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Results for “Multiple system atrophy”

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PubMed1997

Loss of large myelinated nerve fibres of the recurrent laryngeal nerve in patients with multiple system atrophy and vocal cord palsy.

OBJECTIVES: Vocal cord palsy seen in some patients with multiple system atrophy may result from neuronopathy of the recurrent laryngeal nerve. METHODS: Six controls and six patients with multiple system atrophy, four with and two without vocal cord palsy were studied. The number of myelinated nerve fibres were counted and fibre diameter histograms were established for the motor and sensory divisions of the laryngeal branch of the recurrent laryngeal nerve. RESULTS: Although both groups of patients with multiple system atrophy showed selective loss of the myelinated fibres in the motor branch, the change was greater in those with vocal cord palsy than in those without. The small myelinated nerve fibres (diameter < 7 microm) were decreased in number in both multiple system atrophy groups, whereas the large myelinated nerve fibres (diameter < 8 microm) were decreased only in those with vocal cord palsy, and preserved in those without the symptom. CONCLUSION: In multiple system atrophy, the small myelinated fibres innervating the vocal cord are affected first, without obvious clinical signs. The patient develops vocal cord palsy only after the loss of the large myelinated fibres, which mostly comprise the alpha motor axons that innervate the intrinsic laryngeal muscles.

Atrophy↗

PubMed2001

Biochemical changes in multiple system atrophy detected with positron emission tomography.

Multiple system atrophy (MSA) is a sporadic neurodegenerative disorder manifested by parkinsonism and dysfunction of autonomic, cerebellar, urinary, and pyramidal systems. The most frequent presentation is with a combination of parkinsonism and autonomic dysfunction, but cerebellar ataxia with autonomic failure occurs frequently as well. Striatonigral degeneration (SND) and sporadic olivopontocerebellar atrophy (sOPCA) can progress to include autonomic failure and thus may be forms of MSA, but it is not known whether all such cases progress to MSA. Utilizing positron emission tomography (PET) with various ligands, my colleagues and I have investigated the biochemical changes in sOPCA and MSA to understand the relationship between these disorders. An initial study revealed decreased local cerebral metabolic rates for glucose in the brainstem, cerebellum, putamen, thalamus and cerebral cortex in both MSA and sOPCA, suggesting that many sOPCA patients would evolve to develop MSA. Later studies confirmed this by demonstrating decreased monoaminergic nigrostriatal terminals in both sOPCA and MSA patients. The studies suggest that the ligand used might be helpful in determining the risk that an individual patient with sOPCA will progress to develop MSA. An investigation of the course of sOPCA patients observed clinically over several years revealed that approximately one-fourth of them progress to MSA within five years. Studies of gamma-aminobutyric acid type A/benzodiazepine neurotransmitter receptors revealed that these sites are largely preserved in sOPCA and MSA, indicating that symptomatic pharmacological therapy may be possible in these disorders.

Journal Article↗

PubMed1989

Multiple system atrophy--the nature of the beast.

Multiple system atrophy (MSA) is generally considered a rare disease, but may account for up to 10% of patients with Parkinsonism. The profusion of names for this disease, which may present to general physicians, psychiatrists, cardiologists, autonomic specialists, general neurologists and those with a special interest in Parkinsonism (this author's own perspective) or cerebellar disorders, together with ignorance of its protean manifestations, may account for its underrecognition and misdiagnosis. In this article, the history and nosology of the condition are considered, and provisional diagnostic criteria are advanced. The usefulness (or otherwise) of ancillary investigations is addressed, and the shortcomings of current methods of treatment are stressed. As with idiopathic Parkinson's disease, the ultimate goal of eradicating the disease entails better diagnosis in order to establish the cause, and thence to develop a radical treatment capable of preventing or arresting the disease process.

Autonomic Nervous System Diseases↗

PubMed1992

[Magnetic resonance imaging in multiple system atrophy].

We studied 18 patients with multiple system atrophy (MSA) by high field strength MRI: 6 striatonigral degeneration (SND), 4 Shy-Drager syndrome (SDS), and 8 olivo-ponto-cerebellar atrophy (OPCA). We also studied 30 Parkinson's disease (PD) and 10 age-matched controls. The diagnoses of SND, SDS, and OPCA were based on criteria after Hirayama et al (1985). Bradykinesia, rigidity, and tremor were assessed with the summed scores of the signs used as the extrapyramidal scores. The mean extrapyramidal scores were not significantly different in patients with SND, SDS, OPCA, and PD. MRI studies were performed on 1.5 tesla MRI unit, using a T2 weighted spin echo pulse sequence (TR2500 ms/TE40 ms). The width of the pars compacta signal in all subjects was measured by the method of Duguid et al (1986). Intensity profiles were made on a straight line perpendicular to the pars compacta through the center of the red nucleus on an image of the midbrain. We measured the width of the valley at half-height between the peaks of intensity representing the red nucleus and the crus cerebri-pars reticulata complex and used this measurement as an index of the width of the pars compacta signal. The mean widths of the pars compacta signal were: 2.8 +/- 0.4 mm (SND), 2.8 +/- 0.7 mm (SDS), 3.6 +/- 0.6 mm (OPCA), 2.7 +/- 0.3 mm (PD), and 4.3 +/- 0.6 mm (control). The mean widths of the pars compacta signal in PD, SND, and SDS were significantly narrower than that in the control group (p < 0.05), while the OPCA group was not significantly narrower.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1997

[Malignant syndrome in multiple system atrophy].

Five of fourteen patients with multiple system atrophy (MSA) experienced a total of eight episodes of malignant syndrome, three episodes in 1, two episodes in 1 patient, and a single episode in each of the other patients. Four patients had extrapyramidal symptoms and required antiparkinson therapy, including dopaminergic agonists. Five episodes occurred in the summer season. Two were caused by decreased or irregular doses of antiparkinson drugs, one by administration of an antidepressant drug, three by complications, and two by elevation of body temperature of environmental origin. A patient without parkinsonism became febrile after administration of droxidopa, which may be a central pyrogenic substance that acts via the noradrenergic system. Administration of dopaminergic drugs and dantrolene sodium was followed by recovery in four episodes in three patients. One patient manifested dysautonomia after recovery from the malignant syndrome. Another patient with high serum creatine kinase levels and myoglobinuria developed renal failure requiring hemodialysis. Another patient died of DIC. Besides withdrawal of dopaminergic agents, which alter monoaminergic neuron activity, stress to the body and heating by a variety of factors tend to trigger the malignant syndrome in MSA.

Adult↗

PubMed1981

Laryngeal electromyography in multiple system atrophy with autonomic failure.

In five patients with multiple system atrophy, electromyographic studies using a new surface electrode technique, showed unequivocal evidence of denervation of the posterior crico-arytenoid muscle, and in three of them, of the interarytenoid muscle or crico-pharyngeal sphincter. By comparison, only minor electromyographic abnormalities were found in one-third of patients with other extra-pyramidal syndromes, though in two cases, the abnormalities were of a different kind from those found in multiple system atrophy.

Aged↗

PubMed2001

Peripheral neuropathy as the presenting feature of multiple system atrophy.

The authors report a case of multiple system atrophy (MSA) with an onset as a peripheral nerve involvement. Their patient, a 55-year-old man, had a 3-year history of distal weakness and atrophy in upper limbs with dysesthesia in the feet. Other identifiable causes of peripheral neuropathy were ruled out. The authors postulate that peripheral nervous system impairment can anticipate the typical appearance of MSA, and they suggest that, in peripheral neuropathies with autonomic system dysfunction, after excluding main causes of autonomic neuropathy, MSA may need to be suspected.

Autonomic Nervous System↗

PubMed2000

Orthostatic Hypotension in Multiple System Atrophy.

There is no known way to cure multiple system atrophy or even to slow its insidious progression, but there are opportunities to treat many of its symptoms and complications. Of all the autonomic disturbances it causes, orthostatic hypotension is associated with one of the better chances for successful intervention. Milder symptoms may be treated by nonpharmacologic means (eg, changes in diet and activities and compressive maneuvers and devices). Syncopal patients will require treatment with medications. Midodrine is the most effective drug. It is more physiologic and better tolerated, but much more expensive, than fludrocortisone. The latter is often beneficial but frequently causes lower limb edema. Low-dose propranolol is occasionally useful. Other drugs worth trying are indomethacin and intranasal desmopressin. The variety of measures available to treat orthostatic hypotension in multiple system atrophy affords physicians some prospect of easing the suffering of many patients with this otherwise disabling and discouraging disorder.

Journal Article↗

PubMed2002

Not paralysis, but dystonia causes stridor in multiple system atrophy.

Electromyography (EMG) was performed in 10 patients with multiple system atrophy, laryngeal or pharyngeal symptoms, or both. In patients with stridor, EMG during quiet breathing revealed persistent tonic activity in both abductor and adductor vocal cord muscles. In patients with dysphagia, the cricopharyngeal muscle showed persistent EMG activity throughout all phases of swallowing. Botulinum toxin injection into the adductor muscle determined subjective improvement and reduced tonic EMG activity. Therefore, the cause of stridor in multiple system atrophy is dystonia of the vocal cords.

Adult↗

PubMedDate not supplied

[Sleep disorders in multiple system atrophy].

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.

Humans↗

PubMed2002

[Multiple system atrophy speech assessment: study of five cases].

Multiple system atrophy (MSA) is characterized by parkinsonian, cerebellar and pyramidal features along with autonomic dysfunction in different combinations. Onset of dysarthria during the first year of the manifestation of a parkinsonian syndrome suggests the diagnosis of MSA. The aim of this study was to characterize the voice and the speech of patients with MSA. We studied five MSA patients with a mean age of 51.2 years. Each patient was submitted to a neurological and a specific speech and voice assessment. The latter consisted of the following: clinical interview, myofunctional examination, and perceptual speech evaluation. Speech and voice complaints occurred at an average time of 1.1 year after the onset of the motor symptomatology. All MSA patients had the mixed type of dysarthrophonia, where hypokinetic, ataxic and spastic components were seen in each of the patients, although hypokinetic component predominated among the others. Our findings are different from what is commonly seen in Parkinson's disease in which hypokinetic component is the only abnormal finding. We think that specific speech and voice assessment is important to establish the diagnosis and to choose the best management of MSA patients.

Adult↗

PubMed2006

Cerebral autoregulation is preserved in multiple system atrophy: A transcranial Doppler study.

Patients with multiple system atrophy (MSA) present large changes in blood pressure (BP) due to autonomic disturbances. We analyzed how this change may influence dynamic cerebral autoregulation (DCA). Simultaneous recordings of arterial BP (Finapres) and middle cerebral artery (MCA) blood flow velocity (BFV) (transcranial Doppler) were performed in 10 patients with MSA (61 +/- 12 yr of age) and 12 healthy volunteers (61 +/- 11 yr of age): cerebral BFV response to oscillations in mean BP was studied in the supine position by cross-spectral analysis of mean BP and mean MCA BFV. The DCA was also studied during the decrease in BP the first seconds when standing up from a sitting position by the assessment of the cerebrovascular resistance index (CR; mean BP/mean MCA BFV ratio). The MCA BFV/BP cross-spectral analysis showed a phase for the mid-frequency band (0.07-0.2 Hz) significantly larger in MSA, suggesting more active autoregulation in response to larger changes in BP. Changes in CR reflecting the rate of autoregulation, when standing did not differ between the two groups. These data suggest that dynamic cerebral autoregulation is preserved in MSA.

Aged↗

PubMed1995

Nerve conduction studies, skeletal muscle EMG, and sphincter EMG in multiple system atrophy.

Although autonomic failure, parkinsonism, and cerebellar and pyramidal signs are well documented in multiple system atrophy, much less is known about the frequency and severity of involvement of the peripheral nervous system. The frequency and nature of peripheral nerve involvement has therefore been determined in 74 patients with multiple system atrophy using nerve conduction studies and skeletal muscle EMG. These findings were compared with those on sphincter EMG. Ninety per cent of the patients had an abnormal sphincter EMG, indicating denervation and reinnervation consistent with anterior horn cell loss in Onuf's nucleus, but only 40% had either abnormal nerve conduction studies (mixed sensorimotor axonal neuropathy in 17.5%) or abnormal skeletal muscle EMG (suggesting partial denervation in 22.5%). These data indicate a remarkable selective vulnerability of the anterior horn cells of Onuf's nucleus innervating external sphincter muscles relative to those supplying skeletal muscle in patients with multiple system atrophy. If this selective pattern of involvement can be explained it may be a clue to pathogenetic mechanisms in multiple system atrophy.

Adult↗

PubMed1998

[Clinical diagnostic criteria for multiple system atrophy].

What are the distinctive criteria for the diagnosis of multiple system atrophy? The combination of motor, dysautonomic, neuropsychological and eyes movements may help to distinguish, at a early stage, multiple system atrophy from other neurodegenerative diseases such as Parkinson's disease, progressive supranuclear palsy or corticobasal degeneration. Thus, these criteria are suitable both for clinical care and drug trials.

Cognition Disorders↗

PubMed1999

Multiple system atrophy with remarkable frontal lobe atrophy.

The autopsy findings of a multiple system atrophy (MSA) patient with remarkable frontal lobe atrophy are described. The patient was a 65-year-old woman with a 13-year history of untreatable parkinsonism, dysautonomia and progressive motor aphasia. The brain weight was 810 g, and there was remarkable atrophy of the cerebrum predominantly in the frontal lobe, striatum, pons and cerebellum. Microscopic examination revealed a preserved cortical structure with laminar gliosis in the sixth layer of the precentral and superior frontal gyri of the frontal lobe, and postcentral gyrus and inferior parietal lobule of the parietal lobe. The second layer of the cortices of these regions were also revealed to be in a spongy state, and mild cell loss was seen in the fifth and six layers. The frontal lobe white matter showed a mild loss of myelinated fibers and axons, and mild gliosis. Glial cytoplasmic inclusions (GCIs) were abundantly observed in the deep layer of the cortex in the regions mentioned above, and were more abundant in the white matter of the frontal and parietal lobes, callosal body, and internal, external and extreme capsules. There was severe degeneration in the olivopontocerebellar and striatonigral systems, and GCIs in widespread regions of the brain. No Pick bodies, Lewy bodies, ballooned neurons, senile plaques, or significant amounts of neurofibrillary tangles were detected. There were no vascular changes. Thus, this was a verified MSA patient with progressive aphasia and remarkable frontal lobe atrophy. We indicate a possible involvement of the cerebral lobes in MSA.

Aged↗

PubMed1993

Oligodendroglial and neuronal inclusions in multiple system atrophy.

Argyrophilic cytoplasmic inclusions of oligodendrocytes have been described in cases of multiple system atrophy (olivopontocerebellar atrophy, striatonigral degeneration, and Shy-Drager syndrome). The oligodendroglial cytoplasmic inclusions are immunolabeled with antiubiquitin antibodies. Ultrastructurally, they appear as granule-associated filaments. They have not been found in other neurological diseases or in normal brains, and are now considered to be early and specific markers of multiple system atrophy. More recently, similar argyrophilic inclusion bodies have been reported in the cytoplasm of neurons and in both oligodendroglial and neuronal nuclei of multiple system atrophy brains. Neuronal and oligodendroglial cytoplasmic inclusions have identical ultrastructural characteristics, but different antigenic properties. The chemical nature of the inclusions is presently unknown and their significance remains controversial: they could be a primary event in the course of the degenerative process or merely an epiphenomenon of some disordered cytoskeletal metabolism.

Corpus Striatum↗

PubMed2005

Metaiodobenzylguanidine (MIBG) scintigraphy at various parts of the body in Parkinson's disease and multiple system atrophy.

We compared MIBG uptake at various parts of the body in controls and patients with Parkinson's disease and multiple system atrophy. In the heart, MIBG uptake in Parkinson's disease (early H/M: 1.668+/-0.325, late H/M: 1.500+/-0.402) was less than that in multiple system atrophy (early H/M: 2.395+/-0.186, late H/M: 2.530+/-0.391) and controls (early H/M: 2.635+/-0.508, late H/M: 2.575+/-0.635) (early: P<0.0001, late: P<0.0001). There were no significant differences in uptake by the lung, thyroid, or liver in the three groups. Only on early images, uptake in the shoulder in multiple system atrophy (early S/M: 0.473+/-0.78) and Parkinson's disease (early S/M: 0.470+/-0.710) was decreased compared to that in controls (early S/M: 0.560+/-0.118) (P=0.0252). MIBG is reported to be taken up in the terminal part of sympathetic nerves and demonstrates sympathetic nerve activity, especially on late images. The cause of differences between the heart and other parts of the body remains unknown. We consider the following possibilities: (a) differences in the sympathetic nervous system between Parkinson's disease and multiple system atrophy are more subtle in organs other than the heart; (b) the cause of MIBG uptake reduction by the heart in Parkinson's disease involves factors in addition to sympathetic nervous system damage; and (c) MIBG uptake by organs other than the heart involves not only the sympathetic nervous system but also non-neuronal components. In conclusion, MIBG uptake by organs other than the heart cannot differentiate Parkinson's disease from multiple system atrophy at present.

3-Iodobenzylguanidine↗

PubMed1996

Early diagnosis and stage classification of vocal cord abductor paralysis in patients with multiple system atrophy.

OBJECTIVES: Vocal cord abductor paralysis (VCAP) is a life threatening complication which may cause nocturnal sudden death in patients with multiple system atrophy. However, the early diagnosis of VCAP is often difficult to make on routine laryngoscopy performed during wakefulness, as stridor, which is the sole symptom of VCAP in the early stage, develops only during sleep. The aim was to investigate laryngeal dysfunction in patients with multiple system atrophy while awake and asleep. METHODS: Seven patients with multiple system atrophy with nocturnal stridor and five control patients were studied. Vocal cord movement was analysed by laryngoscopy while the patients were awake and also during sleep induced by intravenous diazepam. RESULTS: When awake, for the seven patients with multiple system atrophy normal movement of the vocal cords occurred in three, mild abduction restriction in three, and paradoxical movement in one. When asleep, however, all showed obvious paradoxical movement with high pitched inspiratory stridor. In controls, there were no differences in the vocal cord movement between wakefulness and sleep. From these findings, VCAP could be divided into four stages: stage 0 (normal) with normal vocal cord movement during both wakefulness and sleep, stage 1 (mild VCAP) with normal movement during wakefulness and paradoxical movement during sleep, stage 2 (moderately severe VCAP) with abduction restriction during wakefulness and paradoxical movement during sleep, and stage 3 (severe VCAP) with an almost midline position for the vocal cords during both wakefulness and sleep. CONCLUSIONS: Laryngoscopy during sleep can disclose subclinical VCAP, making an early diagnosis of VCAP in patients with multiple system atrophy. Stage 2 of VCAP seems to be a suitable stage for tracheostomy in patients with multiple system atrophy.

Aged↗