Clinical characteristics of autoimmune GFAP astrocytopathy

Akio Kimura; Akira Takekoshi; Nobuaki Yoshikura; Yuichi Hayashi; Takayoshi Shimohata

doi:10.1016/j.jneuroim.2019.04.004

Clinical characteristics of autoimmune GFAP astrocytopathy

J Neuroimmunol. 2019 Jul 15:332:91-98. doi: 10.1016/j.jneuroim.2019.04.004. Epub 2019 Apr 9.

Authors

Akio Kimura¹, Akira Takekoshi², Nobuaki Yoshikura², Yuichi Hayashi², Takayoshi Shimohata²

Affiliations

¹ Department of Neurology and Geriatrics, Gifu University Graduate School of Medicine, Gifu, Japan. Electronic address: kimura1@gifu-u.ac.jp.
² Department of Neurology and Geriatrics, Gifu University Graduate School of Medicine, Gifu, Japan.

PMID: 30991306
DOI: 10.1016/j.jneuroim.2019.04.004

Abstract

The clinical features of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy remain to be elucidated. We describe here the clinical features of 14 patients with GFAP astrocytopathy confirmed by detection of GFAP-IgG in cerebrospinal fluid (CSF). The novel findings of this study are as follows. First, over half of the patients presented with movement disorders (tremor, myoclonus, and ataxia), autonomic dysfunction (mainly urinary dysfunction), and hyponatremia. Second, most patients showed transient elevation of adenosine deaminase activity levels in CSF. Finally, some patients showed bilateral hyperintensities in the posterior part of the thalamus on brain magnetic resonance imaging.

Keywords: Adenosine deaminase activity (ADA); Astrocytopathy; Autoantibody; Glial fibrillary acidic protein (GFAP); Movement disorder.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adenosine Deaminase / cerebrospinal fluid
Adolescent
Adrenal Cortex Hormones / therapeutic use
Adult
Aged
Aged, 80 and over
Astrocytes / immunology*
Autoantigens / immunology*
Autoimmune Diseases of the Nervous System / drug therapy
Autoimmune Diseases of the Nervous System / immunology*
Cerebrospinal Fluid Proteins / analysis
Diagnosis-Related Groups
Female
Glial Fibrillary Acidic Protein / immunology*
Humans
Hyponatremia / drug therapy
Hyponatremia / immunology*
Inflammation
Japan
Magnetic Resonance Imaging
Male
Middle Aged
Movement Disorders / drug therapy
Movement Disorders / immunology*
Nervous System Diseases / drug therapy
Nervous System Diseases / immunology*
Nervous System Diseases / pathology
Neuroimaging
Thalamus / immunology
Thalamus / pathology
Urination Disorders / drug therapy
Urination Disorders / immunology*
Young Adult

Substances

Adrenal Cortex Hormones
Autoantigens
Cerebrospinal Fluid Proteins
GFAP protein, human
Glial Fibrillary Acidic Protein
ADA protein, human
Adenosine Deaminase