Successful management of steroid-refractory Morvan's syndrome 3 months after onset with therapeutic plasma exchange

Abstract

A 52-year-old man presented with behavioral and cognitive symptoms and involuntary movements. Serum CASPR-2 antibodies were strongly positive, and the nerve conduction study showed hyperexcitability. Thus, it was diagnosed as a case of Morvan's syndrome, detected after 3 months. The failed therapy with steroids led to the initiation of the therapeutic plasma exchange (TPE). Five cycles of TPE were completed to improve dramatically the cognitive and behavioral issues as well as the involuntary movements. He was initiated on rituximab for long-term maintenance therapy. In conclusion, TPE can be initiated even after 3 months of onset to achieve significant recovery. Although it is challenging, it is still a tolerated procedure with good outcomes.

Keywords

• apheresis
• morvan's syndrome
• therapeutic plasma exchange