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geri:dementia:progressive-supranuclear-palsy-psp [September 2018]
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 +====== Progressive Supranuclear Palsy (PSP) ======
  
 +{{INLINETOC}}
 +===== Primer =====
 +**Progressive Supranuclear Palsy** (PSP) is a neurodegenerative disorder characterized by early postural instability,​ leading to falls, and a characteristic vertical supranuclear-gaze palsy on physical exam. It is classified as an atypical parkinsonian syndrome (or Parkinson'​s Plus).
 +
 +== Symptoms ==
 +Pseudobulbar signs may be present, and retropulsion is often prominent. Cognitive testing will show psychomotor slowing, poor working memory, and executive dysfunction.
 +===== Diagnostic Criteria =====
 +
 +==== NINDS-SPSP Criteria ====
 +The National Institute of Neurological Disorders and Stroke and Society for PSP (NINDS-SPSP) criteria are the most widely used criteria for the diagnosis of PSP.[([[https://​www.ncbi.nlm.nih.gov/​pubmed/​8710059|Litvan,​ I., Agid, Y., Calne, D., Campbell, G., Dubois, B., Duvoisin, R. C., ... & Hallett, M. (1996). Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) report of the NINDS-SPSP international workshop. Neurology, 47(1), 1-9.]])]
 +
 +<panel type="​info"​ title="​ NINDS-SPSP PSP Criteria"​ subtitle="​Litvan,​ I., et al. Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) report of the NINDS-SPSP international workshop. Neurology 47.1 (1996): 1-9." no-body="​true">​
 +
 +^               ^ Symptoms ​                                                                                                                      ^
 +^ Probable PSP  | Vertical supranuclear gaze palsy plus postural instability and falls within the first year of symptom onset to diagnosis. ​     |
 +^ Possible PSP  | Supranuclear gaze palsy or a combination of slow vertical saccades and postural instability with falls within the first year.  |
 +</​panel>​
 +
 +==== MDS Criteria ====
 +The International Parkinson and Movement Disorder Society (MDS)-endorsed PSP Study Group set out to provide an evidence and consensus-based revision of the NINDS-SPSP criteria in 2017, with a much more comprehensive set of criteria.[([[https://​www.ncbi.nlm.nih.gov/​pmc/​articles/​PMC5516529/​|Hoglinger,​ Gunter U. et al. “Clinical Diagnosis of Progressive Supranuclear Palsy: The Movement Disorder Society Criteria.” Movement disorders :​ official journal of the Movement Disorder Society 32.6 (2017): 853–864. PMC. Web. 16 Sept. 2018.]])]
 + 
 +<panel type="​info"​ title="​Basic Features: Core Inclusion and Exclusion Criteria"​ subtitle="​Höglinger,​ Günter U., et al. Clinical diagnosis of progressive supranuclear palsy: the movement disorder society criteria. Movement Disorders 32.6 (2017): 853-864."​ no-body="​true">​
 +^ Mandatory inclusion criteria ​ | 1. Sporadic occurrence \\ 2. Age 40 or older at onset of first PSP-related symptom \\ 3. Gradual progression of PSP-related symptoms ​                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                      |
 +^ Mandatory exclusion criteria ​ | //Clinical findings//​\\ 1. Predominant,​ otherwise unexplained impairment of episodic memory, suggestive of [[geri:​dementia:​alzheimers|]]\\ 2. Predominant,​ otherwise unexplained autonomic failure, e.g., orthostatic hypotension (orthostatic reduction in blood pressure after 3 minutes standing ​ 30 mm Hg systolic or  15 mm Hg diastolic), suggestive of multiple system atrophy or Lewy body disease\\ 3. Predominant,​ otherwise unexplained visual hallucinations or fluctuations in alertness, suggestive of dementia with Lewy bodies\\ 4. Predominant,​ otherwise unexplained multisegmental upper and lower motor neuron signs, suggestive of motor neuron disease (pure upper motor neuron signs are not an exclusion criterion)\\ 5. Sudden onset or step-wise or rapid progression of symptoms, in conjunction with corresponding imaging or laboratory findings, suggestive of vascular etiology, autoimmune encephalitis,​ metabolic encephalopathies,​ or prion disease \\ 6. History of encephalitis\\ 7. Prominent appendicular ataxia\\ 8. Identifiable cause of postural instability,​ e.g., primary sensory deficit, vestibular dysfunction,​\\ severe spasticity, or lower motor neuron syndrome \\ \\ //Imaging findings//​\\ 1. Severe leukoencephalopathy,​ evidenced by cerebral imaging\\ 2. Relevant structural abnormality,​ e.g., normal pressure or obstructive hydrocephalus;​ basal ganglia, diencephalic,​ mesencephalic,​ pontine or medullary infarctions,​ hemorrhages,​ hypoxic-ischemic lesions, tumors, or malformations ​ |
 +</​panel>​
 +
 +
 +<panel type="​info"​ title="​Core Clinical Features"​ subtitle="​Höglinger,​ Günter U., et al. Clinical diagnosis of progressive supranuclear palsy: the movement disorder society criteria. Movement Disorders 32.6 (2017): 853-864."​ footer="​Presumed levels of certainty (1 [highest], 2 [mid], and 3 [lowest]) that contribute to the diagnosis of PSP" no-body="​true">​
 +^ Levels of Certainty ​ ^ Ocular Motor Dysfunction ​                                           ^ Postural Instability ​                                             ^ Akinesia ​                                                                          ^ Cognitive Dysfunction ​                                                                                                              ^
 +^ Level 1              | **O1**: Vertical supranuclear gaze palsy                                | **P1**: Repeated unprovoked falls within 3 years                      | **A1**: Progressive gait freezing\\ within 3 years                                     | **C1**: Speech/​language disorder (i.e. - nonfluent/​agrammatic variant of [[geri:​dementia:​primary-progressive-aphasia-ppa|primary progressive aphasia]] or progressive apraxia of speech) ​ |
 +^ Level 2              | **O2**: Slow velocity of vertical saccades ​                             | **P2**: Tendency to fall on the pull-test within 3 year               | **A2**: [[geri:​parkinsons|Parkinsonism]],​ akinetic-rigid,​\\ predominantly axial, and\\ levodopa resistant ​ | **C2**: [[geri:​dementia:​frontotemporal|Frontal cognitive/​behavioural presentation]] ​                                                                                      |
 +^ Level 3              | **O3**: Frequent macro square wave jerks or\\ "​eyelid opening apraxia" ​ | **P3**: More than two steps backward on the pull-test within 3 years  | **A3**: [[geri:​parkinsons|Parkinsonism]],​ with tremor and/or asymmetric and/or levodopa responsive ​        | **C3**: [[geri:​dementia:​corticobasal-degeneration-cbd|Corticobasal syndrome]] ​                                                                                                          |
 +</​panel>​
 +===== Pathophysiology =====
 +PSP is due to the intracerebral aggregation of the microtubule-associated protein tau (MAPT), predominantly involving isoforms with four microtubule-binding repeats (4R-tau), in neurofibrillary tangles, oligoden- drocytic coils, and, specifically,​ astrocytic tufts. The definite diagnosis of PSP requires a post-mortem neuropathological examination.