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Olivopontocerebellar Atrophies (Olivopontocerebellar Atrophy)

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; Ataxias, 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; Degenerations, Olivo-Ponto-Cerebellar; Degenerations, Olivopontocerebellar; Dejerine Thomas Syndrome; Familial Olivopontocerebellar Atrophies; 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, Idiopathic; Olivopontocerebellar Atrophies, Inherited; Olivopontocerebellar Atrophies, Nonfamilial; Olivopontocerebellar Atrophy, Familial; Olivopontocerebellar Atrophy, Inherited; Olivopontocerebellar Atrophy, Nonfamilial; Olivopontocerebellar Degenerations; Pontoolivocerebellar Atrophies; Presenile Ataxias; Syndrome, Dejerine-Thomas; Dejerine-Thomas Syndrome; Olivopontocerebellar Atrophy, Idiopathic
Networked: 166 relevant articles (4 outcomes, 17 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Atrophy
2. Multiple System Atrophy
3. Friedreich Ataxia (Friedreich's Ataxia)
4. Olivopontocerebellar Atrophies (Olivopontocerebellar Atrophy)
5. Striatonigral Degeneration

Experts

1. Poewe, Werner: 3 articles (10/2015 - 03/2005)
2. Stefanova, Nadia: 3 articles (10/2015 - 03/2005)
3. Wenning, Gregor K: 3 articles (10/2015 - 03/2005)
4. Ferrer, I: 3 articles (06/2005 - 09/2001)
5. Georgievska, Biljana: 2 articles (10/2015 - 05/2012)
6. Kaindlstorfer, Christine: 1 article (10/2015)
7. Mather, Robert J: 1 article (10/2015)
8. Kugler, Alan R: 1 article (10/2015)
9. Sommer, Patrick: 1 article (10/2015)
10. Takahashi, Makio: 1 article (05/2013)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Olivopontocerebellar Atrophies:
1. Dopamine (Intropin)FDA LinkGeneric
2. Buspirone (Buspar)FDA LinkGeneric
3. Glutamate Receptors (Glutamate Receptor)IBA
4. Amantadine (Aman)FDA LinkGeneric
5. Thyrotropin-Releasing Hormone (Protirelin)FDA Link
6. N-Methylaspartate (NMDA)IBA
7. 5-Hydroxytryptophan (5 Hydroxytryptophan)IBA
8. Estrogens (Estrogen)FDA Link
9. SilverIBA
06/01/1992 - "Cytoskeletal protein abnormalities in patients with olivopontocerebellar atrophy--an immunocytochemical and Gallyas silver impregnation study."
04/01/1994 - "In this study a semiquantitative mapping of oligodendroglial cytoplasmic inclusions (GCIs), a feature of oligodendroglial degeneration in multiple system atrophy (MSA), was undertaken by means of a sensitive silver technique in 14 brains and 11 spinal cords of patients with various combinations of striatonigral degeneration, olivopontocerebellar atrophy and autonomic failure. "
03/01/1992 - "A highly sensitive silver technique for glial cytoplasmic inclusions in olivopontocerebellar atrophy (OPCA) was applied to 15 subjects with neurodegenerative disorders including 4 patients with OPCA, 4 patients with Joseph disease and with 3 normal control subjects, and the argyrophilic structures in the OPCA cases were immunocytochemically examined. "
02/01/1992 - "In 8 brains of patients with various combinations of striatonigral degeneration, olivopontocerebellar atrophy and Shy-Drager syndrome, inclusion bodies were demonstrated in the cytoplasm and nucleus of both neuronal and oligodendroglial cells and in neuronal processes by means of silver staining, immunocytochemistry and electron microscopy. "
12/01/1989 - "Glial cytoplasmic inclusions (GCIs) were demonstrated by silver staining, immunocytochemistry and by electron microscopy in the central nervous system (CNS) of 11 patients with various combinations of striatonigral degeneration, olivopontocerebellar atrophy and Shy-Drager syndrome. "
10. Glucose (Dextrose)FDA LinkGeneric

Therapies and Procedures

1. Tracheostomy
2. Vaginal Hysterectomy
3. Cholecystectomy
4. Anesthesia