Autoimmune GFAP astrocytopathy

The reported symptoms are: • myelitis (68.4%) • headache (63.2%) • abnormal vision (63.2%) • fever (52.6%) • ataxia (36.8%) • psychosis (31.6%) • dyskinesia (15.8%) • dementia (15.8%) • seizure (10.5%) Under MRI these patients show a characteristic radial enhancing and laminar patterns. In an early report, most patients had brain abnormalities (89.5%), of which eight (42.1%) revealed the characteristic radial enhancing and laminar patterns. Cortical abnormalities were found in one-fifth of patients (21.1%). Other abnormalities were found in the hypothalamus, midbrain, pons, medulla cerebellum, meninges, and skull. Eleven patients had longitudinally extensive spinal cord lesions. CSF abnormalities were detected in all patients. ==Clinical courses==

GFAP autoimmunity comprises a spectrum of presentations of meningoencephalomyelitis. Specifically, some courses can be described as relapsing autoimmune meningoencephalomyelitis. The clinical presentations include: • meningoencephalomyelitis • encephalitis • movement disorder (choreoathetosis or myoclonus) • anti-epileptic drugs (AED)-resistant epilepsy • cerebellar ataxia • myelitis • optic neuritis Some clinical courses could be coincident with neuromyelitis optica clinical cases. ==Causes==

The reason that anti-GFAP autoantibodies appear is currently unknown. There is the possibility that GFAP is not pathogenic, but just an unspecific biomarker of several heterogeneous CNS inflammations. According to this hypothesis, GFAP antibody itself does not induce pathological changes; it is only a biomarker for the process of immune inflammation ==Diagnosis==

Currently, it is diagnosed by the presence of anti-GFAP autoantibodies in CNS. Detection of GFAP-IgG in CSF by IFA and confirmation by GFAPα-CBA is recommended. ==Treatment==

Steroids and immunosuppressive treatment have been tried with limited effects. ==References==