Corticobasal degeneration

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Corticobasal degeneration Classification and external resources
DiseasesDB	33284

Corticobasal degeneration (CBD) is a sporadic progressive neurodegenerative disease associated with atrophy of the cerebral cortex and the basal ganglia. Onset is gradual. The first symptoms usually involve asymmetric changes in motor function, such as dystonia and myoclonus. Some patients experience alien hand syndrome. Language function can also be affected. CBD can expand to include extrapyramidal motor dysfunction (e.g. rigidity, tremor) and some cognitive dysfuntion (dementia). Upon autopsy, CBD brains present with frontal atrophy and glial abnormalities (astrocytic plaques). In addition, tau (a microtubule stabilizing protein that also aggregates in Alzheimer's disease) immunoreactivity can be detected in the white matter.

CBD is a pathological diagnosis. Many patients present clinically with a corticobasal syndrome characterized by features of progressive asymmetric brain degeneration, but are found to have other pathologies such as progressive supranuclear palsy (PSP) or Alzheimer's disease (AD). Many of the symptoms and pathology of CBD are similar to the more common disease, Progressive Supranuclear Palsy (PSP). Ballooned neurons are the most telling feature of CBD to allow distinction from PSP. Since CBD and Alzheimer's disease share tau pathology, scientists are hopeful that if a medical treatment that reduced tau aggregation was developed for Alzheimer's disease, it would also be useful for CBD patients. Such a treatment does not currently exist.

References

• Gibb WR, Luthert PJ, Marsden CD. (1989) "Corticobasal degeneration." Brain. 112 ( Pt 5):1171-92. PMID 2478251

See also

• Alzheimer's disease
• Dementia with Lewy bodies
• Frontotemporal lobar degenerationde:Kortikobasale Degeneration

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Acknowledgement and Attribution Regarding Sources of Content

Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .