Objective: We present a 56-year-old man patient who diagnosed CJD with CBS-like onset.

Background: Creutzfeldt-Jakob disease (CJD) is an untreatable rare human prion disease characterized by rapidly progressive dementia along with various neurological features, including myoclonus and sometimes other movement disorders  such as chorea, tremor, dystonia, and parkinsonism, including a progressive supranuclear palsy like and corticobasal syndrome (CBS) phenotype.

Methods: The patient had six months  history of motor disorder of left arm. On the examination it was found left-sided hemiparkinsonism with ideomotoric apraxia, alien limb, and positive cathcing reflex- typical features of corticobasal syndrome (CBS). MRI report was minimal global atrophy. The Standardized Mini-Mental State Examination (MMSE) score was 28/30.

Results: After six months he admitted with progressive amnesia and bilateral parkinsonism. The new MRI- DWI was shown hyperintense areas in bilateral  cortex, basal ganglia and thalamus. It was typical for CJD (cortical ribbon). Also, it was shown periodic sharp wave complexes on the EEG. But, it was negative 14-3-3 protein in the cerebrospinal fluid. The new score of MMSE was 12/30.

Conclusions: This case illustrates that CJD can mimic a rare neurodegenerative disorder like CBS as especially clinical signs.

References:

1. Necpal J, Stelzer M, KosIova S, Patarak M. A Corticobasal Syndrome Variant of Familial Creutzfeldt-Jakob Disease with Stroke-Like Onset Case Reports in Neurological Medicine Volume 2016, Article ID 4167391

1. Zhang Y, Minoshima S, Vesselle H, Lewis DH A case of Creutzfeldt-Jakob disease mimicking corticobasal degeneration: FDG PET, SPECT, and MRI findings. Clin Nucl Med. 2012 Jul;37(7):e173-5. doi: 10.1097/RLU.0b013e31824c5f0e.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/creutzfeldt-jakob-disease-with-corticobasal-syndrome-like-onset-case-report/