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q 3 - lausmlestep
#1
A 78-year-old man came to your office seeking a second opinion regarding his Parkinson's disease (PD). Three years ago he was diagnosed with PD, and despite treatment, his condition became worse. He has an unsteady gait, which has been progressively worse over the past five years. For the past 3 years, he has also had difficulty seeing. He complains of frequent falls, occasional urinary incontinence, and difficulties in maintaining an erection. Both his parents had Parkinson's disease. Physical examination findings are remarkable for postural instability and gait unsteadiness. He has a significant bradykinesia, and the face is hypomimic. The neck has an extended posture, and there is rigidity of the limbs and axial muscles. The speech is dysarthric, and the jaw jerk and gag reflexes are exaggerated. There is paralysis of vertical and horizontal gaze, with preservation of the oculocephalic and oculovestibular reflexes. What is the most likely cause of his condition?

(A) Parkinson's disease
(B) Progressive supranuclear palsy
© Shy-Drager syndrome
(D) Postencephalitic parkinsonism
(E) Familial parkinsonism
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#2
(B) Progressive supranuclear palsy

Explanation:

This patient presents with progressive supranuclear palsy, which accounts for 8% of all parkinsonian patients evaluated in a Parkinson's disease clinic. Progressive supranuclear palsy has an onset after 70 years of age.
Initial symptoms consist of a gradual onset of postural instability, unsteady gait, and supranuclear vertical ophthalmoparesis, initially expressed by an impairment of downward gaze. Later, upward and lateral conjugate gaze become impaired. They can exhibit axial rigidity, nuchal dystonia, and a rigid facial expression. Dementia is a sign of late disease. The symptoms don't respond to antiparkinsonian medications.

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