The 12/15/09 issue of the journal Neurology contains an interesting case report from Case Western (Cleveland) of a 49-year-old man who was found to have the dementia form of PSP upon brain autopsy. That form of PSP is called “Richardson syndrome (RS).”
I don’t believe this man was diagnosed during life with PSP because he presented with atypical features for PSP: “young age at onset, absence of falls, and the presenting complaint of horizontal diplopia (due to vergence abnormalities). His cognitive impairment was suggestive of frontotemporal dementia. However, vertical saccades were slow at presentation.”
The report notes that the patient clapped exactly three times. This must refer to the “clap test,” which has now been discounted as a neurological test for PSP.
The patient received extensive testing, including neuro-ophthalmological testing. The clinicians have gone back through the patient’s clinical records after death to try to solve the mystery of how they missed this patient’s PSP. The researchers conclude “that careful examination of the speed (more than amplitude) of vertical saccades in patients with undiagnosed parkinsonian disorders remains the cornerstone for recognition of PSP and differentiation from other parkinsonian
disorders.”
The citation is copied below. You can purchase the article (probably costs $30). I did obtain it but think copyright law precludes copying it here.
The text refers to images of brain tissue available as a result of the brain autopsy. You can find those images at the Neurology journal’s website here:
http://neurology.org/cgi/content/full/73/24/2122/DC1 (6 images available for free)
Robin
Neurology. 2009 Dec 15;73(24):2122-4.
Evolution of oculomotor and clinical findings in autopsy-proven Richardson syndrome.
Hardwick A, Rucker JC, Cohen ML, Friedland RP, Gustaw-Rothenberg K, Riley DE, Leigh RJ.
Department of Neurology, Veterans Affairs Medical Center, Case Western Reserve University, Cleveland, OH, USA.
From the Departments of Neurology (A.H., K.G.-R., D.E.R., R.J.L.) and Neuropathology (M.L.C.), University Hospital, and Daroff-Dell’Osso Laboratory (R.J.L.), Veterans Affairs Medical Center, Case Western Reserve University, Cleveland, OH; Department of Neurology (J.C.R.), University of Louisville School of Medicine, Louisville KY; and Mount Sinai Medical Center (R.P.F.), New York, NY.
PubMed ID#: 20018641 (there’s nothing viewable at pubmed.gov on this article but you can link the the journal Neurology’s website, neurology.org, if you want to purchase the short article)