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PubMed1990

Neurotensin receptors in Parkinson's disease and progressive supranuclear palsy: an autoradiographic study in basal ganglia.

The technique of receptor autoradiography was used to study the distribution of neurotensin receptors in post mortem brain tissues from patients affected by Parkinson's disease, progressive supranuclear palsy and from age-matched controls. [125I]Neurotensin was used as ligand. Significant receptor decreases were found in the substantia nigra, both pars compacta and reticulata, and in the putamen in Parkinson's disease and progressive supranuclear palsy. In addition, significant decreases of neurotensin receptors were found in the ventral tegmental area, nucleus accumbens and dorsal part of caudate head in patients with Parkinson's disease but not in patients with progressive supranuclear palsy, indicating differential involvement of neurotensin receptors in these two neurological disorders. In addition, both in Parkinson's disease and progressive supranuclear palsy the decrement of striatal neurotensin binding sites was less than expected from the reported decrease of dopamine content in this nucleus, suggesting only partial localization of neurotensin receptors on mesostriatal dopaminergic projections.

Aged↗
PubMed1996

Progressive supranuclear palsy: a clinicopathological study of 21 cases.

The symptoms and signs used to diagnose progressive supranuclear palsy (PSP) should be easily identifiable by neuropathologists and neurologists as well as by movement disorder experts. The presence, at the time of death, of symptoms and signs that are used in published clinical criteria for the diagnosis of this disorder was searched for in 21 pathologically confirmed typical PSP cases. The following items, present in at least 80% of pathologically confirmed cases, can be considered as the most accurate clinical data for the diagnosis of PSP: non-familial parkinsonism, not improved by L-dopa therapy, with vertical voluntary gaze palsy; postural instability and falls; pseudobulbar palsy and dementia with frontal lobe-like syndrome; and a progressive course of less than 10 years. The definite diagnosis of PSP must be clinicopathological, and these minimal clinical data may be used for this purpose.

Adult↗
PubMed1988

Neuropathologic findings in progressive supranuclear palsy. A brief review with two additional case reports.

Recent studies utilizing electron microscopy and immunohistochemistry have added to the understanding of the pathology of progressive supranuclear palsy. This article reviews the neuropathologic findings of progressive supranuclear palsy and includes two new case reports. Findings in the patients reported here include extensive neuronal loss with reactive gliosis in deep subcortical gray matter such as globus pallidus, subthalamic nuclei, substantia nigra, red nucleus, dentate nucleus, and inferior olives. Neurofibrillary tangles were also noted in thalamus, hypothalamus, striatum, nucleus basalis, locus ceruleus, basis pontis nuclei, midbrain, pontine, and medullary reticular formation as well as in various cranial nerve nuclei.

Aged↗
PubMed1993

Serotoninergic terminal transporters are differentially affected in Parkinson's disease and progressive supranuclear palsy: an autoradiographic study with [3H]citalopram.

Receptor autoradiography with [3H]citalopram as ligand was used to study the distribution of serotonin uptake binding sites in post mortem brain tissues from patients with Parkinson's disease, progressive supranuclear palsy and from age-matched controls. Significant decreases in [3H]citalopram binding sites were found in the cerebral cortex of patients with Parkinson's disease and progressive supranuclear palsy. Densities of [3H]citalopram binding sites were significantly reduced in all components of the basal ganglia of Parkinson's disease but only in the head of caudate nucleus of progressive supranuclear palsy patients. The density of [3H]citalopram binding sites in the raphe nuclei of Parkinson's disease was comparable to control values. Our results suggest that serotoninergic terminals are differentially affected in Parkinson's disease and in progressive supranuclear palsy.

Aged↗
PubMed1995

Distribution of tangles and threads in the cerebral cortex in progressive supranuclear palsy.

Recent studies have described silver- and tau-positive glia and threads in the degenerating lesions of progressive supranuclear palsy. In this study, Gallyas-Braak silver impregnation and several immunohistochemical techniques were employed to examine the distribution of tangles, abnormal glia and threads in the cerebral cortex of nine cases of progressive supranuclear palsy. In addition to neurofibrillary tangles, argentophilic glia and threads were impregnated exclusively by Gallyas-Braak technique. This technique demonstrated two types of glia profiles: tightly coiled intra-cytoplasmic profiles surrounding nuclei (coiled profiles) and thorn-like profiles with radial ramifications (thorn-like profiles). Thorn-like profiles are possibly in astrocytes and were detected in the cerebral cortex, while coiled profiles are possibly in oligodendroglia and were detected both in the cerebral cortex and subcortical white matter. Topographically, many neurofibrillary tangles were constantly seen in the frontal cortex and in the pre-central gyrus. Numerous neurofibrillary tangles were detected in the entorhinal cortex of the two brains. Argentophilic glia and threads were also frequent both in the frontal cortex and the precentral gyrus; however, they were more frequent in the pre-central gyrus that in the frontal cortex in four of the eight cases examined. In two brains, argentophilic threads were distributed widely in the cerebral cortex and white matter except for the temporal cortex. In immunohistochemical studies, argentophilic glia and threads were mostly positive for Tau 2, and a small number of them were weakly positive for ubiquitin and paired helical filament protein. The immunoproperties of these abnormal glia and threads seemed to be virtually identical to those of neurofibrillary tangles.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗
PubMed1993

[Comparison of the vesicourethral function between progressive supranuclear palsy and Parkinson's disease].

We compared the vesicourethral function between progressive supranuclear palsy (7 cases) and Parkinson's disease (vesicourethral function study in 8 patients with dysuria and questionnaire study in 44 patients). The frequency of urinary incontinence in the 44 patients with Parkinson's disease was 38.7%. Vesicourethral function study showed hypersensitivity, low bladder capacity, detrusor hyperreflexia and normal sphincter EMG. The frequency of urinary incontinence in supranuclear palsy was 85.7%. Vesicourethral function study showed hyposensitivity and detrusor hyperreflexia in 6 cases, and 4 of these 6 cases had residual urine due to impaired detrusor contraction in micturition phase and/or abnormal sphincter EMG. Compliance was normal, but bladder capacity was decreased in progressive cases. Impaired contraction and hyposensitivity made it more difficult to control the urinary incontinence in supranuclear palsy than in Parkinson's disease. Dementia and lower ADL were frequently accompanied with supranuclear palsy (rare in Parkinson's disease). These were other factors that worsen the control of incontinence.

Female↗
PubMed1992

[Progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of a case and review of the literature].

Progressive supranuclear palsy (PSP) was first recognized as a distinct syndrome by Richardson, Steele and Olszewski roughly a quarter century ago. Subsequent clinical experience has corroborated and enlarged their original observations. PSP has become familiar as a chronic progressive disorder with extrapyramidal rigity, bradykinesia, gait impairment, bulbar palsy, dementia and a characteristic supranuclear ophthalmoplegia. It is a significant cause of parkinsonism and its etiology remains obscure. The case of a patient from Santa Catarina who presented definite clinical evidences of this syndrome is reported. This is the first description in this southern Brazilian State, where at least 50 more patients should exist, if we may extrapolate the prevalence rate of this condition in developed countries to this well developed area of Brazil. A review of the literature was undertaken with emphasis on recent clinical and therapeutic aspects of PSP.

Aged↗
PubMed2000

Computerized posturography analysis of progressive supranuclear palsy: a case-control comparison with Parkinson's disease and healthy controls.

BACKGROUND: Progressive supranuclear palsy (PSP) is a neurodegenerative disorder that is frequently mistaken for Parkinson's disease (PD) in its early stages. OBJECTIVE: To compare balance measures using computerized posturography in patients with early PSP and early PD. METHODS: We performed computerized posturography (SMART Balance Master; NeuroCom International, Inc, Clackamas, Ore) in 20 patients with clinically diagnosed mild to moderate PSP (ambulatory) and compared results with those from 20 patients with PD of similar age and disease duration who were not receiving medications, and from 20 healthy age- and sex-matched controls. Sensory organization testing (SOT), limits of stability (LOS), and toes-up perturbations (4 degrees at 50 degrees per second) were tested while receiving and not receiving a combination of oral carbidopa (25 mg) and levodopa (250 mg) in the PSP group. Clinical assessment included Unified Parkinson's Disease Rating Scale, Performance-oriented assessments, and functional reach. RESULTS: When compared with the PD and control groups, total LOS time (P < .001) and path sway (P < .001) were significantly prolonged in PSP. Total SOT showed significantly worse scores in PSP compared with PD and control groups (F(2,57) = 29.6; P < .001). Univariate follow-up tests comparing PSP and PD showed differences in the following conditions: eyes open and visual sway (P = .003), eyes open and platform sway (P = .003), eyes closed and platform sway (P < .001), and eyes open and platform and visual sway (P < .001). Medium- and long-latency responses to perturbation were similar, but a larger number in the PSP group lacked short-latency responses (chi(2) = 11.3; P = .002). Levodopa administration did not significantly improve any aspect of posturography testing in PSP. In differentiating PSP from PD, LOS time and SOT condition of eyes open and platform and visual sway were nearly 100% sensitive and 100% specific (canonical correlation, 0.91). CONCLUSIONS: Computerized posturography testing reliably differentiated early PSP from early PD and age-matched controls. The PSP group demonstrated severely contracted limits of stability with probable deficits in motor programming. Results of SOT in PSP suggested a vestibular pattern and overreliance on visual cues, even when incorrect. The absence of short-latency responses (monosynaptic reflex arch) suggests an additional disturbance in the spinal cord or peripheral nervous system. Arch Neurol. 2000;57:1464-1469

Aged↗
PubMed1989

Physostigmine treatment of progressive supranuclear palsy.

Cognitive and extrapyramidal effects of cholinomimetic therapy were evaluated in 8 patients with progressive supranuclear palsy. Each was randomized to a 10-day double-blind crossover trial of physostigmine and placebo. Physostigmine treatment was associated with marginal and inconsistent changes in long-term memory, suggesting that cholinergic therapy alone is insufficient to restore cognitive function. Motor scores remained unchanged.

Aged↗
PubMed2002

Tau accumulation in astrocytes in progressive supranuclear palsy is a degenerative rather than a reactive process.

Tau-immunoreactive astrocytes in progressive supranuclear palsy (PSP) have a distinctive morphology and are referred to as tufted astrocytes (TA). We hypothesized that TA may be a degenerative change in reactive astrocytes. To test this hypothesis we examined the relationship of TA to gliosis in PSP. We first examined the distribution of gliosis [glial fibrillary acid protein (GFAP)-positive astrocytes], TA, neurofibrillary tangles (NFT) and pretangles in brain sections of neuropathologically pure PSP cases. Second, we examined PSP cases complicated by infarcts or Alzheimer's disease, since these cases would have reactive astrocytes associated with lesions. We used double immunostaining for GFAP and tau for cases with vascular lesions, and triple immunostaining for GFAP, tau and beta-amyloid protein for sections with senile plaques. There was no correlation between the distribution of gliosis and TA, with gliosis prominent in globus pallidus and subthalamic nucleus, and TA prominent in motor cortex and striatum. On the other hand, gliosis paralleled the distribution of NFT, but not the distribution of pretangles, suggesting that NFT contributes to gliosis in PSP. Although reactive astrocytes were present around infarcts and senile plaques, TA were not associated with these lesions. Tau accumulation in astrocytes in PSP was not preferential to (and was actually independent of) reactive astrocytes. This is consistent with the notion that tau accumulation in astrocytes is a degenerative rather than reactive process. Unlike NFT, astrocytic degeneration does not seem to contribute to gliosis or neuronal loss in PSP, and its clinical significance remains unclear.

Aged↗
PubMed2002

Frequency, distribution and characteristics of progressive supranuclear palsy in Italy: preliminary observations.

We report the number of pathologically proven cases of progressive supranuclear palsy, described in the Italian neurological literature from 1961 until now. A discussion of the diagnostic value of downward gaze palsy is made. A comparison with the number of similar cases described in the rest of the world and with the number of Parkinsonian patients who died in the same region in the corresponding year is attempted.

Humans↗
PubMed2006

No evidence of CRHR1 gene involvement in progressive supranuclear palsy.

Several genes have been located in the chromosomal region 17q21 genetically associated with progressive supranuclear palsy (PSP). Corticotropin releasing hormone receptor 1 (CRHR1) is a gene included in this region. In order to investigate the possible involvement of CRHR1 in PSP pathogenesis, we measured the globus pallidus mRNA expression of this gene using real-time PCR in 12 PSP comparing with several control groups composed by 10 Alzheimer's disease, 5 cerebrovascular disease and 6 healthy controls subjects. We furthermore sequenced directly the entire coding region of CRHR1 of two histopathologically confirmed PSP patients. Expression pattern of CRHR1 in globus pallidus was similar in all groups. We did not find any coding non-synonymous mutation in the patients analysed. Our results do not support an involvement of CRHR1 gene in PSP pathogenesis.

Age of Onset↗
PubMed1999

Progressive supranuclear palsy.

Richardson observed an unusual clinical syndrome in the 1950s, which he later designated progressive supranuclear palsy (PSP). Over the past 25 years, although knowledge of this disorder has gradually improved, its cause is still unknown, pathogenesis is unclear, and there is still no definitive treatment for this disorder. This article reviews the epidemiology, clinical features, diagnostic criteria, neuropathology, neuroimaging, and treatment of PSP.

Brain↗
PubMed1999

Prevalence of progressive supranuclear palsy and multiple system atrophy: a cross-sectional study.

BACKGROUND: The prevalence of progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) in the general population is unknown. Clinicopathological studies of patients diagnosed as having Parkinson's disease suggest that the two disorders may be underdiagnosed. We investigated the population prevalence of these disorders. METHODS: We screened computerised records of 15 general practices in London, UK, for patients with specific diagnostic labels suggestive of Parkinson's disease or parkinsonism and all patients who had ever received antiparkinsonian medication. We assessed eligible patients by review of records, interview, physical examination, and video recordings of neurological signs for independent diagnostic confirmation. We diagnosed parkinsonian disorders according to published criteria and further reviewed patients with atypical features after 1 year. FINDINGS: The participation rate was 84%. The age-adjusted prevalence for PSP was 6.4 per 100,000 (five probable and one possible case [95% CI 2.3-10.6]) and for MSA 4.4 per 100,000 (two probable and two possible cases [1.2-7.6]). An additional four atypical patients were identified at 1 year but did not fulfil criteria for PSP or MSA. INTERPRETATION: Our results suggest that the true prevalence of PSP and MSA has been underestimated, since many patients in the community remain undiagnosed or misdiagnosed.

Aged↗
PubMed2001

Abnormal signals on proton density-weighted MRI of the superior cerebellar peduncle in progressive supranuclear palsy.

OBJECTIVE: To assess MRI signal abnormalities of the superior cerebellar peduncle (SCP) in progressive supranuclear palsy (PSP) patients. MATERIAL AND METHODS: Signal changes were examined on proton density-weighted images (PDWI) and on T2-weighted images (T2WI) of SCP in 9 PSP patients, and findings were compared to those in 20 Parkinson's disease patients and 20 age-matched control subjects. RESULTS: We observed effacement or lack of clarity of the low signal on PDWI in SCP in 4 of 9 PSP patients, but not in any of the Parkinson's disease patients or control subjects. These signal changes were not observed on T2WI. CONCLUSIONS: The signal changes on PDWI may be a specific finding reflecting demyelination and gliosis of SCP in PSP. Our findings suggest that evaluation of SCP on PDWI may be helpful in the diagnosis of PSP patients.

Aged↗
PubMed1992

Immunohistochemical, ultrastructural and immunoelectron microscopic studies of spinal cord neurofibrillary tangles in progressive supranuclear palsy.

Immunohistochemical, ultrastructural and immunoelectron microscopic studies of spinal cord neurofibrillary tangles (NFTs) in progressive supranuclear palsy (PSP) were performed. The spinal cord NFTs reacted with antibodies to tau protein (tau-2), ubiquitin and Alzheimer neurofibrillary tangles (ANTs, Ab 39). Ultrastructurally, the NFTs consisted of bundles of straight fibrils. In longitudinal sections, the individual NFT fibrils appeared as straight fibrils with a diameter of approximately 15 nm. In cross sections, circular structures approximately 15 nm in diameter were seen, and some had a central density. Electron microscopic examination of specimens stained with the antibodies and by the modified Bielschowsky method revealed the products of the tau, ubiquitin and ANTs immunoreactions and silver deposits on the NFT fibrils. This is the first demonstration of the ultrastructure of spinal cord NFTs in PSP.

Adult↗
PubMed2004

Sequence analysis of tau 3'untranslated region and saitohin gene in sporadic progressive supranuclear palsy.

BACKGROUND: The extended tau H1 haplotype has previously been described in association with progressive supranuclear palsy (PSP). Recently, a new gene called saitohin (STH), nested within an intron of tau, has been discovered. The Q7R polymorphism of STH appears to be related to late onset Alzheimer's disease. OBJECTIVES: To search for genetic changes in the 3'untranslated region (3'UTR) of tau and adjacent sequence LOC147077, and in the coding region of STH in PSP patients. METHODS: The study included 57 PSP patients and 83 healthy controls. The genetic analysis of each region was performed through sequencing. The Q7R polymorphism was studied through restriction enzyme and electrophoresis analysis. RESULTS: No mutations were found in the regions analysed. The QQ genotype of the STH polymorphism was over-represented in participants with PSP (91.5%) compared with control subjects (47%) (p< or =0.00001). This genotype co-segregated with the H1/H1 haplotype in our PSP cases. CONCLUSIONS: Our results do not support a major role for the tau 3'UTR in PSP genetics. The QQ genotype of STH confers susceptibility for PSP and is in linkage disequilibrium with the H1/H1 haplotype.

3' Untranslated Regions↗