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Results for “Supranuclear Palsy, Progressive”

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PubMed2004

Update on progressive supranuclear palsy.

A better understanding of the challenges in the diagnosis of progressive supranuclear palsy (PSP), the most common atypical parkinsonian neurodegenerative disorder, has led to improving its diagnostic accuracy. Similarly, a better understanding of the role of tau mutations, mitochondrial complex I inhibitors, oxidative injury, and inflammation in neurodegeneration has led to an unraveling of potential risk factors for the development of PSP. These advances are explained in detail in this review.

Animals↗

PubMed1988

Orienting of visual attention in progressive supranuclear palsy.

Orienting of visual attention was studied in 8 patients with progressive supranuclear palsy (PSP) and 8 parkinsonian control subjects. While maintaining fixation on the centre of a visual display, subjects made simple reaction time (RT) key press responses on detecting visual targets which appeared above, below, to the left or right, equidistant from fixation. On each trial the target was preceded by a preparatory cue, either a peripheral luminance change or a central arrow, to summon attention to one of the four locations. The orienting of attention was measured as a facilitation in detection RT at the cued location. For the parkinsonian controls, this facilitation was equal for horizontal and vertical directions, whereas for both types of cues, PSP patients were slower moving attention in the vertical than in the horizontal plane. Midbrain retinotectal pathways are important not only for controlling eye movements, but also for orienting attention.

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PubMed1989

Brain iron in progressive supranuclear palsy: clinical, magnetic resonance imaging, and neuropathological findings.

A patient with progressive supranuclear palsy demonstrated a region-specific decrease in T2 signal during high-field-strength brain magnetic resonance imaging. At autopsy the T2-signal hypointensity was found to correspond topographically to increased deposition of ferric iron. The potential clinical, radiologic, and pathophysiologic implications of these findings are discussed.

Brain↗

PubMed1987

Progressive supranuclear palsy and a multi-infarct state.

In 58 patients with progressive supranuclear palsy (PSP), 19 (32.8%) had CT, MRI, or autopsy evidence of a multi-infarct (MI) state. The clinical findings in the infarct syndrome were similar to idiopathic PSP. Five MI-PSP patients had had a stroke, four had focal dystonia, two had hemiparesis, and one had an intention tremor of recent onset. In contrast, only 5.9% (12.9% of those with CT or MRI) of 426 Parkinson's disease patients had evidence of strokes. One case of PSP studied pathologically was attributed to cerebral amyloid angiopathy.

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PubMed1987

Reductions in corticotropin releasing factor-like immunoreactivity in cerebral cortex in Alzheimer's disease, Parkinson's disease, and progressive supranuclear palsy.

Dementias occurring in Alzheimer's disease, Parkinson's disease, and progressive supranuclear palsy are associated with dysfunction and death of neurons in a variety of cell populations, including cholinergic, monoaminergic, and peptidergic systems. In the present investigation of these three disorders, we demonstrated decreased levels of corticotropin releasing factor (CRF)-like immunoreactivity in the frontal, temporal, and occipital poles of the neocortex. Moreover, reductions in peptidergic immunoreactivity correlated with reductions in the activity of choline acetyltransferase, the enzyme that catalyzes the formation of acetylcholine. The reduction in cortical CRF levels may be due to abnormalities of intrinsic cortical neurons or to dysfunction in neurons that contain CRF and innervate cortex.

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PubMed1994

[Widespread argentophilic structures in progressive supranuclear palsy--an autopsy case report].

We report an autopsy case of progressive supranuclear palsy (PSP) with a five-year clinical course. A 67-year-old man was suffering from a gait disturbance and mental deterioration. Neurological examination at the age of 71 revealed pseudobulbar palsy, horizontal ophthalmoplegia, and truncal dystonia, and a diagnosis of PSP was made. Mental deterioration including forgetfulness and character change was also noted, and the patient sometimes exhibited intermittent stuporous states. Cranial computed tomography and magnetic resonance images revealed moderate brain atrophy, predominantly in the frontal lobes. The patient died of bronchopneumonia at the age of 71. Neuropathological examination confirmed typical pathological changes of PSP, such as neuronal loss, neurofibrillary tangles, and fibrillary gliosis in the subcortical nuclei. Gallyas-Braak silver impregnation revealed neurofibrillary tangles, silver-positive glia and thread-like structures in degenerating subcortical nuclei. In addition to these classical lesions, the argentophilic structures were detected in the cerebral cortex, cortical white matter and cerebellar white matter. In the cerebral cortex, they were abundant mostly in the precentral gyrus and subcortical white matter. Immunohistochemical studies revealed that most silver-positive structures were also tau 2 antibody-positive. Thus, these argentophilic structures seemed to be closely related to abnormal tau protein. Their distribution in this case implies that lesions related to abnormal tau protein may occur more extensively in the brains of PSP than expected.

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PubMed1993

[Vesicourethral function study and application of urinary alarm in progressive supranuclear palsy].

We performed a vesicourethral function study on seven patients with progressive supranuclear palsy. In storage phase, 6 patients had decreased urinary sensation and overactive detrusor. Although bladder compliance was normal in all patients, maximum cystometric capacity was decreased in 3 patients. In micturition phase, detrusor contraction was underactive in 4 patients and acontractile in 1 patient. Sphincter electromyogram showed detrusor-sphincter-dyssynergia in 1 patient, no decrease in 3 patients and synergistic decrease in 1 patient. Six patients had urinary incontinence partially due to those neurological abnormality, partially due to dementia and lower activity of daily living. To facilitate the care of such functional incontinence, we devised a urinary alarm. The urinary alarm is a device to detect urine in a diaper. One can know the micturition in a diaper without being informed of micturition by the patient and change diapers as soon as possible. It was also useful to examine their frequency/volume chart.

Activities of Daily Living↗

PubMed1997

[Progressive supranuclear palsy with macular degeneration--report of three cases].

Three cases of progressive supranuclear palsy (PSP) with decreased visual acuity were reported. Case 1 was a 50-year-old man, case 2 a 60-year old woman, and case 3 a 66-year-old woman. They all had vertical gaze palsy and unexplained falls, and were diagnosed as PSP by NINDS-SPSP clinical criteria. All cases had symptoms of difficulty in vision; therefore, detailed ophthalmological tests were performed. Ophthalmoscopic findings of macula revealed atrophy in case 1 and 2, and brownish discoloration in case 3. Fluorescein fundus angiography done in case 1 showed abnormal staining at the macula. Multifocal electroretinography performed in case 3 showed absence of big response in the macula. These results indicated macular degeneration. None of three cases had cataract. And visual evoked potentials of case 2 and 3 showed prolonged P100 latency. From these results, we suspected that decreased visual acuity of our cases were due to macular degeneration and difficulty to catch the object at the fovea because of gaze palsy. There had been no reports of macular degeneration with PSP, but we might have overlooked these findings, because of dementia, gaze palsy, and apraxia of lid opening. The possibility of macular degeneration is raised as a part of neurological signs is PSP.

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PubMed1997

Progressive supranuclear palsy and corticobasal degeneration.

The clinical and neuropathological features characteristic of progressive supranuclear palsy and corticobasal degeneration are described in detail. These disorders are not as rare as previously believed, but are poorly recognized. In patients with parkinsonism, a high index of clinical suspicion, sometimes complemented by specific laboratory tests, should improve diagnostic accuracy. Biological treatments are at present not available, but suggested symptomatic therapies may improve the quality of life of patients with these disorders.

Basal Ganglia↗

PubMed1989

[Ocular motor abnormalities in progressive supranuclear palsy].

Eleven patients, 7 males and 4 females, of progressive supranuclear palsy (PSP) were examined neuro-otologically for the purpose of elucidating the characteristics of ocular motor abnormalities. All cases were admitted to our hospital and age at onset was from 52 to 71 years old, duration of illness was 2 to 11 years. Range of voluntary eye movements and abnormal eye movements including nystagmus were examined on naked eyes and with electronystagmography (ENG). Smooth pursuit movements and saccadic eye movements were tested both horizontally and vertically by using visual tracking method with ENG recordings. Optokinetic nystagmus test and caloric test with visual suppression test were also performed. These neurotological examinations were made repetitively in 5 cases and their progressions were observed. Vertical gaze palsy and convergence palsy were observed in all cases as the initial symptom. In this study downward gaze was more severely disturbed than upward gaze. Using ENG, saccadic eye movements (saccades) were disturbed earlier than smooth pursuit movements. Hypometric saccades and decreased saccadic velocity were common abnormalities. In the later stage of the disease, horizontal eye movements were also disturbed. In four cases bilateral adduction palsy was added to vertical gaze paralysis so that the lesion of the MLF to oculomotor nucleus was suggested to exist. These voluntary eye movements were worsened gradually as the disease progressed. By using ENG we could find so called abnormal eye movements more frequently than the previous reports. Eight patients demonstrated horizontal gaze nystagmus, and rebound nystagmus were observed in four cases.(ABSTRACT TRUNCATED AT 250 WORDS)

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PubMed1989

Occurrence of 15-nm-wide straight tubules in neocortical neurons in progressive supranuclear palsy.

Ultrastructural investigations were carried out on the cerebral neocortex in two cases of progressive supranuclear palsy. In both cases, characteristic 15-nm-wide straight tubules were observed in the neurons. The numbers of cells containing the straight tubules and of tubules in individual cells were small. However, the occurrence of the tubules strongly suggests that the cerebral neocortex is also exposed to the disease process in progressive supranuclear palsy.

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PubMed1998

Progressive supranuclear palsy: a survey of the disease course.

BACKGROUND: The most accurate knowledge about progressive supranuclear palsy (PSP) comes from small sample studies that preclude precise estimation of the proportion of PSP patients affected with various symptoms and the examination of factors predicting survival time. OBJECTIVE: To describe the course of PSP in a large clinically diagnosed sample of PSP patients and to identify factors predicting survival time. METHODS: We surveyed the caregivers of 318 living and 119 deceased patients with progressive supranuclear palsy. The main outcome measures were a principal symptom severity questionnaire and a signs and symptoms questionnaire. RESULTS: The estimated age of PSP symptom onset depends critically on how symptom onset is defined, with estimates differing by as much as 1.5 years. Men and women were represented equally (51.6% versus 48.4%) in the living sample, but men formed 61.8% of the deceased sample. Men were diagnosed later than women following symptom onset (33.4 versus 24.1 months) and died earlier following the diagnosis (37.0 versus 47.6 months). Motor and visual symptoms appeared first, followed by emotional and personality problems, cognitive impairment, and sleep changes. Whereas motor symptoms eventually affected almost every patient, emotional/personality and cognitive symptoms did not. The early onset, presence of falls, slowness, and inability to move eyes downward early in the development of the disease predicted survival time. CONCLUSION: PSP is a rapidly progressive disease dominated by motor symptoms, and it affects men more frequently than women.

Affective Symptoms↗

PubMed2002

[Acquired stuttering as an early symptom in a patient with progressive supranuclear palsy].

We report a 57-year-old man with progressive supranuclear palsy (PSP) showing acquired stuttering (AS) as an early symptom. He had noticed micrographia at age 48, and thereafter he began to suffer from progressive speech disturbance at age 49. Neurological examination at age 57 revealed prominent acquired stuttering, supranuclear vertical gaze palsy, axial rigidity, disturbance of postural reflex, motor perseveration, and catalepsy, but no aphasia. A brain MRI study demonstrated atrophy of the midbrain tegmentum and dilatation of the third ventricle with a few lacunar infarcts in the basal ganglia. Anti-parkinsonian drugs were ineffective. We diagnosed his illness as PSP. His speech was characterized by repetition of sounds and syllables. It was more dominant at repetition of sentences than at that of words, and was improved with rhythmic stimulation. Although pathophysiology underlying AS remains unknown, the AS in this patient seems to be closely related to his motor perseveration and catalepsy. These symptoms may be caused by the disturbance of dopaminergic system from midbrain to limbic system and frontal cortex as reported in PSP. In conclusion, As can be an early and prominent symptom which may be related to characteristic impairment of the dopaminergic system.

Catalepsy↗

PubMed1988

Selective decrease of large neurons in the neostriatum in progressive supranuclear palsy.

In order to evaluate the quantitative changes in the neostriatum in progressive supranuclear palsy (PSP), sections of the caudate head (CN) and putamen (PT) from 4 PSP patients were stained with Klüver-Barrera, and the cell body and nuclear area of the neurons were measured by a digitizer. Obtained results were compared to those of 6 age-matched control and 4 Alzheimer's disease and senile dementia of Alzheimer type (AD/SDAT) subjects which were previously reported. The number of large neurons (nuclear area greater than 101 microns 2) in PSP was about 40% (P less than 0.01) and 30% (P less than 0.01) of that of the controls in CN and PT, respectively. In contrast, the number of small neurons in PSP (nuclear area less than 100 microns 2) was well preserved. The values were quite similar to those in AD/SDAT. The implications and possible significance are discussed.

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PubMed2004

Failure in heat-shock protein expression in response to UBB+1 protein in progressive supranuclear palsy in humans.

UBB+1 protein is an aberrant ubiquitin associated with progressive supranuclear palsy (PSP). It leads to proteasome inhibition, heat-shock protein (HSP) expression and apoptosis in cell cultures. Despite UBB+1 polyubiquitination (an indication of proteasome inhibition), we demonstrate that UBB+1 and HSP40/HSP70 immunoreactivity do not co-localize in the pons of patients with PSP. As HSPs are involved in both normal tau and proteasome function, these findings may be relevant to the aetiology of PSP and other tauopathies.

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PubMed2005

Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson's syndrome and PSP-parkinsonism.

The clinical diagnosis of progressive supranuclear palsy (PSP) relies on the identification of characteristic signs and symptoms. A proportion of pathologically diagnosed cases do not develop these classic features, prove difficult to diagnose during life and are considered as atypical PSP. The aim of this study was to examine the apparent clinical dichotomy between typical and atypical PSP, and to compare the biochemical and genetic characteristics of these groups. In 103 consecutive cases of pathologically confirmed PSP, we have identified two clinical phenotypes by factor analysis which we have named Richardson's syndrome (RS) and PSP-parkinsonism (PSP-P). Cases of RS syndrome made up 54% of all cases, and were characterized by the early onset of postural instability and falls, supranuclear vertical gaze palsy and cognitive dysfunction. A second group of 33 (32%) were characterized by asymmetric onset, tremor, a moderate initial therapeutic response to levodopa and were frequently confused with Parkinson's disease (PSP-P). Fourteen cases (14%) could not be separated according to these criteria. In RS, two-thirds of cases were men, whereas the sex distribution in PSP-P was even. Disease duration in RS was significantly shorter (5.9 versus 9.1 years, P < 0.001) and age at death earlier (72.1 versus 75.5 years, P = 0.01) than in PSP-P. The isoform composition of insoluble tangle-tau isolated from the basal pons also differed significantly. In RS, the mean four-repeat:three-repeat tau ratio was 2.84 and in PSP-P it was 1.63 (P < 0.003). The effect of the H1,H1 PSP susceptibility genotype appeared stronger in RS than in PSP-P (odds ratio 13.2 versus 4.5). The difference in genotype frequencies between the clinical subgroups was not significant. There were no differences in apolipoprotein E genotypes. The classic clinical description of PSP, which includes supranuclear gaze palsy, early falls and dementia, does not adequately describe one-third of cases in this series of pathologically confirmed cases. We propose that PSP-P represents a second discrete clinical phenotype that needs to be clinically distinguished from classical PSP (RS). The different tau isoform deposition in the basal pons suggests that this may ultimately prove to be a discrete nosological entity.

Age of Onset↗

PubMed2005

Population based mortality and quality of death certification in progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome).

OBJECTIVE: To estimate the mortality of progressive supranuclear palsy (PSP) and to assess the quality of death certification in patients with PSP who died in England and Wales. METHODS: An analysis was conducted of ICD-9 (International Classification of Diseases, version 9) coded deaths obtained through the Office of National Statistics over an eight year period. RESULTS: The crude annual mortality rate was 1.77 (95% confidence interval, 1.64 to 1.90) cases per million, using the mid-1996 population estimate for England and Wales. Annual mortality increased over time, possibly as a result of increased incidence or increased awareness of the disorder. Forty nine death certificates from deceased patients previously diagnosed clinically showed that the commonest proximate cause of death was pneumonia, occurring in 45% of cases (22/49). The underlying cause of death was cited as pneumonia in 14% of cases (7/49). PSP was mentioned in only 65% of death certificates (32/49). Eight of the 49 cases (16%) underwent necropsy and results were available for five of these cases. PSP was confirmed pathologically in four; the remaining case was diagnosed as Parkinson's disease. CONCLUSIONS: Further research is needed to establish the reasons for the observed increase in mortality. Determining the population mortality rate for PSP using the ICD-9 coding system is problematic but is likely to improve following the introduction of ICD-10 updated codes and coding rules.

Autopsy↗

PubMed2005

Diffusion tensor imaging and voxel based morphometry study in early progressive supranuclear palsy.

BACKGROUND: A comprehensive characterisation of grey and white matter changes in progressive supranuclear palsy (PSP), the second most common extrapyramidal syndrome after Parkinson disease, is still not available. OBJECTIVE: To evaluate grey and white matter changes in mild PSP patients by voxel based morphometry (VBM) and diffusion tensor imaging (DTI), respectively. METHODS: 14 mild PSP patients and 14 healthy controls entered the study and underwent a clinical and neuropsychological evaluation according with a standardised assessment. Each subject had a structural magnetic resonance imaging (MRI) study. Processing analysis of MRI data was carried out according to optimised VBM and fractional anisotropy was determined. RESULTS: Compared with the controls, in PSP patients VBM analysis showed a significant clusters of reduced grey matter in premotor cortex, frontal operculum, anterior insula, hippocampus, and parahippocampal gyrus, bilaterally. With regard to subcortical brain regions, the pulvinar, dorsomedial and anterior nuclei of the thalamus, and superior and inferior culliculum were affected bilaterally. A bilateral decrease in fractional anisotropy in superior longitudinal fasciculus, anterior part of corpus callosum, arcuate fascicolus, posterior thalamic radiations, and internal capsule, probably involving the cortico-bulbar tracts, was present in PSP patients. CONCLUSIONS: These data provide evidence for both grey and white matter degeneration in PSP from the early disease stage. These structural changes suggest that atrophy of cortical and subcortical structures and neurodegeneration of specific fibre tracts contribute to neurological deficits in PSP.

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