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J Neuroophthalmol. 2017 Jun;37(2):149-153. doi: 10.1097/WNO.0000000000000472.

Short Follow-up Bias Confounds Estimates of the "Typical" Clinical Course of Susac Syndrome.

Author information

1
Department of Neurology (IV, SP), Brigham and Women's Hospital, Boston, MA; and Department of Neurology (IV, SP), Harvard Medical School, Boston, MA.

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.

PMID:
27984352
DOI:
10.1097/WNO.0000000000000472
[Indexed for MEDLINE]

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