Progressive Supranuclear Palsy PSP

PSP is also known as Steele-Richardson-Olszewski syndrome. It is a degenerative disorder of subcortical nuclei, including the subthalamic nucleus, substantia nigra, globus pallidus, caudate, putamen, and peria-queductal gray. Clinically, persons with PSP show paralysis involving motor neurons of the eye, leading to impaired downward ocular gaze and other ocular symptoms. Clinical diagnosis requires documentation of at least two of the following signs: axial dystonia (abnormal pattern of muscle tonus in the axial musculature) and rigidity (especially of the neck), pseudobulbar palsy (manifested as sudden crying or laughing without apparent cause or accompanying experienced emotion), bradykinesia (slowness in the initiation of movement), signs of frontal lobe dysfunction (e.g., motor perseveration, difficulty in behavioral set-shifting in response to changing task demands), postural instability with backward falling, and sometimes dysarthria (difficulty in the motor aspects of speech). Cognitive deficits, documented by neuropsy-chological testing, include memory loss, slowed information processing speed, apathy and depression, irritability, and executive function deficits.

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