Abstract Details

Title Swallowing function in a long-term survivor with Creutzfeldt-Jakob disease

Topic Aging, Dementia, and Behavioral Neurology

Presentation(s) Aging and Dementia Posters (7:00 AM-5:00 PM)

Poster/Presentation
Number 098

Objective Herein, we reported the serial evaluation of swallowing function of a long-term survivor of V180I gCJD using videofluoroscopic examination of swallowing (VF).

Background Swallowing function in long-term survivors with Creutzfeldt-Jakob disease (CJD) remains unknown.

Design/Methods N/A

Results A 69-year-old woman was admitted to our hospital after 4 months of experiencing memory disturbances and memory disturbances without any family history. Neurological examination revealed dementia, bradykinesia and frontal signs. Diffusion-weighted MRI revealed bilateral cortical hyperintensity in the frontal, temporal, and parietal cortices, and PRNP gene analysis revealed a V180I mutation. Her condition slowly deteriorated to a state of akinetic mutism. Her dysphagia gradually progressed, and she received percutaneous gastrostomy 42 months after the onset. Her family eagerly wanted her to eat by her mouth. VF was performed at 27, 31, 39, 79 and 87 months after the onset. Although bolus transport from oral cavity to pharynx gradually worsened and initiation of the pharyngeal swallow was gradually delayed, the pharyngeal swallowing function was preserved. MRI revealed no apparent atrophy of brainstem, and single photon emission computed tomography showed preserved regional cerebral blood flow in the brainstem. At the age of 76, the patient was still alive and continued to intake food orally with assistance.

Conclusions Swallowing function of patients with V180I gCJD might be preserved even in the akinetic mutism state. The pathophysiology of dysphagia is considered to show a pseudobulbar palsy pattern. Continuing oral intake might be meaningful for families and caregivers with appropriate care.

Authors/Disclosures
Kenjiro Kunieda (Gifu university) PRESENTER	Kenjiro Kunieda has nothing to disclose.
Yuichi Hayashi (Department of Neurology, Gifu University Grad)	Yuichi Hayashi has nothing to disclose.
	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
Takayoshi Shimohata, MD, F�鶹��ýӳ�� (Department of Neurology, Gifu University)	Dr. Shimohata has nothing to disclose.

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