Progressive supranuclear palsy

Progressive supranuclear palsy
A neurologic disorder of unknown origin that gradually destroys cells in many areas of the brain, leading to serious and permanent problems with the control of gait and balance. The most obvious sign of the disease is an inability to aim the eyes properly, which occurs because of damage in the area of the brain that coordinates eye movements. Some patients describe this effect as a blurring. Another common visual problem is an inability to maintain eye contact during a conversation. This can give the mistaken impression that the patient is hostile or uninterested. Patients also often show alterations of mood and behavior, including depression and apathy as well as progressive mild dementia. The disease is "progressive" because it worsens over time; "supranuclear" because the main problem is not in the nuclei (clusters of cells in the brainstem) that directly control eye movements, but in higher centers that control the nuclei; and "palsy," which means weakness, in this case of eye movement. Progressive supranuclear palsy (PSP) characteristically begins with loss of balance. Nearly all patients eventually develop the characteristic difficulty in moving the eyes up and down, the sign that often arouses a doctor's suspicion of the correct diagnosis. Although PSP gets progressively worse, no one dies from PSP itself. Difficulty swallowing can eventually permit aspiration of food into the trachea (windpipe). PSP may also be complicated by the effects of immobility, especially pneumonia, and by injuries from falls. PSP is the disease that led to the death of the British comedian Dudley Moore in 2002. He developed pneumonia as a terminal event. PSP affects about 20,000 Americans, one in every 100,000 people over the age of 60. Patients are usually in middle or advanced age. Men are affected more often than women. There is currently no effective treatment for PSP. In some patients the slowness, stiffness, and balance problems of PSP may respond to antiparkinsonian agents such as levodopa, or levodopa combined with anticholinergic agents or amantadine, but the effect is usually temporary. The speech, vision, and swallowing difficulties usually do not respond to any drug treatment. Antidepressant medications have been used with some modest success in PSP. The most commonly used of these drugs are fluoxetine (Prozac), amitriptyline (Elavil), and imipramine (Tofranil). The anti-PSP benefit of these drugs seems not to be related to their ability to relieve depression.

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progressive supranuclear palsy n an uncommon neurological disorder that is of unknown etiology, that typically occurs from late middle age onward, and that is marked by loss of voluntary vertical eye movement, muscular rigidity and dystonia of the neck and trunk, pseudobulbar paralysis, bradykinesia, and dementia called also supranuclear palsy

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a progressive neurological disorder, having onset during the sixth decade, characterized by supranuclear ophthalmoplegia, especially paralysis of the downward gaze, pseudobulbar paralysis, dysarthria, dystonic rigidity of the neck and trunk, and dementia. Called also Steele-Richardson-Olszewski syndrome.

Medical dictionary. 2011.

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  • Progressive supranuclear palsy — Infobox Disease Name = Progressive supranuclear palsy Caption = DiseasesDB = 10723 ICD10 = ICD10|G|23|1|g|20 ICD9 = ICD9|333.0 ICDO = OMIM = 601104 MedlinePlus = eMedicineSubj = neuro eMedicineTopic = 328 MeshID = D013494 Progressive supranuclear …   Wikipedia

  • progressive supranuclear palsy — a progressive neurological disorder resulting from degeneration of the motor neurones, basal ganglia, and brainstem. Starting in late middle age, it is characterized by a staring facial expression due to impaired ability to move the eyes up and… …   Medical dictionary

  • progressive supranuclear palsy — Steele Richardson Olszewski syndrome a progressive neurological disorder resulting from degeneration of the motor neurones, basal ganglia, and brainstem. Starting in late middle age, it is characterized by a staring facial expression due to… …   The new mediacal dictionary

  • progressive supranuclear palsy — /suprəˈnjukliə/ (say soohpruh nyoohkleeuh) noun a cerebral degenerative disease of late adulthood, typified by nerve problems affecting the muscles of the eyes, tongue, throat, etc., and causing rigidity of the limbs, and dementia. Abbrev.: PSP …  

  • supranuclear palsy — n PROGRESSIVE SUPRANUCLEAR PALSY …   Medical dictionary

  • Progressive supranukleäre Blickparese — Klassifikation nach ICD 10 G23.1 Progressive supranukleäre Ophthalmoplegie (Steele Richardson Olszewski Syndrom) …   Deutsch Wikipedia

  • Progressive Supranukleäre Blickparese — Klassifikation nach ICD 10 G23.1 Progressive supranukleäre Ophthalmoplegie (Steele Richardson Olszewski Syndrom) …   Deutsch Wikipedia

  • Progressive supranukleäre Ophthalmoplegie — Klassifikation nach ICD 10 G23.1 Progressive supranukleäre Ophthalmoplegie (Steele Richardson Olszewski Syndrom) …   Deutsch Wikipedia

  • Palsy — Paralysis, generally partial, whereby a local body area is incapable of voluntary movement (motor function). For example, Bell’s palsy is localized paralysis of the muscles on one side of the face. The word “palsy” is a corruption (and… …   Medical dictionary

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