Atypical presentation of anti-Ma2-associated encephalitis with choreiform movement

Ma2 antibody-associated encephalitis is an inflammatory brain disease that associates with a systemic tumor in more than 90% of patients, most commonly a testicular germ-cell tumor, lung cancer, or breast cancer. The Ma2 antibody-mediated autoimmune encephalitis presents mostly as limbic, mesodiencephalic, or brain stem encephalitis. Cranial MRI often detects T2-hyperintense lesions that may progress to atrophy. However, other areas of the CNS, such as the brainstem, the thalamus, the hypothalamus, the cerebellum, or the basal ganglia may also be affected. Approximately one-third of patients with Ma2 antibody-associated encephalitis initially show no abnormalities in MRI. Roughly two-thirds of cases present abnormalities in the CSF, such as pleocytosis, protein increase, and positive oligoclonal bands.1 In cases of paraneoplastic encephalitis, tumor therapy is crucial for improvement and prognosis. Immunotherapy using high-dose IV steroids, IV immunoglobulins, plasma exchange, rituximab, and cyclophosphamide is recommended.2,3 Here, we present an unusual clinical presentation of a Ma2-associated autoimmune encephalitis.
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: Chorea Clinical/Scientific Notes Source Type: research