European Journal of Biomedical and Pharmaceutical Sciences

CASPR2 ASSOCIATED LIMBIC EN CEPHALITIS WITH FEAT URES OF MORVAN SYNDROME: A CASE REP ORT

Ashna Zeaba V. T.*, Lekshmi Gopal U.

ABSTRACT

Morvan syndrome is a rare autoimmune neurological disorder characterized by neuromyotonia, autonomic dysfunction, and central nervous system involvement, often associated with CASPR2 antibodies. We present a 77-year-old male with CASPR2-associated autoimmune limbic encephalitis and facial-brachial dystonic seizures (FBDS), who exhibited seizures, autonomic dysfunction, and possible peripheral nerve hyperexcitability, raising suspicion for Morvan syndrome. Neurological imaging showed diffuse cortical atrophy with chronic small vessel ischemic changes, while serum VGKC panel revealed mildly positive CASPR2 antibodies. A tilt table test confirmed orthostatic hypotension without compensatory tachycardia. The patient was treated with intravenous methylprednisolone and rituximab, leading to clinical improvement with seizure resolution and autonomic stabilization. This case highlights the importance of early recognition and immunosuppressive therapy in CASPR2-spectrum disorders, as well as the need for a multidisciplinary approach in managing autoimmune neurological syndromes.

Keywords: Morvan syndrome, CASPR2 encephalitis, facial-brachial dystonic seizures, autoimmune limbic encephalitis, neuromyotonia, dysautonomia, case report.