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1.
Konagaya M Konagaya Y Sakai M Matsuoka Y Hashizume Y 《Journal of the neurological sciences》2002,195(2):123-127
Nine patients with multiple system atrophy (MSA) were studied based on MRI findings of cerebral hemispheric involvement. The age at onset was 56.4+/-8.6 (mean+/-S.D.) years, duration of illness at the first MRI study 2.1+/-1.1 years, duration of illness at the last study 9.7+/-2.6 years, and the follow-up duration 7.6+/-2.3 years. Controls were 85 neurologically intact persons (60.2+/-11.1 years age). In the MRI study, measurements of the ratio of each area to the intracranial area were performed for the cerebral hemisphere, frontal, temporal and parietal-occipital lobes. A significant progression of atrophy to under the normal limit was observed in the cerebrum, frontal and temporal lobes. Besides the typical pathological lesions in MSA, five autopsied patients revealed frontal lobe atrophy with mild gliosis, mild demyelination and glial cytoplasmic inclusions (GCIs). One of these patients showed remarkable frontal lobe atrophy with degenerative changes in the cerebral cortex. We observed the involvement of the cerebral hemisphere, especially the frontal lobe. 相似文献
2.
Brenneis C Egger K Scherfler C Seppi K Schocke M Poewe W Wenning GK 《Journal of neurology》2007,254(2):191-196
In this study, we aimed to determine the progression of brain atrophy in the parkinson variant of multiple system atrophy (MSA-P). Voxel-based morphometry was applied to two consecutive high resolution MR images of 14 patients with probable MSA-P in comparison to 14 patients with Parkinson's disease (PD). The time interval between baseline and follow-up investigations (1.0 +/- 0.5 SD years in MSAP and 1.4 +/- 0.6 SD years in PD patients) was introduced as covariate in the statistical analysis. Additionally, correlation analyses were performed between the progression maps and clinical data. We observed marked progression of brain atrophy in the MSA-P cohort, the regions including striatum, mesencephalon, thalamus and cerebellum, but also cortical regions such as the primary sensorimotor cortex, supplementary motor area, lateral premotor cortex, medial frontal gyrus, middle frontal gyrus, orbito-frontal cortex,insula, posterior parietal cortex and hippocampus. Short disease duration was correlated with progression of atrophy in the striatum whereas longer disease duration was correlated with increasing atrophy in the cortical areas and cerebellar hemispheres. The UPDRS-III score was not significantly correlated with any brain region. Our data suggest that cortical atrophy is prominent in MSA-P and early degeneration of the basal ganglia drives late onset cortical atrophy. 相似文献
3.
Probst-Cousin S Kayser C Heuss D Neundörfer B 《Fortschritte der Neurologie-Psychiatrie》2000,68(1):25-36
Multiple system atrophy represents an enigmatic, clinico-pathologically defined neurodegenerative disease. According to findings of the last three decades, the historically derived, previously used terms olivopontocerebellar atrophy, striatonigral degeneration, and Shy-Drager-syndrome should be avoided in clinical use because of both the clinical and morphological overlap between these syndromes. Complex neurodegenerative syndromes other than multiple system atrophy according to recently developed criteria, should rather be referred to as multiple system degenerations. 相似文献
4.
F. Tison G. K. Wenning M. A. Volonte W. R. Poewe P. Henry N. P. Quinn 《Journal of neurology》1996,243(2):153-156
Pain is a recognized feature of idiopathic Parkinson’s disease (IPD) but has never been studied in multiple system atrophy
(MSA), the commonest cause of atypical parkinsonism. We retrospectively analysed histories of pain in 100 consecutive cases
of clinically probable MSA. Details were obtained from the medical records of 100 patients with MSA, comprising 82 with the
striatonigral degeneration (SND) type and 18 with the olivopontocerebellar atrophy (OPCA) type of MSA. Pain was reported in
47% of the MSA patients. It was classified as rheumatic in 64% of MSA patients reporting pain, sensory in 28%, dystonic in
21%, and levodopa-related in 16%, mostly related to off-period or diphasic dystonias. There was a mixed pain syndrome in 19%
of these patients. Pain was significantly more commonly reported by females (P=0.02), and by patients with levodopa-induced dyskinesias (P=0.02). No other clinical feature differentiated MSA patients who reported pain from those who did not. The mean delay between
disease onset and onset of pain was 2.9 years, but pain was reported at the time of, or before, disease onset in about 30%
of patients. The overall prevalence of pain in MSA was similar to that reported in IPD, but the distribution of pain categories
was different. 相似文献
5.
Soma H Yabe I Takei A Fujiki N Yanagihara T Sasaki H 《Journal of the neurological sciences》2006,240(1-2):107-110
We investigated the family histories of 157 Japanese patients with probable or possible multiple system atrophy (MSA). A family history of neurodegenerative disorders was only detected in three MSA patients (1.9%). We evaluated these patients by careful neurological examination, neuroimaging studies, and genetic studies to exclude hereditary spinocerebellar ataxia with a similar clinical phenotype to MSA. The results indicated that one of them had a family history of MSA. Although the familial presence of neurodegenerative disorders is rare in MSA patients, the existence of such cases suggests that MSA may have a genetic background. 相似文献
6.
Dystonia in multiple system atrophy 总被引:2,自引:0,他引:2
Riley DE 《Journal of neurology, neurosurgery, and psychiatry》2002,72(3):300-303
OBJECTIVE: To delineate the frequency and nature of dystonia in multiple system atrophy (MSA). METHODS: A cohort of 24 patients with clinically probable MSA over the past 10 years were prospectively followed up. Motor features were either dominated by parkinsonism (MSA-P subtype, n=18) or cerebellar ataxia (MSA-C, n=6). Classification of dystonic features and their changes with time was based on clinical observation during 6-12 monthly follow up visits. Parkinsonian features and complications of drug therapy were assessed. Most patients (22/24) died during the observation period. Neuropathological examination was confirmatory in all of the five necropsied patients. RESULTS: At first neurological visit dystonia was present in 11 (46%) patients all of whom had been levodopa naive at this time point. Six patients (25%) exhibited cervical dystonia (antecollis) (MSA-P n=4, MSA-C n=2), five patients (21%) showed unilateral limb dystonia (MSA-P n=4; MSA-C n=1). A definite initial response to levodopa treatment was seen in 15/18 patients with MSA-P, but in none of the six patients with MSA-C. A subgroup of 12 patients with MSA-P developed levodopa induced dyskinesias 2.3 years (range 0.5-4) after initiation of levodopa therapy. Most patients had peak dose craniocervical dystonia; however, some patients experienced limb or generalised dystonia. Isolated peak dose limb chorea occurred in only one patient. CONCLUSION: The prospective clinical study suggests that dystonia is common in untreated MSA-P. This finding may reflect younger age at disease onset and putaminal pathology in MSA-P. Levodopa induced dyskinesias were almost exclusively dystonic affecting predominantly craniocervical musculature. Future studies are required to elucidate the underlying pathophysiology of dystonia in MSA. 相似文献
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Anette Schrag MD PhD Gregor K. Wenning MD PhD Niall Quinn MD Yoav Ben‐Shlomo MD PhD 《Movement disorders》2008,23(2):294-296
We here report survival in patients with multiple system atrophy (MSA) in a large, prospectively studied group of patients with MSA. Eighty‐five of 100 patients were known to have died. Three patients were rediagnosed as having PD. Twenty‐four patients came to autopsy, which showed MSA in 22 and idiopathic Parkinson's disease in 2. The median survival time was 8.6 and 7.3 years for men and women, respectively (hazard ratio for women was 1.49, 95% CI 0.97–2.31, P = 0.07). Except for rediagnosis as PD, no predictive factors for better survival could be identified. These data confirm the relatively poor prognosis of MSA of less than 9 years on average. © 2007 Movement Disorder Society 相似文献
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10.
Cerebral atrophy in multiple system atrophy by MRI 总被引:4,自引:0,他引:4
Horimoto Y Aiba I Yasuda T Ohkawa Y Katayama T Yokokawa Y Goto A Ito Y 《Journal of the neurological sciences》2000,173(2):109-112
Cranial magnetic resonance images (MRI) of the cerebral areas of 40 patients with multiple system atrophy (MSA) and of 61 age-matched controls were analyzed. The cerebral area of MSA patients was 131. 95+/-15.89 cm(2) (mean+/-S.D.), which was significantly smaller than that of normal controls at 149.01+/-10.93 cm(2) (P<0.0001). All 23 MSA cases subjected to the MRI study over a 1-year period showed progressive cerebral atrophy, and the atrophy rate was 2.46+/-1. 66%/year. There were no significant differences within the MSA subtypes or between gender. The progression of cerebral atrophy in MSA correlated more with duration (r=-0.634) than age (r=-0.421). We conclude that MRI findings throughout the course of MSA suggest progressive cerebral atrophy, which is common in all subtypes and reflects duration of the disease rather than age. 相似文献
11.
Kim HJ Jeon BS Lee JY Yun JY Kim YE Paek SH 《Journal of the neurological sciences》2012,318(1-2):168-170
Ehlers-Danlos Syndrome is a rare group of inheritable disorders resulting in abnormal collagen production, leading to skin fragility, joint hypermobility and easy bruising. Six major subtypes have been identified, of which Type IV most often leads to neurovascular complications, may lead to inner organ rupture and overall has the worst prognosis. Early recognition followed by genetic testing is key, since this diagnosis will guide decision making in the management of complications, influence the choice of antiplatelet medications versus anticoagulants and allow for potentially affected family members to be identified, undergo genetic testing and reproductive counseling. We here report the case of a 50 year old woman with a fulminant presentation of Ehlers Danlos Syndrome Type IV, including bilateral carotid and vertebral artery dissection, multiple strokes and liver rupture. Of note, this patient did not have a known history or obvious clinical features of connective tissue disease. Genetic testing confirmed the diagnosis. Review of her family history revealed multiple family members with a history of aortic dissection or aneurysm rupture. This case illustrates that Ehlers Danlos Syndrome Type IV is an important differential diagnosis even in adult patients without a known history of connective tissue disease and no prior complications. 相似文献
12.
Accumulation of tubular structures in oligodendroglial and neuronal cells as the basic alteration in multiple system atrophy. 总被引:11,自引:0,他引:11
In 8 brains of patients with various combinations of striatonigral degeneration, olivopontocerebellar atrophy and Shy-Drager syndrome, inclusion bodies were demonstrated in the cytoplasm and nucleus of both neuronal and oligodendroglial cells and in neuronal processes by means of silver staining, immunocytochemistry and electron microscopy. Differing from oligodendroglial cytoplasmic inclusions recognized by anti-ubiquitin, anti-alpha- and anti-beta-tubulin, and anti-tau antibodies, neuronal cytoplasmic inclusions were stained only by anti-ubiquitin antibody but not with those raised against cytoskeletal proteins. Tubular structures forming the inclusion bodies irrespective of their glial or neuronal location, have fuzzy cover and side extensions which make them similar to the linear structures described in motor neuron diseases. Our study proves that the accumulation of abnormal tubular structures in both oligodendrocytes and neurons is the basic pathological alteration in multiple system atrophy and defines multiple system atrophy as a group of diseases with similar cellular pathology or as a nosological entity. 相似文献
13.
N. Vanacore V. Bonifati G. Fabbrini C. Colosimo G. De Michele R. Marconi D. Nicholl N. Locuratolo G. Talarico S. Romano F. Stocchi U. Bonuccelli M. De Mari P. Vieregge G. Meco 《Neurological sciences》2001,22(1):97-99
Multiple system atrophy (MSA) is a form of atypical parkinsonism with unknown etiology. The epidemiological studies conducted up to now on this disease are scarce. The incidence rate is about 0.6 cases per 100 000 persons per year. The prevalence rates show 4–5 cases per 100 000 persons. In Italy, about 4900 prevalent cases have been estimated. The mean onset age is about 54 years; the median survival is 7–9 years. Only one case-control study has been performed on this disease. This study showed an increased risk MSA associated with occupational exposure to organic solvents, plastic monomers and additives, pesticides and metals. Smoking habits seem to be less frequent in MSA cases (as in Parkinson's disease cases) than in healthy controls. Quinn's clinical criteria and those of the Consensus Conference promoted by the American Academy of Neurology are in fair agreement. We have performed a case-control study on 73 MSA cases, 146 hospital controls and 73 population controls. 相似文献
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Multiple system atrophy (MSA) is a sporadic, adult‐onset neurodegenerative disease, which is characterized by striatonigral degeneration, olivopontocerebellar atrophy, and preganglionic autonomic lesions in any combination. The histological hallmark is the presence of argyrophilic fibrillary inclusions in the oligodendrocytes, referred to as glial cytoplasmic inclusions (GCIs). Fibrillary inclusions are also found in the neuronal somata, axons, and nucleus. Neuronal cytoplasmic inclusions are frequently found in the pontine and inferior olivary nuclei. Since the discovery of α‐synuclein as a major component of glial and neuronal inclusions in MSA, two neurodegenerative processes have been considered in this disease: one is due to the widespread occurrence of GCIs associated with oligodendroglia–myelin degeneration (oligodendrogliopathy) in the central nervous system, and the other is due to the filamentous aggregation of α‐synuclein in the neurons in several brain regions. These two degenerative processes might synergistically cause neuronal depletion in MSA. 相似文献
16.
In a patient with multiple system atrophy, the ataxia was temporarily exacerbated by cigarette smoking. The phenomenon is attributed to a direct effect of nicotine on the central nervous system, rather than being secondary to autonomic changes. Its prevalence and specificity are currently unknown. 相似文献
17.
Sleep disorders are so common in multiple system atrophy that they should be considered an integral part of the disease. Sleep fragmentation occurs in more than half of the patients, with sleep onset and sleep maintenance insomnia. Periodic leg movements of sleep are also common, although they are generally asymptomatic. REM sleep behavior disorder, a parasomnia in which the patient presents vigorous movements associated with intense dreaming dreaming during REM sleep, is detected polysomnographically in almost all the patients with variable severity. Nocturnal stridor is produced by obstruction on the glottis level. The cause of stridor is unknown but two alternative explanations have been proposed: paralysis of the muscles opening the vocal chords and excessive contraction of the muscles that close them. A combination of both, however, is the most likely explanation. Nocturnal stridor is associated with decreased survival and its treatment is based on continuous positive air pressure (CPAP) when it occurs only during sleep or tracheostomy when it worsens and becomes also diurnal. 相似文献
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19.
Sphincter electromyography and multiple system atrophy 总被引:2,自引:0,他引:2
Electromyographic studies of the sphincter in patients with multiple system atrophy have shown increased duration and polyphasia of motor unit potentials. These electrophysiological markers have been used to argue for the selective degeneration of sacral motor neurons in Onuf's nucleus in patients with multiple system atrophy. Studies comparing sphincter electromyographic changes in patients with multiple system atrophy and Parkinson's disease have shown significant differences between these two patient populations. Despite the controversy surrounding this claim, recent studies using quantitative electromyographic techniques support the view that reinnervation of the anal sphincter muscles may be a useful diagnostic marker for distinguishing multiple system atrophy from Parkinson's disease. A critical review of these data is needed to assess the validity and reliability of electromyographic changes in multiple system atrophy. 相似文献
20.
Ghorayeb I Bioulac B Tison F 《Journal of neural transmission (Vienna, Austria : 1996)》2005,112(12):1669-1675
Summary. Complaints about sleep disorders and excessive daytime sleepiness are common among patients with multiple system atrophy.
The diffuse neurodegenerative process that encompasses the key structures involved in the regulation of the sleep/wake transition
and respiratory function may account for these complaints and for the most frequent polysomnographic findings in MSA, i.e.,
sleep-related breathing disturbances and REM sleep behaviour disorder, which are both treatable conditions. Nocturnal stridor
is an inspiratory sound produced by complex vocal cord muscle dysfunction. Often occurring with sleep apnoea, stridor is associated
with decreased survival. REM sleep behaviour disorder, a parasomnia characterized by loss of normal skeletal muscle atonia
during REM sleep with prominent motor activity, is detected in almost all patients. The pathophysiology of both disorders
is partially elucidated but increasing evidence points to the role of basal ganglia dysfunction. 相似文献