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CJD Update from Texas

Posted by Gary Finch on June 12, 2013

The Infectious Disease Control Unit is one division of The Department of State (Texas) Health Services. In January of 2013, they released a report on CJD. It was well written, informative, and worth passing on to embalmers. This is especially true since some of our suppliers advocate embalming a CJD case. In my newsletter, I argue over the insanity of their position.

Overview

  Creutzfeldt-Jakob disease (CJD) is a rare, fetal 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 latrogenic.

  The organism/Etiologic Agent is a Prion (infectious protein).

Transmission

      Sporadic CJD (sCJD) – mode of transmission is unknown.

      Familial CJD (fCJD) – inherited

      Varian 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 three months.

  Familial CJD (fCJD) – Symptoms and duration of illness vary depending on types 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 oclonus, 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 and Prevention

  There is no known effective treatment or prevention.

Recent Texas Trends

  CJD is a rare, invariably fatal neurodegenative 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. This is likely attributed to the rarity of the disease and lack of pre-mortem diagnostics. Confirmation of CJD diagnosis requires neuropath logical 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. More information at www.cjdsurveillance.com.



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