Progressive Supranuclear Palsy

Progressive Supranuclear Palsy
SYMPTOMS Diplopia, Poor downward vision, Difficulty reading
SIGNS Slow vertical saccades (First ocular sign), Abnormal pursuits, Impairment of voluntary vertical gazes (Downward gaze affected before upward gaze but eventually all voluntary eye movements will be lost), Apraxia of eyelid opening, Retraction of upper lids, Blepharospasm, Convergence dysfunction, Square wave jerks, Loss of Bell’s phenomenon with intact vestibular ocular response, Dry eyes due to decreased blink rate
WORK-UP Pupils | EOMs | Full eye exam with dilation | Cranial nerve testing | Test for Bell’s phenomenon | Test for vestibular ocular response | Visual field | OC
TREATMENT Begin artificial tears for dry eyes
Patient should undergo vision rehabilitation and vision restorative training (glasses, prism, tint, etc). Mirror-prism glasses can help but patient should not be prescribed multifocal or progressive lenses
Refer to neuro-ophthalmologist/neurologist for further evaluation and treatment
No effective treatment for progressive supranuclear palsy (L-dopa is ineffective)
Refer patient to occupational therapist
FOLLOW-UP Monitor every 4-6 months to evaluate visual symptoms
ADDITIONAL LAB | TESTS MRI with and without contrast of the brain Neurological evaluation
ETIOLOGY Progressive disease that affects the brainstem reticular formation and ocular motor supranucleus. Thought to occur due to an accumulation of neurofibrillary tangles
DIFFERENTIAL DX Parkinson’s disease, Hydrocephalus, Lewy body dementia, Whipple’s disease
NOTES Systemic signs include imbalance, stiff steps, slow movements, slurred speech, difficulty swallowing food, irritable personality, masked expression, apathy, and nuchal rigidity
Mortality rate is 100% within 10 years