A treatable case of autoimmune GFAP astrocytopathy presenting chronic progressive cognitive impairment
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LETTER TO THE EDITOR
A treatable case of autoimmune GFAP astrocytopathy presenting chronic progressive cognitive impairment Takahiro Natori 1 & Kazumasa Shindo 1 & Akihiro Okumura 2 & Akio Kimura 3 & Yoshihisa Takiyama 1 Received: 15 February 2020 / Accepted: 7 May 2020 # Fondazione Società Italiana di Neurologia 2020
Dear editor, Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A) is an inflammatory central nervous system disorder [1, 2]. GFAP-A is diagnosed by the confirmation of antibodies for GFAP in cerebrospinal fluid (CSF), and presents the characteristic finding of linear perivascular radial gadolinium enhancement of the cerebral white matter on magnetic resonance imaging (MRI) [1, 2]. Most patients with GFAP-A exhibit acute- or subacute-onset meningoencephalitis or meningoencephalomyelitis with pleocytosis and elevated protein in CSF, and are treated with immunotherapeutics such as corticosteroids, intravenous immunoglobulin, and immunosuppressants [1, 2]. We report here a rare case of GFAP-A presenting progressive chronic cognitive impairment with comfortable treatment. A 68-year-old right-handed Japanese woman was admitted to our hospital for examination and treatment of progressive cognitive impairment. At age 67, she could not remember the date or place, or how to use daily items such as slippers, and these symptoms gradually worsened. She has had urinary incontinence from 6 months ago. Neurological examination revealed brisk tendon reflexes in the upper and lower extremities and bilateral Babinski signs. Truncal or limb weakness, cerebellar ataxia, involuntary movements, sensory disturbance, visual symptoms, neck stiffness, or meningeal irritation was absent. The Mini-Mental State Examination (MMSE) and Hasegawa’s dementia scalerevised (HDS-R) scores were 13/30 and 6/30, respectively.
* Yoshihisa Takiyama [email protected] 1
Department of Neurology, University of Yamanashi, Yamanashi 409-3898, Japan
2
Department of Radiology, University of Yamanashi, Yamanashi 409-3898, Japan
3
Department of Neurology, Gifu University Graduate School of Medicine, Gifu 501-1194, Japan
Because of severe cognitive impairment, we could not evaluate her using the Wechsler Adult Intelligence Scale. The laboratory data including thyroid function, cortisol, ACTH, syphilis, and angiotensin-converting enzyme were unremarkable. The vitamin B12 concentration was slightly low (158 pg/ml; normal range, 180-914 pg/ml). Serum was negative for anti-nuclear antibodies, anti-neutrophil cytoplasmic antibody, anti-aquaporin 4 antibody, anti-myelin oligodendrocyte glycoprotein antibody, and anti-N-methyl-D-aspartate receptor antibody. Serum tumor marker screening revealed that CYFRA was slightly elevated (3.74 ng/ml; normal range, < 2.8 ng/ml), but others (SCC, AFP, CEA, CA19-9, NSE, SLX, Pro GRP, and IL-2R) were unremarkable. CSF examination showed mild lymphocytic pleocytosis (29/μl of monocytic cells) and elevated protein (144 mg/dl), and the IgG index was 0.92 (normal range, 0.34–0.68). Gram or ac
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