Too bad this full article is in Japanese as it might be a very good review of corticobasal syndrome.
Most impressive to me about the abstract is the long list of disorders that can look like corticobasal syndrome — CBD, AD, PSP, FTLD with TDP-43, Pick’s disease, Lewy body disease, CJD, etc.
Clearly, the only way to be sure of the diagnosis is for the brain to be examined upon death. If you haven’t made arrangements for that but would like to, please let me know, as we can help with this. Half of our local group members are involved with brain donation!
Robin
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Brain & Nerve. 2012 Apr;64(4):462-73.
[Corticobasal syndrome: recent advances and future directions].[Article in Japanese]
Aiba I.
Department of Neurology, National Hospital Organization Higashinagoya National Hospital.
Abstract
Corticobasal degeneration (CBD) is a progressive neurodegenerative disorder described by Rebeiz et al. It is characterized by progressive, asymmetric, cortical (eg, apraxia, alien limb phenomena, cortical sensory loss, and myoclonus), and extrapyramidal (eg, rigidity, bradykinesia, dystonia, and tremor) dysfunction.
However, CBD has many clinical phenotypes, and the features used for predicting CBD have low sensitivity.
Therefore, the term corticobasal syndrome (CBS) has been used to characterize such clinical features, whereas the term CBD is used to refer to the pathological disorder.
The most frequent causes of CBS are CBD, followed by Alzheimer’s disease, progressive supranuclear palsy, frontotemporal lobar degeneration with TDP-43 pathology (sporadic and familial), Pick’s disease, Lewy body disease, frontotemporal lobar degeneration with fused in sarcoma-positive inclusions, Creutzfeldt-Jakob disease, and mutations in the microtubule-associated protein tau (MAPT) and progranulin (GRN) genes.
The topography of neurodegeneration dictates the clinical syndrome not according to the underlying pathology.
Researchers have attempted to develop fluid biomarkers or imaging analysis for diagnosing CBS. The aim of this review was to highlight recent advances in CBS diagnosis and discuss future directions.
PubMed ID#: 22481519 (see pubmed.gov for this English-language abstract only)