Neurocognitive Profile of a Woman with Susac's Syndrome: Further Evidence of Cognitive Variability.

OBJECTIVE: Susac's syndrome is a rare autoimmune disease characterized by encephalopathy, retinal artery occlusions, hearing loss, and lesions to the corpus callosum. To date, only four papers (five cases) have described the neuropsychological effects of the syndrome. Extant case reports of Susac's syndrome have documented varying levels of cognitive impairment; some studies have identified diffuse cerebral dysfunction, while others describe more focal impairments in attention, memory, and executive functioning.

METHOD: The goal of this case study was to provide comprehensive neurocognitive data obtained from another case of confirmed Susac's syndrome. As such, we present the case of a 42-year-old woman with a two-year history of Susac's syndrome. At the time of the neuropsychological evaluation, the patient described ongoing difficulties with memory, word-finding problems, problems with math, and poor problem-solving. Physical/sensory changes included hearing loss, tinnitus, and migraines.

RESULTS: Neuropsychological test results revealed the patient to be a woman of estimated average to high average premorbid intellect who exhibited a number of focal inefficiencies in the context of a generally intact profile. Particular cognitive weaknesses emerged on select tasks involving visuoconstruction, encoding of a wordlist, and bilateral speeded finger oscillations. She also committed three failures to maintain set on a task of cognitive flexibility. There was no evidence of memory deficits.

CONCLUSIONS: Our findings provide further evidence of cognitive interindividual variability in a confirmed case of Susac's syndrome.