Creutzfeldt-Jakob Disease May Present Early With Unusual Bulbar Predominance

Malik Ghannam; Roshni Patel; Kent Nichols; Andrew Smith; Jetter Robertson; Brent M. Berry

doi:10.1097/NRL.0000000000000321

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Creutzfeldt-Jakob Disease May Present Early With Unusual Bulbar Predominance

Journal article

Peer reviewed

Creutzfeldt-Jakob Disease May Present Early With Unusual Bulbar Predominance

Malik Ghannam, Roshni Patel, Kent Nichols, Andrew Smith, Jetter Robertson and Brent M. Berry

The Neurologist (Baltimore, Md.), Vol.26(2), pp.69-72

03/04/2021

DOI: 10.1097/NRL.0000000000000321

PMID: 33646993

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Abstract

Introduction: Creutzfeldt-Jakob disease (CJD) is a prion protein disorder of significant consequence and currently incurable. Diagnosis can be challenging early in the disease course. CJD can present in many ways but often fits a pattern of cognitive problems, cerebellar disturbance, behavioral/psychological changes, and perhaps myoclonus. Case Report: We herein present the case of a 69-year-old White male with subacute progressive bulbar and limb weakness over ten weeks period. Early on, he was diagnosed with amyotrophic lateral sclerosis versus autoimmune-related bulbar neuropathy and treated as such. However, he continued to deteriorate clinically that prompted another admission, upon readmission, his cerebrospinal fluid RTQuick and 14-3-3 from the National Prion Disease Pathology Surveillance Center (NPDPSC) did eventually return positive. Hence he was diagnosed with CJD. Conclusions: CJD may present with progressive bulbar symptoms similar to acute inflammatory demyelinating polyradiculoneuropathy (MF variant), motor neuron disease, or autoimmune brainstem encephalitis. It becomes even higher on the differentials especially with no response to immunotherapy.

Clinical Neurology

Life Sciences & Biomedicine

Neurosciences & Neurology

Science & Technology

Details

Title: Subtitle: Creutzfeldt-Jakob Disease May Present Early With Unusual Bulbar Predominance
Creators: Malik Ghannam - University of Minnesota
Roshni Patel - University of Minnesota
Kent Nichols - University of Minnesota
Andrew Smith - University of Minnesota
Jetter Robertson - University of Minnesota
Brent M. Berry - University of Minnesota
Resource Type: Journal article
Publication Details: The Neurologist (Baltimore, Md.), Vol.26(2), pp.69-72
Publisher: Lippincott Williams & Wilkins
DOI: 10.1097/NRL.0000000000000321
PMID: 33646993
ISSN: 1074-7931
eISSN: 2331-2637
Number of pages: 4
Language: English
Date published: 03/04/2021
Academic Unit: Neurology
Record Identifier: 9984585426502771

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