ArticleFrontiers in neurology2023
Neuropathological features of levodopa-responsive parkinsonism in multiple system atrophy: an autopsy case report and comparative neuropathological study.
Article in Frontiers in neurology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers, 1 of them a synthesis that pooled it.
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Who cites it
4 citing papers in PubMed, 1 synthesis or guideline pooled it, 3 citations in OpenAlex.
- Dopaminergic responsiveness and dopaminergic challenge tests of Parkinson's disease: a systematic review and meta-analysis.Journal of neurology · 2025Pooled it
- Gut microbiome dysbiosis, short-chain fatty acid depletion and implications for neuroinflammation in atypical parkinsonian syndromes - a systematic review of patient cohorts.Journal of Parkinson's disease · 2026Review
- Persistent parkinsonism with dopaminergic denervation following axicabtagene ciloleucel therapy for diffuse large B cell lymphoma: a case report.International journal of hematology · 2026Article
- Late-onset multiple system atrophy: Neuropathological features associated with slow disease progression.Brain pathology (Zurich, Switzerland) · 2025Article
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Authors and funding
9 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Background: In typical patients with multiple system atrophy with predominant parkinsonism (MSA-P) levodopa is ineffective. However, there are some of these patients who respond well to levodopa treatment. Levodopa efficacy in MSA-P patients is thought to be related to the degree of putaminal damage, but the pathological causation between the putaminal involvement and levodopa efficacy has not been established in detail. Objective: This study aimed to evaluate the neuropathological features of the nigrostriatal dopaminergic system in a "levodopa-responsive" MSA-P patient in comparison with "levodopa-unresponsive" conventional MSA-P patients. Materials and methods: Clinicopathological findings were assessed in a 53-year-old Japanese man with MSA who presented with asymmetric parkinsonism, levodopa response, and later wearing-off phenomenon. During autopsy, the nigrostriatal pathology of presynaptic and postsynaptic dopaminergic receptor density and α-synuclein status were investigated. The other two patients with MSA-P were examined using the same pathological protocol. Results: Four years after the onset, the patient died of sudden cardiopulmonary arrest. On autopsy, numerous α-synuclein-positive glial cytoplasmic inclusions in the basal ganglia, pons, and cerebellum were identified. The number of neurons in the putamen and immunoreactivity for dopamine receptors were well-preserved. In contrast, significant neuronal loss and decreased dopamine receptor immunoreactivity in the putamen were observed in the "levodopa-unresponsive" MSA-P control patients. These putaminal pathology results were consistent with the findings of premortem magnetic resonance imaging (MRI). All three patients similarly exhibited severe neuronal loss in the substantia nigra and decreased immunoreactivity for dopamine transporter. Conclusion: Levodopa responsiveness in patients with MSA-P may be corroborated by the normal putamen on MRI and the preserved postsynaptic nigrostriatal dopaminergic system on pathological examination. The results presented in this study may provide a rationale for continuation of levodopa treatment in patients diagnosed with MSA-P.
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