Progressive Supranuclear Palsy. Shirley H. Wray, M.D., Ph.D. Professor of Neurology, Harvard Medical School Director, Unit for Neurovisual Disorders Massachusetts General Hospital. Criteria for Dx PSP. Gradually progressive disorder Onset at age 40 years old or later
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Shirley H. Wray, M.D., Ph.D.
Professor of Neurology, Harvard Medical School
Director, Unit for Neurovisual Disorders
Massachusetts General Hospital
Gradually progressive disorder
Onset at age 40 years old or later
Vertical supranuclear palsy
Slowing of vertical saccades
Postural instability with falls in the first year of disease onset
Symmetric akinesia or rigidity
Abnormal neck posture, especially retrocollis
Poor or absent response of parkinsonism to levodopa therapy
Early dysphagia and dysarthria
Early onset of cognitive impairment, including at least 2 of these: apathy, decreased verbal fluency, impaired abstract reasoning, and utilization behavior
Slow vertical saccades, especially down, with a preserved range of movement, may be the first sign of the disorder; later, loss of vertical saccades and quick phases
Horizontal saccades become slow and hypometric
Disruption of steady gaze by horizontal saccadic intrusions (square-wave jerks)
Impaired smooth pursuit, vertically (reduced range) and horizontally (with catch-up saccades)
Smooth eye-head tracking may be relatively preserved, especially vertically
Preservation of vestibulo-ocular reflex
Horizontal disconjugacy suggesting INO
Loss of convergence
Ultimately, all eye movements may be lost, but vestibular movements are the last to go
Eyelid disorders: delay in lid opening, lid lag, repetitive blinking in response to flashlight stimulus (failure to habituate), blepharospasm
Tonic head deviation opposite to direction of body rotation (vestibulocollic reflex)
Inability to clap just three times (applause sign)
Leigh RJ, Zee DS. Diagnosis of Central Disorders of Ocular Motility. Chpt 12: 598-718. The Neurology of Eye Movements, Fourth Edition. Oxford University Press, NY, 2006.
Figure 1: Sagittal T2-weighted MR in another patient with PSP shows the tectal plate is markedly thinned and atrophic. Courtesy Anne Osborn, MD.
Figure 2. Single photon emission tomography (PET) scan of a patient with PSP demonstrating frontal lobe hypoperfusion.
Figure 3. PET in a patient with PSP. Showing area of hypoperfusion (blue).
Figure 4: PSP_pale locus ceruleus
Figure 5: PSP_pale substantia nigra
Figure 6: PSP-the-globose Tangle
Figure 7: PSP-tau-globose-tangle
Figure 8: PSP-tau-tufted
Alzheimer’s disease PSP
Dementia pugilistica MSA
Guam ALS/PD Corticobasogangli-
Pick disease onic degeneration
Argyrophilic grain FTDP-17
disease Postencephalatic PD
Nieman-Pick, type C Autosomal recess-
SSPE ive PD
Freidman DI, Jankovic, J, McCrary III JA. Neuro-ophthalmic findings in progressive supranuclear palsy. J Clin Neuro-ophthalmol. 1992 Jun; 12(2):104-109.
Growden JH, Rossor MN. The Dementias. Blue Books of Practical Neurology. Butterworth-Heinemann 1998; vol 19.
Stanford PM, Halliday GM, Brooks WS, Kwok JBJ, Storey CE, Creasey H, Morris JGL, Fulham MJ, Schofield PR. Progressive supranuclear palsy pathology caused by a novel silent mutation in exon 10 of the tau gene: explanation of the disease phenotype caused by tau gene mutations. Brain 2000; 123(Pt 5): 880-893.