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Results for “Supranuclear Palsy, Progressive”

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PubMed1987

Computed tomographic findings in progressive supranuclear palsy.

CT findings of 6 patients with progressive supranuclear palsy (PSP) are described, with emphasis on their supratentorial changes in comparison with those of control subjects and patients with Parkinson's disease (PD). As estimated from CT films, the lateral ventricles, third ventricle and prepontine cistern were significantly enlarged in PSP patients compared with both controls and PD patients. It is suggested that the patients with PSP have not only infratentorial but also supratentorial lesions.

Aged↗

PubMed2002

Progressive supranuclear palsy: where are we now?

This review provides an update on progressive supranuclear palsy (PSP, or Steele-Richardson-Olszewski disease), an adult-onset neurodegenerative disorder characterised by early postural instability, which leads to falls, and a vertical supranuclear-gaze palsy. Recent epidemiological studies have shown that the disorder is more common than previously recognised, that it is commonly misdiagnosed, and that it may present to a wide range of hospital specialists. The diagnosis of PSP hinges on clinical acumen. Attempts to identify a suitable biomarker in the CSF or a specific and sensitive imaging or neurophysiological technique have so far failed to have a significant effect on the diagnostic process. Better understanding of the molecular pathology of PSP has highlighted the importance of tau-protein accumulation and tau-genotype susceptibility in its pathogenesis. No drug treatment significantly and consistently benefits patients, and novel therapies are urgently required.

Brain↗

PubMed1999

Progressive supranuclear palsy with dementia: cortical pathology.

Patients with progressive supranuclear palsy (PSP) often develop dementia, and cortical pathology has been documented in PSP. However, there are no reports correlating dementia in PSP with cortical pathology. We hypothesized that cases of PSP presenting with cognitive impairment would have more severe cortical tau pathology than those without. We compared 7 cases of PSP presenting with cognitive deficits (group 1) with 4 cases of PSP that did not (group 2). The subcortical tau pathology was almost identical in both groups. The cortical tau pathology was strikingly different in group 1, in which it was on average moderate, compared with group 2, in which it was minimal. The accumulation of cortical neuronoglial tau in PSP cases with dementia suggests that neurofibrillary pathology is central to the cause of dementia in PSP.

Aged↗

PubMed1988

Risk factors for progressive supranuclear palsy.

To explore possible risk factors for progressive supranuclear palsy (PSP), we conducted a case-control study of 50 cases in New Jersey. Two neurologists confirmed the diagnosis in the 41 living patients. Two hospital controls were matched by age, sex, race, date of death, and relationship of next-to-kin to each case. A structured interview was administered in person to the next-of-kin of cases and controls. Genetic, viral, toxic, medical, surgical, and personality factors were investigated. Cases lived in areas with low population as adults significantly more frequently than controls. The study identified no other factors associated with PSP including a history of stroke, hypertension, or smoking.

Adult↗

PubMed1992

[CSF monoamine metabolites in patients with progressive supranuclear palsy].

Neurochemical abnormalities in the patients with progressive supranuclear palsy (PSP) are not well understood. We investigated CSF levels of the monoamine metabolites HVA, 5HIAA and MHPG in 5 patients with PSP in order to investigate, especially dopaminergic, serotonergic and noradrenergic metabolism. Results were as follows (value; mena +/- S.D.). [table: see text] The levels of HVA were significantly lower than those in controls. These findings suggest that there is a hypofunction of dopaminergic neurons in PSP patients.

Aged↗

PubMed1989

[Treatment of progressive supranuclear palsy].

The neuropharmacological and neurochemical features of Progressive Supranuclear Palsy (PSP) are reviewed, together with the results obtained by various therapeutic trials. PSP is a neurodegenerative disease which often causes Parkinsonian symptoms but dopaminomimetic drugs have given rise to poor improvement, in spite of dopamine decrease observed in PSP patients' nigrostriatal region. The uselessness of L-DOPA therapy in PSP patients may explain the numerous failures encountered in PSP patients misdiagnosed as Parkinsonian. On the basis of the recent discovery of striatal dopaminergic receptor abnormalities and of interaction between various neurotransmitters, the authors suggest some possible therapeutic substances that might improve the outcome of the disease.

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PubMed2002

Progressive supranuclear palsy: clinical and genetic aspects.

PURPOSE OF THE REVIEW: This review focuses on the recent additions to the literature in the clinical and genetic aspects of progressive supranuclear palsy. RECENT FINDINGS: Clinical features of progressive supranuclear palsy are reasonably well established and known to be quite characteristic. Recent epidemiological studies suggest that the disorder is more common than previously considered and that it is frequently misdiagnosed. New laboratory and novel imaging techniques are being tested and cerebrospinal fluid levels of tau protein have been found helpful in diagnosis. Pathological and biochemical studies in progressive supranuclear palsy brains have shown the predominance of hyperphosphorylated tau isoforms which contain the sequence encoded by exon 10 (4R) aggregated into filaments. Familial tauopathies linked to tau gene mutations showing clinical and neuropathological overlap with sporadic progressive supranuclear palsy have been described. Despite recent discoveries of the strong genetic association of sporadic progressive supranuclear palsy with tau gene polymorphisms, a specific risk allele for developing the palsy has not yet been identified yet. SUMMARY: Recent clinical studies and clinicopathological correlations are contributing significantly to the delineation of the clinical features of progressive supranuclear palsy. These features and the appropriate use of laboratory tests allow for an earlier identification of the disease and a more accurate premortem diagnosis. However, no specific biological markers for the disorder are available yet, and consequently diagnosis in the early stages or when some of the characteristic signs and symptoms are missing, remains a major challenge. Despite the recent advances in the understanding of genetic factors involved in progressive supranuclear palsy, the cause of the disease still remains unknown. Biochemical studies in brains from progressive supranuclear palsy patients provide a potential helpful instrument to improve the characterization of this disorder.

Humans↗

PubMed2002

An R5L tau mutation in a subject with a progressive supranuclear palsy phenotype.

MAPT, the gene encoding tau, was screened for mutations in 96 progressive supranuclear palsy subjects. A point mutation (R5L) was identified in a single progressive supranuclear palsy subject that was not in the other progressive supranuclear palsy subjects or in 96 controls. Functionally, this mutation alters the ability of tau to promote microtubule assembly. Analysis of soluble tau from different brain regions indicates that the mutation does not affect the ratio of tau isoforms synthesized. Aggregated insoluble tau from subcortical regions was predominantly four-repeat tau with no or one amino terminal insert (0N4R and 1N4R). Insoluble tau from cortical regions also contained 1N3R tau. Thus, the R5L mutation causes a progressive supranuclear palsy phenotype, presumably by a gain-of-function mechanism.

Amino Acid Sequence↗

PubMed1992

[A case of progressive supranuclear palsy presenting mouth opening difficulty with tonic contraction of the orbicularis oris muscle].

A 72-year-old man developed supranuclear ophthalmoplegia, bradykinesia, rigidity, unsteady gait, dementia, dysphagia, retrocollis, grasp reflex and apraxia of eyelid opening. These findings were compatible with progressive supranuclear palsy (PSP). At the age of 66, he presented a peculiar phenomenon characterized by simultaneous tonic contraction of the orbicularis oris muscle (OOM) and the palatal muscles elicited by pronouncing "pa", which resulted in difficulty of voluntary opening of the mouth and the rhinopharynx. Therefore, the respiration air reciprocated between the lung and the closed mouth. The expiratory pressure puffed out the cheeks, while the lips remained tightly closed. While the respiratory movements and the pressure increased by degree, the OOM contracted more strongly in proportion to the pressure. Sixty to ninety seconds after the elicitation, the pressure overcame the contraction of the OOM and the course of the phenomenon was completed. The electromyograms showed that the OOM activity was prolonged after initial voluntary contraction, remaining thus after a tracheostomy for pneumonia at the age of 72, and that it increased in response to the pressure. Apraxia of eyelid opening, one of the other symptoms, resembled this phenomenon in terms of the aspect of difficulty of voluntary mouth opening. The "holding" phase of grasp reflex, yet another symptom, resembled it in the recruitment of the OOM activity. The phenomenon is not common in patients with PSP. However, we concluded that it may be included among the symptoms of PSP because it has similar characteristics to apraxia of eyelid opening and grasp reflex, which are not uncommon in patients with PSP.

Aged↗

PubMed1998

Pharmacological therapy in progressive supranuclear palsy.

BACKGROUND: To our knowledge, previous reports on drug treatment in progressive supranuclear palsy have not evaluated autopsy-confirmed cases. OBJECTIVE: To evaluate pharmacological treatment responses from detailed clinical records in patients with autopsy-confirmed progressive supranuclear palsy. SUBJECTS AND METHODS: We reviewed medical records for clinical presentation and pharmacological response in 12 patients with autopsy-confirmed progressive supranuclear palsy diagnosed using the National Institute of Neurological Disorders and Stroke pathologic criteria. For each drug class, exposure, global positive response, and specific positive response (parkinsonism, other movement disorders, or gaze dysfunction) were recorded. RESULTS: Drug classes examined were dopaminergics (all patients), tricyclics (3 patients), methysergide maleate (3 patients), 5-hydroxytryptophan (2 patients), and anticholinergics and selective serotonin inhibitors (1 patient). Positive clinical response was detected in 7 of the patients receiving dopaminergic drugs and in 1 patient each receiving tricyclics, methysergide, and 5-hydroxytryptophan, respectively. None of the patients responded markedly however, and there was no persistent beneficial effect. Use of dopaminergic drugs most frequently improved parkinsonian features, but disabling adverse effects included orthostatic hypotension (6 patients), hallucinations and delusions (3 patients), gastrointestinal complaints (3 patients), and dizziness (1 patient). Only 1 patient developed dyskinesia. CONCLUSION: Use of antiparkinsonian medications and other neurotransmitter replacement therapies was largely ineffective and caused frequent adverse effects in this series of patients with autopsy-confirmed with progressive supranuclear palsy.

5-Hydroxytryptophan↗

PubMed1984

Progressive supranuclear palsy and In111-DTPA cisternography.

Three cases of progressive supranuclear palsy are reported in which In111-DTPA cisternography showed slow diffusion, ventricular reflux and failure of isotope clearance. The clinical diagnosis of progressive supranuclear palsy was confirmed histologically in two of these patients. The possible causes of the cisternographic changes and their relationship to the changes of CSF dynamics in progressive supranuclear palsy are discussed.

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PubMed1997

Botulinum toxin treatment of apraxia of eyelid opening in progressive supranuclear palsy: report of two cases.

We report two patients, with postural instability and dystonic parkinsonism whose adjunctive disabling feature was blindness due to an inability to reopen the eyes after voluntary closure of the eyelids, as in apraxia of lid opening (ALO). Supranuclear downgaze paresis permitted the diagnosis of progressive supranuclear palsy (PSP) in one case. Electromyographic studies showed a loss of normal reciprocal inhibition between the levator palpebrae and the pretarsal portion of the orbicularis oculi, with a cocontraction of these two antagonist muscles. The evoked blink reflex, tested with the paired shock technique, showed enhanced recovery of R2 response. Botulinum toxin A injections directed toward the junction of the preseptal and pretarsal parts of the palpebral orbicularis oculi muscle improved eyelid motility in both patients. Successive static and dynamic balance training and development of compensatory strategies for visual scanning deficits reduced gait imbalance, the number of falls, and the disability level as measured on the Northwestern University Disability Scale.

Aged↗

PubMed1975

Internuclear ophthalmoplegia in progressive supranuclear palsy.

Internuclear ophthalmoplegia has not previously been described in progressive supranuclear palsy. The present report draws attention to the occurrence of varying degrees of anterior internuclear ophthalmoplegia in 4 out of 13 cases of this condition studied over a 4-yr period. This finding suggests that the medial longitudinal fasciculus may be involved in the degenerative process in some cases of progressive supranuclear palsy.

Aged↗

PubMed1998

Truncal muscle tonus in progressive supranuclear palsy.

OBJECTIVE: To elucidate the character and distribution of the abnormal muscle tonus in the body axis in progressive supranuclear palsy. Although neck hypertonus has been well described in progressive supranuclear palsy, little is known about the involvement of the truncal muscles. METHODS: Muscle tonus of the neck and trunk was separately investigated in 13 patients with progressive supranuclear palsy by clinical examination and surface EMG during passive rotation. Muscle hypertonus was graded according to a four point scale, and subjected to statistical analysis. The results were compared with those from 13 age matched patients with Parkinson's disease and six healthy volunteers. RESULTS: In all but one patient with progressive supranuclear palsy, there was a distinct difference in muscle tonus between the neck and trunk. A tonic shortening reaction characteristic of dystonia and an increased tonic stretch reflex (rigidity) were present in the neck muscles of patients with progressive supranuclear palsy, whereas only normal to moderately increased tonus was noted in the truncal muscles (neck v trunk, shortening reaction p=0.0001; stretch reflex p=0.0241). Follow up studies disclosed an increase in axial muscle tonus with predilection for the neck in three of four patients. In the 13 patients with Parkinson's disease, however, no significant difference was found in muscle rigidity between the neck and trunk. CONCLUSION: Mild changes in truncal muscle tonus with prominent neck dystonia and rigidity are characteristic of progressive supranuclear palsy. It is suggested that separate clinical evaluation of muscle tonus in the neck and trunk may be helpful for distinguishing progressive supranuclear palsy from Parkinson's disease.

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PubMed1989

Memory impairment in patients with progressive supranuclear palsy.

Verbal memory was compared in 12 patients with progressive supranuclear palsy and 12 healthy control subjects matched for age, sex, and education. Learning, consolidation, and retrieval were significantly impaired in patients with progressive supranuclear palsy. Information scanning, which requires the use of short-term memory processes, remained intact. Duration of symptoms and degree of motor dysfunction correlated with intrusions during learning. No relation between central dopamine metabolism and memory dysfunction could be established.

Aged↗

PubMed1991

Autonomic nervous function in progressive supranuclear palsy.

Autonomic nervous function was assessed in 11 patients with progressive supranuclear palsy, 26 patients with Parkinson's disease, matched for age, medications, disease severity, and disease duration, and 19 age-matched controls. Results of both parasympathetic (heart rate response to deep breathing and Valsalva maneuver) and sympathetic (blood pressure decrease on standing and increase on sustained handgrip) tests were abnormal in both patient groups. Abnormalities in the group of patients with progressive supranuclear palsy were similar to those in the group with Parkinson's disease but were more pronounced. Autonomic dysfunction may have to be considered a feature of progressive supranuclear palsy.

Aged↗

PubMed1990

[Progressive supranuclear palsy: clinical report on 7 cases and review of literature].

Progressive supranuclear palsy is sufficiently rare and difficult to diagnose that it escaped clinical recognition until 1964, when Steele, Richardson, and Olszewski clarified it as a pathologic entity. From January 1981 to June 1989, 7 patients who fulfilled the Golbe's criteria of progressive supranuclear palsy were admitted to the neurological department of National Taiwan University Hospital. The mean age at onset was 60 years (range: 53-69 years), and the mean age at diagnosis was 63 years), and the mean age at diagnosis was 63 years (range: 55-71 years). The study failed to identify any specific risk factors associated with progressive supranuclear palsy. Unsteady gait, vertical gaze palsy, pseudobulbar palsy, parkinsonian features and dementia were noted in all cases. The brain computed tomography revealed mild cortical atrophy and ventricular dilatation in 5 patients. Additionally, electroencephalography revealed diffuse theta waves in 4 patients and temporal theta waves in 2 patients, but these findings were nonspecific. Nystagmograms were performed in 3 patients, and hypometric saccades were noted in all of these patients, and uninhibited neurogenic bladder was proven by cystometry in 2 of these patients. All patients were treated with levodopa, but none of the patients showed any beneficial effects. Two patients died of aspiration pneumonia; the average duration from onset to death was about 4 years.

Aged↗

PubMed1997

The clinical and pathologic hallmarks of progressive supranuclear palsy.

This article reviews recent studies evaluating the clinicopathologic markers of progressive supranuclear palsy, which have helped establish standardized clinical and pathologic diagnostic criteria. Although these criteria increase diagnostic accuracy, the clinical and pathologic overlap between progressive supranuclear palsy and other disorders remains. Factors that may contribute toward managing progressive supranuclear palsy patients better are discussed and the etiopathogenicity of the disorder is hypothesized.

Biomarkers↗