Adult-onset Alexander disease with typical "tadpole" brainstem atrophy and unusual bilateral basal ganglia involvement: a case report and review of the literature |
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| Authors: | Michito Namekawa Yoshihisa Takiyama Junko Honda Haruo Shimazaki Kumi Sakoe Imaharu Nakano |
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| Institution: | (1) Department of Neurology, Jichi Medical University, Tochigi, Japan;(2) Department of Neurology, Interdisciplinary Graduate School of Medicine and Engineering University of Yamanashi, Yamanashi, Japan;(3) Department of Hematology, Interdisciplinary Graduate School of Medicine and Engineering University of Yamanashi, Yamanashi, Japan |
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| Abstract: | Background Alexander disease (ALX) is a rare neurological disorder characterized by white matter degeneration and cytoplasmic inclusions
in astrocytes called Rosenthal fibers, labeled by antibodies against glial fibrillary acidic protein (GFAP). Three subtypes
are distinguished according to age at onset: infantile (under age 2), juvenile (age 2 to 12) and adult (over age 12). Following
the identification of heterozygous mutations in GFAP that cause this disease, cases of adult-onset ALX have been increasingly reported. |
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