Abstract

When the MDS diagnostic criteria for progressive supranuclear palsy (PSP) were published in 2017 [ [1] Höglinger G.U. Responde G. Stamelou M. et al. Clinical diagnosis of progressive supranuclear palsy: the Movement Disorder Society criteria. Mov. Disord. 2017; : 853-864 Crossref PubMed Scopus (638) Google Scholar ], the authors stated that they would be followed by a video-tutorial to facilitate their application. The video-tutorial appears in this issue of Parkinsonism and Related Disorders [ [2] Iankova V. Respondek G. Saranza G. Painous C. Cámara A. Compta Y. Aiba I. Balint B. Giagkou N. Josephs K.A. Otsuki M. Golbe L.I. Bhatia K.P. Stamelou M. LangE A.E. Höglinger G. Video-turorial for the movement disorder society criteria for progressive supranuclear palsy. Park. Relat. Disord. 2020; https://doi.org/10.1016/j.parkreldis.2020.06.030 Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar ] and represents an important step forward in improving the clinical diagnosis of PSP, particularly since the diagnosis has become increasingly complex. In their 1964 paper describing PSP subtitled “A heterogeneous degeneration involving the Brain Stem, Basal Ganglia and Cerebellum …” Steele, Richardson and Olszewski were prescient in stating that “It is possible that further observations may broaden the clinical spectrum of the disease” [ [3] Steele J.C. Richardson J.C. 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; : 333-359 Crossref PubMed Scopus (1332) Google Scholar ]. When the NINDS-SPSP criteria were published 32 years later [ [4] Litvan I I. Agid Y. Calne D. et al. Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology. 1996; : 1-9 Crossref PubMed Scopus (1943) Google Scholar ], PSP was still defined clinically according to the original 1964 description with the hallmark features being early loss of postural righting reflexes with falls, and supranuclear vertical ophthalmoplegia (SVO). Yet, well before 1996, the clinical spectrum of PSP was, as predicted, already broadening; for instance, SVO was not always present early in the course of PSP and this and other atypical features created “problems” in the diagnosis [ [5] Perkin G.D. Lees A.J. Stern G.M. Kocen R.S. Problems in the diagnosis of progressive supranuclear palsy. (Steele--Richardson--Olszewski syndrome). Can. J. Neurol. Sci. 1978; 5: 168-173 Crossref PubMed Scopus (46) Google Scholar ]. Subsequent autopsy studies further expanded the phenotype of PSP including onset without early postural instability and falls, as well as PSP starting as asymmetric parkinsonism, pure akinesia, dementia.

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