Objective:

We examined the frequency and the type of movement disorders in the published literature among patients with Susac syndrome. 

Background:

Susac syndrome is a rare disease characterized by an inflammatory microangiopathy limited to the vessels of the brain, eyes, and ears. Young women are primarily affected.   Recent findings support a primitive vasculitis affecting the cerebral, retinal, and cochlear small arteries, although the mechanism remains unclear. The diagnosis is based on the identification of the triad: subacute encephalopathy, bilateral occlusion of the retinal arterial branches on fundoscopy, and hearing loss. Movement abnormalities are still underreported in the disease, though. We outline the types and prevalence of movement disorders found in the literature. 

Design/Methods:

A thorough literature review was conducted using PubMed and Google Scholar, in accordance with PRISMA criteria. Movement disorders, tremors, chorea, dystonia, ataxia, myoclonus, nystagmus, and Susac syndrome are the terms that have been used. We considered ten studies in our analysis.

Results:

A total of 189 patients were included, with a mean age of 31 ± 2.16 years and 72.5% being female. Ataxia was identified in 84.7% of cases (160 out of 189 patients), while eye movement abnormalities, including nystagmus or saccadic ocular pursuit, were present in 15.3% (29 out of 189 patients). Some patients exhibited more than one type of movement disorder.

Conclusions:

Among those with Susac syndrome, ataxias are among the most reported movement disorders. Clinicians should be aware of these symptoms to aid in differential diagnosis.