Concomitant PSP and MSA (4 Cases)

This recently-published article is about one case of concomitant PSP and MSA in the Queen Square Brain Bank in the UK. (That’s one out of 120 cases of path-confirmed PSP.) And the article mentions that there are three other (independent) cases. One of these three is a Mayo Jax case; it was one case out of 290 path-confirmed PSP cases. So, that answers the question as to whether this is even possible!

Here are the highlights:

* “The apparent rarity of cases of coexistent PSP and MSA initially suggests they might be due to mere coincidence, rather than the result of any biological overlap.”

* “Grading of PSP tau pathology provided a score of 6, consistent with moderately severe tau pathology typical of Richardson’s syndrome [29].” [Robin’s note: this indicates that the RS form of PSP has more severe tau pathology than the other common form, PSP-P.]

* “Nevertheless, taking into consideration the rarity of MSA and PSP, in addition to the small number of cases that come to autopsy, the findings of 4 cases reported to-date is higher than expected.”

* “Whether these cases represent a pre-clinical stage of MSA, or simply a biological coincidence is yet unknown. The occurrence of pre-symptomatic MSA should be confirmed in prospective studies in which detailed neurological evaluation has been performed in the final months of life.”

* “Clinically our patient fulfilled the NINDS-SPSP operational criteria for the diagnosis of probable PSP and the only feature that might have suggested MSA was the urinary incontinence which started 20 years before death and resolved. The lack of overt cerebellar features of MSA is consistent with mild olivo-ponto-cerebellar pathology, whereas the prevailing parkinsonian syndrome is in line with more severe striato-nigral-degeneration, or the baseline PSP pathology.”

* “The rarity of pathologically proven cases of combined PSP and MSA does not provide sufficient evidence to confirm a common pathogenic mechanism for both diseases, but our findings suggest that systematic screening for PSP and MSA changes in atypical parkinsonian patients and control brain bank donors is warranted.”

The abstract follows.


Neuroscience Letters. 2009 Oct 14. [Epub ahead of print]

Concomitant progressive supranuclear palsy and multiple system atrophy: More than a simple twist of fate?

Silveira-Moriyama L, González AM, O’Sullivan S, Williams DR, Massey L, Parkkinen L, Ahmed Z, de Silva R, Chacón JR, Revesz T, Lees AJ, Holton JL.
Reta Lila Weston Institute for Neurological Studies, UCL Institute of Neurology, London, UK.

Progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) are both rare neurodegenerative diseases.

In the Queen Square Brain Bank, from 2001 to 2008, we received 120 cases of pathologically confirmed PSP and 36 of MSA, and one had concomitant PSP and MSA pathology.

The clinical symptoms in this case were compatible with PSP and did not predict the dual pathology.

The growing number of collective case reports, including the one reported here, might suggest an increased prevalence of concomitant PSP and MSA than what would be expected by chance.

PubMed ID#: 19835934