Objective: To investigate the availability of levodopa for pathologically confirmed multiple system atrophy (MSA) patients and determine the clinicopathological features of levodopa responders with MSA.

Background: MSA patients can show parkinsonism in addition to cerebellar ataxia, autonomic dysfunction, and other neurological symptoms. While levodopa or dopamine agonists are ineffective in most patients with MSA, some MSA patients show a small response [1].

Method: We obtained clinical data, including efficacies of levodopa and dopamine agonists, magnetic resonance (MR) findings, and pathological findings retrospectively from the medical records of 12 pathologically confirmed MSA patients (M/F = 6/6).

Results: The mean age at onset was 61.5 ± 8.2 (44–73) years. The mean disease duration was 8.0 ± 4.8 (3–15) years. Eleven patients had MSA (7 had predominant parkinsonism (MSA-P) and 4 had predominant cerebellar ataxia (MSA-C)). One patient was clinically diagnosed with progressive supranuclear palsy (PSP) and showed concomitant pathology of MSA and PSP. Nine patients received levodopa treatment (maximum dose 300–900 mg), which was effective in four patients. All levodopa responders had MSA-P. One patient showed wearing-off phenomenon. In three patients, levodopa was either poorly effective or ineffective. The medical data of two patients contained no information on levodopa efficacy. A dopamine agonist was mildly effective in one MSA-C patient. Of the levodopa responders, two patients showed outstanding autonomic dysfunction relatively later in their clinical courses and became bed-ridden after more than 10 years from the disease onset time. In two patients, one dopamine responder and one dopamine-agonist responder, putaminal rims appeared on MR images in the late stage of the disease (7–10 years from onset). Neurodegeneration of the putamen was relatively mild.

Conclusion: This study revealed that levodopa availability was more than 30% in patients with MSA who were confirmed to not have Parkinson’s disease. The putamen may be relatively preserved from neurodegeneration in responders to levodopa or dopamine agonists, as shown in a previous study.1 In addition, levodopa responders without early occurrence of autonomic dysfunction might be able to maintain daily life activities for a prolonged time.

References: [1] Ozawa T, et al. The spectrum of pathological involvement of the striatonigral and olivopontocerebellar systems in multiple system atrophy: clinicopathological correlations. Brain 2004;127:2657-2671.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/availability-of-levodopa-and-clinicopathological-features-of-patients-with-multiple-system-atrophy/