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Results for “Multiple System Atrophy”

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PubMed2006

[Clinical characteristics of multiple system atrophy in serbian population].

BACKGROUND/AIM: Mulstiple system atrophy (MSA) is a neurodegenerative central nervous system disorder, characterized by any combination of extrapyramidal, cerebellar, pyramidal or autonomic disturbance. The aims of our study were to define clinical characteristics of MSA patients in our population, to account for neuroradiological and electrophysiological profile of the disease and to evaluate one-dose levodopa response. METHODS: We have diagnosed 29 patients as MSA, with disease duration from the first symptom 5 years on average on examination. The examinating procedure included an anamnesis and complete neurological investigations, as well as neurootological and neuroradiological examinations. The study included the patients of the Institute of Neurology of the Clinical Center of Serbia in the period of 1996-2001, who completed both clinical and diagnostic criteria for a possible and probable MSA. RESULTS: Autonomic disturbances were documented in 93.1%, whereas extrapyramidal symptoms were seen in additional 89.3% with symmetrical onset in 60%. Levodopa response was poor or moderate. Cerebellar signs were present in 63%, while pyramidal signs occured in 78.7%. There was no a cognitive deterioration (MMSE > 24). CT scan and MRI showed cerebellar and brainstem atrophy, as well as diffuse cortical atrophy. CONCLUSION: Failure of additional diagnostic procedures to distinguish MSA patients required a precise understanding of their clinical specificities. Our results support this statement.

Antiparkinson Agents↗
PubMed1997

The acoustic startle response is normal in patients with multiple system atrophy.

We investigated the acoustic startle response in eight patients with MSA and compared the results with those from a group of age matched healthy subjects. Onset latency and amplitude of the responses obtained in the orbicularis oculi, masseter and sternocleidomastoid muscles were not different in patients and control subjects. We conclude that, in spite of the pathological derangement described in brainstem reticular nuclei in MSA, the neuronal circuits mediating the auditory startle reflex are functionally preserved.

Acoustic Stimulation↗
PubMed2005

Pathological hypersexuality predominantly linked to adjuvant dopamine agonist therapy in Parkinson's disease and multiple system atrophy.

Pathological hypersexuality developed in 13 patients with PD and two patients ultimately diagnosed clinically with MSA. Hypersexuality began within 8 months after starting dopamine agonist therapy in 14 of 15 cases, including four on agonist monotherapy. It resolved in the four cases where the agonist was stopped, despite continued levodopa therapy. This was not an isolated behavioral problem in most, with additional compulsive or addictive behaviors coinciding in nine patients (60%). A systematic literature review of pathological hypersexuality in PD revealed similar medication histories; combining these cases with our series, 26 of 29 patients (90%) were on adjuvant dopamine agonists.

Adult↗
PubMedDate not supplied

Immunohistochemical localization of phosphoinositide 3-kinase in brains with multiple system atrophy.

Oligodendrocytes have been shown to display some morphological characteristics of apoptosis in MSA. The accumulated evidence shows that phosphoinositide 3-kinase (PI3K) is closely associated with the regulation of apoptosis. Thus, we examined immunohistochemically PI3K in the cerebellum and pons from autopsy samples with MSA. In control tissues, PI3K was immunostained in some neurons and a few oligodendrocytes. In MSA samples, the larger number of oligodendrocytes was observed in the pons and cerebellum. Furthermore, some neurons were strongly immunolabeled in MSA samples. The recent study has shown that PI3K phosphorylates PKB/Akt which phosphorylates BAD resulting in the cessation of apoptotic process. The present results suggest that PI3K is upregulated in oligodendrocytes and some neurons in MSA, possibly in response to the apoptotic signals to these cells.

Aged↗
PubMed1999

Multiple system atrophy.

MSA is a complex disorder, with regard to its pathology and cause as well as its clinical diagnosis and treatment. Although a number of clinical treatments may improve quality of life for these patients, given the widespread pathology present, symptomatic treatment, particularly that involving neurotransmitter replacement, is likely to remain difficult. Truly effective treatment for these patients is likely to depend on an understanding of the underlying pathogenic mechanisms and methods to halt or reverse disease progression. A firm understanding of the classification of these disorders is the first step to understanding the relevant pathogenic mechanisms. The finding of intracytoplasmic glial inclusion bodies provides a compelling piece of evidence that SND, OPCA, and SDS do, in fact, belong to one nosologic entity. These inclusions do not seem to be present in familial cases of OPCA; thus, they may provide a means to improve diagnostic specificity as well as sensitivity. With the ability to define clearly the entity of MSA, an understanding of the pathophysiology can be developed along with other degenerative neurologic diseases, including Parkinson's disease, Alzheimer's disease, amyotrophic lateral sclerosis, and Huntington's disease.

Brain↗
PubMed2003

Cutaneous sympathetic function in patients with multiple system atrophy.

Some procedures increase the sweat output (SSwR; sympathetic sweat response) and reduce the cutaneous blood flow (SVR; skin vasomotor reflex) in the hand. We evaluated SSwRs and SVRs to deep inspiration, mental arithmetic, exercise, and tactile stimulation in 40 MSA patients and 15 healthy controls. We also conducted head-up tilt tests and R-R interval variation tests (CV(R-R)). SSwRs were present in all controls, but absent in 19 (47.5 %) of the MSA patients. The mean SSwR amplitudes in the MSA group were significantly lower than those in the control group. SVRs were evoked in all subjects except 3 MSA patients. There were no marked differences in SVR amplitudes between the two groups. Orthostatic hypotension and low CV(R-R) values were seen in 18 (45 %) and 13 (32.5 %) of the MSA patients, respectively. SSwR amplitudes correlated significantly with postural fall in blood pressure and CV(R-R) values in the MSA group. SSwRs were absent in about half of the MSA patients, and the SSwR results correlated with those of the cardiovascular autonomic tests. The SVRs were not severely disturbed in the MSA patients. We considered SSwR a useful index for the detection of autonomic dysfunction in MSA.

Blood Flow Velocity↗
PubMed2000

Postural and action myoclonus in patients with parkinsonian type multiple system atrophy.

Patients with a parkinsonian syndrome and features of multisystem atrophy (pMSA) may exhibit abnormal movements of the hands and fingers, which are reported in the literature either as "jerky" tremor or myoclonus. We studied clinically and electrophysiologically these movements in 11 consecutive patients with pMSA. No abnormal movements were observed when the patients were at complete rest, except for a characteristic parkinsonian "pill-rolling" tremor in one patient. Abnormal small-amplitude, nonrhythmic movements involving just one or a few fingers, or more rarely the whole hand, were observed in nine patients when holding a posture or at the beginning of an action. Accelerometric recordings showed small-amplitude irregular oscillations which, contrary to those of patients with tremor, had no predominant peak in the Fast Fourier frequency spectrum analysis. Electromyographic recordings in the forearm and hand muscles showed brief jerks of less than 100 ms duration which were synchronous in antagonist muscles of the forearm and alternated with brief periods of silence. Electrical stimulation of the digital nerves evoked consistent reflex responses in the wrist flexor and extensor muscles at a latency of 55.3+/-4.1 ms (range, 50-63 ms). Routine electroencephalographic (EEG) and somatosensory evoked potentials to median nerve stimulation were normal. Back-averaging of the EEG activity time-locked to the jerks was performed in two patients with no evidence of abnormal cortical activity. Two patients had episodes of transient respiratory failure related to pneumonia. This caused a long-lasting enhancement of the abnormal hand and finger movements, which became larger and more widespread, with features of posthypoxic myoclonus. We conclude that the abnormal hand and finger movements of patients with pMSA are a form of postural and action myoclonus, and can be described as mini-polymyoclonus.

Aged↗