Creutzfeldt-Jakob Disease

ICD-9 046.1;ICD-10 A81.0

Creutzfeldt - Jakob disease (CJD) is a rare, fatal brain disorder consisting of four types: sporadic (most common - makes up 85 - 95% of all CJD cases - sometimes referred to as classic), familial (represents 5-15%of all CJD cases), variant, and iatrogenic.

Organism/Etiologic Agent

Prion (infectious protein)

Transmission

Sporadic CJD (sCJD) – mode of transmission is unknown

Familial CJD (fCJD) – inherited

Variant CJD (vCJD) – believed to be associated with consumption of beef products from cows contaminated with the agent of mad cow disease.

Iatrogenic CJD (iCJD) - transmission can occur through certain medical or surgical procedures.

Symptoms

Sporadic CJD (sCJD) Symptoms may include rapidly progressive dementia, myoclonus, ataxia, vision and speech difficulties. Typically occurs in persons greater than 55 years of age. Average duration of illness is 3 months.

Familial CJD (fCJD) Symptoms and duration of illness vary depending on type of mutation. Symptoms may be similar to sCJD.

Variant CJD (vCJD) Symptoms may include early psychiatric symptoms and dysaesthesia/paresthsias progressing to chorea/dystonia or myoclonus, dementia, ataxia, and/or akinetic mutism. Typically occurs in persons less than 55 years of age (average age 28). Average duration of illness is 13 months.

Iatrogenic CJD (iCJD) Symptoms vary depending on portal of entry. If transmission occurs directly to brain symptoms will be similar to sCJD.

Treatment & Prevention

There is no known effective treatment or prevention.

Recent Texas Trends

CJD is a rare, invariably fatal neurodegenerative disease with a rate of .5 to 1.5 cases per million inhabitants per year. In Texas, there is an average of 15 deaths reported per year. CJD is presumably under-reported and misdiagnosed which can be attributed to rarity of the disease and lack of pre-mortem diagnostics. Confirmation of CJD diagnosis requires neuropathological examination of brain tissue, preferably by autopsy, to confirm a case. Autopsy is available free through the National Prion Disease Pathology Surveillance Center for all suspect cases of CJD. Please see www.cjdsurveillance.com for more information.

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