GAD65 Autoimmune Encephalitis

Abstract

We describe a patient who presented with subacute onset of short-memory impairment, disorientation, and gait instability, with progressive deterioration. Workup demonstrated glutamic acid decarboxylase antibody-related encephalitis. Aggressive immunotherapy with high-dose intravenous corticoids, followed by slow oral taper, plasmapheresis, rituximab, and cyclophosphamide did not halt disease progression. During follow-up, she developed a frontotemporal dementia phenotype. Serial imaging showed the appearance of marked atrophy of the frontal and anterior temporal regions. We conclude that glutamic acid decarboxylase antibodyrelated encephalitis may rarely present with a treatment-refractory frontotemporal phenotype.

Presenile dementia concomitant with Sjogren's syndrome：A Case Report

“…Based on an extensive body of literature, it is widely acknowledged that a small subset of patients experiencing cognitive impairment exhibit the presence of sensitized T-lymphocytes and/or anti-nuclear antibodies (ANAs), which have been implicated in the "attack" on the central nervous system (CNS). The presence of "dopamine-producing cells" within the extrapyramidal system (EPS) and the hippocampus situated in the limbic system of the brain (Figure 2) contributes to the manifestation of symptoms associated with Parkinson's disease (PD) and Alzheimer's disease (AD) [1,2] .…”