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Título

FIRST DEMYELINATING EVENT WITH ATYPICAL EVOLUTION AFTER VACCINATION IN A PEDIATRIC PATIENT: A CASE REPORT

Apresentação do caso

CASE REPORT: Girl, 4 years old, healthy. Admitted with right hemiplegia, right central facial paralysis and aphasia over 15 days. Vaccines for triple viral and influenza given in the previous month. No recent infection. Magnetic Resonance Imaging (MRI) with angiography showed hypersignal on T2/FLAIR in the nucleus-thalamo-capsular region, corona radiata and external capsule on the left, without vasculopathy. Negative serology for anti-NMDAr, cultures also negative. Normal ocular fundoscopy. Partial improvement after pulse therapy. Evolved with progressive worsening of symptoms over two weeks, encephalopathy and seizures requiring new hospitalization. Human immunoglobulin 2g/kg was infused, but patient persisted with global deficits. Serology for human immunodeficiency virus and rheumatological tests negative. New MRI showed extensive lesion on the left of frontotemporal region, corpus callosum and thalamus on the right, compatible with demyelination. After 1 week of immunoglobulin a new pulse therapy was performed. Discharged with residual symptoms of right hemiplegia, mild dysphagia and motor aphasia.

Discussão

DISCUSSION: Acute Disseminated Encephalomyelitis (ADEM) is defined as the first episode of demyelization with multifocal deficits and encephalopathy. Typically occurs after infection or immunization. Symptoms improve in a few days, usually recovery in a month and with good response to immunotherapy. MRI shows in the most cases generalized injuries, especially in the basal ganglia and thalamus bilaterally. The patient described started with a single unilateral lesion that evolved in more than a month with bilateral injuries and encephalopathy. She has a recent vaccination history and lesions in a topography compatible with ADEM. She showed limited response to immunotherapy, maintaining residual symptoms. 32 to 50% of children and adolescents with a first acquired demyelination event evolve to multiple sclerosis in 5 years. Tests for diagnosis of multiple sclerosis, anti-MOG and neuromyelitis spectrum are requested, considering that atypical cases of these pathologies have already been reported and treatment is individualized.

Comentários finais

FINAL CONSIDERATIONS: An initial presentation with localized symptoms and a single lesion on imaging don’t exclude demyelinating events. Long-term follow-up and specific serologies will define chronic causes. KEYWORDS: Demyelination diseases; Encephalomyelitis; Immunotherapy.

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Declaração de conflito de interesses de TODOS os autores

All authors declare that they have no conflicts of interest.

Área

Neuroimunologia, esclerose múltipla e outras doenças desmielinizantes