Article ; Online: GAD65 Autoimmune Encephalitis: A Cause of Rapidly Evolving Frontotemporal Atrophy.
Alzheimer disease and associated disorders
2021 Volume 36, Issue 1, Page(s) 80–82
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 ... ...
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 antibody-related encephalitis may rarely present with a treatment-refractory frontotemporal phenotype. |
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MeSH term(s) | Atrophy ; Autoantibodies ; Encephalitis ; Female ; Glutamate Decarboxylase/therapeutic use ; Hashimoto Disease ; Humans |
Chemical Substances | Autoantibodies ; Glutamate Decarboxylase (EC 4.1.1.15) |
Language | English |
Publishing date | 2021-06-16 |
Publishing country | United States |
Document type | Journal Article |
ZDB-ID | 1002700-2 |
ISSN | 1546-4156 ; 0893-0341 |
ISSN (online) | 1546-4156 |
ISSN | 0893-0341 |
DOI | 10.1097/WAD.0000000000000463 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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