Dados do Trabalho
ANTI-MOG-ASSOCIATED DISEASE: OVERLAPPING SYNDROMES
Apresentação do caso
A 9-year-old previously healthy girl was referred to our emergency with psychomotor retardation, headache, and vomiting for the last 40 days. She has had a fever in the first 2 weeks and brief self-resolved episodes of right arm limbs weakness, dysphagia, and urinary incontinence. Head CT and MRI performed within 10 days of symptoms were normal. On neurologic exam, she presented marked fluctuation in consciousness, pyramidal liberation syndrome on both legs and cerebellar ataxia. Another CT was performed to exclude intracranial hypertension. Laboratory screening for metabolic disturbances came normal. Lumbar puncture revealed 8 cells with lymphomononuclear predominance, 0 red blood cells, 30mg/dl protein, 48mg/dl glucose (glycemia 91mg/dl), negative cultures, and positive oligoclonal bands. After 75 days of the first symptom, we managed to do a brain and spinal cord MRI which showed small nodular foci of T2/Flair hyperintensity on the anterior branch of the left internal capsule, cerebellum, pons, medulla oblongata and periventricular (leukodystrophy-like phenotype), as well as T2-hyperintensity with enhancement on C2, C3-C4 and T6-T7, enhancement of thoracic and lumbar roots, cauda equina and leptomeningeal enhancement at conus medullaris. A cell-based assay for serum MOG-IgG became positive. An electroencephalogram showed mild to moderate dysfunction and focal epilepsy. The patient received methylprednisolone 1g for 5 days, followed by human immunoglobulin 2g/kg for 2 days, and, later, a second course of pulse therapy, so she was discharged asymptomatic.
Anti-MOG associated disease (MOGAD) is one of the acquired demyelinating central nervous system disorders most common in children with different phenotypes, which can occur in isolation or various combinations. They are acute disseminated encephalomyelitis (ADEM), optic neuritis, myelitis, cortical encephalitis, cerebellitis, and brainstem syndromes. Initial brain or spinal cord MRI in MOGAD can be normal in up to 10% of cases despite severe acute disability, which, added to the possibility of overlapping syndromes, including an opening in the form of encephalitis with later evolution to a demyelinating condition, makes the most challenging diagnosis.
The case reinforces the possibility that patients with anti-MOG-associated cortical encephalitis may experience previous, concomitant, or subsequent clinical and/or radiological demyelinating events.
Referências (se houver)
MOGAD. Anti-MOG. Encephalitis. Demyelinating
Fonte de Fomento (se houver)
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Neuroimunologia, esclerose múltipla e outras doenças desmielinizantes
Hospital das Clinicas da Faculdade de Medicina de Ribeirão Preto - São Paulo - Brasil
Eduardo Silveira Marques Branco, Amanda Povoa de Paiva, Bruno Antunes Contrucci, Roberta Fantauzzi Borges, Vanessa Limeira Pontes de Lucena, Ana Paula Ribeiro Faria, Maiave Micaelle Figueredo de Matos, Maria Avanise Yumi Minami, Ana Paula de Andrade Hamad