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Título

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) with PNS involvement: a case report

RESUMO

Case presentation: GLF, male, 13 years old. The patient came for consultation in June 2020. At the age of three, he had a generalized tonic-clonic febrile seizure with post-ictal drowsiness. From then on, a difficulty in school learning was noticed. At the age of 7, he evolved with bilateral visual impairment with pain at eye mobilization. Two years later, he started with distal weakness and frequent falls. He had no family history. The patient was oriented, but with childish behavior for age. Mini-mental state exam: 22/30. He also presented flaccid tone and global hypotrophy associated with symmetrical tetraparesis predominantly crural. Deep tendon reflexes and cutaneous-plantar reflex were absent. Cerebellar gait with dysdiadochokinesia and dysmetria was evident. Distal hypopalesthesia was noticed. Visual acuity: 20/300 in the left eye and 20/200 in the right eye. Fundoscopy showed bilateral optic nerve atrophy. Considering the phenotype of presentation, findings in complementary exams and epidemiological profile, we chose to measure the anti-MOG IgG, with a positive result, corroborating a diagnosis of Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) with PNS involvement, manifested in our patient as polyradiculoneuropathy. Discussion: MOGAD is an immune-mediated demyelinating inflammatory disorder of the central nervous system predominantly affecting the optic nerves, brain, and spinal cord. The disease has a predilection for children. Oligodendrocyte myelin glycoprotein (MOG) is a protein on the surface of oligodendrocytes that has historically been considered as a potential antibody target in multiple sclerosis, until in 2007 it was described as a different disease phenotype. According to a database review (PUBMED), there are a few studies that corroborate the occurrence of peripheral impairment associated with MOGAD, present in 7% of MOGAD patients in an Australian cohort. There are some hypotheses to justify the concomitant central and peripheral demyelination processes: one of the most accepted claims that MOG is found in an isoform that can be released into the CSF, triggering or “spreading” autoimmunity when drained into the PNS. Final comments: The presence of central demyelination syndrome and SNP involvement may be associated with MOGAD and may respond to immunotherapy, and should not discourage the search for MOGAD in patients with an appropriate clinical setting.

Palavras Chave

Myelin oligodendrocyte glycoprotein antibody-associated disease; Peripheral nervous system; Anti-MOG IgG