SEARCH · PubMed Health

Results for “Multiple system atrophy”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 253 records · Page 14Linked to original sources

PubMed2001

Reduced morning cortisol secretion in patients with multiple system atrophy.

To determine whether daytime or nighttime cortisol secretion is aberrant in patients with multiple system atrophy (MSA), the plasma cortisol concentrations in 20 patients with MSA were measured at 4-hr intervals for one day. Ten age-matched healthy individuals and 20 patients with cerebral infarction but without hypothalamic lesions were used as controls. The results showed that morning (8 A.M.) cortisol concentrations in patients with MSA were significantly lower than those in the control subjects. This phenomenon is highly associated with dysfunction in the autonomic nervous system in MSA.

Autonomic Nervous System↗

PubMed1993

Multiple system atrophy and progressive supranuclear palsy. Diminished striatal D2 dopamine receptor activity demonstrated by 123I-IBZM single photon emission computed tomography.

OBJECTIVE: To measure D2 dopamine receptors in the striatum in patients with multiple system atrophy and progressive supranuclear palsy by I 3-iodo-6-methoxybenzamide labeled with iodine I 123 (123I-IBZM) single photon emission computed tomography and differentiate them from control subjects. DESIGN: Survey with the following as retrospective criterion standards: (1) parkinsonism, (2) negative apomorphine test, and (3) no or only slight reaction to dopaminergic medication. SETTING: Ambulatory or hospitalized care in an academic referral center. PATIENTS AND CONTROL SUBJECTS: Twenty-one patients with parkinsonism not responding to dopaminergic medication (17 with multiple system atrophy and four with progressive supranuclear palsy) and 21 control subjects without parkinsonism. INTERVENTION: In vivo imaging by single photon emission computed tomography using the D2 dopamine receptor specific radioligand 123I-IBZM. MAIN OUTCOME MEASURE: Striatum/occipital cortex ratio of count rate density as semiquantitative measurement for striatal D2 dopamine receptor density. RESULTS: A highly significant loss of striatal uptake of 123I-IBZM was observed in the patients in comparison to the control subjects with little or no overlap between values. CONCLUSIONS: The hypothesized loss of D2 receptors in multiple system atrophy has been confirmed. Use of 123I-IBZM single photon emission computed tomography may be a cost-effective alternative to positron emission tomography in the differential diagnosis of parkinsonism and in the selection of patients for dopaminergic therapy.

Adult↗

PubMed1999

[An autopsy case of multiple system atrophy presenting with rapid progression of autonomic disturbance].

We report an autopsy case of multiple system atrophy (MSA) presenting with rapid progression of autonomic disturbance. He was admitted to our hospital because of gait disturbance and dysarthria. The patient was a Japanese man, who first noticed gait disturbance and dysarthria at age 58, followed by syncope 3 months later. He developed urinary incontinence and frequency of urination 8 months after the disease onset. His gait disturbance, dysarthria, syncope, and urinary symptoms progressed, and he was admitted to the department of neurology 1 year after the onset of the disease. He was clinically diagnosed as having MSA and was followed in the outpatient office. He deteriorated rapidly and was readmitted to the department of neurology 19 months after the onset of the disease. Physical examination showed orthostatic hypotension. Neurological examination revealed nystagmus, dysarthria of cerebellar type, increased deep tendon reflexes, bilateral positive Babinski signs, ataxic and spastic gait, mild right hypesthesia and hypalgesia, impotence, constipation, and urinary incontinence. Routine blood examination showed slight anemia, elevated BUN, GOT, and blood sugar. Electrocardiography revealed sinus tachycardia and chest rentogenogram showed cardiac enlargement. Brain MRI showed atrophy of cerebellum and pons, and lacunae in basal ganglia. Autonomic function tests revealed abnormal in head-up tilt test, and CVR-R in May and November 1995. However 123I-MIBG myocardial scintigraphy showed normal uptake of MIBG in May 1995 and decreased uptake in November 1995. He deteriorated rapidly and died in May 1996. Autopsy findings revealed not only prominent olivopontocerebellar and slight striatonigral lesions, but also autonomic lesions with massive appearance of glial cytoplasmic inclusions. He was pathologically verified as having MSA. In the present patient, autonomic nervous system, especially cardiac sympathetic nerve, deteriorated rapidly, which might result in short duration of the illness.

3-Iodobenzylguanidine↗

PubMed1997

Value of sphincter electromyography in the diagnosis of multiple system atrophy.

It is clinically important, to distinguish between idiopathic Parkinson's disease (IPD) and multiple system atrophy (MSA) not only because of the implications for prognosis but also because urinary incontinence is often an early troublesome feature of MSA and by making the correct neurological diagnosis inappropriate urological surgery may be avoided. Onuf's nucleus in the sacral cord is the location of the anterior horn cells innervating the sphincters, and it is among central nervous system sites affected by neuronal cell loss in MSA but not in IPD. A systematic analysis of motor units recorded from the sphincter looking for changes of chronic reinnervation has therefore been used to distinguish between these conditions. Sphincter electromyography (EMG) was carried out in 126 patients with suspected MSA with review of their case notes up to 2 years later. Of those in whom a diagnosis of MSA was made, 82% had had an abnormal sphincter EMG.

Adult↗

PubMed2003

Riluzole and blood pressure in multiple system atrophy.

Riluzole is a neuroprotective agent that is currently tested for the treatment of multiple system atrophy (MSA). Riluzole may influence afferent and efferent parts of the baroreflex due to glutamate antagonistic effects. The effect of riluzole on the efferent part may be unmasked in MSA patients with dysfunction of afferent structures of the baroreflex. We compared the effect of a single dose of 200 mg riluzole with placebo in 10 patients with probable MSA. Brachial blood pressure and heart rate were recorded at baseline and for 120 minutes every 5 minutes after ingestion of riluzole. For determination of spontaneous baroreflex sensitivity, continuous finger blood pressure and ECG were recorded. Cardiac stroke volume was monitored using impedance cardiography. The change in blood pressure over a two hour period was significantly greater with riluzole than with placebo (5 +/- 5/2 +/- 3 mmHg with placebo, 16 +/- 6/10 +/- 2 mmHg with riluzole, p < 0.001 by ANOVA). Systemic vascular resistance increased 32 +/- 6% with riluzole. Baroreflex sensitivity, the high and low frequency components of heart rate variability, and the low frequency component of systolic blood pressure variability were not different between placebo and riluzole treatment. We conclude that in MSA patients, manipulation of glutamatergic transmission with riluzole elicits a moderate pressor response. The response is explained by a marked increase in systemic vascular resistance. We propose that decreased inhibition of efferent sympathetic neurons may contribute to the response.

Aged↗

PubMed2005

Heredity in multiple system atrophy.

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.

Adult↗

PubMed2005

Sleep disorders in multiple system atrophy.

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.

Animals↗

PubMed2006

Cognitive function in multiple system atrophy of the cerebellar type.

Dementia represents an exclusion criterion for the diagnosis of multiple system atrophy (MSA), but there have been reports of fronto-executive dysfunction in patients with MSA of the striatonigral type (MSA-P). To study the cognitive profile of MSA, 20 patients with MSA of the cerebellar type (MSA-C) were subjected to an extensive neuropsychological test battery comprising tests for IQ, attention, verbal and visuospatial memory, as well as executive function. There was evidence for impaired verbal memory and verbal fluency. Test performance was not related to the severity of motor disability. Regarding the similar cognitive syndrome of MSA-P, the otherwise subclinical problems in MSA-C result from subcortical rather than from cerebellar dysfunction.

Age of Onset↗

PubMed2000

Evoked potentials in multiple system atrophy (MSA).

OBJECTIVES: To study the involvement of pyramidal tracts and sensory pathways in multiple system atrophy (MSA). MATERIALS AND METHODS: Evoked potential studies were performed in 45 MSA patients suffering from either MSA of cerebellar type (MSA-C) or MSA of parkinsonian type (MSA-P). RESULTS: Motor evoked potentials were normal in all MSA patients, whereas visual and somatosensory evoked potential abnormalities were found in about 40% of the MSA patients with no significant difference between the cerebellar (MSA-C) and parkinsonian (MSA-P) subgroup. Abnormal latencies of wave III in brainstem auditory evoked potentials were significantly more frequent in MSA-C. CONCLUSIONS: Abnormalities of somatosensory, visual and auditory evoked potentials are frequent findings in MSA, whereas abnormal motor evoked potentials are not a characteristic feature of the disease.

Adult↗

PubMed2001

Stimulation of growth hormone release in multiple system atrophy, Parkinson's disease and idiopathic cerebellar ataxia.

Clonidine has been proposed to differentiate multiple system atrophy (MSA) from idiopathic Parkinson's disease (IPD), as it does not increase growth hormone (GH) release in MSA. We studied GH release in response to clonidine in 7 IPD patients, 6 MSA patients, 4 patients affected by idiopathic late-onset cerebellar ataxia (ILOCA) and 8 healthy controls. In addition, we investigated the effects of GH releasing hormone plus arginine (GHRH-Arg) on GH release in the same patients. Both clonidine and GHRH-Arg raised serum GH levels in all groups examined. Clonidine failed to differentiate MSA from IPD and ILOCA. GHRH-Arg showed a lower increase of serum GH in MSA patients than in other groups, even if such difference was not statistically significant. We suggest that stimulation of GH release with GHRH-Arg rather than clonidine could differentiate MSA from IPD and ILOCA, but this hypothesis would need to be confirmed by further investigations.

Adrenergic alpha-Agonists↗

PubMed2004

Accumulation of phosphorylated alpha-synuclein in the brain and peripheral ganglia of patients with multiple system atrophy.

We immunohistochemically examined the brain and peripheral sympathetic ganglia from eight patients with multiple system atrophy (MSA), using an antibody specific for phosphorylated alpha-synuclein (anti-PSer129). Phosphorylated alpha-synuclein was deposited in five cellular locations: oligodendroglial cytoplasm and nucleus, and neuronal cytoplasm, processes and nucleus. Many neuronal cytoplasmic inclusions (NCIs) were found in the pontine and inferior olivary nuclei and, to a lesser extent, in the substantia nigra, locus ceruleus, and neocortical and hippocampal neurons. NCIs were also found in the sympathetic ganglia in two out of the eight cases. Moreover, anti-PSer129 immunohistochemistry revealed extensive neuropil pathology; swollen neurites were abundant in the pontine nucleus, delicate neurites were observed in the deeper layers of the cerebral cortex and thalamus, and neuropil threads and dot-like structures were distributed in the basal ganglia and brainstem. Diffuse neuronal cytoplasmic staining (pre-NCI) was frequently found in the pontine and inferior olivary nuclei. Thus, the widespread accumulation of phosphorylated alpha-synuclein in both glial and neuronal cells is a pathological feature in patients suffering from MSA.

Aged↗

PubMed2005

Impaired circadian rhythm of gastric myoelectrical activity in patients with multiple system atrophy.

In order to evaluate gastric motility and its circadian rhythm in patients with multiple system atrophy (MSA) and healthy control subjects, we measured gastric myoelectrical activity (GMA) for 24 hours using a cutaneous electrogastrogram (EGG) recorder in 14 MSA patients and 9 age-matched controls. We analyzed six 10-minute segments of EGG before and after each meal and two 20-minute EGG segments during sleep; three parameters were used for the analysis: dominant frequency (DF), instability coefficient of dominant frequency (ICDF), and dominant power (DP). DF increased during daytime and decreased during sleep in the control, while this circadian variation was blunted in the patients with MSA. The average DF of the eight segments in the MSA patients did not differ from that of the control. Both MSA patients and control subjects did not show the circadian variation of ICDF and DP. The average ICDF of the eight segments in the patients with MSA was significantly decreased when compared with that of the control (p < 0.01). No differences were observed in DP between the two groups. This study indicates that the healthy subjects appear to have a circadian rhythm of DF, and the patients with MSA appear to have impaired circadian rhythm of DF and decreased ICDF possibly due to the degeneration of the central autonomic neurons.

Circadian Rhythm↗

PubMed2003

[Clinical and electromyographical analysis of peculiarities of parkinsonian syndrome in multiple system atrophy and Parkinson's disease].

Because differential diagnosis between Parkinson's disease (PD) and multiple system atrophy (MSA) may be difficult due to overlapping of clinical features, especially at the early stage of the diseases, a search for additional clinical and instrumental markers increasing reliability of etiological diagnosis appears actual. The article presents the results of comprehensive comparison of parkinsonian signs and spectral electromyography (sEMG) data in 18 patients with MSA, diagnosed clinically according to criteria of Gilman et al (1998), and in 21 PD patients. Though no between-group differences in total expression of parkinsonian signs evaluated with The Unified Parkinson's Disease Rating Scale, part III, were found, MSA patients demonstrated progressive motor deficits, severer hypokinesia in the distal regions of the extremities, more pronounced impairment of axial movements, postural instability and gait disturbances but milder resting tremor, as compared to the PD patients (p < 0.05). Besides, the MSA patients were more frequently resistant to levodopa and had axial drug-induced dyskinesia (p < 0.05). An analysis of sEMG data revealed that MSA patients had a more prominent peak in 10-17 Hz frequency band (for arm muscles) and in Hz 7-14 (for leg muscles) as well as higher amplitudes peak frequencies (p < 0.05) positively correlated to hypokinesia severity. The results may be useful for differential diagnosis between PD and MSA.

Diagnosis, Differential↗

PubMed2004

Differentiation of idiopathic Parkinson's disease, multiple system atrophy, progressive supranuclear palsy, and healthy controls using magnetization transfer imaging.

The differentiation of multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) from idiopathic Parkinson's disease (IPD) is difficult. Magnetization transfer imaging (MTI), a measure that correlates with myelination and axonal density, was employed in this study in the attempt to distinguish between these disorders. Measurements were carried out in 15 patients with IPD, 12 patients with MSA, 10 patients with PSP, and in 20 aged-matched healthy control subjects. The main finding was a change in the magnetization transfer ratio in the globus pallidus, putamen, caudate nucleus, substantia nigra, and white matter in IPD, MSA, and PSP patients, matching the pathological features of the underlying disorder. Furthermore, stepwise linear discriminant analysis provided a good classification of the individual patients into the different disease groups. All IPD patients and control subjects were correctly separated from the MSA and PSP cohort, and all PSP patients and 11 of 12 MSA patients were correctly separated from the IPD and control cohort. There was also a fairly good discrimination of IPD patients from control subjects and of MSA from PSP patients. In conclusion, MTI revealed degenerative changes in patients with different parkinsonian syndromes matching the underlying pathological features of the different diseases, underlining the high potential of this method in distinguishing MSA and PSP from IPD.

Aged↗

PubMed1992

Immunocytochemical and ultrastructural studies of neuronal and oligodendroglial cytoplasmic inclusions in multiple system atrophy. 1. Neuronal cytoplasmic inclusions.

Neuronal alterations in five cases of multiple system atrophy (MSA) were investigated histologically, immunocytochemically and ultrastructurally. Argentophilic neuronal cytoplasmic inclusions (NCIs) were observed in all cases. They were distributed, in order of decreasing frequency, in the pontine nucleus, striatum, subiculum, amygdala, hippocampus, dentate fascia, substantia nigra and inferior olivary nucleus. Anti-ubiquitin antibodies visualized many thickened neurites in the degenerating gray matter as well as NCIs. Some NCIs were also recognized by anti-phosphorylated neurofilament antibodies. Ultrastructurally, NCIs consisted of a meshwork of granule-associated filaments, the diameter ranging from 18 to 28 nm, that were mixed with neurofilaments. The granule-associated filaments were also present in the axoplasm of myelinated fibers. Our studies demonstrate widespread distribution of NCIs in the central nervous system of MSA. The same pathological process that forms the granule-associated filaments in axons may also be responsible for the formation of ubiquitin-positive thickened neurites. These axonal alterations, as well as neuronal perikaryal changes, may play an important role in the impaired neuronal function in MSA.

Adult↗

PubMed1993

Multiple system atrophy. Clinical and MR observations on 42 cases.

Probable or possible multiple system atrophy (MSA) was diagnosed on strict clinical criteria in 42 patients: 20 with combined parkinsonism and cerebellar ataxia, 9 with striatonigral degeneration (SND) and 13 with olivopontocerebellar atrophy (OPCA). All patients were then studied with 0.5 and/or 1.5 Tesla magnetic resonance (MR) units. MR imaged putaminal abnormalities in all 9 patients with SND and posterior fossa abnormalities consistent with OPCA in all 13 patients with this diagnosis. Of the 20 patients with parkinsonism and cerebellar involvement, classified as probable MSA, 7 presented putaminal abnormalities only, 3 abnormalities consistent with OPCA only and 10 a combination of both. These findings show strong MRI support for the clinical diagnosis of MSA.

Adult↗

PubMed1996

Depression in multiple system atrophy and in idiopathic Parkinson's disease: a pilot comparative study.

Multiple system atrophy (MSA) is a disease causing parkinsonism in which response to levodopa is classically absent, poor, or transient. Idiopathic Parkinson's disease (IPD) itself, which responds favorably to levodopa, has been associated with the development of disease-related depression. Over and above the clinical and pathological characteristics of IPD, MSA causes additional, more widespread, clinical and pathological deficits. We have compared motor disability and mood in 12 patients with MSA and 12 with IPD. There was more severe motor disability, but no clinical evidence of depression among the MSA patients studied, and their Beck Depression Inventory scores did not differ significantly from the group with IPD. We conclude that depression does not appear to be more common in MSA than in IPD.

Activities of Daily Living↗

PubMed1996

Paroxysmal staring and masticatory automatisms during postural hypotension in a patient with multiple system atrophy.

PURPOSE: We studied a 51-year-old man with multiple system atrophy and autonomic insufficiency. He had repeated episodes of loss of contact, staring, and masticatory automatisms. METHODS: Blood pressure during these events documented a systolic pressure of 60 mm Hg. Cardiovascular reflex tests provided evidence of autonomic failure. Head computed tomography (CT) revealed moderate, diffuse cortical and cerebellar atrophy. RESULTS: These events were strictly related to blood pressure decreases and could be reproduced consistently by having the patient sit up after a meal. Ictal polygraphic recordings showed EEG changes consistent with anoxia, preceded by sudden hypotension with fixed heart rate. CONCLUSIONS: Cerebral anoxia during a syncopal attack may therefore precipitate transient, sudden neurologic dysfunction that closely mimics complex partial seizures. Masticatory automatisms may represent a release phenomenon resulting from inactivation of neocortical structures by cerebral anoxia or reticular disconnection.

Automatism↗