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Título

Eight-and-a-half syndrome as a rare initial presentation of Multiple Esclerosis (MS)

RESUMO

A 22-year-old male with no previous medical history is admitted with a new onset strabismus and double vision, both installed three days prior to the consultation. He had no history of trauma, head or ocular pain, vision loss or systemic symptoms. Ophthalmology was the first specialty evaluating the patient, concluding no alterations in their field. Neurological examination showed an ocular misalignment at primary gaze, with right eye exotropia, right conjugate gaze palsy, impairment of right eye adduction and complete right facial nerve palsy. Remaining neurological assessment was preserved. Initial blood investigation tests were negative for inflammatory markers, serological status and rheumatological panel. The Cerebrospinal fluid analysis showed 1 leukocyte/mm³ and 24mg/dL proteins. Magnetic resonance imaging identified several T1-weighted hypointense images with no gadolinium enhancement compatible with “black-holes” in periventricular white matter, as long as T1-weighted hyperintense lesions on cervical spinal cord and right facial colliculus site at the pons, also with no gadolinium enhancement. Patient was treated for MS outbreak with Methylprednisolone 1g IV for five days with complete resolution of diplopia and partial recovery of the eye movements and strabismus. As disease modifying therapy, the patient was put under Natalizumab schedule.

MS is an autoimmune demyelinating disease whose lesions can potentially cause irreversible disabilities when untreated. Among these, neuronal pathways to ocular extrinsic motility are commonly affected. Nevertheless, the presentation of eight-and-a-half syndrome is a very rare and intriguing presentation where a single lesion compromises three pons structures: medial longitudinal fasciculus, leading to homolateral eye adduction deficit (called internuclear ophthalmoplegia); abducens nuclei, impairing homolateral eye abduction and fascicular facial nerve fibers at the genu site where they arch behind abducens nerve, clinically presented as “periferic” pattern facial palsy besides being a central lesion.

In our case we illustrate how the knowledge of anatomical pathways is crucial to clinical neurology, useful for identifying patterns which help correct diagnosis and treatments. Despite continuous improvement, MS remains a challenge to clinicians and neurologists because of its extensive variety of initial presentations, sometimes making the correct diagnosis a true hardship.

Palavras Chave

Área

Neuroimunologia