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Creutzfeldt-Jakob disease not related to a common venue--New Jersey, 1995-2004.
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On May 7, this report was posted as an MMWR Early Release on the MMWR website (http://www.cdc.gov/mmwr).
Beginning in June 2003, the New Jersey Department of Health and Senior Services (NJDHSS) and CDC were notified of a suspected cluster of deaths caused by Creutzfeldt-Jakob disease (CJD) in persons reportedly linked to Garden State Racetrack in Cherry Hill, New Jersey. Concerns were raised that these deaths might have resulted from consumption of meat contaminated with the agent causing bovine spongiform encephalopathy (BSE, commonly called "mad cow disease") served at ...
<6 mos]
Suspected CJD deaths under investigation
15 1995 70-74 Pennsylvania Under investigation
16 1995 60-64 Pennsylvania Under investigation
17 1996 65-69 Delaware Under investigation
* As of May 2, 2004.
([dagger]) Cases were classified as definite or probable CJD based on
the World Health Organization diagnostic criteria (1).
([section]) Various subtypes of CJD have differing clinical and
pathologic phenotypes that correlate with the genotype at codon 129
of the prion protein gene (M=methionine; V=valine) and the size of
protease-resistant prion protein (Type 1 or 2). All cases of vCJD to
date are homozygous for M at codon 129 and have the Type 2 pattern.
([paragraph]) Genotype based on characteristic neuropathology.
** Death certificate included CJD.
([dagger][dagger]) Protein 14-3-3 is a group of proteins released into
the cerebrospinal fluid (CSF) during neuronal death that, in the
appropriate clinical setting, can be used as a diagnostic marker for
CJD.
TABLE 2. Clinical and pathologic characteristics distinguishing
variant Creutzfeldt-Jakob disease (vCJD) from classic CJD
Characteristic vCJD
Median age at death 28 yrs
Median duration of illness 13-14 mos
Clinical signs and symptoms Prominent psychiatric/behavioral
symptoms; painful dysesthesias;
delayed neurologic signs
Periodic sharp waves on Absent
electroencephalogram
"Pulvinar sign" on MRI * Present in>
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