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3. Diseases with spinal cord and/or cerebellum involvement: For each of the following diseases, describe (as appropriate) etiology/pathogenesis of lesions, location of lesions, clinical features and course of the disease, diagnostic tests, inheritance pattern and genetic mechanism, gross pathology, microscopic pathology

  • Amyotrophic lateral sclerosis
  • Friedreich ataxia

4. Prion diseases: 

  • Discuss characteristics and replication mechanism (conformational change) of the "prion," the agent in transmissible spongiform encephalopathies.
  • Know that prion diseases can be sporadic, genetic, or infectious. 
  • Understand the role of codon 129 of the PrP gene in determining disease characteristics.
  • For Creutzfeldt-Jakob (C-J) disease, describe age of onset, pathogenesis, clinical signs and symptoms, gross and microscopic pathology. Understand the general characteristics of other prion diseases, including variant CDJ,  in humans.