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From: TSS ()
Subject: Involvement of the peripheral nervous system in human prion diseases including dural graft associated Creutzfeldt–Jakob disease
Date: February 17, 2005 at 9:38 am PST

-------- Original Message --------
Subject: Involvement of the peripheral nervous system in human prion diseases including dural graft associated Creutzfeldt–Jakob disease
Date: Wed, 16 Feb 2005 21:11:26 -0600
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@LISTSERV.KALIV.UNI-KARLSRUHE.DE


##################### Bovine Spongiform Encephalopathy #####################

Journal of Neurology Neurosurgery and Psychiatry 2005;76:325-329
© 2005 BMJ Publishing Group Ltd

------------------------------------------------------------------------


PAPER


Involvement of the peripheral nervous system in human prion diseases
including dural graft associated Creutzfeldt–Jakob disease

C Ishida1, S Okino1, T Kitamoto2 and M Yamada1

1 Department of Neurology and Neurobiology of Aging, Kanazawa University
Graduate School of Medical Science, Kanazawa, Japan
2 Department of Neurological Science, Tohoku University Graduate School
of Medicine, Sendai, Japan

Correspondence to:
Dr Masahito Yamada
Department of Neurology and Neurobiology of Aging, Kanazawa University
Graduate School of Medical Science, Takara-machi, 13-1, Kanazawa,
Ishikawa 920-8640, Japan; m-yamada@med.kanazawa-u.ac.jp


Objective: To investigate abnormal prion protein (PrP) deposition in the
peripheral nervous system (PNS) in human prion diseases.

Methods: Eight patients with prion diseases were examined: three with
sporadic Creutzfeldt–Jakob disease (sCJD), two with dural graft
associated CJD (dCJD), one with Gerstmann–Sträussler–Scheinker disease
(GSS) with a PrP P102L mutation (GSS102), and two with a P105L mutation
(GSS105). An atypical case of sCJD with PrP plaques in the brain
presented clinically with peripheral neuropathy, and showed
demyelination in 12% of the teased fibres of the sural nerve. The PNS
was investigated by immunohistochemical and western blotting analyses of
PrP.

Results: In immunohistochemical studies, granular PrP deposits were
detected in some neurones of dorsal root ganglia and a few fibres of
peripheral nerves and spinal posterior roots in one sCJD and two dCJD
patients, but not in GSS102 or GSS105 patients. The atypical case of
sCJD with peripheral neuropathy showed no obvious PrP deposition in the
nerves. Western blotting analysis of the PNS from the dCJD patients
revealed a small amount of protease K resistant PrP in the dorsal root
ganglia and peripheral nerves.

Conclusions: Abnormal PrP deposition occurs in the dorsal root ganglia
and peripheral nerves in sCJD and dCJD. The PrP deposits in the PNS are
not correlated with clinical manifestation of peripheral neuropathy in CJD.

------------------------------------------------------------------------

Abbreviations: CJD, Creutzfeldt–Jakob disease; dCJD, dural graft
associated CJD; GSS, Gerstmann–Sträussler–Scheinker disease; PNS,
peripheral nervous system; PrP, prion protein; sCJD, sporadic CJD

Keywords: Creutzfeldt–Jakob disease; neuropathy; peripheral nervous
system; prion disease


http://jnnp.bmjjournals.com/cgi/content/abstract/76/3/325?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=prion&searchid=1108609455773_4700&stored_search=&FIRSTINDEX=0&volume=76&issue=3&journalcode=jnnp

TSS

######### https://listserv.kaliv.uni-karlsruhe.de/warc/bse-l.html ##########






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