Subacute Sclerosing Panencephalitis

A rare, slowly progressive encephalitis caused by chronic infection with the MEASLES VIRUS. The condition occurs primarily in children and young adults, approximately 2-8 years after the initial infection. A gradual decline in intellectual abilities and behavioral alterations are followed by progressive MYOCLONUS; MUSCLE SPASTICITY; SEIZURES; DEMENTIA; autonomic dysfunction; and ATAXIA. DEATH usually occurs 1-3 years after disease onset. Pathologic features include perivascular cuffing, eosinophilic cytoplasmic inclusions, neurophagia, and fibrous gliosis. It is caused by the SSPE virus, which is a defective variant of MEASLES VIRUS. (From Adams et al., Principles of Neurology, 6th ed, pp767-8)

Also Known As:

SSPE; Subacute Sclerosing Leukoencephalitis; Inclusion Body Encephalitis, Measles; Leukoencephalitis, Van Bogaert's; Sclerosing Leukoencephalitis, Subacute; Sclerosing Panencephalitis, Subacute; Leukoencephalitides, Subacute Sclerosing; Leukoencephalitis, Van Bogaert; Leukoencephalitis, Van Bogaerts; Panencephalitides, Subacute Sclerosing; Sclerosing Leukoencephalitides, Subacute; Sclerosing Panencephalitides, Subacute; Subacute Sclerosing Leukoencephalitides; Subacute Sclerosing Panencephalitides; Van Bogaert Leukoencephalitis; Van Bogaerts Leukoencephalitis; Encephalitis, Inclusion Body, Measles; Leukoencephalitis, Subacute Sclerosing; Measles Inclusion Body Encephalitis; Panencephalitis, Subacute Sclerosing; Van Bogaert's Leukoencephalitis

Infections: 539155
- Virus Diseases: 42919
  - RNA Virus Infections: 309
    - Mononegavirales Infections: 1
      - Paramyxoviridae Infections: 1496
        Morbillivirus Infections: 28
        Measles: 9320
        Subacute Sclerosing Panencephalitis: 966
  - Central Nervous System Viral Diseases: 47
    - Viral Encephalitis: 480
      - Subacute Sclerosing Panencephalitis: 966
  - Slow Virus Diseases: 9
    - Subacute Sclerosing Panencephalitis: 966
- Central Nervous System Infections: 1428
  - Infectious Encephalitis: 58
    - Viral Encephalitis: 480
      - Subacute Sclerosing Panencephalitis: 966
  - Central Nervous System Viral Diseases: 47
    - Viral Encephalitis: 480
      - Subacute Sclerosing Panencephalitis: 966
Nervous System Diseases: 14737
- Neuroinflammatory Diseases: 19509
  - Encephalitis: 8670
    - Infectious Encephalitis: 58
      - Viral Encephalitis: 480
        Subacute Sclerosing Panencephalitis: 966
- Central Nervous System Diseases: 4371
  - Brain Diseases: 16141
    - Encephalitis: 8670
      - Infectious Encephalitis: 58
        Viral Encephalitis: 480
        Subacute Sclerosing Panencephalitis: 966
  - Central Nervous System Infections: 1428
    - Infectious Encephalitis: 58
      - Viral Encephalitis: 480
        Subacute Sclerosing Panencephalitis: 966
    - Central Nervous System Viral Diseases: 47
      - Viral Encephalitis: 480
        Subacute Sclerosing Panencephalitis: 966

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