Autoimmune GFAP astrocytopathy. Lecture and clinical cases

Autoimmune GFAP astrocytopathy is a rare neurological disorder first described in 2016, characterized by immune-mediated damage to astrocytes in the central nervous system. Associated with antibodies to glial fibrillary acidic protein (GFAP), the disease presents diverse clinical symptoms, including meningoencephalomyelitis and visual system involvement. Timely diagnosis and treatment are crucial due to the high efficacy of corticosteroids and the potential for relapses. The most common manifestations are meningoencephalomyelitis (55–60 % of cases) and meningoencephalitis (40–45 % of cases). Symptoms include headache, fever, nausea, vomiting, delirium, epileptic seizures, and motor disturbances (ataxia, tremor, myoclonus). Visual system involvement occurs in 25 % of patients, presenting as optic disc edema, blurred vision, and rarely, optic neuritis. In children, the clinical picture resembles that of adults but more frequently includes encephalitis and epileptic seizures. The article presents two clinical cases. The first involves a 14-year-old girl with meningoencephalomyelitis who developed visual impairment, ataxia, and other neurological symptoms following a viral infection. Magnetic resonance imaging revealed multiple demyelination foci, and cerebrospinal fluid analysis confirmed GFAP antibodies. The second case involves a 6-year-old girl with sudden vision loss after a viral infection, diagnosed with optic disc edema and GFAP antibodies in the cerebrospinal fluid. The primary diagnostic marker is the detection of GFAP antibodies in cerebrospinal fluid. Magnetic resonance imaging findings include linear perivascular enhancement in white matter and T2/FLAIR hyperintense lesions. Differential diagnosis includes multiple sclerosis, sarcoidosis, infectious diseases, and other autoimmune conditions. Corticosteroids (methylprednisolone, prednisolone) are the cornerstone of treatment, showing high efficacy. Intravenous immunoglobulin and plasma exchange are used for non-responsive cases. Immunosuppressants (mycophenolate mofetil, rituximab) are employed to prevent relapses. Treatment duration ranges from 2 to 5 years, with lifelong therapy considered in cases of relapse. With adequate treatment, the prognosis is generally favorable. Most patients achieve significant improvement, although relapses occur in 20–50 % of cases. Children tend to have better short-term outcomes compared to adults. Autoimmune GFAP astrocytopathy remains poorly understood, necessitating further research to elucidate its pathogenesis and optimize treatment strategies. The presented cases underscore the importance of early diagnosis and personalized therapy.