Advances in progressive supranuclear palsy

new diagnostic criteria, biomarkers, and therapeutic approaches

Adam L. Boxer, Jin Tai Yu, Lawrence Golbe, Irene Litvan, Anthony E. Lang, Günter U. Höglinger

Research output: Contribution to journalReview article

71 Citations (Scopus)

Abstract

Progressive supranuclear palsy (PSP), previously believed to be a common cause of atypical parkinsonism, is now recognised as a range of motor and behavioural syndromes that are associated with a characteristic 4-repeat tau neuropathology. New research criteria that recognise early presentations of PSP and operationalise diagnosis of the full spectrum of clinical phenotypes have been reported. The Movement Disorders Society PSP diagnostic criteria include syndromes with few or mild symptoms that are suggestive of underlying PSP pathology and could provide an opportunity for earlier therapeutic interventions in the future. These criteria also include definitions for variant PSP syndromes with different patterns of movement, language, or behavioural features than have been conclusively associated with PSP pathology. Data from new diagnostic biomarkers can be combined with the clinical features of disease to increase the specificity of the new criteria for underlying PSP pathology. Because PSP is associated with tau protein abnormalities, there is growing interest in clinical trials of new tau-directed therapies. These therapies are hypothesised to have disease-modifying effects by reducing the concentration of toxic forms of tau in the brain or by compensating for loss of tau function. Since tau pathology is also central to Alzheimer's disease and chronic traumatic encephalopathy, a successful tau therapeutic for PSP might inform treatment of other neurodegenerative diseases.

Original languageEnglish (US)
Pages (from-to)552-563
Number of pages12
JournalThe Lancet Neurology
Volume16
Issue number7
DOIs
StatePublished - Jul 1 2017

Fingerprint

Progressive Supranuclear Palsy
Biomarkers
Pathology
Therapeutics
tau Proteins
Poisons
Movement Disorders
Parkinsonian Disorders
Neurodegenerative Diseases
Alzheimer Disease
Language
Clinical Trials
Phenotype

All Science Journal Classification (ASJC) codes

  • Clinical Neurology

Cite this

Boxer, Adam L. ; Yu, Jin Tai ; Golbe, Lawrence ; Litvan, Irene ; Lang, Anthony E. ; Höglinger, Günter U. / Advances in progressive supranuclear palsy : new diagnostic criteria, biomarkers, and therapeutic approaches. In: The Lancet Neurology. 2017 ; Vol. 16, No. 7. pp. 552-563.
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Advances in progressive supranuclear palsy : new diagnostic criteria, biomarkers, and therapeutic approaches. / Boxer, Adam L.; Yu, Jin Tai; Golbe, Lawrence; Litvan, Irene; Lang, Anthony E.; Höglinger, Günter U.

In: The Lancet Neurology, Vol. 16, No. 7, 01.07.2017, p. 552-563.

Research output: Contribution to journalReview article

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