Sphincter EMG in possible multiple system atrophy: to do or not to do?
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Anal Canal↗
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Results for “Multiple System Atrophy”
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Dystonia in multiple system atrophy.
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Diagnosis, Differential↗
Cardiac uptake of [123I]MIBG separates PD from multiple system atrophy.
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3-Iodobenzylguanidine↗
Case 27-2004: multiple-system atrophy.
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Female↗
Exaggerated auditory startle responses in multiple system atrophy: a comparative study of parkinson and cerebellar subtypes.
OBJECTIVE: Auditory startle responses (ASRs) have recently been reported to be exaggerated in cranial and peripheral nerve supplied muscles of patients with multiple system atrophy (MSA). ASRs displayed increased probability, amplitude and duration, shorter onset latency, and reduced habituation in comparison with healthy subjects. In order to investigate whether certain ASR features may differentiate MSA subtypes, the authors studied ASRs in 21 MSA patients (olivopontocerebellar type, MSA-C: n = 8, striatonigral type, MSA-P: n = 13), and 17 age-matched normal controls. METHODS: ASRs were elicited by binaural high-intensity auditory stimuli which differed randomly in tonal frequency and intensity (250 Hz, 90 db; 500 Hz, 105 db; 750 Hz, 105 db; 1000 Hz, 110 db normal hearing level), presented through tubal insert phones. Reflex electromyographic activity was simultaneously recorded with surface electrodes from masseter, orbicularis oculi, sternocleidomastoid, biceps brachii, abductor pollicis brevis, rectus femoris, tibialis anterior, and soleus muscles. RESULTS: Eighteen MSA patients (86%) had exaggerated ASRs as compared to normal subjects. At group level, indices of ASR disinhibition including increased ASR probability (in extremity muscles), shortened onset latency, and enlarged response magnitude were significantly more marked in MSA-P as compared to MSA-C patients. ASR probability showed habituation in normal subjects, less in MSA-P. and none in MSA-C patients. Three MSA-patients had no ASRs except in orbicularis oculi muscle. CONCLUSIONS: Although absent ASRs may occur in some MSA patients, most of them exhibit exaggerated ASRs. This finding may reflect disinhibition of lower brainstem nuclei due to the degenerative disorder. ASRs were significantly more disinhibited in MSA-P versus MSA-C. suggesting involvement of different neural structures in the two MSA-subtypes.
Acoustic Stimulation↗
Clinical/metabolic correlations in multiple system atrophy. A fludeoxyglucose F 18 positron emission tomographic study.
OBJECTIVE: To evaluate the regional cerebral metabolic involvement; the relationships among regional brain metabolism, clinical features, and quantitative measures of disease severity; and the patterns of brain involvement that can be related to the different types of onset: striatonigral degeneration vs olivopontocerebellar atrophy. DESIGN: Fludeoxyglucose F 18 positron emission tomography (PET) studies performed in patients with multiple system atrophy (MSA) were evaluated for their clinical features at the onset of the disease and at the time of the PET study. CASES: Seventeen patients diagnosed as having probable MSA and 10 age-matched controls. RESULTS: The hypometabolism in the putamen-pallidum complex and in the cerebellum was the best discriminant for disease classification. The efficacy of levodopa treatment was positively correlated with the metabolic activity of the putamen-pallidum complex. The patients with olivopontocerebellar atrophy type (N = 8) had a prevalent hypometabolism in the cerebellum, while the patients with striatonigral degeneration type (N = 9) had a prevalent impairment in the pallidum-putamen complex. We demonstrated a negative correlation between (1) severity of parkinsonism and metabolic values of putamen and caudate; (2) severity of cerebellar signs and metabolism in the cerebellum; and (3) autonomic dysfunction and metabolic activity in the thalamus, frontal, and temporal regions, bilaterally. CONCLUSIONS: These findings support the selective metabolic reduction in the putamen and cerebellum as a marker of MSA. The clinical/metabolic correlations, demonstrating the expected dependence of extrapyramidal and cerebellar signs by dysfunction of basal ganglia and cerebellum, also support a possible involvement of central nervous system structures in autonomic control.
Atrophy↗
[Iodine 123-labeled meta-iodobenzylguanidine myocardial scintigraphy in the cases of idiopathic Parkinson's disease, multiple system atrophy, and progressive supranuclear palsy].
A 20-25% rate of antemortem misdiagnosis of idiopathic Parkinson's disease (PD) suggests that it may be difficult to clinically differentiate PD from other degenerative diseases of the central nervous system, such as progressive supranuclear palsy (PSP) or multiple system atrophy (MSA). Iodine-123 meta-iodobenzylguanidine ([123I] MIBG), an analogue of norepinephrine, is a tracer for functioning of sympathetic neurons. To investigate cardiac sympathetic function in PD, MSA, and PSP, [123I] MIBG myocardial scintigraphy was performed in 25 patients with PD, 25 patients with MSA, 14 patients with PSP, and 20 control subjects. In planar imaging studies, the heart-to-mediastinum average count ratio (H/M) was calculated for both early and delayed images. The mean value of H/M in patients with PD was significantly lower than in those with MSA, PSP, or no disease (p < 0.0001). Regardless of disease severity or intensity of anti-parkinsonian pharmacotherapy, mean values for H/M were always low in patients with PD. The mean values of H/M in patients with MSA and PSP were significantly lower than in controls (p < 0.01). There was no significant difference between the mean value of H/M in MSA with orthostatic hypotension (OH) and that in MSA without OH, and also there was no significant difference between the mean value of H/M in MSA with striatonigral degeneration and that in MSA with olivopontocerebellar atrophy. Although the mean value of H/M in PSP with amitriptyline treatment was significantly lower than that in PSP patients without amitriptyline treatment (p < 0.005), there was no significant difference between the mean value of H/M in PSP patients without amitriptyline treatment and that in controls. There was no correlation between H/M and disease duration in those three akinetic-rigid disorders that we have studied here. Thus, PD may have an abnormality of cardiac sympathetic function which has not been detected by previous cardiovascular autonomic studies. Particularly in early stages, [123I] MIBG myocardial scintigraphy may help to differentiate PD from MSA and PSP.
3-Iodobenzylguanidine↗
Glial cytoplasmic inclusions in white matter oligodendrocytes of multiple system atrophy brains contain insoluble alpha-synuclein.
Recently, alpha-synuclein was shown to be a structural component of the filaments in Lewy bodies (LBs) of Parkinson's disease (PD), dementia with LBs (DLB) as well as the LB variant of Alzheimer's disease, and this suggests that alpha-synuclein could play a mechanistic role in the pathogenesis of these disorders. To determine whether alpha-synuclein is a building block of inclusions in other neurodegenerative movement disorders, we examined brains from patients with multiple system atrophy (MSA) and detected alpha-synuclein, but not beta- or gamma-synuclein, in glial cytoplasmic inclusions (GCIs) throughout the MSA brain. In MSA white matter, alpha-synuclein-positive GCIs were restricted to oligodendrocytes, and alpha-synuclein was localized to the filaments in GCIs by immunoelectron microscopy. Finally, we demonstrated that insoluble alpha-synuclein accumulated selectively in MSA white matter with alpha-synuclein-positive GCIs. Taken together with evidence that LBs contain insoluble alpha-synuclein, our data suggest that a reduction in the solubility of alpha-synuclein may induce this protein to form filaments that aggregate into cytoplasmic inclusions, which contribute to the dysfunction or death of glial cells as well as neurons in neurodegenerative disorders with different phenotypes.
Actin Cytoskeleton↗
Transient left ventricular apical ballooning at the onset of multiple system atrophy.
A 64-year-old woman was admitted to our hospital for persistent lipothymia and exertional dyspnoea and was treated for worsening asthma. During hospital stay, she manifested typical chest pain, with electrocardiographic and echocardiographic abnormalities suggesting acute myocardial infarction. Coronary angiography demonstrated normal coronary arteries and left ventriculography revealed apical akinesis. Creatine kinase levels showed a slight increase in spite of the severe ventricular abnormalities. The electrocardiographic and echocardiographic evolution, along with the favourable outcome, led us to diagnose tako-tsubo cardiomyopathy. Afterwards, severe autonomic dysfunction with multiple system atrophy was diagnosed. Impaired multivessel coronary microcirculation is thought to be one causative mechanism of tako-tsubo-like left ventricular dysfunction, and catecholamines are likely to play a role. In our case, sympathetic neurocirculatory failure was indicative of altered sympathoneural activity. We suggest that the prescribed therapy contributed to the development of this syndrome; in particular dopamine for hypotension and corticosteroids for suspected asthma stimulated heart sympathetic terminals.
Coronary Angiography↗
QT interval and QT dispersion in multiple system atrophy (Shy-Drager syndrome).
To evaluate the influence of autonomic function on the QT interval and QT dispersion, 18 patients (10 males and 8 females; mean age 61 +/- 9 years) with multiple system atrophy (MSA, Shy-Drager syndrome) were studied. Cardiovascular tests were performed to assess the degree of autonomic dysfunction. The QT interval, corrected QT (QTc), QT dispersion (QTd), corrected and adjusted QTd were calculated from a standard 12-lead electrocardiogram. Fifteen healthy subjects matched for sex and age were studied as controls. Nine MSA patients showed severe autonomic dysfunction with orthostatic hypotension. In the remaining patients definite autonomic impairment was found. No statistically significant difference was found in QTd and only a trend towards higher values of maximal QTc was found in patients compared with controls. QTc prolongation, defined as greater than the mean +/- 2 SD of the controls, was detected only in three out of the 18 MSA patients (17%). No correlation was found between the severity of autonomic impairment and repolarization parameters. Our data suggest that chronic autonomic impairment in patients with MSA does not significantly affect ventricular repolarization and ventricular dispersion.
Adult↗
Distinction of idiopathic Parkinson's disease from multiple-system atrophy by stimulation of growth-hormone release with clonidine.
BACKGROUND: Idiopathic Parkinson's disease is a common neurodegenerative disease that is difficult to distinguish from other parkinsonian syndromes such as multiple-system atrophy (MSA). In MSA, autonomic dysfunction is common and is associated with either parkinsonian or cerebellar features, or both. Differentiation of idiopathic Parkinson's disease from MSA is important because prognosis, complications, and response to therapy vary according to disorder. Our aim was to find out whether clonidine/growth hormone (GH) testing distinguishes idiopathic Parkinson's disease from MSA. METHODS: Clonidine is a centrally active alpha 2-adrenoceptor agonist that raises concentrations of GH in serum in healthy people and those with pure autonomic failure (with peripheral lesions), but not in those with MSA (with a central autonomic deficit). We investigated the effects of clonidine on 14 people with idiopathic Parkinson's disease (without autonomic deficits). 31 people with MSA of the three different clinical forms (parkinsonian, cerebellar, and mixed), 19 people with pure autonomic failure, and 27 healthy participants. In nine people with parkinsonian MSA (MSA-P), the GH response to levodopa was also assessed. FINDINGS: Clonidine raised serum GH concentrations in patients with idiopathic Parkinson's disease (median increase 8.98 [IQR 6.6-16.6] mU/L), normal participants (13.2 [7.0-18.6] mU/L), and patients with pure autonomic failure (12.5 [5.6-18.2] mU/L). In those with MSA who had central autonomic failure, GH concentrations were unchanged (MSA-P; 0.41 [-0.30 to 2.09] mU/L and cerebellar MSA [MSA-C] 1.67 [0-4.49] mU/L). The GH response to clonidine in idiopathic Parkinson's disease was significantly different from that in MSA-P (p < 0.0002). In MSA-P, the dopamine precursor levodopa raised GH concentrations (from mean 2.7 [SE 1.0] mU/L to mean 18.2 [6.0] mU/L, p < 0.05) and GH-releasing hormone (GHRH) concentrations (from mean 20.6 [3.25] ng/L to mean 68.0 [10.6] ng/L, p < 0.05), excluding dysfunction of pituitary somatotrophs or GHRH neurons as a cause for the absent GH response to clonidine in MSA. INTERPRETATION: The GH responses to clonidine clearly differentiated idiopathic Parkinson's disease from MSA-C and MSA-P. Together with the levodopa studies they indicated a specific alpha 2-adrenoceptor-hypothalamic deficit in MSA. The clonidine-GH test may provide further insight into central neurotransmitter and alpha 2-adrenoceptor-hypothalamic abnormalities in MSA.
Adrenergic alpha-Agonists↗
The use of lisuride in the treatment of multiple system atrophy with autonomic failure (Shy-Drager syndrome).
In a controlled trial lisuride, an ergolene derivative with dopamine receptor agonist properties was given maximum tolerated doses (2.4 mg/day) to seven patients with multiple system atrophy with autonomic failure (Shy-Drager syndrome). Improvement in Parkinsonian features occurred in only one patient and another patient who had been deriving marked benefit from levodopa treatment before the study began failed to respond to large doses of lisuride. Psychiatric side effects (including nightmares, isolated visual hallucinations and toxic confusional states) were the dose-limiting factor in six patients. A modest reduction in orthostatic hypotension occurred in two patients, one of whom had experienced an aggravation of this disturbance on levodopa and bromocriptine. Destruction of post-synaptic dopamine receptors and damage to central noradrenergic systems may offer an explanation for the lack of therapeutic effect of lisuride.
Adult↗
[Magnetic resonance spectroscopy in Parkinson's disease and multiple system atrophy].
Recent advances in magnetic resonance spectroscopy(MRS) allow to assay noninvasively key molecules of brain metabolism in living patients. There are several reports of MRS in Parkinson's disease(PD) and multiple system atrophy(MSA). 1H-MRS of the striatum revealed the reduced NAA/Cr and Cho/Cr ratio in MSA patients and the preserved NAA/Cr and Cho/Cr ratio in PD patients. The reduced NAA/Cr ratio probably reflects striatal neuronal loss. 1H-MRS of the striatum showed increased Cho/Cr ratio in the "on" state compared with that in the "off" state in the PD patients. The increased Cho/Cr ratio may reflect some membrane alteration or change of choline metabolism in PD with the "wearing-off" phenomena. Although studies are still preliminary, MRS shows great possibility for aiding in the differential diagnosis of parkinsonism and it will contribute to a better understanding of the pathogenesis of PD and MSA.
Aspartic Acid↗
Effect of ApoE and tau on age of onset of progressive supranuclear palsy and multiple system atrophy.
In addition to allelic association between genetic polymorphisms and diseases, modulation of age of onset is a well recognised effect of disease susceptibility genes. The ApoE epsilon4 allele is associated with an earlier age of onset of sporadic and familial Alzheimer's disease. It has been suggested that the tau genotype influences the age of onset of progressive supranuclear palsy (PSP), a form of atypical parkinsonism caused by tau neurofibrillary tangle deposition. We have therefore analysed the relationship between tau and ApoE genotypes and the age of onset of PSP and another form of atypical parkinsonism, multiple system atrophy (MSA). We have not found any effect of possession of the tau H1 haplotype or the ApoE epsilon4 allele on the age of onset of MSA or PSP.
Age of Onset↗
The alpha-synucleinopathies: Parkinson's disease, dementia with Lewy bodies, and multiple system atrophy.
Parkinson's disease is the second most common neurodegenerative disease, after Alzheimer's disease. Neuropathologically, it is characterized by the degeneration of populations of nerve cells that develop filamentous inclusions in the form of Lewy bodies and Lewy neurites. Recent work has shown that the filamentous inclusions of Parkinson's disease are made of the protein alpha-synuclein and that rare, familial forms of Parkinson's disease are caused by missense mutations in the alpha-synuclein gene. Besides Parkinson's disease, the filamentous inclusions of two additional neurodegenerative diseases, namely, dementia with Lewy bodies and multiple system atrophy, have also been found to be made of alpha-synuclein. Recombinant alpha-synuclein has been shown to assemble into filaments with similar morphologies to those found in the human diseases and with a cross-beta fiber diffraction pattern. The new work has established the alpha-synucleinopathies as a major class of neurodegenerative disease.
Brain↗
Dystonia in Parkinson's disease, multiple system atrophy, and progressive supranuclear palsy.
Adult-onset dystonia-parkinsonism is a syndrome in search of a pathology. We therefore reviewed the literature on dystonic manifestations in autopsy-proven cases of multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and idiopathic Parkinson's disease (PD). Only 6 of 140 autopsy reports of MSA remarked on the presence of dystonia in life, but personal observations suggest prominent antecollis may develop at some stage in up to 1/2 of sufferers. Similarly, very few (15/118) clinicopathologic observations on PSP included convincing dystonic manifestations, in contrast to some clinical reports where blepharospasm and early limb dystonia were prominent. Virtually any form of focal and segmental dystonia may sometimes occur with clinically diagnosed PD, with occasional descriptions of hemidystonia-hemiparkinsonism. However, there is pathologic confirmation of this diagnosis in only 1 case. With many patients thought clinically to have PD proving pathologically to have another cause for their parkinsonism, the true frequency and the range of dystonic manifestations acceptable in PD remain unknown.
Brain Diseases↗
[A clinico-MRI study of extrapyramidal symptoms in multiple system atrophy--linear hyperintensity in the outer margin of the putamen].
We did a clinico-MRI study concerning extrapyramidal symptoms and T2-weighted MRI findings of the putamen in twenty patients with multiple system atrophy (MSA) as well as twenty-five with idiopathic Parkinson's disease. Nine out of twenty MSA patients showed extrapyramidal symptoms. And we could not observe cerebellar ataxia in two of these patients because of severe rigidity and akinesia. Eight out of nine MSA patients with extrapyramidal symptoms showed linear hyperintensity in the outer margin of the putamen. This abnormal intensity was bilateral and symmetric in most patients. However, in MSA patients without extrapyramidal symptoms, only one patient showed the linear hyperintensity. We could not find such abnormal intensity in any patients with Parkinson's disease. On proton density MRI, the signal intensity in the lesion was higher than that in the gray matter, which leads the speculation that the hyperintensity is gliosis of the putamen or increased extracellular fluid space caused by severe shrinkage of the putamen. This characteristic MRI finding is useful to distinguish MSA with extrapyramidal symptoms from Parkinson's disease.
Aged↗
Diffuse Lewy body disease presenting as multiple system atrophy.
OBJECTIVES: The majority of patients with diffuse Lewy body disease have cognitive or psychiatric manifestations as part of their initial presentation. A sizable minority present with parkinsonian features alone. Autonomic features may also occur, typically after the development of cognitive changes. We aim to demonstrate that diffuse Lewy body disease may rarely also present with parkinsonism accompanied by marked autonomic dysfunction in the absence of significant cognitive or psychiatric abnormalities. METHODS: Case report based on a retrospective chart review and neuropathological examination. RESULTS: We report on a patient in whom a clinical diagnosis of multiple system atrophy was made based on a presentation of parkinsonism with prominent and early autonomic involvement. The former included postural tremor, rigidity and bradykinesia, while the latter consisted of repeated falls due to orthostasis and the subsequent development of urinary incontinence midway through the course of her illness. She was poorly tolerant of dopaminergic therapy due to accentuated orthostasis. Benefit from levodopa was limited and only evident when attempted withdrawal resulted in increased rigidity. There was no history of spontaneous or drug-induced hallucinations, delusions or fluctuating cognition, and in contrast to the prominence and progression of her parkinsonian and autonomic features over the first several years, cognitive impairment did not occur until the final stages of her illness, seven years after the onset of initial symptoms. Neuropathological examination revealed numerous Lewy bodies in both neocortical as well as subcortical structures consistent with a diagnosis of diffuse Lewy body disease. There was marked neuronal loss in the substantia nigra as well as the autonomic nuclei of the brainstem and spinal cord. CONCLUSIONS: In addition to cognitive, psychiatric, and parkinsonian presentations, diffuse Lewy body disease may present with parkinsonism and prominent autonomic dysfunction, fulfilling proposed criteria for the striatonigral form of MSA.
Aged↗