A rare case of glial fibrillary acidic protein astrocytopathy that resolved spontaneously within a self-limited course

Glial fibrillary acidic protein astrocytopathy is a form of autoimmune meningoencephalomyelitis. The presence of antibodies in spinal fluid against glial fibrillary acidic protein is necessary to diagnose the disease. There is no standard treatment and few cases of glial fibrillary acidic protein astrocytopathy have been reported. A 31-year-old healthy Japanese man presented to our emergency department with a 7-day history of fever and headache. He was in good general condition, without abnormalities on physical examination, and a general hematological examination revealed hyponatremia (130 mEq/L). Five days later, he was followed up and new subjective symptoms were noted: tremor in the right hand, constipation, sweating, and lightheadedness. Cerebrospinal fluid examination revealed a cell count of 57/mu L (96 % mononuclear cells, 4 % multinuclear cells), elevated protein level (103 mg/dL), elevated adenosine deaminase level (15.0 U/L), negative polymerase chain reaction test results for herpes simplex virus and Mycobacterium tuberculosis, negative cerebrospinal fluid culture, and negative cerebrospinal fluid anti-acid bacteria culture, indicating aseptic meningitis. T1-weighted contrastenhanced magnetic resonance imaging of the head showed a linear contrast effect perpendicular to the lateral ventricular wall and along the perivascular vessels spreading radially. Based on the presence of hyponatremia, history of movement disorder and autonomic symptoms, high adenosine deaminase level in cerebrospinal fluid, and findings on contrast-enhanced magnetic resonance imaging of the head, we suspected glial fibrillary acidic protein astrocytopathy and assessed anti-glial fibrillary acidic protein alpha antibody in cerebrospinal fluid, which was positive, and diagnosed glial fibrillary acidic protein astrocytopathy. After careful follow-up with symptomatic treatment without immunosuppressive therapy, the fever, headache, tremor, and autonomic symptoms were improved over time. Contrast-enhanced magnetic resonance imaging of the head and findings of cerebrospinal fluid also showed improvement. glial fibrillary acidic protein astrocytopathy should be a differential diagnosis in patients with aseptic meningitis with movement disorders or autonomic symptoms and elevated cerebrospinal fluid adenosine deaminase. Careful follow-up without immunosuppressive treatment should be considered for patients with minimal neurologic symptoms as glial fibrillary acidic protein astrocytopathy may have a self-limiting course and resolve.

Automated summary

Glial fibrillary acidic protein astrocytopathy is an autoimmune meningoencephalomyelitis characterized by fever, headache, movement disorders, and autonomic symptoms. Diagnosis is based on the presence of antibodies against glial fibrillary acidic protein in spinal fluid. There is no standard treatment for this disease.