Best Post of March 2019: Glial cytoplasmic inclusions filling putaminal pencillary fibers in a case of multiple system atrophy (striatonigral type)

The next in our "Best of the Month" series is from Thursday, March 14, 2019, which is simply comprised of a nice representative photomicrograph of an MSA-P case:

Putamen (alpha-synuclein immunohistochemistry)

Glial cytoplasmic inclusions filling putaminal pencillary fibers in a case of multiple system atrophy (striatonigral type)

Putamen (alpha-synuclein immunohistochemistry)

Mutations in COQ2 in Familial and Sporadic Multiple System Atrophy

Researchers from the Multiple System Atrophy (MSA) Research Collaborative in Japan just published online in the New England Journal of Medicine findings providing evidence that functionally impaired variants of the COQ2 gene (involved in the biosynthetic pathway for coenzyme Q10) are associated with an increased risk of developing MSA. This group previously identified multiplex families with MSA, indicating a genetic component in a disease that had previously been considered a non-genetic disorder.

More on the JNEN article

I was never clear on exactly which cells contained the glial cell inclusions (GCIs) in Multiple System Atrophy. This article clears that issue up. I quote: "GCIs are faintly eosinophilic, sickle-shaped, oval or conical inclusions that displace the nucleus eccentrically. Their localization to microglia has been established by double staining techniques." There you have it!

JNEN article on synucleinopathies

In the introduction to the article cited yesterday, the authors describe two subtypes of multiple system atrophy (MSA-P and MSA-C). What about the type in which primary autonomic failure is the predominant presentation? Does the dysautonomic subtype (formerly known as Shy-Drager syndrome) have a specific MSA subtype designation? And, where would one find neuropathologic evidence of disease in that subtype? That information is missing from the introduction. Given that this is supposed to be a review article, I would think that that aspect of MSA would be addressed.

Protein aggregation in the synucleinopathies

The current issue of the Journal of Neuropathology and Experimental Neurology has a review article about alpha-synuclein aggregation mechanisms in the major synucleinopathies: Parkinson's disease, Dementia with Lewy Bodies, and Multiple System Atrophy. The article was written by Drs. Katrin Beyer and Aurelio Ariza from Barcelona, Spain. I'll have more to say about this article in future posts.