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Results for “Supranuclear Palsy, Progressive”

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PubMed2002

Morphological and biochemical correlations of abnormal tau filaments in progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) is characterized by specific filamentous tau inclusions present in 3 types of cells including oligodendrocytes (coiled bodies), astrocytes (tufted astrocytes), and neurons (neurofibrillary tangles; NFTs). To correlate the morphological features and biochemical composition of tau in the inclusions, we examined tau filament-enriched fractions isolated from selected brain regions. Frontal and cerebellar white matter manifested a predominance of coiled bodies. The isolated fractions contained straight, 14-nm-wide filaments of relatively smooth appearance. Caudate nucleus and motor cortex with numerous tufted astrocytes contained mostly straight, but irregular, 22-nm-wide filaments with jagged contours. Perirhinal cortex and hippocampus, rich in NFTs, contained 22-nm-wide filaments that were twisted at 80-nm intervals. Among the regions, those with tufted astrocytes showed the most heterogeneity in the ultrastructure of filaments. In all regions, isolated filaments were immunolabeled with PHF-1, Tau 46, and AT8. Fractions from all regions showed 2 PHF-1 immunoreactive bands of 64 and 68 kDa, while an additional band of 60 kDa was detected in NFT-enriched regions. All fractions, in varying extents, showed Tau-1-immunoreactive bands between 45-64 kDa. The results indicate that the 3 types of PSP tau inclusions vary in the ultrastructure although with some overlapping features. Neuronal and glial inclusions also vary in the biochemical profile of tau protein. These differences may depend on the metabolism of tau in the diseased oligodendrocytes, astrocytes, and neurons.

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PubMed2004

Creutzfeldt-Jakob disease presenting as progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) is a neurodegenerative disorder characterized by an akinetic rigid syndrome with vertical supranuclear ophthalmoplegia, early falls, and levodopa resistance. The pathological substrate of PSP consists of filamentous tau degenerative lesions affecting neurons and glia. Other disorders can present with a similar clinical picture, most commonly corticobasal degeneration and multiple system atrophy. Non-neurodegenerative disorders are rare causes of the PSP syndrome. In this report we describe clinical and pathological features of two cases of Creutzfeldt-Jakob disease (CJD) presenting with the PSP syndrome and discuss which features may help prevent misdiagnosis. To our knowledge, this is the first report of cases of CJD with autopsy confirmation that presented with a PSP syndrome.

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PubMed2006

Aging and oxidative stress in progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) is a neurodegenerative disorder, which may possibly be induced by oxidative stress. However, the age-related alteration of the endogenous antioxidant system is not well understood. To better understand this, we measured Cu/Zn-superoxide dismutase (SOD), glutathione peroxidase (GPx), and 4-hydroxynonenal (HNE)-conjugated GPx in cerebrospinal fluid of PSP patients by enzyme linked immunosorbent assay. A significant increase in the Cu/Zn-SOD level was detected in PSP group compared with controls. The levels of Cu/Zn-SOD and GPx in PSP group showed positive correlations with age. Two-thirds of total GPx was present as the HNE-conjugated form with positive correlation in PSP group. In conclusion, the endogenous antioxidant system of PSP patients appears to be activated with aging, however, it might be unable to function effectively because of conjugation with HNE.

Age Factors↗

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.

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PubMed2002

Sequence analysis of tau in familial and sporadic progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) is a tau deposition neurodegenerative disorder which usually occurs in sporadic form and is associated with a common variant of the tau gene. Rare familial forms of PSP have been described. Recently familial frontotemporal dementia linked to chromosome 17 (FTDP-17) has been shown to be due to mutations in tau and there may be a clinical and pathological overlap between PSP and FTDP-17. In this study we have analysed the tau sequence in two small families with PSP, and a number of clinically typical and atypical sporadic cases with pathological confirmation of the diagnosis. The tau mutations described in FTDP-17 were not found in the most clinically diagnosed patients with PSP. This suggests that usually FTDP-17 and PSP, including the rare familial form of PSP, are likely to be separate conditions and that usually PSP and typical PSP-like syndromes are not due to mutations in tau.

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PubMed2000

Progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) or Steele-Richardson-Olszewski syndrome is a neurodegenerative disease of middle and late age. It is under-diagnosed not only by general physicians but also by neurologists. The cause of PSP is not known. Exposure to toxins and viruses has been proposed in the aetiology of PSP without any concrete evidence. The features of PSP resemble those of Parkinson's disease and the two diseases are often confused. Corticobasal degeneration and multisystem atrophy are other differential diagnoses. Despite certain common features with Parkinson's disease, corticobasal degeneration, and mutisystem atrophy, there are important differences that help to differentiate it from these disorders.

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PubMed1998

Clinical and genetic aspects of progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) is, after Parkinson's disease, the most common form of degenerative parkinsonism. Several clinical features are used in the recognition of this disorder as well as in the differentiation from related disorders. Clinical criteria that could increase diagnostic accuracy in research studies are also emphasized. Due to a better understanding of the genetic aspects of PSP, recent studies have suggested that it is a recessive disorder in linkage disequilibrium with the tau (tau) gene, rather than a sporadic disorder. In addition, the recent identification of mutations in the tau gene associated with a similar neurodegenerative condition (frontotemporal dementia and parkinsonism linked to chromosome 17) has further strengthened the argument that tau dysfunction is somehow involved in the pathogenesis of PSP. Nongenetic factors that could trigger or perpetuate the cascade of events leading to neuronal degeneration in PSP are also reviewed.

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PubMed2003

[Cognitive deficits in progressive supranuclear palsy].

Progressive supranuclear palsy (PSP) is one of the most frequent causes of an atypical parkinsonism. The cognitive disturbances in PSP gave rise to the term "subcortical dementia". Cognitive impairment is independent of depression, which is also common in PSP. There is no correlation between cognitive impairment and either disease duration or a level of physical disability. We present a clinical picture and difficulties in PSP diagnosis in three patients--63 to 74 years old, who were hospitalized in the Department of Neurology, Medical Academy in Bialystok. A neuropsychological evaluation revealed significant differences among those patients. The patients presented with: 1. depressive and dementive syndrome, 2. executive dysfunction, 3. slowed information processing with no signs of dementia. Our findings are similar with data presented in literature and confirm the observations that: 1. there is a difference in a degree of cognitive impairment in between the patients with PSP, 2. the most frequent cognitive disturbances in PSP patients are: slowness of thought process and executive dysfunction.

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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.

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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.

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PubMed1995

[Progressive supranuclear palsy].

Progressive supranuclear palsy (PSP) was a distinct clinicopathological syndrome described by Steele, Richardson and Olszewski in 1964. In 1974, Narabayashi and the present author described a syndrome of levodopa unresponsive pure akinesia or freezing without rigidity or tremor affecting gait, hand writing and speech. In a series of subsequent reports in Japan, evidence has indicated that pure akinesia often represents a pre-oculomotor form of PSP. In this sense, the syndrome of pure akinesia/PSP is proposed. The pathogenesis and etiology of PSP and related conditions remain to be cleared.

Diagnosis, Differential↗

PubMedDate not supplied

Familial progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) is a degenerative neurological disease not typically associated with a family history. Two siblings developed identical clinical features consisting of supranuclear vertical ophthalmoplegia, bradykinesia, rigidity, gait disturbance, and dementia. There was no history of encephalitis or of exposure to known chemicals. L-dopa and dopamine agonist therapy were minimally effective. Autopsy of 1 patient revealed the typical pathological findings of PSP: severe neuronal loss with neurofibrillary tangles (NFTs) in the substantia nigra, subthalamic nucleus, and locus ceruleus. Prominent neurofibrillary degeneration of the amygdaloid nucleus and hippocampus was also observed. Scattered neurofibrillary tangles were seen in the cerebral cortices. Cerebellar degeneration was characterized by a loss of neurons in the dentate nucleus associated with neurofibrillary tangles. Lewy bodies and cortical neuritic plaques were notably absent. The existence of a rare familial form of PSP is supported by these 2 siblings.

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PubMed1997

[Progressive supranuclear palsy].

Progressive supranuclear palsy (PSP) is a distinct clinicopathological syndrome described by Steele, Richardson and Olszewski in 1964. Its clinical features include supranuclear ophthalmoplegia, pseudobulbar palsy, dysarthria, nuchal dystonia, and dementia. The neuropathological changes are characteristic and include cell loss, gliosis, and neurofibrillary degeneration in the basal ganglia, brain stem and cerebellum. But, all these clinical features are not present in the early stage and diagnosis of PSP is sometimes difficult. Atypical presentation of PSP includes the case without ophthalmoplegia, with markedly dementia, or pure akinesia. Pure akinesia presents freezing of gait, handwriting and speech without rigidity or tremor, and can be the initial and early symptom-complex of PSP.

Brain↗

PubMed1999

Pramipexole in progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) is a progressive neurodegenerative disorder with no effective treatment. Dopaminergic agents occasionally produce transient symptomatic improvement. The authors report the results of pramipexole treatment (4.5 mg daily) in six patients with PSP (average disease duration, 4.4 years). Patients were treated for 2 months. Patients were evaluated with the Unified Parkinson's Disease Rating Scale, Hoehn and Yahr stage, and Schwab and England Activities of Daily Living Scale at baseline and 2 months. Pramipexole was not efficacious for the symptoms of PSP.

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PubMed1993

Retrospective study of drug response in 87 patients with progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) is a progressive neurodegenerative disease that responds poorly to pharmacologic intervention despite its clinical, neurochemical, and pathologic similarity to Parkinson's disease. We reviewed our experience with drugs used in the treatment of patients with PSP who were followed in the Department of Neurology, University of Medicine and Dentistry of New Jersey--Robert Wood Johnson Medical School. Of 136 patients identified, adequate drug-response data were available for 87 (64%). Benefit and adverse effects of therapy were graded on a 4-point scale: 0, none; 1, minimal; 2, moderate; 3, marked. The three most frequently used drugs were amitriptyline (32% of patients benefited), imipramine (28% of patients benefited) and levodopa/carbidopa (Sinemet) (38% of patients benefited). Levodopa/carbidopa, amantadine, selegiline, and amitriptyline gave the best risk/benefit ratios. Monotherapy tended to show more benefit and fewer adverse effects than polypharmacy.

Amitriptyline↗

PubMed2006

Relationship between neuronal loss and tangle formation in neurons and oligodendroglia in progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) is a progressive degenerative disorder characterized by neuronal loss, gliosis and abnormal fibril formation of abnormally phosphorylated tau protein in neurons and glia cells, but the cause is not clear at present. For the purpose of clarifying the pathological significance of accumulation of tau protein in neurons and oligodendroglia in PSP, we morphologically classified neurofibrillary tangles (NFT) and coiled bodies (CB) in oligodendroglia in three PSP brains into four stages, using double staining for immunohistochemistry with AT8 antibody and modified Gallyas-Braak (GB) staining. AT8-positive neurons without abnormal fibril structure with GB staining were classified as stage I, AT8-positive neurons containing a few fibril structures with GB staining were classified stage II, AT8-positive neurons containing mature fibril structures were classified as stage III, and AT8 negative neurons containing abnormal fibril structures stained only with GB staining were classified as stage IV (ghost tangles). These stages were also assessed for CB. Then we counted the number of cells of each stage in various brain regions to investigate the relationship of NFT and CB with neuronal loss and gliosis. The results showed that there were very few stage IV NFT and CB, which reflect cell death, but that stage III NFT and CB were abundant. Moreover, CB were abundant in regions with severe neuronal loss. These results suggest that appearance of CB is closely associated with degenerative regions.

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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.

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PubMed2003

[Progressive supranuclear palsy].

Progressive supranuclear palsy (PSP) is a parkinsonian syndrome 20 to 30 times less common than Parkinson's disease. PSP and Parkinson's disease share certain symptoms, but also present distinctive features. The histopathological features of PSP are highly specific and enable certain diagnosis. Since no biological marker has been identified, clinical signs must be recognized to establish the probability of the histopathological diagnosis. The NINDS program distinguishes two categories: probable PSP and possible PSP with distinctive characteristics. The sensitivity of probable PSP is only 50 p. 100, but the specificity and positive predictive value are 100 p. cent. For possible PSP, specificity is 83 p. cent (93 p. cent if diagnostic errors are included) and the positive predictive value is lower (83 p. cent). Although there number is smaller, patients with probable PSP are retained for research on new treatments. There have been few, non-controlled therapeutic trials in PSP. No drug and no surgical procedure has been demonstrated to be highly effective. Neuroprotection is a new avenue of research which requires knowledge of the natural course of clinical incapacity. A longitudinal study of 50 patients with probable PSP has provided mid-term results on symptom onset and on the development of three key signs, detected with the standard Unified Parkinson's Disease Rating Scale: incapacity to stand alone without help, incapacity to speak intelligently, incapacity to eat (tube feeding). The Kaplan-Meier curves show that the mean duration of the disease before development of one of these signs is 50 months. Research on protocols designed to stop disease progression should be centered on this period.

Deglutition Disorders↗