Corticobasal degeneration

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Corticobasal degeneration Classification and external resources
DiseasesDB	33284
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Corticobasal degeneration (CBD) is a sporadic progressive neurodegenerative disease associated with atrophy of the cerebral cortex and the basal ganglia. Onset is gradual.

Contents 1 Presentation 2 Histology 3 Differential diagnosis 4 References 5 External links 6 See also

[edit] Presentation

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 dysfunction (dementia).

[edit] Histology

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.

[edit] Differential diagnosis

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.

[edit] References

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

[edit] External links

• FTD Support Forum
• The Association for Frontotemporal Dementias
• UCSF Memory and Aging Center - FTD Resources and FTD Info
• Mayo Clinic - FTD Info
• Pick's Disease Support Group Online
• Frontotemporal Dementia Caregiver Support Center

[edit] See also

• Alzheimer's disease
• Dementia with Lewy bodies
• Frontotemporal lobar degeneration

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