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Progressive supranuclear palsy (PSP) is a late-onset neurodegenerative disease involving the gradual deterioration and death of specific volumes of the brain. [ 1 ] [ 2 ] The condition leads to symptoms including loss of balance , slowing of movement , difficulty moving the eyes , and cognitive impairment . [ 1 ]
This is a list of major and frequently observed neurological disorders (e.g., Alzheimer's disease), symptoms (e.g., back pain), signs (e.g., aphasia) and syndromes (e.g., Aicardi syndrome). There is disagreement over the definitions and criteria used to delineate various disorders and whether some of these conditions should be classified as ...
PSP has been all over the news—here's what to know about it.
The applause sign was first described by Dubois and colleagues in 1995, as “a simple test of motor control that helps to differentiate Progressive supranuclear palsy (PSP) from frontal or striatofrontal degenerative diseases”, [1] but has since appeared in various neurodegenerative conditions involving frontal lobe dysfunction.
Alzheimer's disease, Pick's disease, FTDP-17 and progressive supranuclear palsy can display a corticobasal syndrome. [ 25 ] [ 14 ] It has been suggested that the nomenclature of corticobasal degeneration only be used for naming the disease after it has received verification through postmortem analysis of the neuropathology. [ 4 ]
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Additional features include bradykinesia, early-onset postural instability, increased rigidity in axial muscles, dysautonomia, alien limb syndrome, supranuclear gaze palsy, apraxia, involvement of the cerebellum including the pyramidal cells, and in some instances significant cognitive impairment. [2]
This vertical palsy is supranuclear, so doll's head maneuver should elevate the eyes, but eventually all upward gaze mechanisms fail. In the extreme form, conjugate down gaze in the primary position, or the "setting-sun sign" is observed. Neurosurgeons see this sign most commonly in patients with hydrocephalus. [8]