Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Presenting with Slowly Progressive Myelitis and Longitudinally Extensive Spinal Cord Lesions
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- Kudo Takuya
- Department of Neurology, Gifu University Graduate School of Medicine, Japan
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- Kimura Akio
- Department of Neurology, Gifu University Graduate School of Medicine, Japan
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- Higashida Kazuhiro
- Department of Neurology, Gifu University Graduate School of Medicine, Japan
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- Yamada Megumi
- Department of Neurology, Gifu University Graduate School of Medicine, Japan
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- Hayashi Yuichi
- Department of Neurology, Gifu University Graduate School of Medicine, Japan
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- Shimohata Takayoshi
- Department of Neurology, Gifu University Graduate School of Medicine, Japan
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Abstract
<p>We report a 65-year-old man with autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) who presented with gait disturbance that he had experienced for approximately half a year. On neurological examination, he displayed spastic paraplegia and autonomic dysfunctions including dysuria and constipation. Spinal cord magnetic resonance imaging showed longitudinally extensive spinal cord lesions (LESCLs) extending from the cervical to the thoracic cords. The patient was negative for anti-myelin oligodendrocyte glycoprotein and anti-aquaporin 4 antibodies. Treatment with corticosteroids and intravenous immunoglobulin resulted in a clinical improvement. It is important to distinguish GFAP-A from slowly progressive myelitis with LESCLs. </p>
Journal
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- Internal Medicine
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Internal Medicine 59 (21), 2777-2781, 2020-11-01
The Japanese Society of Internal Medicine
