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PubMed2002

Depletion of mesopontine cholinergic and sparing of raphe neurons in multiple system atrophy.

The authors immunocytochemically identified mesopontine cholinergic and rostral raphe serotonergic neurons in brains obtained at autopsy from four patients with multiple system atrophy (MSA) and four matched controls. There was a severe depletion of cholinergic neurons in the pedunculopontine (20 +/- 2 vs 81 +/- 10 cells/section, p< 0.001) and laterodorsal tegmental nucleus (18 +/- 3 vs 47 +/- 4 cells/section, p < 0.001) in MSA. Whereas there was also depletion of locus ceruleus neurons, there was a striking preservation of rostral raphe neurons in MSA.

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PubMed2006

Multiple system atrophy (MSA): topographic distribution of the alpha-synuclein-associated pathological changes.

To study the topographic distribution of the pathology in multiple system atrophy (MSA). Pattern analysis was carried out using alpha-synuclein immunohistochemistry in 10 MSA cases. The glial cytoplasmic inclusions (GCI) were distributed randomly or in large clusters. The neuronal inclusions (NI) and abnormal neurons were distributed in regular clusters. Clusters of the NI and abnormal neurons were spatially correlated whereas the GCI were not spatially correlated with either the NI or the abnormal neurons. The data suggest that the GCI represent the primary change in MSA and the neuronal pathology develops secondary to the glial pathology.

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PubMed2001

Erythropoietin deficiency and anaemia in multiple system atrophy.

Serum erythropoietin (EPO) levels are partially controlled by the sympathetic outflow to the kidney. We have studied whether patients with multiple system atrophy (MSA), known to be associated with dysautonomia, are EPO-deficient. Eighteen MSA patients were studied along with 32 idiopathic Parkinson's disease (PD) patients, 23 controls with iron-deficiency anaemia, and 18 healthy individuals. Serum creatinine was normal in all groups. Mean haemoglobin (Hb) concentration in MSA patients was 13.7 +/- 1.7 g/dL. Four MSA patients had unexplained anaemia (minimum Hb: 10.5 g/dL) and abnormal autonomic function tests including significant postural hypotension, whereas none of the PD patients was anaemic. Serum EPO levels were suppressed in relation to anaemia in MSA patients compared to elevated EPO levels in iron-deficiency anaemia patients (difference of regression lines P < 0.001), indicating EPO deficiency in the anaemic MSA patients. Serum EPO levels in PD patients were within normal range. A subset of MSA patients has anaemia and postural hypotension, which may be associated with EPO deficiency. This may have therapeutic implications.

Anemia↗
PubMed2005

Progression and prognosis in pure autonomic failure (PAF): comparison with multiple system atrophy.

OBJECTIVE: To clarify the progression of autonomic symptoms and functional deterioration in pure autonomic failure (PAF), particularly in comparison with multiple system atrophy (MSA). METHODS: The investigation involved eight patients with PAF (M/F = 7/1; mean age at onset, 57 years) and 22 with probable MSA matched for age at onset (M/F = 14/8; onset 56 years). Subjects were followed up for neurological symptoms, activities of daily living, and autonomic function for more than seven years. Autonomic functional tests were carried out. RESULTS: In PAF, fainting or sudomotor dysfunction occurred first, followed by constipation and syncope. Urinary dysfunction developed late, and respiratory dysfunction was not evident. This clinical course contrasted sharply with that in MSA, where early urinary dysfunction usually proceeded to sudomotor dysfunction or orthostatic hypotension (p = 0.004), followed by respiratory dysfunction (p = 0.0004). Results of pharmacological tests also distinguished PAF from MSA. Progression and prognosis in patients with PAF did not worsen, unlike the steady progressive autonomic dysfunction in MSA (p < 0.0001, p < 0.0001, p = 0.0009, and p = 0.003, for progression to modified Rankin scale grade III, IV, V, and death, respectively). CONCLUSIONS: The time course and pattern of progression of autonomic failure differed significantly between PAF and MSA. Patients with PAF had slower functional deterioration and a better prognosis.

Activities of Daily Living↗
PubMed2006

Placebo-controlled trial of riluzole in multiple system atrophy.

We performed a placebo-controlled cross-over trial of riluzole (100 mg b.i.d.) in 10 patients with probable multiple system atrophy (MSA) administering riluzole and placebo for 4 weeks each with a 4-week washout period. Outcome measures evaluated short-term anti-Parkinsonian effects using the Unified Parkinson's Disease Rating Scale (UPDRS) subscales (UPDRS-II, activities of daily living; UPDRS-III, motor examination; sum of UPDRS-II and -III) before and at the end of each treatment phase. Delta values were calculated by subtracting the UPDRS scores measured at the end of each treatment arm from those before onset of each medication phase. Riluzole was generally well tolerated. There were no significant anti-Parkinsonian effects of riluzole comparing the UPDRS delta values for both treatment arms using the Wilcoxon signed-rank test. It is unlikely that riluzole treatment could have clinically meaningful anti-Parkinsonian effects in MSA. A trial assessing the disease-modifying potential of riluzole in MSA is underway.

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PubMed2001

Primary involvement of the motor area in association with the nigrostriatal pathway in multiple system atrophy: neuropathological and morphometric evaluations.

To evaluate the changes that occur in the motor and supplementary motor cortices in cases of multiple system atrophy (MSA), we carried out morphological and morphometric studies in 7 cases of MSA and 11 age-matched controls. Neuropathological study revealed presence of glial cytoplasmic inclusions (GCIs) in the cortex and subcortical white matter of the motor and supplementary motor areas, loss of small to medium-sized pyramidal neurons, and astrocytosis in the motor cortex in all cases of MSA, showing a definite predilection in the cortical layers V and VI. The severity of neuronal loss in the motor cortex was highly correlated with the incidence of GCIs and the involvement of the nigrostriatal pathway. Morphometrically, significant reductions of both the thickness of motor cortical layers V and VI and the number of neurons were observed. In addition, a reduction in the number of neurons in the supplementary motor cortex was detected in three out of the seven MSA cases. The results of this study suggest that the motor area is a cardinal target in MSA, and that in association with the nigrostriatal pathway it forms a motor loop degeneration in this disease.

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PubMed2003

Alpha-synuclein accumulates in Purkinje cells in Lewy body disease but not in multiple system atrophy.

Alpha-synuclein has an important role in the pathogenesis of Parkinson disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA), comprising a new disease concept, that of alpha-synucleinopathies. Cerebellar degeneration with Purkinje cell depletion is present in the majority of MSA cases. By contrast, cerebellar pathology has not been demonstrated unequivocally in either PD or DLB. Recent immunohistochemical studies using anti-alpha-synuclein antibodies have shown that LB-type degeneration in PD and DLB is more widespread than previously recognized. To determine whether cerebellar Purkinje cells might be involved in alpha-synuclein pathology, we carried out immunohistochemical examinations of the cerebella of patients with PD (n = 10), DLB (n = 7), MSA (n = 10), Alzheimer disease and other tauopathies (n = 9), and age-matched control subjects (n = 10), using antibodies specific for alpha-synuclein. Although no abnormal accumulation of alpha-synuclein was noted in the Purkinje cell somata, numerous alpha-synuclein-positive, round inclusions were found in the cerebellar white matter in all the patients with PD and DLB. Immunohistochemical and ultrastructural examinations revealed that the majority of these inclusions was located in the Purkinje cell axons and consisted of granulo-filamentous structures. No such inclusions were observed in MSA, tauopathies, or controls. These findings indicate that Purkinje cells are also the victims of a-synuclein pathology in PD and DLB, but not in MSA.

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PubMed2000

Urinary dysfunction and orthostatic hypotension in multiple system atrophy: which is the more common and earlier manifestation?

OBJECTIVES: Urinary dysfunction and orthostatic hypotension are the prominent autonomic features in multiple system atrophy (MSA). A detailed questionnaire was given and autonomic function tests were performed in 121 patients with MSA concerning both urinary and cardiovascular systems. METHODS: Replies to the questionnaire on autonomic symptoms were obtained from 121 patients including three clinical variants; olivopontocerebellar atrophy (OPCA) type in 48, striatonigral degeneration (SND) type in 17, and Shy-Drager type in 56. Urodynamic studies comprised measurement of postmicturition residuals, EMG cystometry, and bethanechol injection. Cardiovascular tests included head up tilt test, measurement of supine plasma noradrenaline (norepinephrine,NA), measurement of R-R variability (CV R-R), and intravenous infusions of NA and isoproterenol. RESULTS: Urinary symptoms (96%) were found to be more common than orthostatic symptoms (43%) (p<0.01) in patients with MSA, particularly with OPCA (p<0.01) and SND (p<0.01) types. In 53 patients with both urinary and orthostatic symptoms, patients who had urinary symptoms first (48%) were more common than those who had orthostatic symptoms first (29%), and there were patients who developed both symptoms simultaneously (23%). Post-micturition residuals were noted in 74% of the patients. EMG cystometry showed detrusor hyperreflexia in 56%, low compliance in 31%, atonic curve in 5%, detrusor-sphincter dyssynergia in 45%, and neurogenic sphincter EMG in 74%. The cystometric curve tended to change from hyperreflexia to low compliance, then atonic curve in repeated tests. Bethanechol injection showed denervation supersensitivity of the bladder in 19%. Cardiovascular tests showed orthostatic hypotension below -30 mm Hg in 41%, low CV R-R below 1.5 in 57%, supine plasma NA below 100 pg/ml in 28%, and denervation supersensitivity of the vessels (alpha in 73%; beta2 in 60%) and of the heart (beta1 in 62%). CONCLUSION: It is likely that urinary dysfunction is more common and often an earlier manifestation than orthostatic hypotension in patients with MSA, although subclinical cardiovascular abnormalities appear in the early stage of the disease. The responsible sites seem to be central and peripheral for both dysfunctions.

Adult↗
PubMed1997

Facial myokymia in multiple system atrophy.

Two patients are described with clinical and neuroimaging features consistent with a diagnosis of multiple system atrophy (MSA). The patients are unusual in that facial myokymia became apparent clinically at some stage in their illness. In each patient, the nature and severity of the involuntary facial movements evolved over the course of the illness. Electrophysiologically the movement pattern was consistent with myokymia, and studies of blink-reflex responses suggested that the myokymic discharges were of brainstem origin. Involuntary facial movements described as facial action myoclonus with electrical characteristics consistent with myoclonus have been described previously in hereditary olivopontocerebellar atrophy (OPCA). Our report describes electrical and clinical features of facial myokymia in MSA with electrical features suggesting hyperexcitability of the facial motorneurons in the brainstem. Such myokymic movements may occur more frequently in MSA than previously recognised but may be missed clinically because of their evolving nature.

Blinking↗
PubMed1995

Octreotide effects on orthostatic hypotension in patients with multiple system atrophy: a controlled study of acute administration.

In nine patients with orthostatic hypotension due to multiple system atrophy, the effects of octreotide (100 micrograms s.c.), a somatostatin analogue, on blood pressure (BP), heart rate (HR), and norepinephrine (NE) plasma levels after 60 degrees head up tilt test were evaluated by a randomly controlled study. Octreotide increased supine BP, tilt-test duration (+28%), and delay to obtain minimal BP during the 60 degrees head up tilt (+50%). Octreotide changed neither HR nor NE plasma levels.

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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↗
PubMed2003

Effect of posture on blood and intraocular pressures in multiple system atrophy, pure autonomic failure, and baroreflex failure.

BACKGROUND: Intraocular pressure (IOP) may be partially under systemic vascular control. This study examined whether a correlation exists between blood and intraocular pressures in patients with autonomic dysfunction. METHODS AND RESULTS: Eleven patients with 3 types of confirmed autonomic dysfunction (multiple system atrophy, pure autonomic failure, and baroreflex failure) were compared with age- and gender-matched controls and had IOP, systolic blood pressure, diastolic blood pressure, heart rates, and calculated mean arterial pressures (MAP) and mean ocular perfusion pressures (MOPP) measured in the supine, sitting, and standing positions. Data were analyzed with a general linear model repeated-measures ANOVA. All pressures for patients showed a dramatic decline (P<0.001) from supine to standing (MAP -31+/-14 mm Hg; IOP -6+/-3 mm Hg; MOPP -25+/-14 mm Hg) compared with controls (MAP +4+/-7 mm Hg; IOP -1+/-2 mm Hg; MOPP +6+/-7 mm Hg). There was no significant change in heart rate from supine to standing for patients compared with controls (P=0.648). Within both the multiple system atrophy (n=5) and pure autonomic failure (n=4) groups, all initial pressures were similar to control pressures in the supine position, whereas patients with baroreflex failure (n=2) had higher mean pressures than respective controls in the supine position. CONCLUSIONS: In autonomic dysfunction, a large decrease in MAP correlated with a large decrease in IOP. These data suggest that the autonomic nervous system, perhaps through an influence on systemic blood pressure, has a significant role in IOP regulation.

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↗
PubMed2005

Sonographic assessment of urinary retention in multiple system atrophy and idiopathic Parkinson's disease.

Sonography of the bladder was performed before and after voluntary voiding in 20 subjects with possible/probable multiple system atrophy (MSA), 20 patients with idiopathic Parkinson's disease (PD), and 20 healthy controls. Mean residual volume was 173 ml (SD, 194) in MSA and significantly increased compared to both PD (21 ml; SD, 49) and controls (13 ml; SD, 12). Incomplete voiding (>100 ml of residual volume) was found in 11 subjects with MSA but only in 1 patient with PD. Positive predictive value of increased residual volume for MSA was 91.6% in this study, whereas negative predictive value was only 67.8. Bladder sonography is an objective, simple, and safe tool that allows one to screen for urinary retention, which is highly suggestive but incompletely sensitive for MSA. Because sonography is easily accessible and rapidly performed, it is feasible for routine assessment of atypical Parkinsonism.

Aged↗
PubMed2005

Myoclonic tremulous movements in multiple system atrophy are a form of cortical myoclonus.

We prospectively studied the clinical and electrophysiological features of myoclonic tremulous movements in patients with multiple system atrophy (MSA). Among 42 consecutive patients, 12 MSA-p (parkinsonian type) and 3 MSA-c (cerebellar type) patients showed small-amplitude myoclonic movements. These movements occurred in the distal part of the arms and fingers, particularly in posture or during voluntary movements. We conducted detailed electrophysiological studies in 11 patients. Electromyographic recordings showed irregular bursts of brief duration often synchronously involving the antagonistic muscles. Somatosensory evoked responses were slightly enlarged in some patients. Long-latency reflexes were enhanced in 7 patients. A jerk-locked averaging technique revealed premyoclonic cortical potentials in 9 patients. These findings suggest that small-amplitude myoclonic movements in MSA are a form of cortical myoclonus.

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