AHA Coding Clinic® for ICD-9 - 2008 Issue 4; VOLUMES 1 AND 2 NEW/REVISED CODES

Slow Virus Infections and Prion Diseases of Central Nervous System

Effective October 1, 2008, category 046 has been revised to include prion diseases of the central nervous system. Prion diseases infecting humans include Creutzfeldt-Jakob disease (CJD), variant Creutzfeldt-Jakob disease (vCJD), Gertsmann-Straussler-Scheinker syndrome (GSS), and fatal familial insomnia (FFI). These diseases, previously thought to be caused by a slow virus, are now widely believed to be caused by proteinaceous infectious particles known as prions. A prion is an altered form of a normal brain protein. Creutzfeldt-Jakob Disease (046.19) is a rare, transmissible, rapidly progressing degenerative neurological disorder that is invariably fatal. It is characterized by progressive dementia and gradual...

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