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At least 19 recordsLinked to original sources

PubMed1987

Progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) was first recognized as a distinct morbid entity by Richardson, Steele and Olszewski a quarter century ago. Subsequent experience has confirmed and extended their original observations. PSP has become familiar as a chronic progressive disorder with extrapyramidal rigidity, bradykinesia, gait impairment, bulbar palsy, dementia and a characteristic supranuclear ophthalmoplegia. It is an important cause of parkinsonism. Its etiology remains obscure. Familial concentrations have not been observed. Some cases exhibit no oculomotor dysfunction. Dementia is usually mild. Recent neuropsychological studies have defined features consistent with frontal lobe cortical dysfunction. Seizures and paroxysmal EEG activity may occur. CT and MRI scans show midbrain atrophy early and later atrophy of the pontine and midbrain tegmentum and the frontal and temporal lobes. PET scans have shown frontal hypometabolism and loss of striatal D-2 dopamine receptors. Postmortem studies have documented involvement of both dopaminergic and cholinergic systems. Treatment remains palliative and unsatisfactory.

Brain↗

PubMed1987

Electrooculographic findings in progressive supranuclear palsy.

Progressive supranuclear palsy is a heterogeneous system degeneration. We studied three patients by means of electrooculography. There were severe disturbances in saccadic and smooth pursuit eye movements, and optokinetic nystagmus in both vertical and horizontal planes. Caloric reactions were absent in all patients. The absence of vestibular reflex responses in contrast to the normal results in doll's head manoeuvre seems contradictory and is discussed. From the results in our patients it is concluded that E.O.G. is an essential contribution in proving a multiple system pathology.

Aged↗

PubMed1992

CSF galanin and neuropeptide Y immunoreactivity in progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) has been associated with degenerative changes in cholinergic and dopaminergic neurons in several brain regions. Since acetylcholine is colocalized with the neuropeptide galanin in certain neuronal populations, we measured the concentration of this neuropeptide and neuropeptide Y in cerebrospinal fluid (CSF) of 11 patients with PSP and in 16 age-matched healthy controls. No significant alterations in the CSF levels of galanin or neuropeptide Y were found.

Aged↗

PubMed1992

Cerebral perfusion in progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) is a neurodegenerative disorder in which structurally preserved cerebral cortex is thought to be functionally disconnected by subcortical lesions. To assess brain functional activity in patients with PSP, we measured regional cerebral perfusion, as estimated by [123I] iofetamine (IMP) and single-photon emission computed tomography (SPECT), in 11 patients with a clinical diagnosis of PSP and 10 healthy control subjects. IMP uptake was measured in 2 basal ganglia and 24 cortical regions. Neuropsychological tests were administered to assess cognitive function. Compared to age-matched normal control subjects, relative IMP uptake was significantly reduced in PSP patients in basal ganglia (21%), superior frontal (25%), anterior parietal (19%), and inferior frontal (18%) regions. Cognitive performance was most abnormal on tests thought to be subserved predominantly by frontal lobes. Our study demonstrates that IMP-SPECT detects physiological abnormalities in the cortex that parallel behavioral impairments in PSP.

Aged↗

PubMed1989

[The reappraisal study of the ultrastructure of Alzheimer's neurofibrillary tangles in three cases of progressive supranuclear palsy].

Progressive supranuclear palsy (PSP) is characterized by symptoms of disturbance of eye movement, nuchal rigidity, parkinsonism and subcortical dementia. Its pathology reveals that Alzheimer's neurofibrillary tangles (NFTs) are situated especially in the nuclei of basal ganglia and brainstem. It has been pointed out that NFTs observed in PSP are composed of straight tubules, the width of which is about 15 nm. This report is to reappraise the ultrastructure of NFTs observed in three cases of PSP. They are case 1; 69-year-old male, case 2; 62-year-old female and case 3; 65-year-old male. The clinical features of three cases were characterized by above-mentioned symptoms. Many NFTs were observed in brainstem of case 1 and 3. NFTs seen in brainstem of case 2 were in a small amount. In case 1 and 2, there were many NFTs in cerebral cortex, especially in parahippocampal gyri and other limbic areas. We examined the portions of frontal cerebral cortex, parahippocampal cortex, the basal nucleus of Meynert, oculomotor nucleus, substantia nigra, pontine nuclei and locus caeruleus of case 1, substantia nigra, red nucleus and inferior olivary nucleus of case 2, and Ammon's horn, globus pallidum, subthalamic nucleus, substantia nigra, oculomotor nucleus, pontine nuclei, locus ceruleus and inferior olivary nucleus of case 3. Ultrastructurally, NFTs of frontal cortex of case 1 consisted of twisted tubules and those of hippocampal and parahippocampal cortices of case 1 consisted of straight and twisted tubules, being observed separately in neurons. The NFTs of brainstem of case 1 and 2 were mainly composed of 12-17 nm straight tubules, and twisted tubules occasionally intermingled with straight components. In locus ceruleus of case 1, a single straight tubule could be seen in the bundles of twisted tubules. NFTs of case 3 were composed of only straight tubules, the width of which was about 15 nm. Twisted components were not observed in neurons of each nuclei of case 3. The characteristic ultrastructure of NFTs in PSP has been an appearance of straight tubules, however, twisted tubules were occasionally observed in this study. There are several case reports which demonstrated an appearance of both straight and twisted components. It is easily presumed that the process of aging takes part in the accumulation of twisted fibrils. However, it is now under consideration why twisted tubules are also observed in PSP. To summarize this study, the cases with many NFTs observed in widespread cerebral cortex may have a tendency to show both straight and twisted tubules.

Aged↗

PubMed1992

[Progressive supranuclear palsy].

Progressive supranuclear palsy, first described as clinical entity by Steele, Richardson and Olszewski, is a degenerative disorder of the central nervous system. Besides progressive supranuclear oculomotor disturbances, other characteristic signs are pseudobulbar paresis, axial rigidity, gait disturbances and subcortical dementia. Misinterpretation in the early stage as Parkinson's disease is frequently seen. A causal therapy is still missing.

Aged↗

PubMed1987

Limb dystonia in progressive supranuclear palsy.

Progressive limb dystonia contributed to disability in 8 of 30 patients with progressive supranuclear palsy (PSP). In five, it was present when the patients were on no medication. In four, it had been present before the distinctive ophthalmoplegia permitted a correct diagnosis. The severity of limb dystonia did not correlate with the severity of either ophthalmoplegia or neck dystonia. The importance of dystonia in the pathophysiology of PSP is emphasized, with regard to both the branchial dystonia that underlies several cardinal features of the disease, and to the frequent occurrence of limb dystonia as an early sign.

Aged↗

PubMedDate not supplied

Central conduction time in progressive supranuclear palsy.

Progressive nuclear palsy (PSP) is a parkinson-like extrapyramidal disorder with pathological evidence of brain stem demyelination. We studied brain stem auditory (BAEP) and somatosensory (SSEP) evoked potentials in 8 patients with progressive supra-nuclear palsy to look for evidence of such central demyelination. We found minor alterations in BAEPs and frequently abnormal SSEPs, suggestive of brain stem white matter involvement in this disorder. Evoked potentials may assist in the differentiation of PSP from other parkinsonian conditions.

Aged↗

PubMedDate not supplied

"Learned helplessness" and the patient with progressive supranuclear palsy.

Progressive supra-nuclear palsy is a neurologically debilitating disease of unknown cause and with no effective treatment. Learned helplessness, a major cause of depression in the institutionalized elderly, is a feeling of worthlessness and powerlessness resulting in a decreased incentive to try. This article provides insight into these two problems and specific nursing interventions to enhance self care.

Aged↗

PubMed1989

[Progressive supranuclear palsy accompanied with progressive subcortical vascular encephalopathy of Binswanger type--a case report].

We report the clinical and neuropathological findings in an autopsy case of progressive dementia, Parkinsonism, pseudobulbar palsy and supranuclear ophthalmoplegia. Since 70 years old, this hypertensive patient developed forgetfulness, unsteady gait and festination. These symptoms rapidly worsened and he was admitted in October 1983, at age 71. He had severe dementia and showed stiff face. Voluntary vertical movement of the eyes was severely disturbed, but reflex vertical movement by the doll's head eye maneuver was not affected. Muscle tone in the limbs increased slightly, and deep tendon reflexes were hyperactive in jaw and the upper extremities. Babinski sign was negative bilaterally. Sensation and coordination remained normal. Although he was not paretic or ataxic, his movements were very slow. He could not stand by himself and easily fell backward. These clinical features resembled those of progressive supranuclear palsy except for severe dementia and rapid progression. Brain CT scan revealed marked dilatation of the lateral ventricles, prominent periventicular lucency and atrophy of brainstem and cerebral cortex. Treatment with levodopa slightly improved his movement, but difficulty in swallowing worsened increasingly. He died of hypoglycemic coma and aspiration pneumonia in September 1984, about two years after the initial symptoms. General autopsy showed severe pneumonia and atrophy of the liver. The brain weighed 1,210g. Atherosclerotic change in the cerebral arteries were mild. Coronal sections of the cerebral hemispheres revealed diffuse ischemic change and multiple small infarctions in the bilateral cerebral white matter. Cortical atrophy was observed in the cerebral hemispheres. The basal ganglia, thalamus, and pons showed status lacunaris. Atrophy of midbrain and depigmentation of the substantia nigra were observed macroscopically.

Aged↗

PubMed1986

Rehabilitation in progressive supranuclear palsy: case report.

Progressive supranuclear palsy (PSP) is a distinct clinicopathologic entity characterized by supranuclear ophthalmoplegia, pseudobulbar palsy, axial dystonia in extension, and subcortical dementia. Although relatively rare, PSP is disabling, thus rehabilitation techniques and management are indicated in nearly every case. This report describes the neurologic presentation, rehabilitation management, and outcome of treatment of a patient with PSP during a 12-month period. The patient required thorough neuromuscular, neuropsychological, speech, swallowing, vision, and social service evaluations prior to the implementation of a rehabilitation program. Therapeutic rehabilitation techniques focused on limb coordination activities, tilt board balancing, ambulation activities, and activities to improve route finding and visual scanning ability. Prism lenses were introduced to compensate for deficits in vertical eye movements. Treatment improved the patient's functional status. Later, as the patient's neurologic status deteriorated, it became necessary to educate the family and caretakers in the ongoing rehabilitation management of the patient.

Bulbar Palsy, Progressive↗

PubMed1988

Progressive supranuclear palsy: extensive neuropil threads in addition to neurofibrillary tangles. Very similar antigenicity of subcortical neuronal pathology in progressive supranuclear palsy and Alzheimer's disease.

Light microscopic immunohistochemical investigations were performed on neurofibrillary tangles (NFT) in four histologically confirmed cases of Alzheimer's disease (AD) and in five patients with a progressive supranuclear palsy (PSP). The antibody panel included antisera to the neuronal microtubule-associated protein, tau, and to isolated paired helical filaments (PHF), as well as mouse monoclonal antibodies (MAbs) to phosphorylated epitopes on high and medium molecular weight neurofilament subunits (RT97 and BF10, respectively). Paraffin sections were also impregnated with the Gallyas silver method, which specifically stains tangles and cortical neuropil threads in AD, but does not stain normal neurofilaments. All tangles in PSP and AD showed consistent immunostaining with antibodies to tau protein and isolated PHF, regardless of their localization. MAbs RT97 and BF10, however, did not stain or only weakly stained, subcortical tangles in PSP and AD, whereas most cortical NFT in AD were intensely immunostained. All tangles in PSP were as heavily impregnated with Gallyas as they were in AD. Furthermore there were extensive networks of Gallyas-positive, tau- and PHF-immunoreactive neurites in subcortical gray areas containing NFT, and bundles of positive axons in white matter tracts interconnecting subcortical nuclei of PSP. Our studies indicate a much more extensive disruption of fibrillar proteins in PSP subcortical neurons than previously reported. They furthermore indicate a very similar antigenic profile of NFT in PSP and AD, as far as subcortical neurons are concerned.

Aged↗

PubMed1987

Elementary motor perseveration in early diagnosis of progressive supranuclear palsy.

Three patients with progressive supranuclear palsy (PSP) with different symptoms at onset presented intense elementary motor perseveration before the appearance of the distinctive features of the illness. In elementary motor perseveration once an element of a movement has begun it is no longer inhibited at the right time and continues unchecked. Perseverations were observed in these patients during tests for dysdiadochokinesia, for reproduction of rhythmic structures and during the drawing of simple geometric shapes. Though this sign has not yet been described in PSP it is possible that an adequate search for its occurrence may show it to be an important sign for early diagnosis and an element for the clinical characterization of PSP.

Female↗

PubMed1990

[Obsessive-compulsive behavior and progressive supranuclear palsy].

A case of progressive supranuclear palsy characterized by a loss of self-activation and a compulsive behaviour of the obsessive type is reported. The pathological examination was remarkable for the intensity of pallidal lesions and their diffusion to both the external and internal segments. While the loss of self-activation seemed to result from a damaged cortico-subcortical circuit forming a limbic loop, the compulsive behaviour of the obsessive type may have resulted from the interruption of a frontal-caudal-pallidal-luysian circuit.

Globus Pallidus↗

PubMed1989

[Palilalia associated with progressive supranuclear palsy].

Two cases of progressive supranuclear palsy (PSP) with palilalia were presented. Case 1: A 64-year-old woman was in good health until age 62 when she noted clumsiness in walking. Subsequently, forgetfulness, abrupt falls and difficulty in swallowing developed. At the age of 63, she tended to repeat same words in conversation. On admission, neurological examination revealed mental deterioration (Hasegawa's scale 19), unsteady emotional state, supranuclear vertical gaze palsy, pseudobulbar palsy, nuchal dystonia, pyramidal tract signs and extrapyramidal signs. Although spontaneous speech production was reduced, she could answer to questions with compulsive repetition of a phrase or word. She always repeated twice or three times without stuttering, logoclonus or "palilalie aphone". Case 2: A 68-year-old, right-handed woman noted double vision since about one year ago. Subsequently, she developed slowness of voluntary movement, forgetfulness and difficulty in walking. Neurological examination showed similar signs as Case 1. She was not palilaic on examination, however we found the description of her palilalia on nurse's working records. Palilalia was not present so constantly as Case 1, but continued for about a year. We suppose that palilalia is not a rare phenomenon in PSP. Although the pathogenesis of palilalia is unknown at present, we speculate the combination of extrapyramidal sign, pseudobulbar palsy and dementia may most contribute the pathogenesis of palilalia on PSP.

Aged↗

PubMed1989

[Progressive supranuclear palsy].

Nine cases of progressive supranuclear palsy are reported in this paper. There are 6 males and 3 females in this series. The average age at admission was 62 years. The clinical features of those cases are: (1) onset at the presenile with gradual progression, (2) supranuclear vertical ophthalmoplegia, especially downgaze paresis, (3) disarthria, (4) gait disturbances, (5) dystonia and rigidity of the limbs, (6) clumsiness and ataxia, (7) masked face, (8) bilateral pyramidal signs and (9) mental disturbances. The CT scan in this series showed dilatation of the ventricular system, enlargement of the Sylvius fissure and cortical sulci. There was also enlargement of quadrigeminal and cisterns in the CT scan. The CT diagnosis were cerebral atrophy in 9 cases and brainstem atrophy in 8 cases in this series.

Adult↗

PubMed1990

Effects of physostigmine on spatial attention in patients with progressive supranuclear palsy.

We tested patients with progressive supranuclear palsy and control subjects on a task of visuopatial attention. Targets preceded by cues on the same side were termed validly cued; and those on the opposite side, invalidly cued. For all subjects, validly cued targets were responded to faster than those that were invalidly cued. The difference between reaction times for invalidly and validly cued targets, which is hypothesized to measure attentional movement, was significantly increased for the patients. The performance of the controls on certain neuropsychological tests was correlated with their attentional ability. These correlations were altered by progressive supranuclear palsy. Physostigmine treatment of the patients induced a speeding of responses to invalidly cued targets as a function of the duration of the disease. These studies show defects in cognition and attention in patients with progressive supranuclear palsy and demonstrate that physostigmine reduces some of the abnormal visual attentional performance.

Aged↗

PubMed1988

Progressive supranuclear palsy: nursing care implications.

Progressive supranuclear palsy (PSP) is an uncommon, but debilitating, neurological disease. Characterized by paralysis of eye movements, difficulty talking and swallowing, body trunk rigidity, gait ataxia, and dementia, the disease progresses rapidly over a 6-8 year time span. Death usually occurs from aspiration pneumonia. To provide meaningful care for the PSP patient and his/her family, the neuroscience nurse must first understand the disease pathophysiology, then the appropriate care interventions.

Bromocriptine↗