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Results for “Multiple System Atrophy”

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PubMed1993

Micturitional disturbance in multiple system atrophy.

Detailed micturitional histories and urodynamic studies were conducted to investigate the micturitional disturbance in multiple system atrophy (MSA). Eighty-six patients with MSA comprised of 14 with striatonigral degeneration (SND), 42 with olivopontocerebellar atrophy (OPCA) and 30 with Shy-Drager syndrome (SDS). The results were as follows. Micturitional symptoms were noted in over 90% of patients with each type of MSA. Dominant symptoms were irritative ones in SND and OPCA, and a combination of irritative and obstructive ones in SDS. Micturitional symptoms in SDS appeared earlier than those in SND or OPCA. The degree of micturitional disturbance was severer in SDS than in SND or OPCA. Micturitional disturbance tended to become worse as the disease progressed. The responsible sites of lesions of micturitional disturbance seemed to be supra- as well as infranuclear lesions of the pelvic and pudendal nerves in MSA. Infranuclear lesions were more prominent in SDS than in SND or OPCA. Follow-up studies of some of the patients with SDS and OPCA suggested that the responsible sites of pelvic nerve lesions changed from supra- to infranuclear lesions during the course of disease.

Autonomic Nervous System↗

PubMed1996

Signal changes on MRI and increases in reactive microgliosis, astrogliosis, and iron in the putamen of two patients with multiple system atrophy.

A correlation of clinical, MRI, and neuropathological data is reported in two patients with multiple system atrophy (MSA). On MRI, patient 1 showed striatal atrophy, reduction of T2 relaxation times within most of the putamen, and a band of hyperintense signal changes in the lateral putamen. In patient 2, MRI disclosed only shortening of the T2 signal in the putamen. Immunohistochemistry showed pronounced reactive microgliosis and astrogliosis in the affected brain regions. In patient 1, the area with the most pronounced microgliosis and astrogliosis most likely correlated with the area of hyperintense signal changes on MRI. This area also contained the highest amount of ferric iron, which was increased in the putamen of patient 1 but not patient 2. It is unlikely that the hypointense signal changes in the putamen are due to an increase of iron alone. Reactive microglial and astroglial cells may play a part in the pathogenesis of MSA.

Adult↗

PubMed1996

Olivopontocerebellar pathology in multiple system atrophy.

Olivopontocerebellar atrophy (OPCA) is widely accepted as part of the neuropathological spectrum of multiple system atrophy (MSA). The distribution of affected sites in the olivopontocerebellar (OPC) system and their interrelationship remain poorly understood due to lack of quantitative studies. To further investigate the OPC pathology in MSA, we performed a morphometric analysis of 20 MSA cases and eight healthy controls. In the MSA cases, mean neuronal cell densities were significantly reduced in (medial and dorsal) accessory and principal inferior olives, pontine nuclei, cerebellar vermis (except nodulus), and hemispheres. Inferior olives and pontine nuclei were more severely affected than cerebellar Purkinje cells in most cases. Cerebellar Purkinje cells were more severely depleted in vermis rather than in hemisphere. There was a poor topographic correlation between neuronal cell loss in inferior olives and cerebellar cortex. These results suggest a primary degeneration of olivopontine nuclei and cerebellar Purkinje cells in OPCA. Inferior olives, pontine nuclei and cerebellar cortex were all significantly more severely affected in cases with a pure or predominating cerebellar syndrome (OPCA type, n = 4) compared to those with pure or predominating parkinsonism (SND type, n = 14). However, although cerebellar signs had been noted in life in only six cases, morphometry revealed OPCA in 17 of the 20 MSA brains.

Adult↗

PubMed1997

Incidence of progressive supranuclear palsy and multiple system atrophy in Olmsted County, Minnesota, 1976 to 1990.

Information on the incidence of progressive supranuclear palsy (PSP) is limited; incidence rates for multiple system atrophy (MSA) are not available. We studied the incidence of PSP and MSA in Olmsted County, Minnesota, for the years 1976 to 1990. This study was part of a larger investigation of all forms of parkinsonism. We used the medical records-linkage system of the Rochester Epidemiology Project to identify all subjects whose records contained documentation of any from of parkinsonism, related neurodegenerative diseases, or tremor of any type. A nurse abstractor screened the records and, when applicable, a neurologist reviewed them to determine the presence or absence of parkinsonism. Cases of parkinsonism were classified using specified diagnostic criteria. Population denominators were derived from census data and were corrected by removing prevalent cases of parkinsonism. Over the 15 years of the study, we found 16 incident cases of PSP and nine incident cases of MSA. No cases of PSP or MSA had onset before age 50 years. The average annual incidence rate (new cases per 100,000 person-years) for ages 50 to 99 years was 5.3 for PSP and 3.0 for MSA. The incidence of PSP increased steeply with age from 1.7 at 50 to 59 years to 14.7 at 80 to 99 years, and was consistently higher in men. Median survival time from symptom onset was 5.3 years for PSP and 8.5 years for MSA. The incidence of PSP increases with age and is consistently higher in men at all ages. PSP and MSA are more common than previously recognized.

Adolescent↗

PubMed1997

Tau immunoreactivity in glial cytoplasmic inclusions in multiple system atrophy.

In order to clarify the manner and significance of tau expression in glial cytoplasmic inclusions (GCIs), ubiquitinated oligodendroglial abnormal structures in multiple system atrophy (MSA), an immunohistochemical study was carried out in the lesions of the pontine nuclei of 10 cases of MSA using antibodies against various epitope locations of tau protein. As a result, tau-2 was constantly but weakly positive in ubiquitinated GCIs in each case (from 28.6 to 66.7%). However, tau-2-immunoreactivity in GCIs was not correlated to the density of ubiquitin-positive GCIs or preserved pontine neurons. Antibodies against tau proteins of N-terminal or C-terminal failed to label GCIs, although a few number of GCIs were occasionally positive for tau-1 after dephosphorylation. In comparison with the knowledge on tau-immunoreactivity of coiled bodies (CBs) in oligodendroglia in progressive supranuclear palsy (PSP) or corticobasal degeneration, GCIs are quite different from CBs which have a wide range of epitope location of tau proteins, including N-terminal and C-terminal. This study suggests that expression of tau proteins in GCIs is not related to the essential neurodegenerative process in MSA but induced by non-specific stress in oligodendroglia, unlike CB in various 'tau diseases' such as PSP.

Aged↗

PubMed1999

Auditory brainstem responses in patients with autonomic failure with Parkinson's disease and multiple system atrophy.

Auditory brainstem responses (ABRs) were examined in six patients with autonomic failure with Parkinson's disease (AF with PD) and 10 patients with autonomic failure with multiple system atrophy (AF with MSA), all of which showed marked parkinsonian features as a principal sign. We designated the central abnormalities of ABRs as prolongation of latencies (wave III or V) and interpeak latencies (IPLs; I-III, I-V, and III-V IPL) or decreased amplitude ratios of wave III or V to those of wave I (less than 1.0). None of the patients with AF with PD showed abnormalities in ABRs. In contrast, in those with AF with MSA, the peak latencies or IPLs were prolonged in two of the 10 patients, and the amplitude ratios of wave III or V to those of wave I were decreased in other two of these patients. Moreover, both prolongation of latencies and a decreased ratio were observed in other one. Overall, five of the 10 patients with AF with MSA showed central abnormalities in ABRs. It is clinically difficult to differentiate AF with PD from AF with MSA, particularly when no cerebellar signs are apparent in AF with MSA patients. When central abnormalities of ABRs are observed in AF patients, AF with MSA should be suspected rather than AF with PD. In conclusion, ABRs provide useful information for the differential diagnosis of AF with PD and AF with MSA.

Aged↗

PubMed1998

[Patho-MR imaging study in the putaminal margin in multiple system atrophy].

The slit hyperintensity of the lateral margin of the putamen in T2 weighted MRI is a characteristic finding in those patients with multiple system atrophy (MSA) involving extrapyramidal system. In spite of some speculations such as demyelination, gliosis, iron deposition or increased extracellular fluid, the nature of the abnormal signal intensity has still been remained uncertain. In this paper, we report the coincidental findings of pathology and magnetic resonance imaging of the putaminal margin in a case of MSA. The patient was sixty three years old woman with nine years history of intreatable parkinsonism, mild ataxia and dysautonomia. At six months prior to her death, 0.5T MRI showed the pontocerebellar system atrophy, slit hyperintensity in the bilateral outer margin and left inner margin of the putamen in T2 weighted image as well as linear hypointensity in T1 weighted image. The neuropathological examinations showed severe degeneration in the olivopontocerebellar and striatonigral systems, and glial cytoplasmic inclusion in widespread regions in the brain. The putamen showed severe degeneration with rarefaction. The intertissue space was observed at the outer putaminal margin in both sides and inner margin in left side, which seemed to be caused by severe shrinkage and rarefaction of the putamen. Thus, slit hyperintensity in the putaminal margin in MSA was disclosed to represent widened intertissue space.

Female↗

PubMed1990

[High-field MR findings of multiple system atrophy].

Magnetic resonance images obtained at 1.5T were reviewed in 23 patients with clinically diagnosed multiple system atrophy (MSA). The patient group consisted of 13 cases with olivoponto-cerebellar atrophy (OPCA), three with striatonigral degeneration (SND), and seven with Shy-Drager syndrome (SDS). In each disorder group, hypointensity of the pars compacta of the substantia nigra was frequently demonstrated as well as the atrophy of the brain stem and cerebellum. Although T2-weighted images depicted hypointensity of the putamen, which was prominent in its posterolateral part, in cases with SND and SDS, it was not encountered in cases with OPCA. Therefore, this finding was considered to facilitate differential diagnosis between OPCA and other two disorders. These hypointensities in the putamen and pars compacta of the substantia nigra may be due to excessive iron deposition and seem to correlate with extrapyramidal tract signs of MSA.

Corpus Striatum↗

PubMed1989

[Multiple system atrophy: an analytic study of 20 cases].

An analytic study was made on 20 cases of multiple system atrophy. The patients included OPCA 12, SND 3, and SDS 5. This study showed that in 90% of the cases, the symptom began in middle life. Both sexes affected similarly and the mean course was about 5 years. Approximately three years after the appearance of the primary symptom, the features of other central nervous system lesions were presented. Comparative study of clinical features of the three types repealed the following: Cerebellar symptom: OPCA greater than SDS greater than SND. Symptoms and signs of extrapyramidal system: SND greater than SDS greater than OPCA, autonomic dysfunction: SDS greater than OPCA greater than SND.

Adult↗

PubMed1993

[A case of multiple system atrophy presenting a regular involuntary movement of the neck muscles synchronous with respiration].

A patient with multiple system atrophy developed a regular, rhythmic involuntary movement of the neck muscles which appeared synchronous with respiration and could be described as "rocking-of-the-head". He had been treated with antiparkinsonian drugs such as L-dopa, L-dopa/carbidopa, amantadine hydrochloride and trihexiphenidyl hydrochloride for approximately one year. A fluoroscopic study assured that the involuntary "rocking-of-the-head" movement synchronized with the diaphragmatic up-and-down movement. A polygraphic study showed that the accelerometer curve which reflected the rocking movement of the head oscillated at approximately 0.7 Hz and synchronized with the surface EMG discharges of the right sternocleidomastoid muscle and the nasal flow curve. This involuntary movement was seen almost all day long as well as asleep but severest usually in the afternoon. Discontinuation of trihexiphenidyl hydrochloride alone made the involuntary movement less severe but never suppressed it completely. Resumption of the drug made the involuntary movement as severe as it had been. Discontinuation of L-dopa, L-dopa/carbidopa and amantadine hydrochloride was followed by disappearance of the involuntary movement in a day or so, in spite of continued intake of trihexiphenidyl hydrochloride. Rigidity and bradykinesia induced by discontinuation of these drugs, however, made it necessary to resume them. As a result the involuntary movement again exacerbated.

Amantadine↗

PubMed1997

Electromyography of the external anal sphincter in patients with Parkinson's disease and multiple system atrophy: frequency of abnormal spontaneous activity and polyphasic motor unit potentials.

Electromyographic studies of the external anal sphincter muscle have received increasing attention in the differential diagnosis of patients with parkinsonism. Based on the fact that the external anal sphincter muscle is partly innervated by fibers that originate in Onuf's nucleus in the segments S2-S4 of the spinal cord, an increased duration of the motor unit potentials (MUPs) and an increased polyphasia may reflect neuronal loss of these lower motor neurons characteristic for multiple system atrophy. We report the results of anal sphincter electromyography in 15 patients with clinically probable multiple system atrophy (MSA) and 10 patients with Parkinson's disease (PD). There was no significant difference between patients with MSA and patients with PD concerning the duration of MUPs, mean number of phases, and rate of polyphasic motor potentials. There was a tendency for a longer mean duration and increased polyphasia in patients with MSA compared to patients with PD. Spontaneous activity was recorded in 11 of 15 patients with MSA occurring especially in patients with MSA of the striatonigral type, but not in patients with PD. In this study, the duration of MUPs and the rate of polyphasia were unreliable criteria in the electrophysiological differential diagnosis of patients with parkinsonism. Abnormal spontaneous activity, although difficult to detect, is a more specific marker for neuronal degeneration of Onuf's nucleus occurring in patients with MSA.

Adult↗

PubMed1999

Neuroendocrine responses to levodopa in multiple system atrophy (MSA).

Hypothalamic dopaminergic pathways are involved in the regulation of growth hormone and prolactin release from the anterior pituitary. Neuroendocrine studies in patients with multiple system atrophy (MSA), in whom there is a reported loss of hypothalamic dopamine, are few and contradictory. We therefore studied the neuroendocrine responses to 250 mg levodopa (plus 25 mg carbidopa) in subjects with MSA (n = 15), and compared them with age- and sex-matched healthy control subjects (n = 8). There were no significant differences in basal or post-levodopa levels of growth hormone (GH), growth hormone-releasing hormone (GHRH), glucose, insulin-like growth factor (IGF-1), or thyroid-stimulating hormone (TSH) between the groups. In patients with MSA, basal levels of prolactin were elevated (21.1 +/- 5.2 ng/mL [mean +/-standard error]) compared with control subjects (12.1 +/- 1.7, p <0.05), and after L-dopa there was increased variability in prolactin response with less suppression compared with control subjects. In conclusion, in patients with MSA, the GHRH and GH responses to L-dopa were preserved and were similar to responses in age-matched control subjects. In contrast, there was impaired dopaminergic suppression of prolactin secretion. In patients with MSA this may represent a selective dysfunction, rather than generalized loss, of tubero-infundibular dopaminergic neurones.

Adult↗

PubMed1998

Distribution of cerebellar cortical lesions in multiple system atrophy: a topographic neuropathological study of three autopsy cases in Japan.

We investigated neuropathologically the distribution of the cerebellar cortical lesions in three Japanese autopsy cases of multiple system atrophy (MSA) using hemisphere specimens. The lesions were classified as mild, moderate or severe. The distribution of cerebellar cortical lesions in all three cases were uniform: the cerebellar cortical lesions were more conspicuous in the vermis than in the hemisphere. These neuropathological findings differ from the established theory that cerebellar lesions of MSA are more pronounced in the hemisphere than in the vermis. The degree of cerebellar cortical lesions in our cases increased in relation to the duration of the disease. Our pathological data may contribute to the morphological differential diagnosis in various neurodegenerative disorders including late cortical cerebellar atrophy. Our neuropathological findings may also make a contribution to the neuroradiological progress in the differential diagnosis of spinocerebellar disease.

Brain Mapping↗

PubMedDate not supplied

Hemiballism in a patient with probable multiple system atrophy.

We report a patient with long-standing asymmetrical parkinsonism, cerebellar ataxia and dysautonomia, suggestive of multiple system atrophy (MSA). However, the patient also developed involuntary repetitive movements similar to ballic dyskinesias and mental deterioration. MRI revealed major involvement of both posterior fossa structures and basal ganglia. The case would be accommodated within a rubric of MSA widened to include involvement of the subthalamic nucleus and the medial part of the pallidum, pathology which may account for the ballic movements. Additionally the patient's cognitive and behavioural disturbances suggest an impairment of striato-prefrontal cortex loop.

Autonomic Nervous System Diseases↗

PubMed2026

Plasma cfDNA hypermethylation at SNCA intron 1 as a potential blood-based epigenetic signal in Parkinson's disease and multiple system atrophy.

BACKGROUND: The accumulation of α-synuclein (SNCA) in the central nervous system is a hallmark of Parkinson's disease (PD) and multiple system atrophy (MSA). SNCA intron 1 methylation is implicated in SNCA transcriptional regulation and may serve as a peripheral epigenetic signal in synucleinopathies. However, studies of SNCA methylation in leukocyte-derived DNA have yielded inconsistent results. We aimed to evaluate whether cell-free DNA (cfDNA)-based SNCA intron 1 methylation differs in PD or MSA compared with normal controls (NC). METHODS: Plasma cfDNA was collected from 105 patients with PD, 50 with MSA, and 114 NC. DNA methylation at CpG sites 10-17 was quantified by bisulfite pyrosequencing. Multivariable linear and logistic regression models, adjusted for age, sex, and education, were used to compare methylation levels and estimate odds ratios (ORs). RESULTS: Patients with PD exhibited hypermethylation at CpG site 14 and higher mean methylation across CpG sites 10-17 compared with NC. Patients with MSA showed hypermethylation at CpG sites 10, 12, 13, and 17 and elevated mean methylation. Elevated mean methylation was also observed in drug-naïve de novo PD and early-stage PD patients. Compared with the lowest tertile, the highest mean methylation tertile was associated with increased odds of PD (OR, 2.49; 95% CI, 1.08-5.92) and MSA (OR, 5.02; 95% CI, 1.58-18.00). CONCLUSION: Plasma cfDNA SNCA intron 1 hypermethylation is associated with PD and MSA and detectable in drug-naïve and early-stage PD. It may represent a peripheral epigenetic alteration and warrants evaluation as an adjunctive signal for early screening.

Humans↗

PubMed1993

[Multiple system atrophy (MSA)].

Sporadic olivopontocerebellar atrophy (OPCA), Shy-Drager syndrome (SDS), striatonigral degeneration (SND) are classified as multiple system atrophy (MSA), based on their clinicopathological findings. These clinical features, neuroimaging including CT, MRI, PET and SPECT findings, autonomic function test, pathological findings and treatments are reviewed. Several advances such as noradrenaline loading test, low signal intensity on T2 weighted image on super-conductive MRI and glial cytoplasmic inclusions in the central nervous system on pathological finding are notable. However, unsatisfactory medical treatment for MSA must be resolved in a near future.

Autonomic Nervous System↗

PubMed1976

[Multiple systemic atrophies, mental retardation, neurogenic amyotrophy and congenital bone fragility. A new neuro-generative disorder].

Five cases of a congenital neurological disorder are reported. Four patients, born after a breech delivery, belong to one sibship while the fifth patient is the only child in another family. The clinical features include quadriplegia, amyotrophy, a peripheral neuropathy, severe mental retardation and a subluxation of the hips. X-rays reveal diffuse osteoporosis and multiple spontaneous fractures. Autopsies in 3 patients showed multiple system atrophies involving the spinal cord and the cerebellum, coarse cerebral gyri and a marked reduction in volume of the white matter. These various pathological features are compared with the lesions found in a few other cases reported in the literature, none of which can be considered to be identical to the ones described. It is therefore felt that the condition under discussion represents a new syndrome to be classified, at least temporarily, within the group of multiple system atrophies.

Atrophy↗

PubMed1993

Fulminant multiple system atrophy in a young adult presenting as motor neuron disease.

A 34-year-old man demonstrated rapidly progressive motor neuron disease and, late in his 9-month clinical course, exhibited ophthalmoplegia and dysautonomic symptoms. Neuropathology showed spinal and bulbar motor neuron disease with severe involvement of extraocular motor nuclei, degeneration of spinal sympathetic and bulbar parasympathetic nuclei, striatonigral degeneration, and early olivopontocerebellar atrophy. This case underscores the diversity of multiple system atrophy and demonstrates an unusually rapid course in a young patient.

Adult↗