Progressive Supranuclear Palsy

HARVARD MEDICAL SCHOOL
DEPARTMENT OF NEUROLOGY
MASSACHUSETTS GENERAL HOSPITAL
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
Vertical supranuclear palsy
Slowing of vertical saccades
Postural instability with falls in the first
year of disease onset

Supportive Criteria for Dx PSP
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

Clinical Features of PSP
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)

Clinical Features of PSP
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

Clinical Features of PSP
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.

Neuroimaging
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).

Pathology
Figure 4: PSP_pale locus ceruleus
Figure 5: PSP_pale substantia nigra

Pathology
Figure 6: PSP-the-globose Tangle

Pathology
Figure 7: PSP-tau-globose-tangle
Figure 8: PSP-tau-tufted

Tauopathies: Clinical Diseases
Alzheimer’s disease
Dementia pugilistica
Guam ALS/PD
Pick disease
Argyrophilic grain
disease
Nieman-Pick, type C
SSPE
PSP
MSA
Corticobasoganglionic
degeneration
FTDP-17
Postencephalatic PD
Autosomal recessive
PD

References
Freidman DI, Jankovic, J, McCrary III JA.
Neuro-ophthalmic findings in progressive
supranuclear palsy. J Clin Neuroophthalmol.
1992 Jun; 12(2):104-109.
Growden JH, Rossor MN. The Dementias.
Blue Books of Practical Neurology.
Butterworth-Heinemann 1998; vol 19.

Richardson JC, Steele J, Oszewski J.
Supranuclear ophthalmoplegia, pseudobulbar
palsy, nuchal dystonia and dementia. A clinical
report on eight cases of heterogenous system
degeneration. Trans Am Neurol Assoc. 1963;
88:25-29.
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.

http://www.library.med.utah.edu/NOVEL