Response to L-DOPA in Multiple System Atrophy

Parati, Eugenio; Fetoni, V.; Geminiani, Giuliano; Soliveri, Paola; Giovannini, P.; Testa, D.; Genitrini, S.; Caraceni, T.; Girotti, F.

doi:10.1097/00002826-199304000-00006

Cited by 66 publications

(28 citation statements)

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“…Furthermore, patients with MSA often have excellent initial responses but frequently develop levodopa related orofacial dyskinesias and lose antiparkinsonian efficacy. Although improvement with levodopa is suggestive of PD, it does not definitively differentiate PD from other parkinsonian disorders 137. One study found that only 77% of patients with pathologically proven PD had a “good” or “excellent” initial response to levodopa 36.…”

Section: Assessment Of Patients With Pdmentioning

confidence: 99%

Parkinson's disease: clinical features and diagnosis

Jankovic

2008

Journal of Neurology, Neurosurgery & Psychiatry

4,382

2,812

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Section: Assessment Of Patients With Pdmentioning

confidence: 99%

Parkinson's disease: clinical features and diagnosis

Jankovic

2008

Journal of Neurology, Neurosurgery & Psychiatry

4,382

2,812

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“…Progressive loss of striatal target cells and striatal output systems probably accounts for the poorly sustained response to levodopa in most MSA patients (134,135). However, other observations suggest that levodopa responsiveness is not solely dependent on the degree of striatal cell loss.…”

Section: Clinicopathological Correlation Parkinsonismmentioning

confidence: 99%

Multiple system atrophy: A review of 203 pathologically proven cases

Tison²,

et al. 1997

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We report the clinicopathological features of 203 cases of pathologically proven multiple system atrophy (MSA) from 108 publications up to February 1995. The majority of patients showed symptoms in their early fifties, and men were more commonly affected than women (ratio of 1.3:1). Most patients suffered from some degree of autonomic failure (74%). Parkinsonism was the most common motor disorder (87%), followed by cerebellar ataxia (54%) and pyramidal signs (49%). The response to levodopa was poor in most patients, but there was a subgroup with a good response, who also often developed axial levodopa-induced dyskinesias. Other characteristic features included severe dysarthria, stridor, and, in a few patients, contractures and dystonia (antecollis). Mild or moderate intellectual impairment occurred in some cases, but severe dementing illness was most unusual. The main pathological change comprised cell loss and gliosis in the putamen, caudate nucleus, external pallidum, substantia nigra, locus ceruleus, inferior olives, pontine nuclei, cerebellar Purkinje cells, and intermediolateral cell columns of the spinal cord. However, other neuronal populations were also involved to varying degrees, such as the thalamus, vestibular nucleus, dorsal vagal nucleus, corticospinal tracts, and anterior horn cells. Characteristic glial and/or neuronal cytoplasmic inclusions were identified in all cases in which they were sought, irrespective of clinical presentation. Akinesia correlated with the degree of nigral and putaminal cell loss, whereas rigidity was related only to the later. Tremor was unrelated to cell loss at any site. Ataxia correlated with the degree of olivopontocerebellar atrophy. Pyramidal signs were associated with pyramidal tract pallor. Our analysis also confirmed an association of postural hypotension with intermediolateral cell column degeneration.

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“…This may explain the differences between the two conditions in responsiveness to dopaminergic therapy. 33,34 The Basal Ganglia Model…”

Section: Figmentioning

confidence: 99%

Neuronal activity in the globus pallidus of multiple system atrophy patients

Pereira¹,

Palter²,

Lang³

et al. 2004

Movement Disorders

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The pathophysiological changes in neural activity that characterize multiple system atrophy (MSA) are largely unknown. We recorded the activity of pallidal neurons in 3 patients with clinical and radiological features of MSA who underwent unilateral microelectrode-guided pallidotomy for disabling parkinsonism. Findings in these patients were compared with 4 control patients with a clinical diagnosis of Parkinson's disease (PD). The position, firing rates, and firing patterns of single neurons in the pallidal complex were analyzed in both MSA and PD patients. The mean spontaneous firing rate of neurons in the internal segment of the globus pallidus internus (GPii) was significantly lower in MSA than in PD patients. There were no significant differences between MSA and PD patients, however, in firing rates of neurons in the external globus pallidus (GPe) or in the external segment of GPi (GPie). In addition, no significant differences in firing pattern were found between MSA and PD patients. In conclusion, this study has shown that firing rates of neurons in GPii but not in GPie and GPe are different in MSA patients compared with that in PD patients, a finding that may reflect the poor clinical results of pallidotomy reported in patients with MSA.

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Response to L-DOPA in Multiple System Atrophy

Cited by 66 publications

References 0 publications

Parkinson's disease: clinical features and diagnosis

Parkinson's disease: clinical features and diagnosis

Multiple system atrophy: A review of 203 pathologically proven cases

Neuronal activity in the globus pallidus of multiple system atrophy patients

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