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Clinical profile and treatment response in patients with CASPR2 antibody-associated neurological disease
Sumanth Shivaram1, Madhu Nagappa1, Doniparthi V Seshagiri1, Anita Mahadevan2, Yashwanth Gangadhar2, TN Sathyaprabha3, Vijay Kumavat4, Rose D Bharath5, Sanjib Sinha1, Arun B Taly1
1 Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, Karnataka, India
2 Department of Neuropathology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, Karnataka, India
3 Department of Neurophysiology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, Karnataka, India
4 Department of Transfusion Medicine and Hematology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, Karnataka, India
5 Department of Neuroimaging and Interventional Radiology (NIIR), National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, Karnataka, India
Correspondence Address:
Sanjib Sinha,
Professor, Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore - 560 029, Karnataka
India
 Source of Support: None, Conflict of Interest: None DOI: 10.4103/aian.AIAN_574_20
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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.
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