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Results for “Multiple System Atrophy”

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PubMed2006

Multiple system atrophy presenting with acute respiratory failure due to diaphragmatic dysfunction.

Sleep related respiratory dysfunction and vocal cord paralysis are considered to be the major factors responsible for respiratory failure in multiple system atrophy (MSA). We report a patient initially presenting with alveolar hypoventilation culminating in respiratory failure, ultimately diagnosed as MSA. No central sleep apnea or marked paralysis of the vocal cords was noted. The most likely cause for the respiratory failure was thought to be the weakness of respiratory musculature. This case emphasizes the need that MSA should be added to the differential diagnosis of unexplained hypercapnic respiratory failure.

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PubMed2000

L-glutamate, L-arginine and L-citrulline levels in cerebrospinal fluid of Parkinson's disease, multiple system atrophy, and Alzheimer's disease patients.

Alterations in neuronal nitric oxide (NO) production may play a role in the pathophysiology of Parkinson's disease (PD) Alzheimer's disease (AD), and multiple system atrophy (MSA). The biosynthesis of NO is dependent on the availability of L-arginine, the substrate for NO-synthase (NOS), and on L-glutamate, which stimulates NO synthesis via the NMDA receptor. In this process L-citrulline is formed. We measured the levels of these amino acids in cerebrospinal fluid (CSF) of 108 PD patients, 12 AD patients, 15 MSA patients and 21 healthy subjects. A slight but statistically significant elevation of CSF L-citrulline was found in MSA patients, while CSF L-glutamate was found to be significantly decreased in AD patients. We found no significant changes in L-arginine levels. Although the relation between the CSF levels of these amino acids and neuronal NO production is still unclear, our findings suggest that AD is associated with a decrease in NO synthesis.

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PubMed1994

Laryngeal electromyography with separated surface electrodes in patients with multiple system atrophy presenting with vocal cord paralysis.

When recording the activity of the posterior cricoarytenoid muscle (PCA) with surface electrodes, there is contamination from the surrounding muscles such as the cricopharyngeal muscle. We therefore devised a new oesophageal catheter electrode of the separate type, having three individual surface electrodes for the PCA, cricopharyngeal muscle and diaphragm. The records obtained with this catheter demonstrated satisfactory separation between PCA and cricopharyngeal muscle activities. We used this catheter in patients with multiple system atrophy presenting with vocal cord paralysis, who were awake or asleep. There were two interesting electromyographical findings, which were inspiratory activity of the adductor muscle (the thyroarytenoid muscle) and fade-out of the abductor muscle, that is, PCA activity during sleep. Although vocal cord paralysis is one of the most serious life-threatening complications, the precise mechanism has not been clarified. We believe that our catheter may be useful in investigating the mechanism of vocal cord paralysis which could cause sudden death in neurodegenerative disorders, including multiple system atrophy.

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PubMed2000

Is clonidine growth hormone stimulation a good test to differentiate multiple system atrophy from idiopathic Parkinson's disease?

Clonidine, a centrally active alpha 2-adrenoreceptor agonist used to lower blood pressure, has been proposed to differentiate central from peripheral autonomic deficits and multiple system atrophy (MSA) from untreated idiopathic Parkinson's disease (IPD). A lack of growth hormone (GH) increase after clonidine infusion is found in patients with MSA, but not in those with IPD or with pure autonomic failure. We studied 19 IPD and 7 MSA patients to assess whether this test could be used in clinical practice to distinguish MSA from IPD, whatever the stage of the disease. Serum GH levels were measured 15, 30, 45 and 60 min after a 10-min infusion of 2 micrograms/kg clonidine. GH levels remained stable after clonidine infusion in all 7 MSA patients but increased in only 12 of the 19 IPD patients, while remaining stable in the other 7. No correlation was found with the presence of orthostatic hypotension. We conclude that the GH response to clonidine infusion has a very high sensitivity (100% in our series and in previous studies) for the diagnosis of MSA. However, this response cannot be used as a diagnostic test because of its poor specificity.

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PubMed2002

T2*-weighted MRI differentiates multiple system atrophy from Parkinson's disease.

To compare the ability of T2*-weighted gradient echo (GE) and T2-weighted fast spin echo images to distinguish between patients with idiopathic PD and multiple system atrophy (MSA), the authors studied 15 patients with probable MSA, 40 patients with PD, and 17 healthy control subjects. Hypointense putaminal signal changes were more often observed in MSA than in PD using T2* but not T2-weighted images, indicating that T2*-weighted GE sequences are of diagnostic value for patients with parkinsonism.

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PubMed1991

Idiopathic Parkinson's disease combined with multiple system atrophy. A clinicopathological report.

The clinical and postmortem findings of a patient with Lewy body pathology combined with multiple-system atrophy are described. When alive the patient was diagnosed as having Parkinson's disease. Pathological examination found evidence of striatonigral and olivopontocerebellar degeneration, together with Lewy bodies at a number of sites characteristic for idiopathic Parkinson's disease. Taken together, the clinical history and histological findings support the coexistence of two disease processes.

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PubMed1997

Multiple system atrophy: a review of 203 pathologically proven cases.

We report the clinicopathological features of 203 cases of pathologically proven multiple system atrophy (MSA) from 108 publications up to February 1995. The majority of patients showed symptoms in their early fifties, and men were more commonly affected than women (ratio of 1.3:1). Most patients suffered from some degree of autonomic failure (74%). Parkinsonism was the most common motor disorder (87%), followed by cerebellar ataxia (54%) and pyramidal signs (49%). The response to levodopa was poor in most patients, but there was a subgroup with a good response, who also often developed axial levodopa-induced dyskinesias. Other characteristic features included severe dysarthria, stridor, and, in a few patients, contractures and dystonia (antecollis). Mild or moderate intellectual impairment occurred in some cases, but severe dementing illness was most unusual. The main pathological change comprised cell loss and gliosis in the putamen, caudate nucleus, external pallidum, substantia nigra, locus ceruleus, inferior olives, pontine nuclei, cerebellar Purkinje cells, and intermediolateral cell columns of the spinal cord. However, other neuronal populations were also involved to varying degrees, such as the thalamus, vestibular nucleus, dorsal vagal nucleus, corticospinal tracts, and anterior horn cells. Characteristic glial and/or neuronal cytoplasmic inclusions were identified in all cases in which they were sought, irrespective of clinical presentation. Akinesia correlated with the degree of nigral and putaminal cell loss, whereas rigidity was related only to the later. Tremor was unrelated to cell loss at any site. Ataxia correlated with the degree of olivopontocerebellar atrophy. Pyramidal signs were associated with pyramidal tract pallor. Our analysis also confirmed an association of postural hypotension with intermediolateral cell column degeneration.

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PubMed1992

Argyrophilic glial intracytoplasmic inclusions in multiple system atrophy: immunocytochemical and ultrastructural study.

Argyrophilic intracytoplasmic inclusions in oligodendrocytes (AGCIs) were seen in all of 15 cases of multiple system atrophy (MSA), and none in other neurodegenerative diseases, including 9 cases of Menzel-type olivopontocerebellar atrophy and 4 cases of Joseph's disease. The inclusions were widespread, not only in the olivopontocerebellar and striatonigral systems but also among fibers connecting their affecting lesions of MSA. Immunohistochemically, they were closely associated with tau, tubulins and microtubule-associated protein 5. Ultrastructurally, they consisted of 30- to 50-nm filaments (not tubules) and electron-dense granules, in varying proportions, and their formation is discussed. The specific occurrence of AGCIs could be a key to approach the pathogenesis of MSA.

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PubMed1995

Autonomic dysfunction in pathologically confirmed multiple system atrophy and idiopathic Parkinson's disease--a retrospective comparison.

INTRODUCTION: Autonomic dysfunction (AD) can be a feature of both multiple system atrophy (MSA) and idiopathic Parkinson's disease (IPD), conditions that are frequently misdiagnosed in life. Most studies on AD in MSA and IPD are based on clinical cases without pathological verification. MATERIAL AND METHODS: We retrospectively analysed AD in 135 pathologically confirmed cases of IPD and in 33 of MSA from the UK PD Society Brain Bank. RESULTS: MSA started at a younger age than IPD (54.4 +/- 10.7 yrs versus 60.6 +/- 10.8 yrs), and AD began earlier in the course of the illness All MSA patients had some degree of AD in life whereas AD was absent in 24% of IPD patients. Although each of five autonomic domains was affected in variable numbers of IPD patients, AD in MSA generally involved more autonomic domains than in IPD, and to a more severe degree, in particular with regard to inspiratory stridor. CONCLUSIONS: These results indicate that the presence of autonomic disturbance alone does not distinguish between MSA and IPD in individual cases. However, the presence of severe AD, of AD preceding parkinsonism, or of inspiratory stridor, are all individually suggestive of MSA.

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PubMed1997

Pain-related and electrically stimulated somatosensory evoked potentials in patients with Machado-Joseph disease and multiple system atrophy.

We investigated the ascending somatosensory pathway for pain in 8 patients with Machado-Joseph disease (MJD) and multiple system atrophy (MSA). Pain-related somatosensory evoked potentials (pain SEPs) by CO2 laser stimulation were examined together with conventional electrically stimulated somatosensory evoked potentials (electric SEPs). The sporadic cerebellar ataxia of patients with MSA showed a normal pattern of pain SEPs as well as electric SEPs. However, pain and electric SEPs were abnormal for the central and/or peripheral ascending pathway in MJD. These abnormalities of pain and electric SEPs in MJD were not related to the clinical severity of sensory impairment, but they indicate that MJD presents a subclinical abnormality for the ascending somatosensory pathways not only for vibratory sense but also for pain sense.

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PubMed2003

[11C](R)-PK11195 PET imaging of microglial activation in multiple system atrophy.

Microglia, the brain's intrinsic macrophages, bind (R)-PK11195 when activated by neuronal injury. The authors used [11C](R)-PK11195 PET to localize in vivo microglial activation in patients with multiple system atrophy (MSA). Increased [11C](R)-PK11195 binding was primarily found in the dorsolateral prefrontal cortex, putamen, pallidum, pons, and substantia nigra, reflecting the known distribution of neuropathologic changes in MSA. Providing an indicator of disease activity, [11C](R)-PK11195 PET can thus be used to characterize the in vivo neuropathology of MSA.

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PubMed2004

Fragile X gene premutation in multiple system atrophy.

Previous reports have suggested that expansion of the CGG repeat located in the fragile X mental retardation 1 (FMR1) gene might be responsible for a significant number of patients with the multiple system atrophy (MSA) phenotype. Analysis of 65 MSA patients found only 4.6% displayed CGG expansions in the suspected range. This is similar to the frequency reported in the normal population, suggesting that this expansion does not play a major role in the MSA phenotype.

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PubMedDate not supplied

Multiple system atrophy: laminar distribution of the pathological changes in frontal and temporal neocortex--a study in ten patients.

OBJECTIVE: To determine the distribution of the pathological changes in the neocortex in multiple-system atrophy (MSA). METHOD: The vertical distribution of the abnormal neurons (neurons with enlarged or atrophic perikarya), surviving neurons, glial cytoplasmic inclusions (GCI) and neuronal cytoplasmic inclusions (NI) were studied in alpha-synuclein-stained material of frontal and temporal cortex in ten cases of MSA. RESULTS: Abnormal neurons exhibited two common patterns of distribution, viz., density was either maximal in the upper cortex or a bimodal distribution was present with a density peak in the upper and lower cortex. The NI were either located in the lower cortex or were more uniformly distributed down the cortical profile. The distribution of the GCI varied considerably between gyri and cases. The density of the glial cell nuclei was maximal in the lower cortex in the majority of gyri. In a number of gyri, there was a positive correlation between the vertical densities of the abnormal neurons, the total number of surviving neurons, and the glial cell nuclei. The vertical densities of the GCI were not correlated with those of the surviving neurons or glial cells but the GCI and NI were positively correlated in a small number of gyri. CONCLUSION: The data suggest that there is significant degeneration of the frontal and temporal lobes in MSA, the lower laminae being affected more significantly than the upper laminae. Cortical degeneration in MSA is likely to be secondary to pathological changes occurring within subcortical areas.

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PubMed2001

High-frequency oscillations in human posterior tibial somatosensory evoked potentials are enhanced in patients with Parkinson's disease and multiple system atrophy.

High-frequency oscillations (HFOs) and the underlying P37 primary somatosensory response evoked by posterior tibial nerve stimulation were recorded in patients with Parkinson's disease (PD) and in those with multiple system atrophy (MSA), as well as in normal controls. In order to increase the signal-to-noise ratio, we averaged a large number of responses (9998 epochs) with a high sampling rate (20 kHz per channel). HFOs were extracted by filtering the wide band-pass recording of the P37 potential with a 600-900 Hz band-pass filter. High-amplitude HFOs were observed in both the PD and MSA patients. Furthermore, the duration of illness was positively correlated with the amplitude of HFOs. The results suggest that HFOs are enhanced by dysfunction of the basal ganglia.

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PubMed2003

T2-low signal intensity in the cortex in multiple system atrophy.

To determine the clinical significance of T2-low signal intensity in the cortex of patients presenting parkinsonism, T2-weighted magnetic resonance (MR) images of the cortex of patients with multiple system atrophy (MSA), Parkinson's disease (PD) and progressive supranuclear palsy (PSP), and compared with those of patients with amyotrophic lateral sclerosis (ALS) and age-matched normal controls. The MR images were gathered and presented randomly to three neurologists who were blind to information on the patients. There was a significant increase in the frequency of T2-low signal intensity in the cortex of patients with ALS and MSA. Particularly in those with MSA, the T2-low signal intensity was observed not only in the motor cortex but also in the frontal association cortex. The cortical T2-low signal intensity in MSA might reflect the spread of degenerative processes in the cortex.

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PubMed2003

Depletion of ventromedullary NK-1 receptor-immunoreactive neurons in multiple system atrophy.

We sought to determine whether there are neurokinin-1 receptor-like-immunoreactive (NK-1R-LI) neurons in human ventrolateral medulla and whether these neurons are more severely involved in multiple system atrophy (MSA) than in Parkinson's disease. Brains were obtained at autopsy from six control subjects, six subjects with clinical diagnosis of MSA and four with Parkinson's disease, both confirmed neuropathologically. Serial 50 microm cryostat sections were obtained throughout the medulla, and every eighth section was processed for NK-1R-LI neurons. Some sections were processed simultaneously for tyrosine hydroxylase or choline acetyltransferase. Abundant NK-1R-LI neurons were identified in the ventrolateral medulla. These neurons were distinct from local cholinergic or catecholaminergic neurons. There was a severe depletion of these NK-1R-LI neurons in all MSA cases compared with controls (6 +/- 1 cells/section versus 49 +/- 2 cells/section in controls). Although there was also a reduction in Parkinson's disease (20 +/- 2 cells/section), this was significantly less severe than in MSA. Our findings suggest that the human ventrolateral medulla contains NK-1R-LI neurons, and the more severe depletion in MSA than in Parkinson's disease may explain the higher incidence of respiratory and cardiovascular abnormalities in the former condition.

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PubMed2006

[Subthalamic stimulation in a patient with multiple system atrophy: a clinicopathological report].

INTRODUCTION: Efficacy of high frequency subthalamic nucleus (STN) stimulation has been demonstrated in idiopathic Parkinson's disease (IPD). However, since it may be difficult to differentiate IPD from multiple system atrophy with parkinsonian presentation (MSA-P), a few cases of MSA-P has been treated by deep brain stimulation (DBS) and showed no sustained improvement of clinical signs. We report a patient with a clinical misdiagnosed MSA-P, later confirmed by neuropathological study, who was improved by DBS for one year. CASE REPORT: A 63-year-old parkinsonian patient had been treated by levodopa for 6 years with a persistent good response. Over one year he progressively developed disabling fluctuations with severe axial syndrome and vegetative non motor symptoms in off periods. After checking usual contraindications, he was included in surgical procedure protocol (bilateral STN stimulation). During the first year after surgery, the clinical status improved with disappearance of non motor fluctuations, a 45 percent decrease of the OFF UPDRS III score, and a 39 percent reduction of the treatment. However after one year, axial symptoms reappeared with recurrent falls, as well as increasing dysarthry and swallowing difficulties which were only slightly improved by levodopa. He developed severe urinary disorders increased by a prostatic adenoma which led to surgical treatment. During the post operative period, 2 years after DBS, he died suddenly from an unexplained cause. A cerebral autopsy was performed and showed a good position of the two electrodes in the STN. Microscopic studies revealed severe neuronal depletion in the substantia nigra but no Lewy bodies. Immunohistochemical methods demonstrated numerous argyrophilic glial cytoplasmic inclusions positive for alpha-synuclein and ubiquitin in the STN, putamen, globus pallidus, pontine nuclei and cerebellar white matter, significant of MSA. CONCLUSION: This case shows that DBS can improve parkinsonian signs in MSA-P with persistent dopa sensitivity. However, probably because of striatal degeneration progression, this improvement is time limited and STN DBS cannot be recommended in MSA.

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PubMed1999

L-threo-3,4-dihydroxyphenylserine enhances the orthostatic responses of plasma renin activity and angiotensin II in multiple system atrophy.

We evaluated the effect of L-threo-3,4-dihydroxyphenylserine (L-threo-DOPS) on the response of blood pressure and vasoactive factors during head-up tilt in patients with multiple system atrophy (MSA). After the daily oral administration of 300 mg L-threo-DOPS for 2 weeks we observed a significant reduction in the decline of systolic blood pressure during 60 degrees head-up-tilt. In concordance with this result, we detected significant increases in postural changes in plasma renin activity and angiotensin II following L-threo-DOPS treatment. The enhanced responses of these vasoactive mediators by L-threo-DOPS during head-up tilt may contribute to the improvement in orthostatic hypotension in patients with MSA.

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