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PubMed2003

Influence of target size on vertical gaze palsy in a pathologically proven case of progressive supranuclear palsy.

We document a new oculomotor phenomenon in a patient with pathologically proven progressive supranuclear gaze palsy (PSP), namely that vertical gaze excursion improves with larger pursuit targets. We used computerised video-oculography during vertical smooth pursuit eye movements (SPEM) of circular targets of diameter 0.16 degrees and 16 degrees, sinusoidally oscillating at 0.08 Hz (peak-to-peak amplitude 49 degrees). Increasing target size improved vertical gaze excursion from 10 degrees to 25 degrees. There was no concomitant increase in slow phase eye velocity. The findings could be explained by a potentiation of the position control mechanism of pursuit by target size due to increased activation of brainstem pursuit-optokinetic pathways and to higher order attentional mechanisms. This observation may be useful in the clinical assessment of PSP patients with severe neck rigidity in whom the doll's head-eye manoeuvre cannot be performed by comparing the degree of vertical gaze palsy during smooth pursuit testing between at least two differently sized targets and observing whether there is a larger excursion in response to a large target such as a newspaper.

Aged↗
PubMed2005

Treatment of progressive supranuclear palsy and corticobasal degeneration.

Success in treating patients with progressive supranuclear palsy and corticobasal degeneration remains exceedingly low. This finding probably relates to the widespread distribution of the pathological changes that account for the varied and complex spectrum of clinical manifestations. Dopaminergic drugs are regularly used for the parkinsonian features; however, these rarely result in more than modest benefit, and when better or sustained responses are obtained, as sometimes occurs in progressive supranuclear palsy, the clinical features are atypical and diagnosis is often delayed or not made in life. A variety of other treatments have been used in both disorders, sometimes directed at other specific features such as dystonia or myoclonus, and these treatments will be reviewed. Greater success in treating these disorders will require advances in our understanding of their cause(s) or the pathogenetic mechanisms underlying the neurodegenerative processes. The similarities in the molecular pathology of these four-repeat tauopathies suggests that important advances in the management of one will have a definite impact on the treatment of the other.

Amantadine↗
PubMed1985

[Frontal syndrome of progressive supranuclear palsy].

Neuropsychological findings in 10 clinical cases of progressive supranuclear palsy are presented. Patients were aged 52 to 80 and the duration of their illness was 1 to 5 years. Severe psychological disturbances e.g. depression or outbursts of irritability were prominent features in 5 patients. Formal neuropsychological testing disclosed mental slowing, impaired attention, reduced verbal fluency and elaborated linguistic abilities, poor abstract thinking and reasoning, mild to moderate memory loss, dynamic apraxia, grasping, motor impersistence, imitation and utilization behaviour. The clinical similarities between these neuropsychological changes and frontal lobe syndromes, together with the lack of cortical involvement, suggest that the "dementia" of progressive supranuclear palsy could be explained by deactivation of the frontal cortex by subcortical lesions. The special importance of pallidal and mesencephalic reticular involvement in the syndrome is hypothetized.

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PubMed1994

Initial letter and semantic category fluency in Alzheimer's disease, Huntington's disease, and progressive supranuclear palsy.

Ten patients with dementia of Alzheimer's type, 10 patients with progressive supranuclear palsy, and 10 patients with Huntington's disease were compared on two types of verbal fluency task--namely, initial letter fluency and category (semantic) fluency. The groups were carefully matched for overall level of dementia on the dementia rating scale, and were compared with 25 age matched normal controls. The controls found letter fluency more difficult than category fluency, and this relative pattern of performance was repeated in the progressive supranuclear palsy and Huntington's disease groups, although both groups were significantly impaired on both tasks. By contrast, patients with Alzheimer's disease performed just as poorly as the progressive supranuclear palsy and Huntington's disease groups on the category tasks, but were significantly less impaired at letter fluency, performing at near normal levels on this task. From these results, it is suggested that the performances of patients with progressive supranuclear palsy and Huntington's disease relate largely to initiation and retrieval problems secondary to disruption of frontostriatal circuits, whereas in Alzheimer's disease, the poorer performance on category fluency is due principally to the breakdown of semantic knowledge, which probably reflects temporal neocortical involvement.

Adult↗
PubMed1998

Significant changes in the tau A0 and A3 alleles in progressive supranuclear palsy and improved genotyping by silver detection.

BACKGROUND: Progressive supranuclear palsy (PSP) is characterized by intraneuronal inclusions of neurofibrillary tangles formed by aggregated tau protein. A significant association between the tau gene A0/A0 genotype and PSP recently has been reported. OBJECTIVES: To determine if a significant association between the tau gene A0/A0 genotype and PSP could be found in an independent population with a genetic background different from that in which the initial association was reported, and to standardize a nonradioactive method for tau gene genotyping. SETTING: Hospital and university research laboratories. SUBJECTS AND METHODS: To facilitate genotyping of the tau gene, we standardized the conditions for silver-based detection of the tau gene dinucleotide polymorphism. Thirty patients from Spain clinically diagnosed as having probable PSP were included in the study and compared with different control groups. RESULTS: A highly significant overrepresentation of the A0/A0 genotype (P<.001) and a decrease in the frequency of the A0/A3 genotype were found in the Spanish patients with PSP compared with the control group. A method based on silver detection was standardized for the genotyping of the tau gene. CONCLUSIONS: The detection of a significant association between the tau gene A0/A0 genotype and PSP in 2 independent populations rules out genetic stratification as an explanation for the association and indicates that the presence of the tau A0/A0 genotype is a risk factor for developing PSP independent of genetic background. Alternatively, the results could be interpreted as a protective effect of the A3 allele.

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PubMed2005

Direct comparison between regional cerebral metabolism in progressive supranuclear palsy and Parkinson's disease.

The differentiation between progressive supranuclear palsy (PSP) and Parkinson's disease (PD) may be difficult, especially in the early stages of disease. Positron emission tomography potentially provides a tool for making such a distinction. To identify key features in the spatial distributions of cerebral glucose metabolism, 18F-fluorodeoxyglucose (FDG) measurements of 10 patients with probable or possible PSP were directly compared with those of 9 PD patients. This analysis was done with statistic parametric mapping. After normalization of global brain uptake, in PSP, relative uptake of FDG was reduced in the caudal (motor) part of the anterior cingulate gyrus (Brodmann's area BA 24; P < 0.05, corrected for multiple comparisons). At a lower threshold, an additional decrease was present in the dorsal mesencephalon. In PD, relative hypometabolism was seen in extrastriate visual, ventrolateral temporal, posterior parietal, and orbitofrontal regions. Only reduction in the right fusiform gyrus and the lateral extrastriate visual cortex reached statistical significance. We concluded that particularly the reduction of medial frontal metabolism may be a valuable diagnostic imaging parameter in distinguishing PSP from PD. For PD, a possible association between occipitotemporal FDG decrease and vulnerability to hallucinations is suggested.

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PubMed1991

A case of progressive supranuclear palsy with widespread senile plaques.

A case of progressive supranuclear palsy (PSP) with frontal lobe atrophy is reported, in which many senile plaques were widely distributed in the neocortex, the entorhinal cortex, the amygdala, and, to a lesser extent, the cerebellar cortex, but not in the hippocampus. Most of the plaques were of the diffuse and primitive types. They were well visualized by beta-protein immunostaining, modified Bielschowsky staining and methenamine silver staining, but were not seen by Bodian staining. The widespread distribution of senile plaques in the cerebral and cerebellar cortices was far beyond that seen in normal aging, and was reminiscent of concomitant Alzheimer's disease (AD). Unlike AD, however, this case had neither senile changes in the hippocampus nor neurofibrillary tangles in the amygdala and entorhinal cortex. It seems that many senile plaques may appear widely in the cerebral cortex and even, to a lesser extent, in the cerebellar cortex of some patients with PSP. Additional case studies using sensitive silver and amyloid antibody preparations are required to elucidate the presence of senile plaques in the cerebral cortex of PSP.

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PubMed2006

Anesthetic management of a patient with progressive supranuclear palsy.

A 74-year-old woman with progressive supranuclear palsy (PSP) was scheduled for laryngotracheal separation surgery. Her neck showed severe backward tilt as a symptom of PSP. Magnetic resonance imaging (MRI) showed a significant airway stenosis due to the neck deformity. In the operating room, awake orotracheal intubation failed because of the neck deformity and airway stenosis. Therefore, tracheotomy was performed for airway management. General anesthesia was induced and maintained with sevoflurane (1.0%-2.5%) and fentanyl (total, 200 microg). Vecuronium (total, 5 mg) was used as a muscle relaxant. Monitoring of the train-of--four ratio in the ulnar nerve was impossible because of contracture of the fingers. Patients with PSP may have some serious associated deformities, and specific management, especially for the airway, may be necessary for general anesthesia.

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PubMed1993

Progressive supranuclear palsy: clinical presentation and rehabilitation of two patients.

Progressive supranuclear palsy (PSP), is an infrequent movement disorder characterized by supranuclear ophthalmoplegia, pseudobulbar palsy, and axial dystonia with frequent and unpredictable falls, usually backward. Median survival from time of diagnosis is two years. When diagnosis is reached, the patients are usually disabled and unable to ambulate independently. No specific rehabilitation protocol has been described for PSP. This article examines our rehabilitation experience with two PSP patients and our management of their frequent and unpredictable falls. The rehabilitation program consisted of a thorough physical evaluation; cognitive and speech evaluation and training; exercises to improve strength and coordination; and static and dynamic balance training. In order to improve safety during ambulation, we provided the patients with a heavy shopping cart or wheelchair that they could grab when in danger of losing their balance. The patients were able to achieve independence in ambulation.

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PubMed1990

Formic acid treatment exposes hidden neurofilament and tau epitopes in abnormal cytoskeletal filaments from patients with progressive supranuclear palsy and Alzheimer's disease.

In cases of progressive supranuclear palsy (PSP) and Alzheimer's disease (AD) the treatment of tissue sections with formic acid (FA) disclosed a neurofilament epitope in subcortical straight (SF) and paired helical (PHF) filaments. The same treatment produced a two-fold increase of tau-reactive neuropil threads and plaque-related neurites in AD cortical tissue. These findings indicate that epitopes of cytoskeletal proteins are hidden by the beta-pleated conformation of SF and PHF components. FA treatment should be used in immunohistochemical detection of intraneuronal abnormal cytoskeletal filaments.

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PubMed2005

Clinical deficits correlate with regional cerebral atrophy in progressive supranuclear palsy.

Most cerebral imaging studies of patients with progressive supranuclear palsy (PSP) have noted subtle atrophy, although the full extent of atrophy and any correlates to clinical features have not been determined. We used voxel-based morphometry analysis of grey matter, white matter and CSF on MRI brain scans to map the statistical probability of regional tissue atrophy in 21 patients with PSP, 17 patients with Parkinson's disease and 23 controls. PSP and Parkinson's disease cohorts were selected to approximate the mid-stages of their respective disease courses. Where regions of significant tissue atrophy were identified in a disease group relative to controls, the probability of tissue loss within those regions was correlated with global indices of motor disability, and behavioural and cognitive disturbance for that disease group. Minimal regional atrophy was observed in Parkinson's disease. PSP could be distinguished from both controls and Parkinson's disease by symmetrical tissue loss in the frontal cortex (maximal in the orbitofrontal and medial frontal cortices), subcortical nuclei (midbrain, caudate and thalamic) as well as periventricular white matter. For PSP, motor deficits correlated with atrophy of the caudate and motor cingulate, while behavioural changes related to atrophy in the orbitofrontal cortex and midbrain. These data suggest that intrinsic neurodegeneration of specific subcortical nuclei and frontal cortical subregions together contribute to motor and behavioural disturbances in PSP and differentiate this disorder from Parkinson's disease within 2-4 years of symptom onset.

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PubMed1996

Treatment of progressive supranuclear palsy with amitriptyline: therapeutic and toxic effects.

BACKGROUND: Progressive supranuclear palsy (PSP) is a parkinsonian-like disorder characterized by postural instability, rigidity, bradykinesia, supranuclear ocular palsy, dysarthria, dysphagia, and dementia. There is no satisfactory treatment. Two patients with advanced (PSP) reported here had clinically meaningful improvement in motor function on low dose amitriptyline (AMI) but developed cognitive and behavioral disturbances at higher doses. CASE REPORTS: During 11 weeks of upward dose titration, a 65-year-old man was partially relieved of severe bradykinesia, dysarthria, and dysphagia at an optimal dose of 40 mg bid (plasma AMI 39 ng/mL) such that he could feed himself, swallow easily, and transfer from wheelchair to toilet. Nocturnal confusion and urinary incontinence appeared at 70 mg bid, then resolved at 40 mg bid. Benefits were sustained during the next 14 months. A 77-year-old man had substantial relief of severe rigidity, bradykinesia, poor balance, and blepharospasm at 10 mg bid, such that he could feed himself and walk independently. At 40 mg bid (plasma AMI 62 ng/mL) 3 weeks later, he became aggressive, irritable, and increasingly confused. He reverted to his untreated state with drug withdrawal, then improved when AMI was reintroduced at 10 mg bid (plasma AMI 17 ng/mL). CONCLUSION: Low dose AMI may improve severe motor dysfunction in PSP significantly. Dosing must be individualized for optimal response and minimal toxicity.

Activities of Daily Living↗
PubMed1994

Preliminary NINDS neuropathologic criteria for Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy).

We present the preliminary neuropathologic criteria for progressive supranuclear palsy (PSP) as proposed at a workshop held at the National Institutes of Health, Bethesda, MD, April 24 and 25, 1993. The criteria distinguish typical, atypical, and combined PSP. A semiquantitative distribution of neurofibrillary tangles is the basis for the diagnosis of PSP. A high density of neurofibrillary tangles and neuropil threads in the basal ganglia and brain-stem is crucial for the diagnosis of typical PSP. Tau-positive astrocytes or their processes in areas of involvement help to confirm the diagnosis. Atypical cases of PSP are variants in which the severity or distribution of abnormalities deviates from the typical pattern. Criteria excluding the diagnosis of typical and atypical PSP are large or numerous infarcts, marked diffuse or focal atrophy, Lewy bodies, changes diagnostic of Alzheimer's disease, oligodendroglial argyrophilic inclusions, Pick bodies, diffuse spongiosis, and prion protein-positive amyloid plaques. The diagnosis of combined PSP is proposed when other neurologic disorders exist concomitantly with PSP.

Brain↗
PubMed2001

Randomized placebo-controlled trial of donepezil in patients with progressive supranuclear palsy.

OBJECTIVE: There is no effective treatment for progressive supranuclear palsy (PSP). Because results of immunochemical and pharmacologic studies suggest that the cholinergic system may play a role in the cognitive and motor features of PSP, the authors investigated the effects of donepezil (10 mg/day), an acetylcholinesterase inhibitor, in 21 patients with PSP (mean age +/- SD; 65.7 +/- 4.7 years) by a randomized, double-blind, placebo-controlled crossover trial. METHODS: Donepezil and placebo were administered for 6 weeks each with a 1-month washout period. Patients were evaluated before and at the end of each treatment phase. Outcome measures evaluated neuropsychiatric, global cognitive, frontal, memory, motor, and activities of daily living (ADL) status. RESULTS: Two patients withdrew during the washout phase because of unrelated medical problems. Donepezil-induced systemic side effects were transient and generally mild. Because of worsening of motor function, three patients received 5 mg/day of donepezil. All patients achieved blood and CSF therapeutic levels of donepezil. While the patients were taking donepezil, their Double Memory Test scores improved, whereas their ADL/mobility scores significantly worsened. CONCLUSION: The findings suggest that acetylcholinesterase inhibitors such as donepezil have at best selective, modest effects on cognition in patients with PSP. In light of its deleterious effects on ADL/mobility, donepezil is not recommended for this patient population.

Aged↗
PubMedDate not supplied

CT in progressive supranuclear palsy.

To determine the usefulness of CT in progressive supranuclear palsy (PSP), the CT scans of 87 patients with extrapyramidal disorders and/or dementia were reviewed. Of these, eight patients whose CT scans showed the findings characteristic of PSP were selected for study. These findings consisted of atrophy of the midbrain and quadrigeminal plate, with prominent interpeduncular, crural, ambient, and quadrigeminal plate cisterns, and dilatation of the aqueduct and posterior third ventricle. All eight patients were found to have PSP while none of the other patients had a clinical diagnosis of PSP. The importance of CT in the diagnosis of PSP, especially in the early phases of the disease, is emphasized.

Adult↗
PubMedDate not supplied

Progressive supranuclear palsy presenting with psychiatric features.

A patient with progressive supranuclear palsy who presented with psychiatric features is reported. His case illustrates the difficulty of early diagnosis of this condition. Associated psychiatric symptoms are common and may precede the occurrence of gaze palsy. Our patient's behavioural problems responded to fluoxetine.

Aged↗
PubMed1996

[Improvement of ophthalmoplegia by 5-hydroxytryptophan in two cases of progressive supranuclear palsy].

We report two patients with clinically diagnosed progressive supranuclear palsy (PSP): a 69-year-old man and a 73-year-old woman. Both patients showed supranuclear ophthalmoplegia, postural instability, pseudobulbar palsy, and Parkinsonism. In the first patient, we administered L-dopa/carbidopa (300 mg/30 mg/ day), which moderately improved gait disturbance, but exerted no beneficial effects on gaze palsy. Then, we administered amitriptyline, bromocriptine, pergolide, l-threo-DOPS or 5-hydroxytryptophan (5-HTP) in addition to L-dopa/carbidopa. The second patient was treated by the monotherapy of L-dopa/carbidopa, amitriptyline, l-threo-DOPS or 5-HTP. We interposed two to three weeks between administration of each drug. In both patients, amitriptyline (75 mg/day) markedly improved both gait disturbance and horizontal gaze palsy. 5-HTP (600 mg/day) also improved horizontal gaze palsy, but failed to alleviate gait disturbance. The results suggest the involvement of impaired serotonergic system in ophthalmoplegia of PSP.

5-Hydroxytryptophan↗