Clinical profile and treatment response in patients with CASPR2 antibody-associated neurological disease
| Autor: | Sumanth Shivaram, Madhu Nagappa, Doniparthi V Seshagiri, Anita Mahadevan, Yashwanth Gangadhar, T N Sathyaprabha, Vijay Kumavat, Rose D Bharath, Sanjib Sinha, Arun B Taly |
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| Jazyk: | angličtina |
| Rok vydání: | 2021 |
| Předmět: | |
| Zdroj: | Annals of Indian Academy of Neurology, Vol 24, Iss 2, Pp 178-185 (2021) |
| Druh dokumentu: | article |
| ISSN: | 0972-2327 1998-3549 |
| DOI: | 10.4103/aian.AIAN_574_20 |
| Popis: | 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. |
| Databáze: | Directory of Open Access Journals |
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