Human prion diseases

Definition

Human prion diseases or transmissible spongiform encephalopathies are rare transmissible diseases affecting the central nervous system. The infectious agents are composed of an abnormal isoform of a host membrane protein called 'prion protein' (PrP). Their common features are a long duration of incubation and lesions limited to the central nervous system without inflammatory or immunologic reaction but with accumulation of an abnormal form of prion protein (PrPsc).

6 subordinate codes

• 8E00Sporadic Creutzfeldt-Jakob Disease
• 8E01Acquired prion disease
• 8E02Genetic prion diseases
• 8E03Variably protease sensitive prionopathy
• 4B4YOther specified diseases of the immune system
• 4B4ZDiseases of the immune system, unspecified

Nearby in Diseases of the nervous system

• 8A00-8A0ZMovement disorders
• 8A20-8A2ZDisorders with neurocognitive impairment as a major feature
• 8A40-8A4ZMultiple sclerosis or other white matter disorders
• 8A60-8A6ZEpilepsy or seizures
• 8A80-8A8ZHeadache disorders
• 8B00-8B2ZCerebrovascular diseases
• 8B40-8B4ZSpinal cord disorders excluding trauma
• 8B60-8B6ZMotor neuron diseases or related disorders