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

Schrag, Anette; Ben‐Shlomo, Yoav; Quinn, NP

doi:10.1016/s0140-6736(99)04137-9

Cited by 576 publications

(356 citation statements)

References 23 publications

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“…Alpha-synuclein deposits have been described in LBD (Spillantini et al 1997). Multiple System Atrophy (MSA) is an extra-pyramidal syndrome characterized by the development of Parkinsonism (typically non -DOPA responsive) with cerebellar, autonomic or pyramidal dysfunction (Quinn 1989), which has an age adjusted prevalence of 4.4\100 000 (Schrag et al 1999). MSA is pathologically characterized by nigro-striatal and olivo-ponto-cerebellar degeneration (Wenning et al 1999).…”

Section: Alpha-synuclein Tau and Parkinsonismmentioning

confidence: 99%

“…This may indicate that these neurodegenerative disorders share a similar pathogenesis. Progressive supranuclear palsy (PSP) accounts for the remainder of cases of non -DOPA responsive forms of Parkinsonism and has an age-adjusted prevalence of 6.6\100 000 (Schrag et al 1999). There are a number of neurodegenerative diseases with Parkinsonism and tangle pathology.…”

Section: Alpha-synuclein Tau and Parkinsonismmentioning

confidence: 99%

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Genetics of Parkinsonism: a review

VAUGHAN¹,

DAVIS²,

WOOD³

2001

Annals of Human Genetics

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Idiopathic Parkinson's disease (IPD), a progressive neurodegenerative disorder, is a common cause of disability. No current therapies modify disease progression. The pathological hallmarks are the presence of Lewy bodies and massive loss of dopaminergic neurons in the pars compacta of the substantia nigra. Two genes (SNCA and parkin) as well as two gene loci have now been implicated in the pathogenesis of familial PD. These represent significant progress in our understanding of the disease, considering the rarity of large families, low heritability in the general population and genetic heterogeneity. Mutations in a further gene, UCHL1, have been described in familial PD although the evidence for its role in PD is less clear. Knowledge of the genes described in PD to date should help to define molecular mechanisms of neurodegeneration in PD, as well as in other diseases where defects in protein handling may be a common feature. Nigral degeneration with Lewy body formation and the resulting clinical picture of PD may represent a final common pathway of a multifactorial disease process in which both environmental and genetic factors have a role. This review discusses the major advances in the field to date and illustrates how the existence of genetic factors has now become firmly established.

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Section: Alpha-synuclein Tau and Parkinsonismmentioning

confidence: 99%

Section: Alpha-synuclein Tau and Parkinsonismmentioning

confidence: 99%

Genetics of Parkinsonism: a review

VAUGHAN¹,

DAVIS²,

WOOD³

2001

Annals of Human Genetics

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“…The disorder has a prevalence of 6.4/100 000 of the population [2,3] and is the second most common syndrome in which 'parkinsonism' is a prominent symptom [4]. The first description of PSP was based on nine cases that exhibited supranuclear ophthalmoplegia, pseudobulbar palsy, dysarthria, and dystonic rigidity of the upper neck and trunk [5,6].…”

Section: Introductionmentioning

confidence: 99%

Spatial patterns of the tau pathology in progressive supranuclear palsy

Armstrong

Cairns

2012

Neurol Sci

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“…Multiple system atrophy (MSA) is a progressive neurodegenerative disease of undetermined aetiology, which affects both sexes 1 with a prevalence ranging between 2.3 and 4.9 per 100 000 in various studies, [2][3][4] and an incidence of 0.6 per 100 000 per year. 5 It usually begins in middle age, 1 hence prevalence rates among patients aged over 55 y are significantly higher (16.8-28.7 per 100 000), 4,6 and probably underestimated as patients in the general population are often misdiagnosed.…”

Section: Introductionmentioning

confidence: 99%

“…5 It usually begins in middle age, 1 hence prevalence rates among patients aged over 55 y are significantly higher (16.8-28.7 per 100 000), 4,6 and probably underestimated as patients in the general population are often misdiagnosed. 4 The median survival from the first symptom is 9.3 y. 1,7 MSA causes parkinsonism and cerebellar autonomic, urinary and pyramidal dysfunction in different combinations.…”

Section: Introductionmentioning

confidence: 99%

Erectile and urinary dysfunction may be the presenting features in patients with multiple system atrophy: a retrospective study

et al. 2003

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Multiple system atrophy (MSA) is a progressive neurodegenerative disease characterized by parkinsonism and cerebellar, autonomic, urinary, and/or pyramidal dysfunction. Urinary and erectile dysfunction (ED) symptoms are prominent early features in men with MSA. Autonomic failure, considered until recently to be the cause of ED in these men, is commonly expressed through symptoms of orthostatic hypotension (OH). The aim of this retrospective study is to examine the chronological relationship between the development of urogenital symptoms and those of OH in patients diagnosed with MSA and discuss its significance in the aetiology of ED in these patients. A total of 71 male patients, referred to a Uro-Neurology department with a diagnosis of 'probable MSA', were reviewed in terms of 'autonomic' symptoms onlyFOH and lower urinary tract symptoms, accompanied by EDFpresent at the time of their referral. Laboratory investigations including anal sphincter EMG and/or autonomic function tests (AFTs) were performed in 75 and 90% of the patients, respectively. At presentation, urinary complaints were recorded in 96% of patients and ED in all patients that this was inquired about. The onset of ED had preceded the onset of bladder symptoms in 58% and the onset of OH symptoms in 91% of these men. Bladder symptoms also preceded symptoms of OH in 76% of patients. Sphincter EMG was abnormal in 91% and AFTs in 77% of the patients tested. Almost all patients with abnormal EMG had troublesome urinary symptoms. AFTs showed similar sensitivity relating to symptoms. At presentation, urogenital symptoms are common in patients with probable MSA and are often not accompanied by symptoms of OH. The earlier occurrence of ED in men with MSA suggests a lack of a causal relationship to hypotension. The notion that MSA possibly affects the dopaminergic mechanism of erectile function is discussed.

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

Cited by 576 publications

References 23 publications

Genetics of Parkinsonism: a review

Genetics of Parkinsonism: a review

Spatial patterns of the tau pathology in progressive supranuclear palsy

Erectile and urinary dysfunction may be the presenting features in patients with multiple system atrophy: a retrospective study

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