Dados do Trabalho


Título

DOUBLE-POSITIVE NEUROMYELITIS OPTICA SPECTRUM DISORDER IN A CHILD FOLLOWING HERPETIC INFECTION: CASE REPORT

Apresentação do caso único

A female patient, 11, in July 2022, presented with progressive back pain, headache, and right sided amaurosis, subsequently developing paresis and tremors in the extremities. During investigation, cerebrospinal fluid (CSF) testing revealed positive IgM for Herpes Virus, and cranial MRI showed signs of central demyelination, with no orbital abnormalities. She was treated with acyclovir, ceftriaxone, and methylprednisolone for 14 days, with partial improvement of symptoms. However, after one week, she returned with a worsening headache and right sided amaurosis. Comparative imaging showed an aggravation on orbital MRI, revealing thickening of optic nerves with high signal on STIR and intense contrast enhancement, predominantly on the right side, suggestive of optic neuritis. Additionally, cranial MRI showed multiple new lesions with high signal on T2/FLAIR, primarily juxtacortical in the cerebral hemispheres, as well as in thalamic nuclei and the posterior fossa, indicative of a demyelinating pathology with temporal and spatial progression. Ophthalmology assessment revealed reduced visual acuity, particularly on the right side, altered pupillary reflex, and fundoscopy with diffuse optic disc edema in the right eye and nasal optic disc edema in the left eye. Treatment was reintroduced with acyclovir, methylprednisolone, and immunoglobulin for 5 days, followed by medical release with prednisolone. Subsequent outpatient visits revealed confirmed positive Myelin Oligodendrocyte Glycoprotein (ANTI-MOG) and Aquaporin 4 (AQP4) antibodies, supporting the hypothesis of neuromyelitis optica spectrum disorder (NMOSD), with double antibody positivity. Maintenance treatment was tapering prednisolone and continuous azathioprine, without new disease relapses.

Discussão

NMOSD is characterized by demyelination, immune-mediation and inflammation that affects the optic nerve and spinal cord, with a variable course. It can be triggered during or after viral infections. It is most commonly associated with positivity for AQP4-IgG antibodies, but it can also present as double-positive for both MOG-IgG and AQP4-IgG antibodies. These cases are rare and not extensively documented.

Comentários finais

Considering that Double-Positive NMOSD is still under study and it is a rare condition, especially in the pediatric population, this case report aims to discuss diagnosis, treatment, and progression of this condition in a pediatric patient following herpesvirus infection of the central nervous system.

Referências

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Palavras Chave

Neuromyelitis optica; Antibodies; Herpes Simplex

Área

Neuroimunologia, esclerose múltipla e outras doenças desmielinizantes

Autores

GABRIELA CRISTINA FACCO, MARIA FERNANDA BATALHA MARQUES, MARIANA COSTA DE CARVALHO SILVA, BEATRIZ SILVA LOMBARDI, PRISCILA FLORÊNCIO OLIVEIRA, LUCAS HENRIQUE FACCO, ADRIANA OLIVEIRA RIBEIRO SANTOS, JOÃO CARLOS DINIZ