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Clinical Overlap between Jakob-Creutzfeldt Disease and Lewy Body Disease

Published online by Cambridge University Press:  02 December 2014

Maria Carmela Tartaglia ,
David Y. Johnson ,
Julie Ngoc Thai ,
Tatiana Cattaruzza ,
Katherine Wong ,
Paul Garcia ,
Stephen J. DeArmond ,
Bruce L. Miller  and
Michael D. Geschwind
Maria Carmela Tartaglia
Affiliation:
Department of Neurology, University of California, San Francisco, San Francisco, California, USA Toronto Western Hospital, University of Toronto, Toronto, Ontario, Canada
David Y. Johnson
Affiliation:
Department of Neurology, University of California, San Francisco, San Francisco, California, USA
Julie Ngoc Thai
Affiliation:
Department of Neurology, University of California, San Francisco, San Francisco, California, USA
Tatiana Cattaruzza
Affiliation:
Department of Internal Medicine and Clinical Neurology, University of Trieste, Trieste, Italy
Katherine Wong
Affiliation:
Department of Neurology, University of California, San Francisco, San Francisco, California, USA
Paul Garcia
Affiliation:
Department of Neurology, University of California, San Francisco, San Francisco, California, USA
Stephen J. DeArmond
Affiliation:
Department of Pathology, University of California, San Francisco, San Francisco, California, USA
Bruce L. Miller
Affiliation:
Department of Neurology, University of California, San Francisco, San Francisco, California, USA
Michael D. Geschwind*
Affiliation:
Department of Neurology, University of California, San Francisco, San Francisco, California, USA
*
Department of Neurology, Memory & Aging Center, 350 Parnassus Avenue, Suite 905, University of California San Francisco, San Francisco, California 94117, USA. Email: mgeschwind@memory.ucsf.edu
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Abstract

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Objective:

Sporadic Jakob-Creutzfeldt disease (sCJD) and dementia with Lewy bodies (DLB) have overlapping clinical symptoms that can lead to their misdiagnosis. We delineated the clinical overlap between sCJD and DLB, and assessed the value of magnetic resonance imaging (MRI) to differentiate between them.

Methods:

Medical records, MRI, electroencephalogram (EEG) and cerebrospinal fluid (CSF) were reviewed from 56 sCJD and 30 DLB subjects.

Results:

46% of sCJD subjects met probable DLB criteria and 40% of DLB subjects met probable CJD criteria. A greater proportion of sCJD subjects had cerebellar signs (66% vs. 10%, p<0.001), myoclonus (64% vs. 30%, p=0.002), and visual symptoms (other than hallucinations) (61% vs. 7%, p<0.001), whereas more DLB subjects had hallucinations (70% vs. 39%, p=0.007) and fluctuations (57% vs. 23%, p=0.002). Cortical and/or basal ganglia MRI diffusion weighted imaging hyperintensities consistent with sCJD were seen in 96% of sCJD subjects but in none with DLB. Logistic regression in sCJD revealed that those meeting probable DLB criteria were more likely to have occipital lobe involvement on MRI (OR 1.4, p=0.058, model p=0.022). Parietal lobe involvement on MRI was a predictor of “Other Focal Cortical signs” (OR 1.9, p=0.021). EEG and CSF assessments lacked sensitivity for sCJD as 48% of sCJD patients had a negative EEG; 67% of the 36 sCJD patents with a CSF evaluation had a negative or inconclusive 14-3-3 result. Too few DLB patients had EEG or CSF to assess their utility.

Conclusion:

Sporadic CJD and DLB have significant symptom overlap. MRI helps differentiate these diseases and is related to the signs/symptoms observed in sCJD.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 39 , Issue 3 , May 2012 , pp. 304 - 310
Copyright
Copyright © The Canadian Journal of Neurological 2012

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