Olivopontocerebellar Atrophies

A group of inherited and sporadic disorders which share progressive ataxia in combination with atrophy of the CEREBELLUM; PONS; and inferior olivary nuclei. Additional clinical features may include MUSCLE RIGIDITY; NYSTAGMUS, PATHOLOGIC; RETINAL DEGENERATION; MUSCLE SPASTICITY; DEMENTIA; URINARY INCONTINENCE; and OPHTHALMOPLEGIA. The familial form has an earlier onset (second decade) and may feature spinal cord atrophy. The sporadic form tends to present in the fifth or sixth decade, and is considered a clinical subtype of MULTIPLE SYSTEM ATROPHY. (From Adams et al., Principles of Neurology, 6th ed, p1085)

Also Known As:

Olivopontocerebellar Atrophy; Olivopontocerebellar Degeneration; Familial Olivopontocerebellar Atrophy; Inherited Olivopontocerebellar Atrophy; Nonfamilial Olivopontocerebellar Atrophy; Olivo-Ponto-Cerebellar Atrophy; Olivo-Ponto-Cerebellar Degeneration; Pontoolivocerebellar Atrophy; Presenile Ataxia; Ataxia, Presenile; Atrophy, Familial Olivopontocerebellar; Atrophy, Idiopathic Olivopontocerebellar; Atrophy, Inherited Olivopontocerebellar; Atrophy, Nonfamilial Olivopontocerebellar; Atrophy, Olivo-Ponto-Cerebellar; Atrophy, Olivopontocerebellar; Atrophy, Pontoolivocerebellar; Degeneration, Olivo-Ponto-Cerebellar; Degeneration, Olivopontocerebellar; Dejerine Thomas Syndrome; Familial Olivopontocerebellar Atrophies; Hypoplasia, Olivopontocerebellar; Idiopathic Olivopontocerebellar Atrophies; Idiopathic Olivopontocerebellar Atrophy; Inherited Olivopontocerebellar Atrophies; Nonfamilial Olivopontocerebellar Atrophies; Olivo Ponto Cerebellar Atrophy; Olivo Ponto Cerebellar Degeneration; Olivo-Ponto-Cerebellar Degenerations; Olivopontocerebellar Atrophies, Familial; Olivopontocerebellar Atrophies, Nonfamilial; Olivopontocerebellar Atrophy, Familial; Olivopontocerebellar Atrophy, Inherited; Olivopontocerebellar Atrophy, Nonfamilial; Olivopontocerebellar Degenerations; Olivopontocerebellar Hypoplasias; Pontoolivocerebellar Atrophies; Presenile Ataxias; Syndrome, Dejerine-Thomas; Dejerine-Thomas Syndrome; Olivopontocerebellar Atrophy, Idiopathic; Olivopontocerebellar Hypoplasia

Nervous System Diseases: 14178
- Neurodegenerative Diseases: 42472
  - Nervous System Heredodegenerative Disorders: 63
    - Spinocerebellar Degenerations: 353
      - Olivopontocerebellar Atrophies: 175
- Central Nervous System Diseases: 4213
  - Brain Diseases: 15694
    - Cerebellar Diseases: 631
      - Spinocerebellar Degenerations: 353
        Olivopontocerebellar Atrophies: 175
    - Basal Ganglia Diseases: 346
      - Multiple System Atrophy: 1925
        Olivopontocerebellar Atrophies: 175
  - Movement Disorders: 4634
    - Multiple System Atrophy: 1925
      - Olivopontocerebellar Atrophies: 175
  - Spinal Cord Diseases: 4314
    - Spinocerebellar Degenerations: 353
      - Olivopontocerebellar Atrophies: 175
- Autonomic Nervous System Diseases: 722
  - Primary Dysautonomias: 5
    - Multiple System Atrophy: 1925
      - Olivopontocerebellar Atrophies: 175
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
- Inborn Genetic Diseases: 11939
  - Nervous System Heredodegenerative Disorders: 63
    - Spinocerebellar Degenerations: 353
      - Olivopontocerebellar Atrophies: 175

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