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Höglinger GU, Respondek G, Stamelou M, Kurz C, Josephs KA, Lang AE, Mollenhauer B, Müller U, Nilsson C, Whitwell JL, Arzberger T, Englund E, Gelpi E, Giese A, Irwin DJ, Meissner WG, Pantelyat A, Rajput A, van Swieten JC, Troakes C, Antonini A, Bhatia KP, Bordelon Y, Compta Y, Corvol JC, Colosimo C, Dickson DW, Dodel R, Ferguson L, Grossman M, Kassubek J, Krismer F, Levin J, Lorenzl S, Morris HR, Nestor P, Oertel WH, Poewe W, Rabinovici G, Rowe JB, Schellenberg GD, Seppi K, van Eimeren T, Wenning GK, Boxer AL, Golbe LI, Litvan I, Movement Disorder Society-endorsed PSP Study Group. Clinical diagnosis of progressive supranuclear palsy: The movement disorder society criteria. Mov Disord. 2017 Jun;32(6):853-864. Epub 2017 May 3 PubMed.
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Professor Höglinger and his colleagues of the MDS-PSP study group are to be congratulated on setting new criteria which are both sensitive and specific, and which optimize the early clinical diagnosis of progressive supranuclear palsy (PSP).
During the 1950s, in Toronto, neurologist J.C. Richardson identified patients with an unfamiliar syndrome of vertical gaze and pseudobulbar palsy, nuchal dystonia, and dementia. He termed the syndrome progressive supranuclear palsy, and with the publication of our paper in 1964, this designation became the name of the disease which had occasioned the syndrome (Steele et al., 1964).
Recently the syndrome he described has become known as Richardson's syndrome. But the name of the disease, which is a 4R tauopathy, remains firmly established as PSP. Some refer to it as SRO or Steele-Richardson-Olszewski syndrome, though I have hoped it could be known simply as Richardson's disease, to acknowledge his seminal observation.
In 1964, when describing this neurodegeneration, neuropathologist Jerzy Olszewski and I were surprised that all seven patients had exhibited the same unusual clinical syndrome despite widespread and heterogeneous neurofibrillary degeneration in subcortical nuclei, the brain stem and cerebellum.
He predicted that others with the same disease would suffer various symptoms, as different nuclei were affected, at different times and to different degrees.
Many years later, his prediction is borne out. In studies of those who meet the pathological criteria of PSP, i.e., a tauopathy which accumulates 4R tau in nerve cells, oligodendrocytic coils and astrocytic tufts, we now know that the majority begin with a symptom other than that of Richardson's syndrome.
The new MDS criteria emphasize these many phenotypic variants, which we were not previously aware of and which were not captured by NINDS/SPSP criteria developed in 1996. They will improve the diagnosis, awareness, and understanding of PSP; they will emphasize the variants of clinical presentation; and they will be helpful in research and clinical practice.
I look forward to a MDS/PSP web base to implement the criteria, and video tutorials to facilitate their application.
References:
STEELE JC, RICHARDSON JC, OLSZEWSKI J. PROGRESSIVE SUPRANUCLEAR PALSY. A HETEROGENEOUS DEGENERATION INVOLVING THE BRAIN STEM, BASAL GANGLIA AND CEREBELLUM WITH VERTICAL GAZE AND PSEUDOBULBAR PALSY, NUCHAL DYSTONIA AND DEMENTIA. Arch Neurol. 1964 Apr;10:333-59. PubMed.
View all comments by John C. SteeleLudwig-Maximilians-Universität München
For the very first time in the field of movement disorders, these criteria allow us to introduce in the clinical terminology a prediction of the underlying pathology to the molecular level.
In my opinion, the diagnostic category of "probable 4 repeat tauopathy" is in a way the logical consequence of the research in the past decade, but in another way it is also a seminal step forward. The new category of “probable 4R-tauopathies,” according to the Movement Disorder Society Criteria - Clinical Diagnosis of Progressive Supranuclear Palsy, comprise patients with possible PSP-SL (oculomotor dysfunction + nonfluent/agrammatic variant of primary progressive aphasia) or PSP-CBS (oculomotor dysfunction + corticobasal syndrome).
View all comments by Johannes LevinMake a Comment
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