Recent developments in MSA (dementia?)

This looks like an important paper on MSA, written by Gregor Wenning, one of Europe’s authorities on MSA.

To me, the most interesting sentences were about whether dementia can occur in MSA.  The authors say:

“The revised consensus criteria regard dementia as a non-supportive feature of MSA [50]. However, recent evidence suggests that dementia can occur in some patients otherwise satisfying the MSA criteria [77]. Further studies are required to assess the frequency and profile of dementia in MSA. These data may support a broader phenotype of MSA that also includes cognitive dysfunction.”

In personal correspondence, Dr. Wenning confirmed that there are no post-mortem confirmed cases of MSA with dementia….so far.  He believes we need to “keep an open mind.”

I’ve copied the abstract below.



Journal of Neurology. 2009 May 27. [Epub ahead of print] Recent developments in multiple system atrophy.
Wenning GK, Stefanova N.
Section of Clinical Neurobiology, Department of Neurology, Innsbruck Medical University, Anichstrasse 35, 6020, Innsbruck, Austria,

Multiple system atrophy (MSA) is a rare late onset neurodegenerative
disorder which presents with autonomic failure and a complicated motor syndrome including atypical parkinsonism, ataxia and pyramidal signs. MSA is a glial alpha-synucleinopathy with rapid progression and currently poor therapeutic management. This paper reviews the clinical features, natural history and novel diagnostic criteria for MSA as well as contemporary knowledge on pathogenesis based on evidence from neuropathological studies and experimental models. An outline of the rationale for managing symptomatic deterioration in MSA is provided together with a summary of novel experimental therapeutic approaches to decrease disease progression.

PubMed ID#: 19471850