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Long-term preservation of pharyngeal swallowing function in MM2-cortical-type sporadic Creutzfeldt-Jakob disease
- Source :
- Prion, article-version (VoR) Version of Record
- Publication Year :
- 2021
- Publisher :
- Informa UK Limited, 2021.
-
Abstract
- Swallowing function in long-term survivors of Creutzfeldt-Jakob disease (CJD) has not been elucidated. Herein, we report a patient with MM2-cortical-type sporadic CJD (MM2C-type sCJD) with long-term preservation of pharyngeal swallowing function using videofluoroscopic (VF) examination of swallowing. A 55-year-old woman was admitted to hospital because of dyscalculia and memory disturbance 3 years after the onset of these symptoms. Neurological examination revealed dementia, extrapyramidal signs, and delusion. Diffusion-weighted MRI revealed bilateral hyperintensity in the basal ganglia and frontal, temporal, and parietal cortices. No mutation with the methionine homozygote at codon 129 was found on PRNP gene analysis. VF was performed 68 months after the onset. Although bolus transport from the oral cavity to the pharynx worsened, the pharyngeal swallowing function was preserved even 68 months after onset. Serial MRI examinations revealed no apparent atrophy of the brainstem. Single photon emission computed tomography revealed that the regional cerebral blood flow in the brainstem was preserved. These findings suggest that pseudobulbar palsy is the pathophysiology underlying dysphagia in long-term survivors of MM2C-type sCJD, probably owing to preserved brainstem function even in a state of akinetic mutism.
- Subjects :
- 0301 basic medicine
pseudobulbar palsy
Pediatrics
medicine.medical_specialty
dysphagia
animal diseases
Case Report
Disease
Biochemistry
Creutzfeldt-Jakob Syndrome
03 medical and health sciences
Cellular and Molecular Neuroscience
0302 clinical medicine
Swallowing
Report
mental disorders
otorhinolaryngologic diseases
medicine
Humans
MM2-cortical-type
Tomography, Emission-Computed, Single-Photon
brainstem function
Sporadic CJD
business.industry
Pharyngeal swallowing
Cell Biology
Sporadic Creutzfeldt-Jakob disease
Middle Aged
Pseudobulbar palsy
medicine.disease
Dysphagia
Creutzfeldt-Jakob disease
Deglutition
nervous system diseases
Diffusion Magnetic Resonance Imaging
030104 developmental biology
Infectious Diseases
Pharynx
Female
medicine.symptom
business
human activities
030217 neurology & neurosurgery
Subjects
Details
- ISSN :
- 1933690X and 19336896
- Volume :
- 15
- Database :
- OpenAIRE
- Journal :
- Prion
- Accession number :
- edsair.doi.dedup.....2d2ce8e318a10f866ad3f48b638d666a