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Burton J, Hertza J, Repp Z, Jupp K, A-074 Evidence of Cognitive Decline in a Patient with CADASIL Without Major Stroke Event, Archives of Clinical Neuropsychology, Volume 35, Issue 6, September 2020, Page 866, https://doi.org/10.1093/arclin/acaa068.074
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Abstract
Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) is a genetic disease that impacts small arteries in the brain and can result in ischemic strokes, seizures, and cognitive decline. This case study of a 61-year-old male illustrates the cognitive profile of an individual with CADASIL who has not suffered a major stroke event. Neuroimaging shows subcortical compromise in a diffuse manner.
The patient was assessed in an out-patient institutional practice. The individual was assessed with a clinical interview and comprehensive Neuropsychological Battery. The battery included cognitive and psychological measures with free-standing and imbedded validity measures.
Cognitive testing demonstrated significant impairment in visuo-motor processing speed, executive planning, cognitive flexibility, verbal fluency, decision making, and visual memory, and suggests that the patients abilities show deterioration of subcortical regions, and is consistent with his CADASIL diagnosis. The patient was diagnosed with Mild Neurocognitive Disorder, due to medical condition.
Individuals with CADASIL often suffer from ischemic strokes and cognitive impairment. This study suggests that despite no major stroke event, individuals with CADASIL may still exhibit cognitive decline especially in subcortical regions. This case supports the theory that progressive cognitive decline in CADASIL patients does not begin as a result of a major stroke and can continue as a distinct cause of vascular neurocognitive disorder.
