Clinical Snapshot
Cortical Signal Changes in Creutzfeldt-Jakob Disease
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A 64-year-old former math teacher presented with an approximately 8-month history of slowly progressive difficulty finding his words, impaired memory, gait instability, as well as impaired fine motor function and use of the right hand. Clinical neurological findings included high-grade cognitive impairment with incomplete orientation, memory deficits, dyscalculia, dysgraphia, and apraxia. The patient exhibited right-sided bradydiadochikinesia, individual instances of non-startle-induced myoclonus, and impaired fine motor function. Electroencephalography showed temporary slowing and was otherwise unremarkable. Magnetic resonance imaging revealed ubiquitous cortical signal enhancement on the diffusion-weighted images with with lowered apparent diffusion coefficient (ADC). Extensive ribbon-like cortical signal changes have a broad differential diagnosis: hypoxic-ischemic lesions (for example, following resuscitation), postictal changes, hypoglycemic attacks, infections, mitochondriopathies, and Creutzfeldt-Jakob disease (CJD). The patient history was in itself suggestive of the prion disease CJD. Cerebrospinal fluid findings (elevated neuron-specific enolase, tau protein and protein 14–3–3, positive PrPSc aggregates) confirmed this. History, course, and imaging were suggestive neither for a surgery or meat consumption-induced nor a genetic disease variant. It is likely that this case represented a sporadic form. The patient died after an approximately 1-year disease course.
Prof. Dr. med. Georg Hagemann, Prof. Dr. med. Marius Hartmann, Klinik für Neurologie, HELIOS Klinikum Berlin-Buch, georg.hagemann@helios-gesundheit.de
Conflict of interest statement: The authors state that no conflict of interest exists.
Translated from the original German by Christine Rye.
Cite this as: Hagemann G, Hartmann M: Cortical signal changes in Creutzfeldt-Jakob disease. Dtsch Arztebl Int 2022; 119: 684. DOI: 10.3238/arztebl.m2022.0168
