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PubMedDate not supplied

Corticobasal degeneration: an autopsy case clinically diagnosed as progressive supranuclear palsy.

We report an autopsy case diagnosed clinically as progressive supranuclear palsy (PSP), but neuropathologically confirmed as corticobasal degeneration (CBD). A 56-year-old Japanese woman slowly developed parkinsonism, dementia, character change, followed by vertical gaze palsy and dystonia. Brain MRI demonstrated diffuse cerebral atrophy with severe shrinkage of the brain stem tegmentum. The SPECT images using 123I-IMP disclosed symmetrical hypoperfusion in the frontal lobes. She died of respiratory failure at the age of 71. Gross inspection of the brain showed diffuse, symmetrical atrophy of the cerebrum and marked atrophy of the Luysian body, globus pallidus, substantia nigra and nuclei of the brain stem tegmentum. Microscopically, neuronal loss and fibrillary gliosis were observed in the Luysian body, globus pallidus, substantia nigra and nuclei of the brain stem tegmentum. The cerebellar dentate nucleus showed mild neuronal loss with some grumose degeneration. Neurofibrillary tangles were found only in the Luysian body, substantia nigra and raphe nuclei, whilst tau-positive inclusions were observed more extensively. Astrocytic plaques and swollen achromatic neurones were found in the postcentral gyrus. There were no tuft-shaped astrocytes in the brain. The clinicopathological similarities and differences between PSP and CBD are discussed.

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PubMedDate not supplied

[Changes in sleep organization in two patients with progressive supranuclear palsy].

Sleep-EEG of two subjects affected by Progressive Supranuclear Palsy was studied. Morphological, reduced and poorly organized sleep transients (spindles) in stage 2 and decreased REMs during desynchronized sleep were observed. The quantitative sleep analysis showed a poor and fragmented sleep with an increase of stage 1 and stage 3 and a decrease of stage 2 and REM sleep.

Electroencephalography↗
PubMed1993

[Asynchronous Bell's phenomenon in progressive supranuclear palsy].

A 60-year-old man suffering from progressive supranuclear palsy showed asynchronous Bell's phenomenon and apraxia of eye-lid opening. He showed synchronous upward rotation of the eyeball at closing eyes. Although apraxia of eye-lid opening was observed in both sides, eye opening of the right side took longer duration than that of the opposite side. When he tried to open his eyes, unilateral Bell's phenomenon was sustained in the side of involuntary eye closing for a several seconds. These findings indicated disorders of the coordination of eye opening-and-closing, which regulate the orbicural muscle of eye, levator muscle of upper eyelid and external ocular muscles. And the mechanism of synchronous eye movement between the both sides was also disturbed. The administration of L-droxydopa was effective to gait disturbance, but not to ocular movement disorder including impaired Bell's phenomenon. The pharmacological results suggested different neuron system between locomotion control and conjugation affected in this disease.

Apraxias↗
PubMed1985

Unusual CT findings in progressive supranuclear palsy. Case report.

We report a patient affected by progressive supranuclear palsy who also presented with bilateral internuclear ophthalmoplegia. The computed tomographic (CT) scan showed the previously described radiological picture characteristic of the syndrome and two additional unusual findings. The significance of these findings is discussed and the importance of the CT scan in the diagnosis of progressive supranuclear palsy is emphasized.

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PubMed1991

Language functions in progressive supranuclear palsy.

Language functions were studied in 6 patients with clinically diagnosed progressive supranuclear palsy who conformed to the characteristic pattern of 'subcortical dementia'. Dysarthria, reading difficulties and disturbances of handwriting were present in all patients. Some patients showed additional deficits including visual dyslexia, constructional dysgraphia and an increased rate of self-corrections and misnamings in object confrontation naming. In most instances, the naming errors referred to an object visually similar to the target object, suggesting that visual misperception is the major cause of the naming disorder. It is concluded that a variety of language impairments may develop secondary to other neurological and neuropsychological changes in progressive supranuclear palsy.

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PubMed2005

Toward future therapies in progressive supranuclear palsy.

There is a stern therapeutic challenge for progressive supranuclear palsy (PSP) that has not yet been met. The lack of randomized, controlled trials and negative outcomes from the vast majority of studies make it impossible to set therapeutic standards, or to give clear recommendations. We review progress to date in this area and briefly consider future potential therapeutic strategies.

Adrenergic Agents↗
PubMed1989

Sleep abnormalities in progressive supranuclear palsy.

We studied sleep patterns for three nights in 10 subjects with moderate to severe progressive supranuclear palsy and correlated the findings with disease severity using quantitative measures of motor, cognitive, and eye movement impairment. All subjects had severe insomnia, spending 2 to 6 hours awake per night; the mean time awake per night for the group was more than 4 hours. Sleep latency became shorter and the number of awakenings increased with greater motor impairment, and total sleep time declined as dementia worsened. These findings indicate that in progressive supranuclear palsy insomnia is related to disease severity. Insomnia associated with progressive supranuclear palsy appears to be worse than the insomnia of Parkinson's disease or Alzheimer's disease and may be due to degenerative changes in brain structures responsible for sleep maintenance.

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PubMed1994

Therapy for progressive supranuclear palsy: past and future.

Dysfunction of multiple brain systems in progressive supranuclear palsy (PSP) has complicated attempts to treat the disease. Neurotransmitter replacement strategies targeting the dopaminergic, cholinergic, and serotonergic systems have been unsuccessful. In order to bypass the degenerated cortico-striato-pallidal loop, we administered the adrenergic agonist idazoxan (IDA) to treat PSP in two randomized double-blind, placebo controlled, crossover studies. Approximately one half of patients enrolled in these studies showed statistically significant improvement in balance and manual dexterity while taking IDA compared to placebo. These results suggest that new therapies that target structures outside of the basal ganglia may be useful for symptomatic treatment of PSP. Applying this strategy and developing treatments that arrest or reverse clinical deterioration in PSP will require improved understanding of the process underlying the illness.

Adrenergic alpha-Antagonists↗
PubMed2001

Progressive supranuclear palsy on Guam.

This is the first report demonstrating that progressive supranuclear palsy (PSP) exists on Guam. This 75-year-old Guamanian Chamorro patient with slight dementia and rigidity with restriction of ocular up gaze was diagnosed as parkinsonism-dementia complex (PDC) of Guam clinically. However, neurofibrillary tangles (NFTs) were scarcely seen in the cerebral cortices and hippocampus, but many NFTs, composed of 15-17 nm straight tubules, were detected in the subthalamic nucleus and brain stem. A large number of tuft-shaped astrocytes were observed in the putamen and motor cortex, and numerous argyrophilic grains were seen in the CA1 and subiculum. These pathological findings are different from those of PDC and consistent with PSP. The present case indicates that PSP and PDC clinically resemble each other, and that precise neuropathological examination is indispensable for the final diagnosis of the patient with parkinsonism, dementia and disturbance of vertical external ocular movement.

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PubMed1993

Micturitional disturbance in progressive supranuclear palsy.

Detailed micturitional histories were taken from nine patients with progressive supranuclear palsy (PSP), and eight of them (89%) had micturitional symptoms including urinary incontinence in seven. Urodynamic studies were performed in six patients and the results were as follows. Three had residual urine of 100 ml on average. Four had detrusor hyperreflexia and one had a low compliance cystometrogram. One had detrusor-sphincter dyssynergia. Motor unit analysis of external sphincter was performed in four patients and two had neurogenic changes. The results were compared with our previous findings in Parkinson's disease and in striato-nigral degeneration (SND), and we found that a severe degree of micturitional disturbance in PSP seems to be as common as in SND, especially in the urinary storage phase, and more frequent than in Parkinson's disease. Supranuclear types of pelvic and pudendal nerve dysfunctions seemed to be mainly responsible for micturitional disturbance in PSP.

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PubMed2004

Differential involvement and heterogeneous phosphorylation of tau isoforms in progressive supranuclear palsy.

We found previously that aggregated insoluble tau protein in progressive supranuclear palsy (PSP) brains exhibits a heterogeneous pattern that is not segregated by the type of clinical presentation. Here we have investigated tau isoform composition from 20 PSP cases and found marked variation between different brains. Cases were classified into three groups, each comprising essentially of (1) 1N4R; (2) 1N4R and 1N3R; or (3) 1N4R, 1N3R and 0N4R tau isoforms. There was also an absence of a simple relationship between isoform composition and the pattern of insoluble tau before dephosphorylation. We conclude that there is distinct molecular heterogeneity in the involvement of tau isoforms in the tau pathology in PSP.

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PubMed2006

Effect of MAPT and APOE on prognosis of progressive supranuclear palsy.

To assess genetic influence on the clinical presentation of progressive supranuclear palsy (PSP), the genetic effect on disease course was examined for variants in the tau gene (MAPT) and the gene for apolipoprotein E (APOE) in 58 cases of pathologically confirmed PSP. Clinical indicators of disease course included age at symptomatic onset (AAO), age at death (AAD), and disease duration (DD) and the genetic effects examined included MAPT haplotypes and APOE genotypes. From linear regression analysis, the MAPT H1/H1 genotype was associated with significantly earlier AAO (P=0.038). The MAPT genotype did not significantly influence DD or AAD. The APOE epsilon4 allele did not significantly influence AAO, AAD, or DD. Male sex was a predictor for earlier AAO (P=0.015). The interaction between MAPT and APOE was not significant for AAD and DD, but a significant negative coefficient was found for AAO suggesting their combination does not have an additive effect. These results support the assertion that the H1/H1 genotype may contribute to the earlier occurrence of clinical symptoms.

Age of Onset↗
PubMed2002

Frontal atrophy correlates with behavioural changes in progressive supranuclear palsy.

Regional brain volumes were measured in 21 patients with progressive supranuclear palsy (PSP), 17 patients with Parkinson's disease and 23 controls using 3D MRI-based volumetry. Cortical, subcortical and ventricular volume measures were correlated with global indices of motor disability and cognitive disturbance. All MRI measures, including hippocampal volume, were preserved in Parkinson's disease. Patients with PSP could be distinguished from both Parkinson's disease and controls by whole brain volume loss, ventricular dilatation and disproportionate atrophy of the frontal cortex. Caudate nucleus volume loss additionally differentiated PSP from controls, but was modest in severity and proportionate to whole brain volume loss. The present study identifies disease-specific differences in the topography of brain atrophy between PSP and Parkinson's disease, and has potential implications for the in vivo radiological differentiation of these two disorders. In PSP, the variance in frontal grey matter volume related to measures of behavioural disturbance, confirming the use of behavioural tests for ante-mortem case differentiation and suggesting that intrinsic cortical deficits contribute to these clinical disturbances.

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PubMedDate not supplied

Cerebral blood flow in corticobasal degeneration and progressive supranuclear palsy.

To compare brain perfusion between corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP), we investigated regional cerebral blood flow (rCBF) semiquantitatively with single-photon emission computed tomography and [123I]iodoamphetamine in six patients with CBD and five with PSP. Compared with 12 age-matched control subjects, the average of the left and right rCBF values for the CBD patients was significantly reduced in the inferior prefrontal, anterior cingulate, medial premotor, sensorimotor, posterior parietal, and superior temporal cortices as well as in the basal ganglia and thalamus, whereas only the medial premotor cortex was significantly hypoperfused in the PSP patients. Compared with the PSP patients, the CBD patients showed significantly decreased rCBF in the inferior prefrontal, sensorimotor, and posterior parietal cortices, but not in the subcortical regions. Compared with the controls, interhemispheric differences of rCBF were significant in the inferior prefrontal, sensorimotor, and posterior parietal cortices of the CBD patients but in only the medial prefrontal cortex of the PSP patients. These results indicate that rCBF reductions are more extensive and asymmetric in CBD than in PSP, although the two diseases share medial frontal involvement.

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PubMed1998

Corticobasal ganglionic degeneration and progressive supranuclear palsy presenting with cognitive decline.

Corticobasal ganglionic degeneration (CBGD) and progressive supranuclear palsy (PSP) were originally described in the sixties as predominantly motor syndromes. Over the years, the detailed study of additional cases of CBGD has shown that it is a distinctive histological entity which can often present as dementia or aphasia. Although some pathological features of CBGD overlap with those of other forms of non-Alzheimer non-Lewy body dementia, the distribution and relative number of these abnormalities and the distinctive pattern of tau immunodeposits allows the distinction of CBGD from Pick's disease and fronto-temporal dementia. In contrast, PSP only rarely presents with prominent dementia or behavioral changes. In these unusual PSP cases, care must be taken to exclude the diagnoses of CBGD and familial tangle-only dementia.

Basal Ganglia Diseases↗
PubMed2005

Natural history of progressive supranuclear palsy: a clinicopathologic study from a population of brain donors.

Progressive supranuclear palsy (PSP) is the second most common parkinsonian syndrome, characterized by a 'diverse' clinical phenotype. Although several different diagnostic guidelines have been proposed, the early and accurate diagnosis of PSP remains problematic and neuropathology is still considered the 'gold standard'. In order to contribute to the better clinical characterization of PSP, we conducted a postmortem study in a cohort of 22 consecutive PSP brain donors and compared our results with those of previously published reports. Our results further demonstrate and expand the varied clinical picture of PSP. Furthermore, we report associations between older age at onset, early dementia, early falls and smoking with shorter PSP duration.

Age Factors↗
PubMed1994

Progressive supranuclear palsy: neuropathologic and clinical heterogeneity.

To investigate potential heterogeneity in progressive supranuclear palsy (PSP), we examined 13 patients with neuropathologically diagnosed PSP. The clinical diagnosis of PSP was made in eight of these individuals, whereas probable AD was the primary diagnosis in the remaining five. In addition to PSP neuropathology, seven of the 13 patients (54%) showed concomitant pathologic changes of Alzheimer's disease (AD), Parkinson's disease (PD), or both disorders. These observations indicate that AD and PD changes coexist with PSP neuropathology in a substantive proportion of patients and provide further evidence of clinical and neuropathologic heterogeneity in neurodegenerative disorders. Moreover, our results suggest that PSP may be underdiagnosed and deserves more prominence in the differential diagnosis of dementing illness.

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