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Results for “Supranuclear Palsy, Progressive”

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At least 19 recordsLinked to original sources

PubMed1995

Neurodegeneration in the limbic and paralimbic system in progressive supranuclear palsy.

Progressive supranuclear palsy is neuropathologically characterized by neuronal degeneration of the basal ganglia, brain stem, and cerebellum. In addition, cortical neuronal degeneration associated with neurofibrillary tangles formation has been identified over wide areas of the brain in patients with progressive supranuclear palsy. We studied the distribution of alpha B-crystallin-positive degenerating neurons in cases with progressive supranuclear palsy, and compared them with those in Pick's disease. Alzheimer's disease, senile dementia of Alzheimer type, and normal aged individuals. A large number of alpha B-crystallin-positive neurons was found in the cerebral cortices of four out of nine patients with progressive supranuclear palsy. In particular, alpha B-crystallin-positive ballooned neurons were frequently observed in deep cortical pyramidal cell layers of the limbic and paralimbic systems in these diseases. The involvement of the limbic and paralimbic systems may thus contribute to personality changes as well as to memory and cognitive impairment in some patients with progressive supranuclear palsy.

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PubMed2000

A case of spontaneous arm levitation in progressive supranuclear palsy.

Progressive supranuclear palsy is one of the parkinsonial syndromes causing atypical parkinsonism. In recent reports, other than subcortical involvement, also cortical structures have been shown to be involved in progressive supranuclear palsy patients. One of the clinical presentations of this involvement is spontaneous arm levitation which is a component of alien limb syndrome. Here we report a clinically diagnosed progressive supranuclear palsy patient with spontaneous arm levitation. Clinically spontaneous levitation of one arm without denial of ownership suggests the presence of spontaneous arm levitation. Spontaneous arm levitation can occur in the setting of progressive supranuclear palsy and it possibly demonstrates the cortical involvement in this disorder.

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PubMed1997

Anterior cingulate and motor network metabolic impairment in progressive supranuclear palsy.

Progressive supranuclear palsy is the prototype of subcortical dementia. Using positron emission tomography and statistical parametric mapping, we compared the glucose metabolic pattern obtained in this subcortical dementia to that observed in elderly healthy controls and in Alzheimer's disease, the prototype of cortical dementia. Progressive supranuclear palsy was characterized by a relative decrease of metabolism in anterior cingulate, adjacent supplementary motor area, precentral cortex, middle prefrontal cortex, midbrain tegmentum, globus pallidus, and ventrolateral and dorsomedial nuclei of thalamus. The data in progressive supranuclear palsy highlight predominant metabolic impairment in brain structures engaged in response selection, in attention for action, and in motor networks.

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PubMed1982

Progressive supranuclear palsy.

Progressive supranuclear palsy has been recognized as a distinct nosological entity for about three decades now. Typically, this progressive neurological disease manifests itself late in the sixth decade with a terminal course of approximately four to six years. Well over one hundred cases have been described in the literature and the heterogeneous nature of progressive supranuclear palsy includes the characteristic vertical ophthalmoplegia, frequent falling and a profound nuchal rigidity. Other features are similar in many respects to those found in Parkinson's disease. The present article reviews the literature on progressive supranuclear palsy with particular reference to its clinical manifestations including the ophthalmoplegia, characteristic sleep disturbances and unique dementia. Also addressed, are neuropathological and epidemiological findings. Finally, conclusions and recommendations for further investigation are offered especially with regard to the neuropsychological nature of this neurological disorder.

Brain↗

PubMed2003

Progressive supranuclear palsy.

Progressive supranuclear palsy, also known as Steele-Richardson-Olszewski syndrome, is an uncommon neurodegenerative parkinsonian disorder that starts in middle and late life, and is frequently misdiagnosed as Parkinson's disease. This review will cover the epidemiology, clinical picture, differential diagnosis and management of patients with progressive supranuclear palsy.

Cognition Disorders↗

PubMed2005

Cholinergic systems in progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) is a progressive neurodegenerative disease characterized by akinetic-rigid features, falls, a supranuclear gaze palsy and subcortical dementia. Pathologically, there is abnormal accumulation of tau protein. Cholinergic deficits are thought to underlie the postural instability and cognitive impairment of PSP, but trials of cholinergic agonists and cholinesterase inhibitors have failed to show improvement in motor function, quality of life and cognitive impairment. The five cortico-basal ganglia loops, linking functionally related areas of the brain, are damaged in PSP, leading to specific clinical deficits. Cholinergic dysfunction is related to loss of cholinergic interneurons in the striatum, compounded by reduced inputs into the circuits from other cholinergic nuclei, such as the pedunculopontine nucleus and nucleus basalis of Meynert. Normal cholinergic transmission requires the presence of intact cholinergic neurons capable of releasing sufficient acetylcholine, and functional muscarinic and nicotinic receptors. Whilst there is evidence from autopsy and in vivo studies of loss of cholinergic neurons in PSP, the receptor status is unknown. This may be critical to understanding the basis for the poor therapeutic response to cholinomimetics. Symptomatic treatment using cholinergic drugs may thus be improved by more specific targeting of cholinergic receptors or nuclei. There is also evidence that cholinergic agents may have disease-modifying effects. This article reviews the key clinical features of PSP, along with normal basal ganglia anatomy and cholinergic transmission. Cholinergic deficits based on clinical and neurochemical parameters are then discussed, before concluding with suggested future directions for cholinergic treatments.

Basal Ganglia↗

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↗

PubMed1974

The 'subcortical dementia' of progressive supranuclear palsy.

Progressive supranuclear palsy (Steele et al.) has a characteristic pattern of dementia: (1) forgetfulness, (2) slowing of thought processes, (3) emotional or personality changes (apathy or depression with occasional outbursts of irritability), and (4) impaired ability to manipulate acquired knowledge. In many neurological disease states associated with subcortical pathology a similar pattern of dementia exists. The neurobehavioural changes of progressive supranuclear palsy thus typify a clinical pattern which may be referred to as subcortical dementia. The subcortical dementias have a striking clinical resemblance to the dementia which occurs after bifrontal lobe disease. However, the subcortical dementias can be clearly distinguished clinically from cortical dementias, other than frontal dementias. We propose as a tentative hypothesis that there may be common pathophysiological mechanisms underlying the subcortical dementias-in particular, disturbances of timing and activation. There are immediate practical implications of this hypothesis: drugs which have an effect on subcortical timing and activating mechanisms may be useful in the treatment of subcortical dementias.

Affective Symptoms↗

PubMed2004

Novel haplotypes in 17q21 are associated with progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are sporadic neurodegenerative diseases presenting as atypical parkinsonian disorders, characterized by the presence of tau-positive neurofibrillary tangles. Recently, an extended haplotype (H1E) of 787.6 kb that comprises several genes including MAPT showed increased association with PSP. The objective of this study was to determine the size of the H1E haplotype associated with PSP and CBD in different populations and to identify specific subhaplotypes in the background of H1E haplotype. Nineteen single nucleotide polymorphisms (SNPs) in the 17q21 region were genotyped in two case-control samples. The SNPs that were associated with higher risk for the disease in the homozygous state delimit a region of more that 1 Mb. Haplotype analyses in the Spanish sample showed that the most frequent haplotype found among the patients (H1E'), which extends 1.04 Mb and contains several genes such as MAPT, CRHR1, IMP5, Saitohin, WTN3, and NSF. A specific subhaplotype (H1E'A) was present in 16% of PSP patients but was not observed in the controls. Furthermore, the H2E'A haplotype, was rarely present in the disease group suggesting that it plays a protective role. The identification of these specific subhaplotypes that modify risk for PSP/CBD supports the hypothesis that a pathogenic allele exists in a subgroup of PSP patients.

Adult↗

PubMed2006

In vivo imaging of microglial activation with [11C](R)-PK11195 PET in progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) is a neurodegenerative disease presenting with voluntary gaze difficulties, early falls, and Parkinsonism. Neuronal loss, associated with intracellular neurofibrillary tangles and activated microglia, is found targeting the basal ganglia, brainstem nuclei, and frontal cortex. [11C](R)-PK11195 PET is a marker of peripheral benzodiazepine binding sites (PBBS) expressed by activated microglia. We have used [11C](R)-PK11195 PET to demonstrate in vivo the degree and distribution of the glial response to the degenerative process in four patients with PSP. Compared to normal age-matched controls, the PSP patient group showed significantly increased mean [11C](R)-PK11195 binding in the basal ganglia, midbrain, the frontal lobe, and the cerebellum. Two of the patients were rescanned after 6 to 10 months and during that time the level of microglial activation remained stable. [11C](R)-PK11195 PET reveals a pattern of increased microglial activation in PSP patients involving cortical and subcortical regions that corresponds well with the known distribution of neuropathological changes. [11C](R)-PK11195 PET, therefore, may help in characterizing in vivo the underlying disease activity in PSP.

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PubMed2007

Diffusion-weighted magnetic resonance imaging differentiates Parkinsonian variant of multiple-system atrophy from progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) and the parkinsonian variant of multiple-system atrophy (MSA-P) may present with a similar phenotype. Magnetic resonance diffusion-weighted imaging (DWI) has been shown to be a sensitive discriminator of MSA-P from Parkinson's disease (PD). We studied 20 PSP, 11 MSA-P, 12 PD patients and 7 healthy controls in order to investigate whether regional apparent diffusion coefficients (rADCs) help distinguish PSP and MSA-P; whether rADCs are correlated with clinical disease severity scores; and the relationship between brainstem and cerebellar volumes and rADCs in PSP and MSA-P. The Unified Parkinson's Disease Rating Scale, Hoehn and Yahr score, Mini Mental State Examination, and frontal assessment battery were recorded in all patients. Regional ADCs were measured in the middle cerebellar peduncle (MCP), caudal and rostral pons, midbrain, decussating fibers of the superior cerebellar peduncle, thalamus, putamen, globus pallidus, caudate nucleus, corpus callosum, frontal and parietal white matter, as well as the centrum semiovale. In MSA-P, rADCs in the MCP and rostral pons were significantly greater than in PSP (P < 0.001 and 0.009) and PD (P < 0.001 and = 0.002). Stepwise logistic regression revealed that the MCP rADC distinguishes MSA-P from PSP with a sensitivity of 91% and a specificity of 84%. Increased brainstem rADCs were associated with motor deficit in MSA-P and PSP. Increased rADCs in the pons and MCP were associated with smaller pontine and cerebellar volumes in MSA-P. rADCs distinguish MSA-P from PSP. These have a clinical correlate and are associated with reduced brainstem and cerebellar volumes.

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PubMed1995

Neuropsychological features of progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) is the epitome of a subcortical dementia process. Due to its relative rarity, there is only a small literature on the neuropsychological consequences of PSP. The findings to date demonstrate that PSP patients have dramatically slowed information processing and motor execution, rapid forgetting, problems in orienting attentional resources, and difficulty in planning and shifting conceptual sets. The pattern and severity of these deficits are unique to PSP and suggest that the study of PSP patients can provide a special insight into brain-behavior relations.

Brain↗

PubMed1995

Psychiatric aspects of progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) is characterized by supranuclear ophthalmoplegia mainly affecting vertical gaze, nuchal dystonia in extension, pseudobulbar palsy, and mental changes. The literature on PSP has been neurologically oriented whereas the psychiatric aspects have been relatively neglected. A review of the literature shows that psychiatric disturbance in PSP is common but with no characteristic pattern. Cognitive impairment, nonspecific affective and behavioral disturbances are commonly found, whereas frank psychosis or bipolar disorder are rare. Misdiagnoses with psychiatric disorders are common and a heightened awareness of the condition is necessary for early diagnosis.

Diagnosis, Differential↗

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.

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PubMed2004

Protein levels of glycogen synthase 3 kinase are normal in progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) is a neurodegenerative disorder characterized by pure neurofibrillary tau pathology involving mainly basal ganglia and brain stem nuclei. One of the kinases involved in tau phosphorylation is glycogen synthase 3 kinase (GSK3). In mammals GSK3 is present in two isoforms, alpha and beta regulated by phosphorylation: phosphorylation of Ser9 in GSK3beta or Ser21 in GSK3alpha leads to inactivation while phosphorylation of Tyr216 in GSK3beta or Tyr279 in GSK3alpha leads to activation. We analyzed the protein levels of GSK3alpha/beta and of the phosphorylated forms GSK3beta S(9), GSK3beta Y(216), GSK3alpha Y(279) in brain tissues of subjects with PSP. The analysis failed to show significant differences of all GSK3 isoforms in PSP in comparison to age-matched control cases. This negative result argues against the role of GSK3 in the pathogenesis of PSP.

Aged↗

PubMedDate not supplied

Psychiatric symptoms in progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) is an unusual neurodegenerative disorder that superficially resembles Parkinson's disease (PD). It is characterized by gaze palsy, bulbar signs, parkinsonian signs, and mental changes. While mental changes are a frequent finding, they have, with the exception of dementia, been poorly defined. In this study, 19 patients with PSP were evaluated psychiatrically and compared with 42 patients with PD. Fifty-two percent of the patients had some degree of dementia, as measured by the Mini-Mental State Exam. Eight (42%) of the PSP patients had other psychiatric diagnoses, mostly relatively mild depression or anxiety, though two patients had more severe depression. Six (32%) patients had pathologic laughing or crying, and four of these had a psychiatric diagnosis other than dementia. The PSP patients did not differ from the PD patients on measures of depression or anxiety and did not have a greater rate of formal psychiatric diagnoses. This study confirms previous reports of dementia as a common feature of PSP. It further suggests that psychiatric disturbances, while common, are generally relatively mild, though more serious psychiatric illness may be seen.

Aged↗

PubMed2000

Mitochondrial dysfunction in cybrid lines expressing mitochondrial genes from patients with progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) is a neurodegenerative movement disorder of unknown etiology. We hypothesized that mitochondrial DNA (mtDNA) aberration could occur in this disease and contribute to its pathogenesis. To address this we created transmitochondrial cytoplasmic hybrid (cybrid) cell lines expressing mitochondrial genes from persons with PSP. The presence of cybrid mtDNA aberration was screened for by biochemical assay of mitochondrial gene products. Relative to a control cybrid set, complex I activity was reduced in PSP cybrid lines (p<0.005). Antioxidant enzyme activities were elevated in PSP cybrid lines. These data suggest that mtDNA aberration occurs in PSP, causes electron transport chain pathology, and can produce oxidative stress. Further study of mitochondrial dysfunction in PSP may yield insights into why neurodegeneration occurs in this disease.

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PubMed2000

Lipoperoxidation is selectively involved in progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) is a neurodegenerative disorder characterized by extensive neurofibrillary tangle (NFT) formation and neuronal loss in selective neuronal populations. Currently, no clues to the biological events underlying the pathological process have emerged. In Alzheimer disease (AD), which shares with PSP the occurrence of NFTs, advanced glycation end products (AGEs) as well as oxidation adducts have been found to be increased in association with neurofibrillary pathology. The presence and the amount of lipid and protein oxidation markers, as well as of pyrraline and pentosidine. 2 major AGEs, was assessed by biochemical, immunochemical, and immunocytochemical analysis in midbrain tissue from 5 PSP cases, 6 sporadic AD cases, and 6 age-matched control cases. The levels of 4-hydroxynonenal (HNE) and thiobarbituric acid reactive substances (TBARS), 2 major products of lipid peroxidation, were significantly increased by 1.6-fold (p < 0.04) and 3.9-fold (p < 0.01), respectively, in PSP compared with control tissues, whereas in AD only TBARS were significantly increased. In PSP tissue the intensity of neuronal HNE immunoreactivity was proportional to the extent of abnormal aggregated tau protein. The amount of protein oxidation products and AGEs was instead similar in PSP and control tissues. In AD, a higher but not significant level of pyrraline and pentosidine was measured, whereas the level of carbonyl groups was doubled. These findings indicate that in PSP, unlike in AD, lipid peroxidation is selectively associated with NFT formation. The intraneuronal accumulation of toxic aldehydes may contribute to hamper tau degradation, leading to its aggregation in the PSP specific abnormal filaments.

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