Joined: Fri Aug 11, 2006 1:46 pm
Location: SF Bay Area (Northern CA)
Pathological stages for Lewy body disorders
Here's an interesting article out of the Sun Health Research Institute near Phoenix. Dr. Beach is the neuropathologist there. He's definitely a rival to Dr. Dickson at Mayo Jax. In this article, Dr. Beach proposes a new pathological staging system for Lewy body disorders, which include PD (Parkinson's Disease) and DLB (Dementia with Lewy Bodies). He and colleagues have studied 417 deceased people (who have donated brain tissue) with diagnoses during life of PD, DLB, incidental Lewy Body disease, and AD with Lewy bodies. He correlates these pathological stages with clinical symptoms (using medical records). "It is suggested that the proposed staging system would improve on its predecessors by allowing classification of a much greater proportion of cases."
I'm assuming that most of those with a clinical diagnosis of DLB end up in stage IV (Neocortical) of the new staging scheme but some may end up with stage III (Brainstem and Limbic), assuming they have a Lewy body disorder. A large percentage of those in our local LBD support group who have been involved in brain donation and elsewhere in the US who have been involved in brain donation actually do NOT have a Lewy body disorder upon brain autopsy.
I'm assuming that most of those with a clinical diagnosis of PD end up in stage III (Brainstem and Limbic) but some may end up in stage IV (Neocortical), assuming they have a Lewy body disorder. The diagnostic accuracy in PD is only 75%.
The abstract follows though probably only those who have been involved in brain donation or will be involved in brain donation will have an interest!
Acta Neuropathologica. 2009 Apr 28. [Epub ahead of print]
Unified staging system for Lewy body disorders: correlation with nigrostriatal degeneration, cognitive impairment and motor dysfunction.
Beach TG, et al; the Arizona Parkinsonâs Disease Consortium.
Sun Health Research Institute, Sun City, AZ
The two current major staging systems in use for Lewy body disorders fail to classify up to 50% of subjects. Both systems do not allow for large numbers of subjects who have Lewy-type alpha-synucleinopathy (LTS) confined to the olfactory bulb or who pass through a limbic-predominant pathway that at least initially bypasses the brainstem.
The results of the current study, based on examination of a standard set of ten brain regions from 417 subjects stained immunohistochemically for alpha-synuclein, suggest a new staging system that, in this study, allows for the classification of all subjects with Lewy body disorders. The autopsied subjects included elderly subjects with Parkinson's disease, dementia with Lewy bodies, incidental Lewy body disease and Alzheimer's disease with Lewy bodies, as well as comparison groups without Lewy bodies.
All subjects were classifiable into one of the following stages:
I. Olfactory Bulb Only;
IIa Brainstem Predominant;
IIb Limbic Predominant;
III Brainstem and Limbic;
Progression of subjects through these stages was accompanied by a generally stepwise worsening in terms of striatal tyrosine hydroxylase concentration, substantia nigra pigmented neuron loss score, Mini Mental State Examination score and score on the Unified Parkinson's Disease Rating Scale Part 3.
Additionally, there were significant correlations between these measures and LTS density scores. It is suggested that the proposed staging system would improve on its predecessors by allowing classification of a much greater proportion of cases.
PubMed ID#: 19399512 (see pubmed.gov for abstract only)