLE) study reported 13%-16% seizure freedom and sustained responder rates over 4 years. It is unclear how much of the treatment benefit during OLE was attributable to CNB, as people could have received other treatments. This study aimed to assess CNB efficacy and safety over an extended follow-up.Methods: This retrospective study was conducted among adults with DRE, treated with CNB between May 2020 and Dec 2023, at the University of California, Davis. Data on demographics, epilepsy history, CNB dose, seizure frequencies, adverse events, and treatment changes were collected. Treatment failure was considered if CNB was withdrawn for any reason or if another epilepsy treatment was needed after starting CNB.Results: Sixty people with epilepsy (PWE) were included, with 78% having focal epilepsy and 11.3% generalized epilepsy. Over the mean follow-up period of 20.5 months, 56.7% had treatment failure, with a median survival time of 17.2 months. Seizure freedom was seen in 15% of participants, and 90% or more seizure reduction in 21% of participants. Of the failures, 44% were due to incomplete efficacy, 38% were due to adverse effects, and the rest due to a combination of both. Seventy-eight percent of PWE who started CNB were taking it at last follow-up.Conclusions: CNB shows clinically meaningful efficacy for at least 1.5 years in people with DRE.","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.8,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145147634","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}

引用次数: 0

Podophyllotoxin Revisited: Unveiling a Rare Cause of Sensory Ataxic Neuropathy. 足臼毒素重访：揭示一种罕见的引起感觉共济失调神经病的原因。

IF 1.8 4区医学

Annals of Indian Academy of Neurology Pub Date : 2025-09-24 DOI: 10.4103/aian.aian_528_25

Dhanush Mallesh, Sahil Mehta, Siddharth Chand, Ravish Padda, Victo Varghese, Ritu Shree, Manoj K Goyal, Vivek Lal

引用次数: 0

First Report of Interaction Among Ponticulus Posticus Anomaly and Stenosis of Carotid, Vertebral Arteries. 后桥异常与颈动脉、椎动脉狭窄相互作用的首次报道。

IF 1.8 4区医学

Annals of Indian Academy of Neurology Pub Date : 2025-09-24 DOI: 10.4103/aian.aian_184_25

Saban Ergene, Ayhan Kanat, Ibrahim Yel, Bulent Ozdemir, Serdar Durmaz, Hasan Gundogdu, Cihangir Erturk, Tugba Yemis, Dogus Hemsinli, Ozan Karakisi, Omer Lutfi Gundogdu, Hizir Kazdal

引用次数: 0

Nasu-Hakola Disease: Crippling the Brain and Bones! 那苏-哈科拉病：破坏大脑和骨骼！

IF 1.8 4区医学

Annals of Indian Academy of Neurology Pub Date : 2025-09-24 DOI: 10.4103/aian.aian_569_25

Kaniti Sowjanya, Ashok Vr Taallapalli, Manas Saxena, Niraj Kumar

引用次数: 0

WARS2-Associated Neuropsychiatric Phenotype in Childhood: A Case-Based Review. 儿童wars2相关的神经精神表型：一项基于病例的回顾。

IF 1.8 4区医学

Annals of Indian Academy of Neurology Pub Date : 2025-09-19 DOI: 10.4103/aian.aian_235_25

Shabnam Kalita, Vishnupriya Veeraraghavan, Kaushik Ragunathan, Jaya Shankar Kaushik

引用次数: 0

Expanding the Clinico-Genetic Scope of Bethlem Myopathy: A Family of Five Patients from Kerala. 扩大Bethlem肌病的临床遗传学范围：来自喀拉拉邦的一个五口患者家庭。

IF 1.8 4区医学

Annals of Indian Academy of Neurology Pub Date : 2025-09-03 DOI: 10.4103/aian.aian_146_25

Jayaram Saibaba, Saranya B Gomathy, Santhakumar Senthilvelan, Ramkumar Sugumaran, Sunil K Narayan

引用次数: 0

Differential Response to Corticosteroids in Immune-Mediated Myokymia vs Grouped Fasciculation Discharges. 免疫介导的肌萎缩症与组束状放电对皮质类固醇的差异反应。

IF 1.8 4区医学

Annals of Indian Academy of Neurology Pub Date : 2025-09-03 DOI: 10.4103/aian.aian_76_25

Rithvik Ramesh, Sundar Shanmugam, Philo Hazeena, Lakshmi Narasimhan Ranganathan, Deepa Avadhani

{"title":"Differential Response to Corticosteroids in Immune-Mediated Myokymia vs Grouped Fasciculation Discharges.","authors":"Rithvik Ramesh, Sundar Shanmugam, Philo Hazeena, Lakshmi Narasimhan Ranganathan, Deepa Avadhani","doi":"10.4103/aian.aian_76_25","DOIUrl":"10.4103/aian.aian_76_25","url":null,"abstract":"Abstract: Myokymia and grouped fasciculation discharges (GFD) are electromyographic manifestations of peripheral nerve hyperexcitability syndromes. This case series explores the differential response to corticosteroids in patients with myokymia compared to those with GFDs. Six patients were included in this report: three with predominant myokymia and three with predominant GFDs, reviewed between March 2023 and February 2024. Electromyographic examinations were performed on various muscle groups, with the gastrocnemius EMG findings serving as an index. The neurologist conducting the EMG was blinded to the clinical history. Patients with predominant myokymia showed a positive response to corticosteroids. In contrast, patients with GFDs, characterized by non-rhythmic, irregular discharges, exhibited a limited response requiring higher immunotherapy. This study potentially highlights the importance of EMG in guiding treatment decisions, demonstrating that patients with myokymia respond well to corticosteroids due to underlying autoantibody-mediated potassium channel dysfunction.","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.8,"publicationDate":"2025-09-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144939921","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}

引用次数: 0

Anti-N-Methyl D-Aspartate Receptor Encephalitis Presenting with Progressive Cerebellar Atrophy. 以进行性小脑萎缩为表现的抗n -甲基d -天冬氨酸受体脑炎。

IF 1.8 4区医学

Annals of Indian Academy of Neurology Pub Date : 2025-08-29 DOI: 10.4103/aian.aian_290_25

S Haritha, P R Sowmini, Aubin M Varghese, S Lakshmanan, Sakthi S Velayutham, Malcolm K Jeyaraj, V Kannan, Viveka R Saravanan, Mugundhan Krishnan

引用次数: 0

Diffusion Restriction in Wilson's Disease: A Radiological Pointer Toward Clinically Severe Disease. 肝豆状核变性弥散受限：临床重症的影像学指标。

IF 1.8 4区医学

Annals of Indian Academy of Neurology Pub Date : 2025-08-29 DOI: 10.4103/aian.aian_483_25

Divyani Garg, Shariq Ahmad Shah, Ayush Agarwal, Divya M Radhakrishnan, Roopa Rajan, Achal Kumar Srivastava, Ajay Garg

{"title":"Diffusion Restriction in Wilson's Disease: A Radiological Pointer Toward Clinically Severe Disease.","authors":"Divyani Garg, Shariq Ahmad Shah, Ayush Agarwal, Divya M Radhakrishnan, Roopa Rajan, Achal Kumar Srivastava, Ajay Garg","doi":"10.4103/aian.aian_483_25","DOIUrl":"https://doi.org/10.4103/aian.aian_483_25","url":null,"abstract":"Background and objectives: Wilson's disease (WD) is a rare autosomal recessive disorder due to abnormal hepatic copper transport, leading to copper accumulation in the liver, brain, and other tissues. Although conventional magnetic resonance imaging (MRI) features are valuable for diagnosis, the role of diffusion-weighted imaging (DWI) remains underexplored in WD. The study aims to assess the prevalence and clinical correlates of diffusion restriction on MRI in WD.Methods: A single-center, retrospective study was conducted. Clinicodemographic and MRI findings of patients diagnosed with WD based on a modified Leipzig score cut-off of 4 were analyzed. Characteristics of patients with diffusion restriction (WDDR+) and without diffusion restriction (WDDR-) were compared using statistical tests.Results: Of 91 patients with WD, 17 (18.7%) demonstrated MRI diffusion restriction. WDDR+ were noted to have a lower median age at symptom onset (14 [9-17] versus 15 [14-23] years; P = 0.020) and presentation (15 [13-20] years versus 19 [15-25.5] years; P = 0.022) compared to WDDR-. WDDR+ had a higher modified Leipzig score compared to WDDR- (5 [4-5] versus 7 [5-8]; P = 0.001). They also had significantly higher Global Assessment Scale (GAS) for WD (32.5 [30-37]) compared to WDDR- (19 [13-22]) (P < 0.001). WDDR+ had significantly higher proportions of patients with dysphagia (10/17, 58.8% versus 6/74, 8.1%; P < 0.001) and portal hypertension (8/17, 47.1% versus 6/74, 8.1%; P < 0.001). WDDR+ also experienced significantly higher rates of early neurological deterioration (END) (23.5% versus 6.6%, P = 0.04).Conclusions: Diffusion restriction may hence serve as a pointer toward more severe neurological and hepatic involvement in WD, indicating the need for close supervision in this group of patients.","PeriodicalId":8036,"journal":{"name":"Annals of Indian Academy of Neurology","volume":" ","pages":""},"PeriodicalIF":1.8,"publicationDate":"2025-08-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144939933","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}

引用次数: 0

Parvovirus B19-Associated Acute Disseminated Encephalomyelitis: A Rare Case with Multiple Cerebral Infarcts and Long-Segment Myelitis. 细小病毒b19相关的急性播散性脑脊髓炎：一例罕见的多发性脑梗死和长段脊髓炎病例。

IF 1.8 4区医学

Annals of Indian Academy of Neurology Pub Date : 2025-08-23 DOI: 10.4103/aian.aian_277_25

Bhavani Madduluri, Punati Harishankar, T Suryprabha, Sabeeha Naaz

引用次数: 0