PubMed Health⌕ Search

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 577 records · Page 32Linked to original sources
PubMed2004

99mTc-TRODAT-1 imaging of multiple system atrophy.

UNLABELLED: Using (99m)Tc-TRODAT-1 ((99m)Tc-[2[[2-[[[3-(4-chlorophenyl)-8-methyl-8-azabicyclo[3,2,1]-oct-2-yl]-methyl](2-mercaptoethyl) amino]ethyl]amino]ethane-thiolato(3-)-N2,N2',S2,S2]oxo-[1R-(exo-exo)])) brain SPECT imaging, we measured striatal dopamine transporters (DATs) activity in multiple system atrophy (MSA) to investigate the possibility of differentiating it from Parkinson's disease (PD) and to correlate the findings with the parkinsonian (MSA-P) and cerebellar (MSA-C) subtypes of MSA. METHODS: Forty-nine patients with probable MSA (30 MSA-P, 19 MSA-C), a disease control group of 36 age- and disease duration-matched patients with probable PD, and a healthy control group of 33 age-matched healthy volunteers participated in a SPECT study. The ratios of specific striatal binding-to-nonspecific occipital binding, including the striatum-to-occipital ratio (S/O), putamen-to-occipital ratio (P/O), caudate nucleus-to-occipital ratio (C/O), and putamen-to-caudate nucleus ratio (P/C), were calculated. The statistical analyses of uptakes among 4 groups used ANOVA followed by Games-Howell's multiple comparisons. The Spearman correlation coefficient between the motor scores of Unified Parkinson's Disease Rating Scale (UPDRS-III) and those binding ratios of the MSA-P and MSA-C groups and the PD group was also performed. RESULTS: The striatal binding was more symmetrically reduced in the MSA-P (asymmetric index, 14.2) and MSA-C (asymmetric index, 8.1) groups, in contrast to the greater asymmetric reduction in the PD group (asymmetric index, 28.6). Overall striatal binding was significantly reduced in the MSA-P (-59.8%), MSA-C (-29.9%), and PD (-58.0%) groups with no overlap between these values and those of the control group. Like the PD group, bilateral P/O, C/O, and S/O ratio values were significantly reduced in the MSA-P and MSA-C groups. Nevertheless, the reduction of bilateral P/O and S/O ratios was more for the MSA-P group than for the MSA-C group. P/C ratios showed that the MSA-P and PD groups had similar patterns of nigral impairment, but the MSA-C group had a different pattern. No correlation between the UPDRS-III scores and (99m)Tc-TRODAT-1 bindings was found in both MSA-P and MSA-C groups; in contrast, a significant negative correlation was noted in the PD group. CONCLUSION: (99m)Tc-TRODAT-1 brain SPECT is capable of scientifically differentiating between the MSA-P and MSA-C subtypes, and MSA-P has more symmetric nigrostriatal damage than that in PD. (99m)Tc-TRODAT-1 brain SPECT imaging probably could provide important information to differentiate MSA from PD.

Corpus Striatum↗
PubMed2004

Failure of caspase inhibition in the double-lesion rat model of striatonigral degeneration (multiple system atrophy).

In the present study we assessed the neuroprotective effects of the pan-caspase inhibitor z-VAD.fmk [N-benzyloxycarbony-valine-alanine-aspartate-(OMe)-fluoromethylketone], and the caspase-3 inhibitor Ac-DEVD.CHO (acetyl-aspartate-chloromethylketone) in the double-lesion rat model of striatonigral degeneration (SND), the core pathology underlying levodopa-unresponsive parkinsonism associated with multiple system atrophy (MSA). Male Wistar rats were divided into three groups, receiving either Ac-DEVD.CHO, z-VAD.fmk or normal saline before lesion surgery, comprising a sequential unilateral quinolinic acid (QA) lesion of the striatum followed by a 6-hydroxydopamine (6-OHDA) lesion of the ipsilateral medial forebrain bundle. At 2 weeks post lesion, all rats underwent testing of spontaneous nocturnal locomotor behavior in an automated Photobeam Activity System (FlexField). Immunohistochemistry was performed with tyrosine hydroxylase, dopamine and cyclic adenosine 3',5'-monophosphate-regulated phosphoprotein and glial fibrillary acidic protein antibodies. Morphometry was performed using computerized image analysis. Behavioral and morphological analysis failed to show striatal or nigral protection in caspase inhibitor-treated animals. Our findings suggest that anti-apoptotic strategies are unrewarding in the SND rat model and, therefore, alternative neuroprotective interventions such as anti-glutamatergic agents or inhibitors of microglial activation should be explored instead.

Amino Acid Chloromethyl Ketones↗
PubMed2006

The validity of a hyperventilation test for an investigation of autonomic failure: assessment in patients with multiple system atrophy and Parkinson's disease.

Although heart rate (HR) responses to hyperventilation (HV) have been used as a cardiovascular autonomic function test, autonomic involvement during HV remains uncertain. To clarify the relationship between autonomic activity and cardiovascular changes during HV, we compared cardiovascular responses during HV among subjects with different autonomic function, namely 16 patients with probable multiple system atrophy (MSA), 16 with possible MSA, 28 with Parkinson's disease (PD) and 28 healthy controls. Abnormalities of cardiovascular responses to head-up postural change and the Valsalva maneuver were definitely present in the order of probable MSA, possible MSA and PD, and abnormal HR and blood pressure (BP) responses during HV were observed in probable MSA and possible MSA, but not in PD. Unlike the significant difference in standard cardiovascular autonomic function tests, the HR and BP responses during HV were equivalent between probable and possible MSA. These findings suggest that cardiovascular control during HV may be affected not only by autonomic activity but also by other factors.

Adult↗
PubMed2004

The role of functional neuroimaging in the differential diagnosis of idiopathic Parkinson's disease and multiple system atrophy.

Parkinsonism is a symptom of a number of neurodegenerative disorders in the elderly. Even though clinical criteria for various parkinsonian disorders have been developed recently, the differential diagnosis of parkinsonian disorders based on clinical symptoms remains unsatisfactory, particularly in early disease stages. Early differential diagnosis on the other hand is important as prognosis and treatment options differ substantially. Multiple system atrophy (MSA) is one of the major differential diagnoses of idiopathic Parkinson's disease (PD). Radiotracer-based imaging methods such as positron emission tomography (PET) remain the established method for differential diagnosis of parkinsonian disorders. The following paper provides a review of different PET imaging methods for the differential diagnosis of PD and MSA patients.

Diagnosis, Differential↗
PubMed2000

Decreased striatal dopamine D2 receptor binding in amyotrophic lateral sclerosis (ALS) and multiple system atrophy (MSA): D2 receptor down-regulation versus striatal cell degeneration.

Recently, decreased striatal dopamine D2-receptor binding was demonstrated in vivo in amyotrophic lateral sclerosis (ALS). To further elucidate the pathogenetic mechanism underlying this D2-receptor deficit, a multi-level comparison was made between 30 sporadic ALS subjects and 24 patients with multiple system atrophy (MSA), a disorder clinically characterized by bradykinesia, neuroradiologically by severe D2-receptor loss, and neuropathologically by degenerating striatal cells. The extent of D2-deficit in ALS and MSA were within the same range, but extrapyramidal signs and symptoms were virtually absent in our ALS patients. Striatal cell loss in general or competitive D2-receptor occupancy could be considered unlikely in ALS. The striatum receives massive glutamatergic input and the pathogenesis of ALS may be related to increased glutamatergic excitotoxicity. As other mechanisms (cell loss, receptor occupancy) could be ruled out, and as animal studies suggest that (excess of) glutamate decreases striatal D2-receptor synthesis, the striatal D2-receptor deficit in ALS is most likely to be caused by a receptor down-regulation.

Amyotrophic Lateral Sclerosis↗
PubMed2001

Decrease of substance P in the parabrachial nucleus of multiple system atrophy.

In the parabrachial nucleus (PBN), which has been known to play an important regulating role for various autonomic functions, many projecting nerve fiber terminals containing substance P (SP) from the nucleus of the solitary tract (NTS) and other areas are found and effect a modulatory influence on the transmission in the PBN. Postmortem brains were obtained from four multiple system atrophy (MSA) patients with autonomic failure and four control patients without any nervous disease, and an immunohistochemical staining for SP was performed on serial 10-microm-thick sections from paraffin-embedded pons including the PBN after immersion fixation in 10% formalin. In the PBN of all MSA patients, a marked decrease in SP-like immunoreactive (SPLI) nerve fiber terminals was revealed compared with the controls. In addition, an obvious astrocytosis was found in the PBN by simultaneous histopathological evaluation, for the preservation of neurons themselves. Therefore, the projecting SP pathway to the PBN may also be primarily involved in the pathophysiological mechanism of the autonomic failure of MSA patients.

Autonomic Nervous System Diseases↗
PubMed2005

Camptocormia in a patient with multiple system atrophy.

The term "camptocormia" describes a severe forward-flexed posture. Although initially used to describe a conversion disorder, early authors also recognized organic camptocormia occurring in old age, or "camptocormie senile," as well as traumatic and arthritic camptocormia. More recently, camptocormia has been described in patients with Parkinson's disease and in an individual with parkinsonism. We describe a case of progressive camptocormia as part of the initial presentation of a patient with multiple system atrophy.

Antiparkinson Agents↗
PubMed2004

The effects of water ingestion on orthostatic hypotension in two groups of chronic autonomic failure: multiple system atrophy and pure autonomic failure.

BACKGROUND: Oral ingestion of water increases seated blood pressure in patients with chronic autonomic failure by mechanisms that remain unclear. As orthostatic hypotension is common in chronic autonomic failure, and is not always adequately controlled by medication, the potential benefits of water ingestion on standing blood pressure were studied in two types of autonomic failure: multiple system atrophy (MSA), in which the lesion is central and pre-ganglionic, and pure autonomic failure (PAF), in which the lesion is post-ganglionic. METHODS: In 14 patients with autonomic failure (seven PAF and seven MSA) standing blood pressure and heart rate were measured before, and 15 and 35 minutes after ingestion of 480 ml distilled water. Patients remained seated for 15 minutes after water ingestion, with beat to beat cardiovascular indices measured with the Portapres II device with subsequent Modelflow analysis. RESULTS: Standing prior to water ingestion caused a significant fall in blood pressure in all patients. After water ingestion there was a rise in seated blood pressure. Seated and standing blood pressure at 15 and 35 minutes after water ingestion was significantly higher than before water, with an improvement in orthostatic symptoms. The time to first significant rise in seated blood pressure occurred at 5 minutes post water ingestion in PAF and at 13 minutes in MSA. These increases were accompanied by increases in total peripheral resistance, reaching significance by 5 minutes in PAF and 13 minutes in MSA. There were no significant changes in cardiac output, stroke volume, or ejection fraction. CONCLUSIONS: Water is thus beneficial in improving standing BP in AF, acting within 15 minutes in both MSA and PAF. The earlier onset of the pressor effect in PAF may reflect the differing lesion site and underlying pathophysiology between these conditions.

Aged↗
PubMed1999

The combination of hypointense and hyperintense signal changes on T2-weighted magnetic resonance imaging sequences: a specific marker of multiple system atrophy?

OBJECTIVE: To compare the frequency and specificity of hypointense magnetic resonance imaging (MRI) signal changes alone with the frequency and specificity of a pathological MRI pattern consisting of a hyperintense lateral rim and a dorsolateral signal attenuation on T2-weighted MRIs in patients with parkinsonism of various origins. PATIENTS: Ninety patients with Parkinson disease (PD) (n = 65), progressive supranuclear palsy (PSP) (n = 10), and multiple system atrophy (MSA) of the striatonigral degeneration type (n = 15) underwent MRI. SETTING: University medical center. RESULTS: Nine of the 15 patients with MSA showed the pattern with hyperintense lateral rim and a dorsolateral hypointense signal attenuation on T2-weighted images within the putamen. This pattern was not found in the 65 patients with PD, nor in the 10 patients with PSP. Only hypointense changes in the putamen were found in 6 patients (9%) with PD, 4 patients (40%) with PSP, and 5 patients (36%) with MSA. CONCLUSIONS: Our data suggest that the pattern consisting of hypointense and hyperintense T2 changes within the putamen is a highly specific MRI sign of MSA, while hypointensity alone remains a sensitive, but nonspecific MRI sign of MSA. In clinically doubtful cases, the appearance of a hypointense and hyperintense signal pattern on MRI makes the diagnosis of PD very unlikely, while hypointense signal changes alone do not exclude idiopathic PD.

Aged↗
PubMed2001

Depletion of cholinergic neurons of the medullary arcuate nucleus in multiple system atrophy.

The human arcuate nucleus (ArcN) has been considered akin to the pontine precerebellar nuclei. However, there is anatomical, functional, and clinical evidence that the ArcN may be the homologue of chemosensitive areas of the ventral medullary surface involved in ventilatory responses to hypercarbia and cerebrospinal fluid acidosis. Acetylcholine has been involved in mechanisms of central chemosensitivity. Loss of ArcN neurons has been reported in patients with multiple system atrophy (MSA), a disorder characterized by disturbed automatic ventilation, but the neurochemical identity of these neurons is undetermined. We sought to determine whether the ArcN contains cholinergic neurons and whether these neurons are depleted in patients with MSA. Medullae were obtained from six patients with MSA, five patients with Parkinson's disease (PD) and six sex- and age-matched controls. Fifty-micron transverse sections obtained through the mid-olivary levels were processed for acetylcholinesterase (AchE), choline acetyltransferase (CAT), and alpha-synuclein immunoreactivity. We found that the ArcN contained CAT-positive neurons. There was a significant decrease in density of cholinergic ArcN neurons in MSA but not in PD patients. alpha-Synuclein-containing inclusions were present in the ArcN of MSA patients. Depletion of cholinergic neurons may provide a substrate for disturbances in automatic respiration in MSA patients.

Aged↗
PubMed1991

Differential effect of L-threo-3,4-dihydroxyphenylserine in pure autonomic failure and multiple system atrophy with autonomic failure.

Treatment with L-threo-3,4-dihydroxyphenylserine (L-threo-dops), a synthetic precursor of norepinephrine, significantly increased upright blood pressure in patients with multiple system atrophy but had no effect on the upright blood pressure of patients with pure autonomic failure. These results suggest that the site of action of L-threo-dops is central and that its pressor effect requires intact peripheral sympathetic neurons.

Adult↗
PubMed1994

Octreotide in the management of orthostatic hypotension in multiple system atrophy: pilot trial of chronic administration.

The aim of the study was to determine the potential effect of octreotide chronic administration on the functional prognosis of patients suffering from multiple system atrophy (MSA) with orthostatic hypotension (OH). Five patients received octreotide (100 micrograms s.c. injections t.i.d.) during 6 months in an open trial. Patients experienced functional improvement after 6 months of treatment. All of them continued to receive octreotide after the 6-month evaluation period (mean duration: 12 months).

Aged↗
PubMed2003

[Regional cerebral blood flow single photon emission tomography (SPECT) and magnetic resonance imaging (MRI) may be useful in the diagnosis of patients with cortico-basal degeneration, progressive supranuclear palsy and multiple system atrophy].

Differential diagnosis of idiopathic Parkinson's Disease is still very difficult. Even in movement disorders centers 25% of patients with clinical diagnosis of Parkinson's disease is misdiagnosed with other neurodegenerative disorders. Clinical symptoms of so called atypical parkinsonian disorders may emerge late in the course of the disease, not at the same time and good or moderate response to levodopa at early stages may be a source of misdiagnosis. Most difficult to differentiate seems to be Progressive supranuclear palsy (PSP), corticobasal degeneration (CBD) and multiple system atrophy (MSA). Authors present in selected cases usefulness of neuroimaging with rCBF SPECT and MRI in clinical diagnosis of these disorders. For PSP typical (although not pathognomonic) is decrease of metabolism and flow in frontal lobes (hypofrontalism), and in CBD asymmetrical, contralateral to the side of dominating symptoms cerebral (frontal, parietal, temporal and within striatum) atrophy. In MSA more useful (but not seen in all cases) is MRI examination with hyperintensities in putamen, pons and cerebellum or cerebellar atrophy. Due to low sensitivity of clinical criteria other tests (EMG of anal sphincter or clonidine test) with specially neuroimaging examination may be helpful in establishing of the diagnosis.

Aged↗
PubMed2003

Cerebrospinal fluid Abeta42 is reduced in multiple system atrophy but normal in Parkinson's disease and progressive supranuclear palsy.

The 42-amino-acid isoform of beta-amyloid Abeta42 in the cerebrospinal fluid (CSF) has recently been proposed as a biochemical marker for Alzheimer's disease (AD) and subcortical white-matter dementia (SWD). In both of these conditions, concentration of CSF-Abeta42 is reduced. We quantified CSF-Abeta42 from patients fulfilling strict clinical criteria for multiple system atrophy (MSA; n = 36), Parkinson's disease (PD; n = 48) and progressive supranuclear palsy (PSP; n = 15). The study groups were consecutively recruited among patients referred to a movement disorder unit, and 32 healthy, age-matched volunteers were used as controls. The CSF concentration of Abeta42 was significantly reduced in the MSA group (P < 0.001), whereas the PD and PSP groups did not differ from controls. On an individual basis, low content of Abeta42 was seen in 9 MSA patients regardless of age and disease duration. Three PD patients with long disease duration also had low concentrations but all PSP patients were normal. We conclude that the reduced CSF-Abeta42 concentration may be a clue to the pathogenesis of MSA. There is a decreased production, or more possible, an increased consumption of CSF-Abeta42. The analysis of this protein may also become a supplement to the clinical differentiation of parkinsonian syndromes in a movement disorder unit.

Aged↗
PubMed2006

[Utility of the study of the vegetative nervous system in the differential diagnosis between Parkinson's disease and multiple system atrophy].

INTRODUCTION: The aim of this study is to show if the exploration of the autonomic nervous system is useful to improve the specificity of clinical criteria of Parkinson's Disease (PD) and Multiple System Atrophy (MSA). PATIENTS AND METHODS: 20 patients with PD and 13 patients with MSA were studied. After 12 hours in off medication, NE and GH were measured in supine position and NE after 5 minutes standing. Later, GH levels were recorded at 15, 30, 45 and 60 minutes after a dose of 0.005 mg/kg of apomorphine. Finally, analysis of the symptoms of autonomic dysfunction and levodopa test were carried out. RESULTS: Sympathetic response to postural changes was significantly higher in patients with PD (NE increase in relation to basal: PD: 170.90 +/- 110.08 pg/ml; MSA: 91.33 +/- 73.79 pg/ml; p = 0.029). No differences were found in the response of GH to apomorphine (GH peak at 45 minutes: PD: 2.37 +/- 2.7 ng/ml; MSA: 1.69 +/- 1.90 ng/ml; ns). The symptoms of autonomic dysfunction were more frequently in patients with MSA. The stridor was specific to MSA. Improvement in motor scores in the levodopa test was higher in patients with PD (PD: 39.7 %; MSA: 17.89; p = 0.019). DISCUSSION: Sympathetic response to postural changes, description of symptoms of autonomic dysfunction, and motor response to levodopa test are useful tools in order to improve specificity of the diagnostic criteria of PD and MSA. The GH test with apomorphine was not useful for a differential diagnosis.

Aged↗
PubMed2005

[Segmental dysfunction of sweating in multiple system atrophy].

There are many different causes of anhidrosis. Sweat glands can be absent, atrophic or blocked; sympathetic innervation may be disturbed, or central nervous system damage may prevent perspiration. We present a patient with multiple system atrophy ( MSA) who presented with unilateral anhidrosis of the trunk. MSA is a sporadic neurodegenerative disorder of adults involving the pyramidal, extrapyramidal, cerebellar and/or autonomic system. The findings in our patient could be explained by a depletion of catecholaminergic sympathetic neurons of the thoracic spinal neurons.

Barbiturates↗
PubMed1997

Iodine-123-epidepride-SPECT: studies in Parkinson's disease, multiple system atrophy and Huntington's disease.

UNLABELLED: Epidepride is a benzamide derivative with very high affinity for D2 receptors, which, in its [123I]-labeled form, can be used for SPECT. The aim of this study was to evaluate the usefulness and accuracy of [123I]epidepride-SPECT for the differential diagnosis of movement disorders. METHODS: SPECT imaging with a triple-headed scintillation camera was performed in 9 patients with Parkinson's disease, 9 patients with probable multiple system atrophy (MSA), 1 patient with progressive supranuclear palsy, 16 patients with Huntington's disease (HD) and 14 controls, 3 hr after the intravenous injection of 3.7 +/- 1.3 mCi of [123I]epidepride. The striatum-to-cerebellum ratio - 1, reflecting the specific-to-nondisplaceable binding ratio, was used as a semiquantitative measure of D2 receptor binding. RESULTS: Kinetic studies showed peak striatal uptake about 3 hr postinjection and a slow decline thereafter. The striatum-to-cerebellum ratio - 1 was significantly reduced in MSA (11.8 +/- 3.9, compared to controls, 19.0 +/- 6.3; p < 0.01) and in patients with HD (8.8 +/- 3.2; p < 0.00005) but normal in Parkinson's disease (15.8 +/- 3.6; not significant). A high interindividual variation of specific striatal epidepride binding (striatum - cerebellum; cpm/mCi x kg) was found in controls and in all patient groups. The interindividual variation of striatum-to-cerebellum ratios was lower but still considerable. In half of the MSA patients, the specific-to-nondisplaceable binding ratio fell within the range of controls. The use of various cortical reference regions did not improve discrimination between MSA and controls or Parkinson's disease patients, respectively. The discrimination of HD patients from controls was better, with overlap in only two cases. In one HD patient, calculation of the striatum-to-cerebellum ratio was almost impossible due to extremely low nonspecific binding. Possible explanations for the large variation of the ratios, resulting in an overlap between controls and different patient groups, are very low counting rates in the reference region and the fact that a transient binding equilibrium may not be achieved after bolus injection of epidepride. CONCLUSION: Epidepride appears to be a useful SPECT ligand for studying dopamine D2 receptors. However, its markedly higher specific-to-nondisplaceable binding ratio in comparison to those of iodobenzamide or other D2 ligands did not result in a better discrimination between different basal ganglia disorders. The calculation of plasma input curves and volumes of distribution might improve the accuracy of [123I]epidepride-SPECT.

Adult↗