progressive supranuclear palsy

PSP cases are often split into two subgroups, PSP-Richardson, the classic type, and PSP-Parkinsonism, where a short-term response to levodopa can be obtained. A degenerative disease of the central nervous system characterized by balance difficulties; OCULAR MOTILITY DISORDERS (supranuclear ophthalmoplegia); DYSARTHRIA; swallowing difficulties; and axial DYSTONIA. Onset is usually in the fifth decade and disease progression occurs over several years. Pathologic findings include neurofibrillary degeneration and neuronal loss in the dorsal MESENCEPHALON; SUBTHALAMIC NUCLEUS; RED NUCLEUS; pallidum; dentate nucleus; and vestibular nuclei. Progressive supranuclear palsy is a degenerative tauopathic disease involving the gradual deterioration and death of specific areas of the brain. The affected brain cells are both neurons and glial cells. The neurons display neurofibrillary tangles, which are clumps of tau protein, a normal part of a brain cell's internal structural skeleton. These tangles are often different from those seen in Alzheimer's disease, but may be structurally similar when they occur in the cerebral cortex. Their chemical composition is usually different, however, and is similar to that of tangles seen in corticobasal degeneration. Lewy bodies are seen in some cases, but it is not clear whether this is a variant or an independent co-existing process. The principal areas of the brain affected are: the basal ganglia, particularly the subthalamic nucleus, substantia nigra and globus pallidus; the brainstem, particularly the portion of the midbrain where "supranuclear" eye movement resides; the cerebral cortex, particularly that of the frontal lobes; the dentate nucleus of the cerebellum; and the spinal cord, particularly the area where some control of the bladder and bowel resides. The initial symptoms in two-thirds of cases are loss of balance, lunging forward when mobilizing, fast walking, bumping into objects or people, and falls. Other common early symptoms are changes in personality, general slowing of movement, and visual symptoms. Later symptoms and signs are dementia (typically including loss of inhibition and ability to organize information), slurring of speech, difficulty swallowing, and difficulty moving the eyes, particularly in the vertical direction. The latter accounts for some of the falls experienced by these patients as they are unable to look up or down. Some of the other signs are poor eyelid function, contracture of the facial muscles, a backward tilt of the head with stiffening of the neck muscles, sleep disruption, urinary incontinence and constipation.

http://purl.obolibrary.org/obo/ND_0000172

PSP cases are often split into two subgroups, PSP-Richardson, the classic type, and PSP-Parkinsonism, where a short-term response to levodopa can be obtained.

Steele-Richardson-Olszewski syndrome

PSP

A degenerative disease of the central nervous system characterized by balance difficulties; OCULAR MOTILITY DISORDERS (supranuclear ophthalmoplegia); DYSARTHRIA; swallowing difficulties; and axial DYSTONIA. Onset is usually in the fifth decade and disease progression occurs over several years. Pathologic findings include neurofibrillary degeneration and neuronal loss in the dorsal MESENCEPHALON; SUBTHALAMIC NUCLEUS; RED NUCLEUS; pallidum; dentate nucleus; and vestibular nuclei.

Progressive supranuclear palsy is a degenerative tauopathic disease involving the gradual deterioration and death of specific areas of the brain. The affected brain cells are both neurons and glial cells. The neurons display neurofibrillary tangles, which are clumps of tau protein, a normal part of a brain cell's internal structural skeleton. These tangles are often different from those seen in Alzheimer's disease, but may be structurally similar when they occur in the cerebral cortex. Their chemical composition is usually different, however, and is similar to that of tangles seen in corticobasal degeneration. Lewy bodies are seen in some cases, but it is not clear whether this is a variant or an independent co-existing process. The principal areas of the brain affected are: the basal ganglia, particularly the subthalamic nucleus, substantia nigra and globus pallidus; the brainstem, particularly the portion of the midbrain where "supranuclear" eye movement resides; the cerebral cortex, particularly that of the frontal lobes; the dentate nucleus of the cerebellum; and the spinal cord, particularly the area where some control of the bladder and bowel resides. The initial symptoms in two-thirds of cases are loss of balance, lunging forward when mobilizing, fast walking, bumping into objects or people, and falls. Other common early symptoms are changes in personality, general slowing of movement, and visual symptoms. Later symptoms and signs are dementia (typically including loss of inhibition and ability to organize information), slurring of speech, difficulty swallowing, and difficulty moving the eyes, particularly in the vertical direction. The latter accounts for some of the falls experienced by these patients as they are unable to look up or down. Some of the other signs are poor eyelid function, contracture of the facial muscles, a backward tilt of the head with stiffening of the neck muscles, sleep disruption, urinary incontinence and constipation.

Alexander P. Cox

http://en.wikipedia.org/wiki/Progressive_supranuclear_palsy

http://neurolex.org/wiki/Category:Progressive_Supranuclear_Palsy

http://ontology.neuinfo.org/NIF/Dysfunction/NIF-Dysfunction.owl#birnlex_12697

progressive supranuclear palsy

ND:0000172

progressive supranuclear palsy

http://purl.obolibrary.org/obo/ND_0000151