Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Peripheral neuropathy associated with severe glial fibrillary acidic protein (GFAP) astrocytopathy: a case report

Nobuhiko Ohashi, M.D., Ph.D., Shota Kamijima, M.D., Rie Watanabe, M.D. and Ko-ichi Tazawa, M.D., Ph.D.

Department of Neurology, Nagano Red Cross Hospital

A 44-year-old man was admitted due to a fever. He developed unconsciousness and respiratory failure, necessitating mechanical ventilation. After the administration of methylprednisolone and intravenous immunoglobulin for suspected autoimmune encephalitis, his consciousness and respiratory state improved. However, he exhibited pronounced tetraparalysis and impaired sensation below the neck. A spinal MRI revealed swelling of the entire spinal cord, indicating myelitis. Deep tendon reflexes were diminished in all extremities, and a nerve conduction study confirmed motor-dominant axonal polyneuropathy. Subsequently, he developed a fever and headache. Brain MRI demonstrated FLAIR hyperintensities in the basal ganglia and brain stem. CSF analysis for anti-glial fibrillary acidic protein (GFAP) antibody turned out positive, leading to the diagnosis of GFAP astrocytopathy. Although the steroid re-administration improved muscle strength in his upper limbs and reduced the range of diminished sensation, severe hemiparalysis remained. Severe GFAP astrocytopathy can be involved with polyneuropathy. Early detection and therapeutic intervention for this condition may lead to a better prognosis.
Full Text of this Article in Japanese PDF (1100K)

(CLINICA NEUROL, 64: 403|407, 2024)
key words: glial fibrillary acidic protein astrocytopathy (GFAP), peripheral neuropathy, polyneuropathy

(Received: 26-Oct-23)