Creutzfeldt-Jakob Disease with Paralysis of the Unilateral Vocal Cord and Soft Palate

Creutzfeldt-Jakob Disease with Paralysis of the Unilateral Vocal Cord and Soft Palate





Jun Hasegawa1), Yuuri Okumura1)2), Etsuko Osumi3), Hideaki Tago4), Yukio Katori2) and Toshimitsu Kobayashi2)




1) Department of Otorhinolaryngology, Public Soma General Hospital
2) Department of Otorhinolaryngology-Head and Neck Surgery, Tohoku University Graduate School of Medicine
3) Department of Neurology, Miyagi National Hospital
4) Department of Neurology, Ishibashi Hospital



(Received July 19, 2011)
(Revision accepted for publication November 5, 2011)



Creutzfeldt-Jakob disease (CJD) is a progressive disease that is characterized by the accumulation of abnormal prion-like proteins in the central nervous system. The cerebral cortex is primarily affected in CJD, leading to spongiform changes and dementia. To date, there have been no reported cases of CJD, with local neuroparalysis discovered at an early stage of the disease. Here, we describe a patient who presented unilateral vocal cord and soft palate paralysis before the progression of CJD. After developing forgetfulness 6 months ago, a 76-year-old woman was presented at department of Otorhinolaryngology in a general hospital for recently developed hoarseness and dysphagia. In the oral and laryngeal endoscopic findings, unilateral paralysis of the vocal cord and soft palate was noted. On videofluorography, the larynx failed to elevate straight on swallowing. The right tongue pharyngeal wall was lax, and some contrast agent was retained in the lower right piriform sinus. The paralysis was thought to be due to the glossopharyngeal nerve or vagal nerve damage, which was caused by peripheral nerve injury or infranuclear palsy. Diffusion-weighted magnetic resonance imaging (MRI) revealed high signals in the cerebral cortical area (a signature feature of CJD). The patient died 2.5 years after the onset of illness. The patient was diagnosed as probable sporadic CJD. Since we could not detect any peripheral organic findings that could cause the paralysis, we suspect that CJD is responsible for the paralysis. In treating CJD patients with neurological signs, exclusive investigation is required to obtain a more detailed picture of the disease.



Creutzfeldt-Jakob disease; diffusion-weighted MRI; prion disease; soft palate paralysis; unilateral vocal cord paralysis





http://www.jstage.jst.go.jp/article/tjem/225/4/225_277/_article





FULL TEXT ;



http://www.jstage.jst.go.jp/article/tjem/225/4/277/_pdf






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Saturday, June 25, 2011


Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque


"BSE-L in North America may have existed for decades"


http://transmissiblespongiformencephalopathy.blogspot.com/2011/06/transmissibility-of-bse-l-and-cattle.html





EFSA Journal 2011 The European Response to BSE: A Success Story

This is an interesting editorial about the Mad Cow Disease debacle, and it's ramifications that will continue to play out for decades to come ;

Monday, October 10, 2011

EFSA Journal 2011 The European Response to BSE: A Success Story

snip...

EFSA and the European Centre for Disease Prevention and Control (ECDC) recently delivered a scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans (EFSA Panel on Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical BSE prions as the only TSE agents demonstrated to be zoonotic so far but the possibility that a small proportion of human cases so far classified as "sporadic" CJD are of zoonotic origin could not be excluded. Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential.

snip...



http://www.efsa.europa.eu/en/efsajournal/pub/e991.htm?emt=1



http://www.efsa.europa.eu/en/efsajournal/doc/e991.pdf




see follow-up here about North America BSE Mad Cow TSE prion risk factors, and the ever emerging strains of Transmissible Spongiform Encephalopathy in many species here in the USA, including humans ;




http://transmissiblespongiformencephalopathy.blogspot.com/2011/10/efsa-journal-2011-european-response-to.html





http://transmissiblespongiformencephalopathy.blogspot.com/






Thursday, August 4, 2011

Terry Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis, Date aired: 27 Jun 2011

(SEE VIDEO)


http://transmissiblespongiformencephalopathy.blogspot.com/2011/08/terry-singeltary-sr-on-creutzfeldt.html




Sunday, August 21, 2011

The British disease, or a disease gone global, The TSE Prion Disease


(SEE VIDEO)



http://transmissiblespongiformencephalopathy.blogspot.com/2011/08/british-disease-or-disease-gone-global.html



Saturday, March 5, 2011

MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA



http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/mad-cow-atypical-cjd-prion-tse-cases.html





TSS