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Clinical profile and treatment response in patients with CASPR2 antibody-associated neurological disease

التفاصيل البيبلوغرافية
العنوان: Clinical profile and treatment response in patients with CASPR2 antibody-associated neurological disease
المؤلفون: Sumanth Shivaram, Madhu Nagappa, Doniparthi V Seshagiri, Anita Mahadevan, Yashwanth Gangadhar, T N Sathyaprabha, Vijay Kumavat, Rose D Bharath, Sanjib Sinha, Arun B Taly
المصدر: Annals of Indian Academy of Neurology, Vol 24, Iss 2, Pp 178-185 (2021)
بيانات النشر: Wolters Kluwer Medknow Publications, 2021.
سنة النشر: 2021
المجموعة: LCC:Neurology. Diseases of the nervous system
مصطلحات موضوعية: autoimmune encephalitis, contactin-associated protein-like 2 (caspr2), morvan syndrome, paraneoplastic neurological disease, voltage-gated potassium channel, Neurology. Diseases of the nervous system, RC346-429
الوصف: Background: The clinical spectrum of contactin-associated protein-like 2 (CASPR2) antibody-associated disease is wide and includes Morvan syndrome. Studies describing treatment and long-term outcome are limited. Aims: We report the clinical profile and emphasize response to treatment and long-term outcome in eight patients with CASPR2-antibody-associated disease. Methods: Clinical, radiological, electrophysiological, treatment, follow-up, and outcome data were collected by retrospective chart review. Results: Clinical manifestations included Morvan syndrome (n = 7) and limbic encephalitis (n = 1). None of the patients were positive for LGI1 antibody. Associated features included myasthenia (n = 1), thymoma (n = 1), and dermatological manifestations (n = 4). Patients were treated with intravenous methylprednisolone and plasma exchange during the acute symptomatic phase followed by pulsed intravenous methyl prednisolone to maintain remission. Mean-modified Rankin score at admission (pre-treatment), discharge, and last follow-up were 3.75, 2.5, and 0.42, respectively. One patient with underlying thymoma and myasthenic crisis died. The other seven patients were followed up for a mean duration of 19.71 months. All of them improved completely. Relapse occurred in one patient after 13 months but responded favorably to steroids. Conclusion: CASPR2 antibody-associated disease has favorable response to immunotherapy with complete improvement and good outcome. Underlying malignancy may be a marker for poor prognosis.
نوع الوثيقة: article
وصف الملف: electronic resource
اللغة: English
تدمد: 0972-2327
1998-3549
Relation: http://www.annalsofian.org/article.asp?issn=0972-2327;year=2021;volume=24;issue=2;spage=178;epage=185;aulast=Shivaram; https://doaj.org/toc/0972-2327; https://doaj.org/toc/1998-3549
DOI: 10.4103/aian.AIAN_574_20
URL الوصول: https://doaj.org/article/1a32bb1b94f7475c987ff366b86bea16
رقم الأكسشن: edsdoj.1a32bb1b94f7475c987ff366b86bea16
قاعدة البيانات: Directory of Open Access Journals
الوصف
تدمد:09722327
19983549
DOI:10.4103/aian.AIAN_574_20