Cognitive disturbances in progressive supranuclear palsy. 1994

I Litvan
Neuroepidemiology Branch, National Institutes of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland.

The cognitive disturbances in progressive supranuclear palsy (PSP) gave rise to the term "subcortical dementia." PSP patients demonstrate prominent recall deficits and moderate forgetfulness although their short-term and implicit perceptual memory processes are intact. PSP patients have both slowed motor responses and dramatically slowed information processing speed. Executive dysfunction appears early in the course of the disease and is relatively severe. The combination of severely slowed information processing and marked executive dysfunction are characteristic of PSP and differentiates it from other dementias. In their landmark description of progressive supranuclear palsy (PSP) as a clinicopathological entity, Steele et al. (1964) reported that cognitive disturbances were present in seven out of their nine patients. Ten years later, Albert et al. (1974) characterized these changes to be part of a "subcortical dementia," They analyzed 5 of their own PSP cases and also reviewed the published literature; they found a common cluster of symptoms, including the presence of forgetfulness, slowness of thought process, emotional or personality changes, and impaired ability to manipulate acquired knowledge. Albert et al. analysis was qualitative, but in the authors' view, clearly differentiated PSP patients from patients with "cortical dementia" who presented with aphasia, apraxia, and/or agnosia. They also suggested that the symptoms found in PSP were similar to those that had previously been described in patients with frontal lobe lesions.

UI MeSH Term Description Entries
D008569 Memory Disorders Disturbances in registering an impression, in the retention of an acquired impression, or in the recall of an impression. Memory impairments are associated with DEMENTIA; CRANIOCEREBRAL TRAUMA; ENCEPHALITIS; ALCOHOLISM (see also ALCOHOL AMNESTIC DISORDER); SCHIZOPHRENIA; and other conditions. Memory Loss,Age-Related Memory Disorders,Memory Deficits,Memory Disorder, Semantic,Memory Disorder, Spatial,Memory Disorders, Age-Related,Retention Disorders, Cognitive,Semantic Memory Disorder,Spatial Memory Disorder,Age Related Memory Disorders,Age-Related Memory Disorder,Cognitive Retention Disorder,Cognitive Retention Disorders,Deficit, Memory,Deficits, Memory,Memory Deficit,Memory Disorder,Memory Disorder, Age-Related,Memory Disorders, Age Related,Memory Disorders, Semantic,Memory Disorders, Spatial,Memory Losses,Retention Disorder, Cognitive,Semantic Memory Disorders,Spatial Memory Disorders
D003072 Cognition Disorders Disorders characterized by disturbances in mental processes related to learning, thinking, reasoning, and judgment. Overinclusion,Disorder, Cognition,Disorders, Cognition
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013494 Supranuclear Palsy, Progressive 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. (From Adams et al., Principles of Neurology, 6th ed, pp1076-7) Ophthalmoplegia, Progressive Supranuclear,Progressive Supranuclear Ophthalmoplegia,Progressive Supranuclear Palsy 1,Steele-Richardson-Olszewski Syndrome,Palsy, Progressive Supranuclear,Progressive Supranuclear Palsy,Richardson's Syndrome,Steele-Richardson-Olszewski Disease,Supranuclear Palsy, Progressive, 1,Progressive Supranuclear Palsies,Richardson Syndrome,Steele Richardson Olszewski Disease,Steele Richardson Olszewski Syndrome,Supranuclear Ophthalmoplegia, Progressive,Supranuclear Palsies, Progressive

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