Criteria
MDS-PSP criteria[3]
The Movement Disorder Society (MDS) criteria, published in 2017, aim to optimise the sensitivity and specificity of progressive supranuclear palsy (PSP) diagnosis, regardless of the phenotypic subtype.[3] A summary of the key elements is included here.
The mandatory inclusion criteria are:
Sporadic occurrence
Age 40 years or older at onset of first PSP-related symptom
Gradual progression of PSP-related symptoms
There are also multiple mandatory exclusion criteria aimed at ruling out other differential diagnoses that can present in a similar way to PSP.
Four functional domains have been identified which summarise the characteristic manifestations of PSP, with three typical clinical features listed under each domain. The level of certainty for the diagnosis is highest for feature 1, intermediate for feature 2, and lowest for feature 3 in each domain.
Ocular motor dysfunction
O1: Vertical supranuclear gaze palsy
O2: Slow velocity of vertical saccades
O3: Frequent macro square wave jerks or 'eyelid-opening apraxia'
Postural instability
P1: Repeated unprovoked falls within the first 3 years
P2: Tendency to fall on the pull-test within the first 3 years
P3: More than two steps backwards on the pull-test within the first 3 years
Akinesia
A1: Progressive gait freezing within the first 3 years
A2: Parkinsonism, akinetic-rigid, predominantly axial, and levodopa-resistant
A3: Parkinsonism, with tremor and/or asymmetric and/or levodopa-responsive
Cognitive dysfunction
C1: Speech/language disorder (i.e., non-fluent/agrammatic variant of primary progressive aphasia or progressive apraxia of speech)
C2: Frontal cognitive/behavioural presentation
C3: Corticobasal syndrome
The degree of diagnostic certainty can then be ascertained. The patient can be diagnosed with probable PSP, possible PSP, or suggestive of PSP depending on the combination of clinical features, as follows:
Probable PSP:
PSP-RS: (O1 or O2) + (P1 or P2)
PSP-PGF: (O1 or O2) + A1
PSP-P: (O1 or O2) + (A2 or A3)
PSP-F: (O1 or O2) + C2
Possible PSP:
PSP-OM: O1
PSP-RS: O2 + P3
PSP-PGF: A1
PSP-SL: (O1 or O2) + C1
PSP-CBS: (O1 or O2) + C3
Suggestive of PSP:
PSP-OM: O2 or O3
PSP-PI: P1 or P2
PSP-RS: O3 + (P2 or P3)
PSP-P: (A2 or A3) + (O3, P1, P2, C1, C2, CC1, CC2, CC3, or CC4)
PSP-SL: C1
PSP-CBS: C3
Key:
PSP-RS: PSP Richardson's syndrome
PSP-PGF: PSP with progressive gait freezing
PSP-P: PSP-parkinsonism
PSP-F: PSP-frontal presentation
PSP-OM: PSP-oculomotor dysfunction
PSP-SL: PSP-speech/language disorder
PSP-CBS: PSP-corticobasal syndrome
PSP-PI: PSP-postural instability
The following supportive features can increase diagnostic confidence but do not qualify as diagnostic criteria.
Clinical clues:
Levodopa resistance
Hypokinetic, spastic dysarthria
Dysphagia
Photophobia
Imaging findings:
Predominant midbrain atrophy or hypometabolism
Postsynaptic striatal dopaminergic degeneration
NINDS-SPSP[28]
The National Institute of Neurological Disorders and Stroke/Society for PSP (NINDS/SPSP) criteria, published in 1996, specified three degrees of diagnostic certainty for PSP: possible PSP, probable PSP, and definite PSP.[28] The criteria have excellent specificity for PSP but are poorly sensitive for the non-Richardson’s syndrome phenotypes of the condition and hence have been superseded in clinical practice by the 2017 MDS criteria.[3]
Possible PSP is defined as a gradually progressive disorder with onset at age 40 years or later and either one of the below present (with no alternative explanation):
Vertical supranuclear gaze palsy, or
Postural instability with falls within the first year of onset together with slowing of vertical saccades
Probable PSP is defined as a gradually progressive disorder with onset at age 40 or later with both of the below present (and no alternative explanation):
Vertical supranuclear gaze palsy, and
Postural instability with falls within the first year
Definite PSP
Definite PSP is clinically probable or possible PSP together with postmortem histopathological evidence of typical PSP.
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