Interdisciplinary Neurosurgery (Mar 2021)
ANTI-MA2 encephalitis mimicking diencephalic demyelinating syndrome
Abstract
Anti Ma 2 encephalitis is an autoimmune encephalitis, usually paraneoplastic, characterized by isolated or combined limbic, diencephalic, or brainstem dysfunction. Limbic encephalitis (LE) is the most common manifestation. Anti-Ma2 LE can also be manifested as a pure psychiatric disturbance associated or not with hypokinesia. Other patients exhibit excessive daytime sleepiness, vertical ophthalmoparesis, or both associated with LE, due to diencephalic and/or upper brainstem involvement. These cases are challenging and demand extensive work-up for differential diagnosis.