Dados do Trabalho

Título

CENTRAL NERVOUS SYSTEM VASCULITIS VERSUS MOYA-MOYA SYNDROME RELATED ACUTE ISCHEMIC STROKE

Apresentação do caso único

A 15yo male with Down syndrome was admitted with an acute disperceptive focal seizure characterized by behavioral arrest, acute aphasia for 3 minutes. He previously reported acute facial asymmetry and decreased visual acuity in the preceding 3 days. His medical history includes corrected congenital cardiopathy, B-cell acute lymphoblastic leukemia with CNS infiltration, treated with chemotherapy, haploidentical hematopoietic stem cell transplantation in 2022, total body radiotherapy and fludarabine. The patient has been out of treatment since July 2022. Additionally, he experienced left third cranial nerve neuropathy due to graft-versus-host disease (GVHD) in 2023. Neurological examination revealed only left deviation of the labial rhyme. MRI showed extensive cortico-subcortical signal alterations and diffusion restriction in the left occipital-temporal region, along with smaller foci with similar alterations in the left hemisphere, particularly in the frontotemporal region. There were also findings of bilateral middle cerebral artery stenosis, moderate internal carotid artery stenosis, and asymmetry in the posterior cerebral and superior cerebellar arteries. Cerebrospinal fluid analysis was normal and negative for neoplastic cells. Initially, CNS vasculitis and Moya-Moya Syndrome (MMS) was suspected and patient received an empirical 5-day course of steroid pulse therapy. Subsequent cerebral angiography confirmed MMS and the patient was referred to Neurosurgery.

Discussão

This case presented a diagnostic challenge of stroke in a child with multiple comorbidities. Acute leukemia is a well-known risk factor for stroke, primarily due to its treatment—chemotherapy and radiotherapy—with an increased risk that persists throughout the patient's life. The patient’s history of focal GVHD, along with cerebral vessel changes and multifocal diffusion restriction on MRI, initially suggested CNS vasculitis. Additionally, Down syndrome and cranial irradiation, which are associated with MMS particularly in adolescence, contributed to the diagnostic complexity. Angiography was crucial in reaching the correct etiological diagnosis and determining the optimal therapeutic approach.

Comentários finais

Acute stroke in children is rare but multifactorial, necessitating specific diagnostic and treatment strategies. Employing appropriate diagnostic tools and considering the patient’s medical history are essential for effective management.

Referências

Rodrigues, M. M., Vilanova, L. C. P., Tratado de neurologia infantil / Marcelo Masruha Rodrigues, Luiz Celso Pereira Vilanova. - 1. ed. -
Rio de Janeiro : Atheneu, 2017.
Abdelgadir, A. et al. A Better Understanding of Moyamoya in Trisomy 21: A Systematic Review. Cureus, mar. 2022; 14(3): e23502. Publicado online em 26 de março de 2022. doi: 10.7759/cureus.23501. Acesso em: 01 de agosto de 2024.

Palavras Chave

moyamoya; stroke; vasculitis