Mayo researchers just published an article on CBD. The abstract is a mix of clinical information and pathological information. If you are like me, you’ll want to skip past the pathological terminology!
The clinical information in the abstract leads me to believe that the article’s content is very similar to Dr. Irene Litvan’s webinar several months ago on CBD. You can find my notes on that webinar here:
http://forum.psp.org/viewtopic.php?t=8575
The abstract mentions the term “Richardson syndrome.” This refers to the most common form of PSP (progressive supranuclear palsy); one of the symptoms of this form is cognitive impairment.
Robin
Nature Reviews. Neurology. 2011 Apr 12. [Epub ahead of print]
Corticobasal degeneration: a pathologically distinct 4R tauopathy.
Kouri N, Whitwell JL, Josephs KA, Rademakers R, Dickson DW.
Department of Neuroscience Mayo Clinic, Jacksonville, FL.
Abstract
Corticobasal degeneration (CBD) is a rare, progressive neurodegenerative disorder with onset in the 5(th) to 7(th) decade of life. It is associated with heterogeneous motor, sensory, behavioral and cognitive symptoms, which make its diagnosis difficult in a living patient.
The etiology of CBD is unknown; however, neuropathological and genetic evidence supports a pathogenetic role for microtubule-associated protein tau.
CBD pathology is characterized by circumscribed cortical atrophy with spongiosis and ballooned neurons; the distribution of these changes dictates the patient’s clinical presentation. Neuronal and glial tau pathology is extensive in gray and white matter of the cortex, basal ganglia, diencephalon and rostral brainstem. Abnormal tau accumulation within astrocytes forms pathognomonic astrocytic plaques.
The classic clinical presentation, termed corticobasal syndrome (CBS), comprises asymmetric progressive rigidity and apraxia with limb dystonia and myoclonus. CBS also occurs in conjunction with other diseases, including Alzheimer disease and progressive supranuclear palsy.
Moreover, the pathology of CBD is associated with clinical presentations other than CBS, including Richardson syndrome, behavioral variant frontotemporal dementia, primary progressive aphasia and posterior cortical syndrome.
Progress in biomarker development to differentiate CBD from other disorders has been slow, but is essential in improving diagnosis and in development of disease-modifying therapies.
PubMed ID#: 21487420 (see pubmed.gov for this abstract only)