Creutzfeldt-Jakob disease with slow progression. A mimickry of progressive supranuclear palsy

Sporadic Creutzfeldt-Jakob disease (sCJD) does not always present with typical clinical signs, such as myoclonus in association with periodic sharp-wave complexes. We present a 67-year old female patient with initial falls and vertical gaze palsy, suggesting the diagnosis of Progressive Supranuclear...

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Published inBulletin de la Société des sciences médicales du Grand-Duché de Luxembourg no. 2; p. 125
Main Authors Huber, F M, Bour, F, Sazdovitch, V, Hauw, J J, Heinemann, U, Zanini, F, Droste, D W, Diederich, N J
Format Journal Article
LanguageEnglish
Published Luxembourg 2007
Subjects
Aged
Atrophy
Brain - pathology
Creutzfeldt-Jakob Syndrome - diagnosis
Creutzfeldt-Jakob Syndrome - physiopathology
Diagnosis, Differential
Disease Progression
Female
Humans
Magnetic Resonance Imaging
Supranuclear Palsy, Progressive - diagnosis
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Summary:Sporadic Creutzfeldt-Jakob disease (sCJD) does not always present with typical clinical signs, such as myoclonus in association with periodic sharp-wave complexes. We present a 67-year old female patient with initial falls and vertical gaze palsy, suggesting the diagnosis of Progressive Supranuclear Palsy (PSP). EEG and MRI were not contributory. Typical clinical and paraclinical CJD signs were only seen after 17 months. The diagnosis was confirmed by autopsy. - CJD can be a neurodegenerative chameleon. The present case adds to the scare literature of slowly evolving CJD mimicking Parkinsonism related to tauopathies.
ISSN:0037-9247