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SYMPTOMS
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Diplopia, Poor downward vision, Difficulty reading
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SIGNS
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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
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WORK-UP
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Pupils | EOMs | Full eye exam with dilation | Cranial nerve testing | Test for Bell’s phenomenon | Test for vestibular ocular response | Visual field | OC
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TREATMENT
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Begin artificial tears for dry eyes
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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
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Refer to neuro-ophthalmologist/neurologist for further evaluation and treatment
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No effective treatment for progressive supranuclear palsy (L-dopa is ineffective)
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Refer patient to occupational therapist
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FOLLOW-UP
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Monitor every 4-6 months to evaluate visual symptoms
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ADDITIONAL LAB | TESTS
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MRI with and without contrast of the brain Neurological evaluation
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ETIOLOGY
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Progressive disease that affects the brainstem reticular formation and ocular motor supranucleus. Thought to occur due to an accumulation of neurofibrillary tangles
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DIFFERENTIAL DX
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Parkinson’s disease, Hydrocephalus, Lewy body dementia, Whipple’s disease
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NOTES
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Systemic signs include imbalance, stiff steps, slow movements, slurred speech, difficulty swallowing food, irritable personality, masked expression, apathy, and nuchal rigidity
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Mortality rate is 100% within 10 years
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