Creutzfeldt-Jakob Syndrome

A rare transmissible encephalopathy most prevalent between the ages of 50 and 70 years. Affected individuals may present with sleep disturbances, personality changes, ATAXIA; APHASIA, visual loss, weakness, muscle atrophy, MYOCLONUS, progressive dementia, and death within one year of disease onset. A familial form exhibiting autosomal dominant inheritance and a new variant CJD (potentially associated with ENCEPHALOPATHY, BOVINE SPONGIFORM) have been described. Pathological features include prominent cerebellar and cerebral cortical spongiform degeneration and the presence of PRIONS. (From N Engl J Med, 1998 Dec 31;339(27))

Also Known As:

Creutzfeldt-Jakob Disease; Encephalopathy, Subacute Spongiform; Subacute Spongiform Encephalopathy; Syndrome, Creutzfeldt-Jakob; CJD (Creutzfeldt-Jakob Disease); Creutzfeldt Jacob Disease; Creutzfeldt-Jakob Disease, Familial; Creutzfeldt-Jakob Disease, New Variant; Creutzfeldt-Jakob Disease, Variant; Familial Creutzfeldt-Jakob Disease; Jakob-Creutzfeldt Disease; Jakob-Creutzfeldt Syndrome; V-CJD (Variant-Creutzfeldt-Jakob Disease); Variant Creutzfeldt-Jakob Disease; CJD (Creutzfeldt Jakob Disease); Creutzfeldt Jakob Disease; Creutzfeldt Jakob Disease, Familial; Creutzfeldt Jakob Disease, New Variant; Creutzfeldt Jakob Disease, Variant; Creutzfeldt Jakob Syndrome; Creutzfeldt-Jakob Diseases, Familial; Disease, Creutzfeldt Jacob; Disease, Creutzfeldt-Jakob; Disease, Familial Creutzfeldt-Jakob; Disease, Jakob-Creutzfeldt; Encephalopathies, Subacute Spongiform; Familial Creutzfeldt Jakob Disease; Familial Creutzfeldt-Jakob Diseases; Jacob Disease, Creutzfeldt; Jakob Creutzfeldt Disease; Jakob Creutzfeldt Syndrome; New Variant Creutzfeldt Jakob Disease; Spongiform Encephalopathies, Subacute; Subacute Spongiform Encephalopathies; Syndrome, Jakob-Creutzfeldt; V CJD (Variant Creutzfeldt Jakob Disease); Variant Creutzfeldt Jakob Disease; New Variant Creutzfeldt-Jakob Disease; Spongiform Encephalopathy, Subacute

Infections: 526564
- Central Nervous System Infections: 1390
  - Prion Diseases: 5345
    - Creutzfeldt-Jakob Syndrome: 2215
Nervous System Diseases: 14178
- Central Nervous System Diseases: 4213
  - Brain Diseases: 15694
    - Dementia: 38184
      - Creutzfeldt-Jakob Syndrome: 2215
  - Central Nervous System Infections: 1390
    - Prion Diseases: 5345
      - Creutzfeldt-Jakob Syndrome: 2215
Mental Disorders: 24177
- Neurocognitive Disorders: 1448
  - Dementia: 38184
    - Creutzfeldt-Jakob Syndrome: 2215

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