Short Follow-up Bias Confounds Estimates of the "Typical" Clinical Course of Susac Syndrome.
J Neuroophthalmol
; 37(2): 149-153, 2017 06.
Article
em En
| MEDLINE
| ID: mdl-27984352
ABSTRACT
BACKGROUND:
To evaluate the validity of the prevailing concept that Susac syndrome (SS), a rare microvasculopathy of the brain, retina, and inner ear, is a self-limiting disease.METHODS:
We performed a literature search to identify all cases of SS reported between 1973 and October 2015. If available, we determined their demographics, duration of follow-up, and the clinical course that was labeled as monocyclic or polycyclic. We attempted to determine the number of relapses and the relapse rate in patients with polycyclic disease.RESULTS:
Our literature search yielded 185 relevant publications reporting 405 cases of SS. The duration of follow-up could be determined in 247/405 cases, with a range 0.5-312 months. The mean was 41 months but the distribution was skewed, with a median of 24 months. Defining the clinical course as monocyclic or polycyclic was possible in 102 patients who were followed for greater than 24 months; 53 were identified as having a polycyclic course. Patients labeled polycyclic were followed longer than those labeled monocyclic (median 62 vs 42 months, P < 0.001). The number or frequency of attacks per patient could not be determined.CONCLUSIONS:
The follow-up of published cases of SS is short, creating an inherent bias toward the impression that the disease is self-limiting. Our findings suggest that stratification of SS into monocyclic, polycyclic, and chronic continuous courses may oversimplify the phenotype of SS; instead, the possibility of a relapsing-remitting course must be considered in all patients with this disorder.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Angiofluoresceinografia
/
Tomografia de Coerência Óptica
/
Síndrome de Susac
Tipo de estudo:
Observational_studies
/
Prognostic_studies
Limite:
Humans
Idioma:
En
Ano de publicação:
2017
Tipo de documento:
Article