Dressing Apraxia as Initial Manifestation of Creutzfeldt-Jakob Disease

Abstract

Background: Creutzfeldt-Jakob disease (CJD) is a rare prion disease characterized by rapidly progressive dementia. Case Report: A 76-year-old woman exhibited pronounced signs and symptoms of dressing apraxia for about seven weeks before the disease progressed and probable CJD was diagnosed supported by imaging and CSF findings. Discussion: Dressing apraxia as the initial manifestation of CJD has been sparsely reported. This remarkably focal syndrome should be considered with view on movement and neuropsychological disorders in early CJD.

Ataxias: Hereditary, Acquired, and Reversible Etiologies

“…[148][149][150] Rapidly progressive aphasia to akinetic mutism and cortical blindness are common cognitive deficits, 146 and other higher cortical dysfunction, such as apraxia or auditory agnosia, can also occur. 149,151 The diagnosis of CJD has dramatically improved because of the recent development of CSF RT-QuIC analysis (sensitivity 92%, specificity 100% at UK National CJD Research and Surveillance Unit). 146,152,153 It is important to consider CJD in the differential diagnosis when encountering patients with cerebellar ataxia, especially for those with a rapidly progressive disease course in combination with myoclonus and dementia.…”