CBS folks –
Probably half of the people I’ve helped with brain donation who had a clinical diagnosis of CBS (or CBD) ended up with a confirmed diagnosis of Alzheimer’s disease (AD) upon brain autopsy. The case of someone recently was the same. One clue in that case was that the person experienced myoclonus which, surprisingly, is more associated with AD than CBD. The Mayo Clinic neuropathology report in this recent case gave the citation to a 2006 article as a reference item. This post is about that 2006 article.
That 2006 article is a case report of a 60-year-old man presenting with “slowly progressive left hemi-Parkinsonism, left hand apraxia, myoclonus, dystonia, visuospatial disturbances, and alien limb phenomenon, resembling corticobasal syndrome.” The man died 8 years after symptom onset. Neuropathological analysis showed that the man had Alzheimer’s Disease. The authors say that the “CBS-like presentation in AD is rare.” Four years later, I don’t think researchers would make the same statement. Indeed, in late 2009, researchers from Mayo, led by Dr. Hu, published research in which 11 of 16 clinical CBS cases ended up having Alzheimer’s Disease upon brain autopsy. One thing I learned from that late 2009 article was that alien limb is not a significant syndrome when differentiating between CBD and AD.
I’ve copied the abstract below.
Robin
Movement Disorders. 2006 Nov;21(11):2018-22.
Alzheimer’s disease presenting as corticobasal syndrome.
Chand P, Grafman J, Dickson D, Ishizawa K, Litvan I.
Department of Neurology, University of Louisville School of Medicine, Louisville, Kentucky 40202, USA.
Abstract
A 60-year-old man presented with slowly progressive left hemi-Parkinsonism, left hand apraxia, myoclonus, dystonia, visuospatial disturbances, and alien limb phenomenon, resembling corticobasal syndrome. Eight years later, neuropathology revealed features of Alzheimer’s disease, with asymmetrical (right more than left) cortical tau burden with image analysis. The videotaped clinical features, neuropsychological aspects, and neuropathological correlates are presented and discussed.
PubMed ID#: 16977625 (see pubmed.gov for the abstract only)