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Published on: June 2, 2022
[Autoimmune limbic encephalitis with concurrent positivity for Caspr2 and GFAPα antibodies]
Akiko Hamamoto1, Tomohito Nakano1, Hisae Sumi1
1Department of Neurology, Higashiosaka City Medical Center.
None:
A 69-year-old man presented with progressive cognitive decline over three months and two episodes of status epilepticus. Brain MRI revealed abnormal signals in the bilateral medial temporal lobes and deep white matter, and brain perfusion scintigraphy demonstrated increased perfusion in these regions. Electroencephalography showed sharp and slow-wave complexes with an F8 focus. Both serum and cerebrospinal fluid were positive for contactin-associated protein 2 (Caspr2) antibodies, and cerebrospinal fluid was positive for glial fibrillary acidic protein α (GFAPα) antibodies. Immunotherapy improved the clinical symptoms and MRI abnormalities, including deep white matter lesions. White matter involvement is rare in Caspr2 antibody-positive limbic encephalitis, and in this case, GFAPα antibodies may have contributed to the white matter lesions.
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