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doi:10.1016/j.forsciint.2004.09.
Copyright © 2004 Elsevier Ireland Ltd All rights reserved.
A Heidenhain variant of Creutzfeldt–Jakob disease: forensic implication
M. Rizzoa
,
Corresponding Author Contact Information
,
E-mail The Corresponding Author , A. Brunib
,
C. Barberioc
,
G. Magroa
and J.F. Foncind
aForensic Medicine Specialist, Catanzaro, Italy
bNeurogenetic Department, Lamezia Terme Hospital, Italy
cDepartment of Legal Medicine, Lamezia Terme Hospital, Italy
dEPHE Neurohistology Unit, Paris, France
Available online 17 November 2004.
Abstract
To investigate whether typical clinical, diagnostic and
neuropathological findings can be identified in a patient with a
postmortem diagnosis of a Heidenhain variant of Creutzfeldt–Jakob
disease (CJD). We report a new case of CJD in a rare variant. A man
admitted to hospital with cefalea and vision disorder. Clinical and
neurological examination showed headache, vision reduction, psychomotor
anxiety and progressive torpor. The patient died 4 h after admission to
hospital. The autopsy findings included marked encephalic vascular
congestion. Hystoneurology examination showed no macroscopic anomaly.
Microscopy findings included neuronal loss, gliosis in striate area with
arachnoid cells and cerebellum microspongiosis. Creutzfeldt–Jakob
disease is a rare neurodegenerative human disorder. The prion hypothesis
as an explanatory model is currently favoured by majority of
researchers. A disease course described by Heidenhain including the
leading symptoms of a visual disorder and rapid progression. This report
emphasize the multidisciplinary role (forensic, neurogenetic and
neurohistologic) for diagnosis and to standardize a protocol to
investigate.
Keywords: Neuropathy; Heidenhain; Hystoneurology
http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B6T6W-4DTKFSF-B&_coverDate=12%2F02%2F2004&_alid=236905071&_rdoc=1&_fmt=&_orig=search&_qd=1&_cdi=5041&_sort=d&view=c&_acct=C000050221&_version=1&_urlVersion=0&_userid=10&md5=e1a0220acf312f61a38e624b84c175f2
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