📋Overview
This condition belongs to a group of rare diseases known as transmissible spongiform encephalopathies, which occur when naturally occurring prion proteins misfold into an abnormal shape. These abnormal proteins accumulate and damage healthy brain tissue, eventually resulting in a microscopic sponge-like appearance within the neural structure.
The disorder leads to a swift decline in neurological health and is the standard clinical term used to describe several related forms of the condition. It is distinct from other neurodegenerative illnesses due to its unique protein-based mechanism and the speed with which it affects the central nervous system.
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Additional Resources
Medical codes (for reference)
UMLS CUI: C0022336Codes are provided for reference and interoperability. They are not a diagnosis.
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