Successful Therapy of a Patient with Prospected Autoimmune Encephalitis and Negative Laboratory Results-Confirmation of Diagnosis by Immunocytochemistry

Abstract

Autoimmune encephalitis (AIE) presents with a broad spectrum of neurological and psychiatric symptoms and can cause persistent brain damage. However, the absence of pathognomonic findings in CT and MRI screening, and the fact that relevant antigens for laboratory detection of autoantibodies mostly are unknown, makes its diagnosis still a challenging task. In the present report the latter problem is overcome by the immunocytochemical detection of autoantibodies using brain sections, which in fact present nearly all neuronal antigens. Furthermore, we used a highly optimized immunoperoxidase technique, which allowed morphological high-resolution analyses of the immunoproducts. The 82-old female patient presented with unsteady gait pattern, ataxia, myoclonus, apraxia, as well as an unclear language. She suffered from panic attacks, cognitive and mnestic deterioration as well as delusions. She was alert but disoriented. Despite of normal MRI and EEG results and normal CSF laboratory data we prospectively diagnosed autoimmune encephalitis. After five days high dose corticosteroid therapy all symptoms disappeared completely. The autoimmune pathogenesis subsequently was verified unequivocally, when the patient´s CSF strongly stained neuronal and glial cell bodies in brain sections. This report should be taken as proof of principle that immunocytochemistry with CSFs of neurological patients provides the possibility to recognize a considerable number morphological details at high resolution. This will provide the possibility to form groups of patients showing similar distributions of immunoreactivities. Such groups may represent distinct nosological entities and finally may allow for elaborating selective treatments for patients with distinct types of autoimmune inflammation of the CNS.