Dados do Trabalho

Título

EIGHT-AND-A-HALF SYNDROME AS INITIAL PRESENTATION OF PEDIATRIC MULTIPLE SCLEROSIS: A CASE REPORT

Apresentação do caso único

A previously neurologically healthy sixteen years old boy notices a rapid-onset double vision when waking up in the morning and a horizontal misalignment of the eyes, with the left eye deviated outward. During the following five days, he complains of a continuous constriction-type headache, as well as vertigo. In the neurological examination, a conjugate gaze palsy to the right side and limited adduction of the right eye with an abduction nystagmus of the left eye, concurrent with a right facial nerve palsy were remarkable, allowing the characterization of a right-sided eight-and-a-half syndrome. After brain and spine magnetic resonance imaging, the time-and-space disseminated white matter lesions fulfilled 2017's McDonald criteria for multiple sclerosis, and differential diagnosis were excluded.

Discussão

Albeit infrequent in the pediatric population, with an average global incidence of 0.87 per 100,000 per year, multiple sclerosis is a relevant neuroimmunological disorder, due to its potential morbid presentation and long-lasting neurological impairment. Eight-and-a-half syndrome is a rare neuro-ophthalmologic condition which is defined as one-and-a-half syndrome plus facial nerve palsy ipsilateral to the side of one eye which has no movement (1.5 + 7 = 8.5). It has an important role in implicating the involvement of the medial dorsal pons, where the medial longitudinal fasciculus (MLF) and the abducens nucleus are encountered, with the fascicular part of the facial nerve surrounding the sixth nerve nucleus paramedially.

Comentários finais

The clinical presentation of eight-and-a-half syndrome arises from the involvement of the abducens nucleus, ipsilateral medial longitudinal fasciculus, and facial nucleus or fascicle within the lower pontine tegmentum. Although the underlying lesion may have various nosologies, such as demyelinating, infectious, vascular and neoplastic, the recognition of the syndrome enables precise localization of the lesion to the ipsilateral lower pontine tegmentum.

Referências

1. Cureus. 2024 Jul 22;16(7):e65138.
2. CNS Drugs. 2022 Jan;36(1):45-59.

Palavras Chave

Eight-and-a-half syndrome; Pediatric multiple sclerosis; Pontine lesions