ArticleNeuroradiology2020
Neuromelanin imaging analyses of the substantia nigra in patients with Machado-Joseph disease.
Article in Neuroradiology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers, 1 of them a synthesis that pooled it.
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Who cites it
6 citing papers in PubMed, 1 synthesis or guideline pooled it, 11 citations in OpenAlex.
- Magnetic Resonance Imaging and Its Clinical Correlation in Spinocerebellar Ataxia Type 3: A Systematic Review.Frontiers in neuroscience · 2022Pooled it
- Altered Brain Iron Depositions of Spinocerebellar Ataxia Type 3: From Pre-Symptomatic to Symptomatic Stage.European journal of neurology · 2025Article
- Substantia nigra degeneration in spinocerebellar ataxia 2 and 7 using neuromelanin-sensitive imaging.European journal of neurology · 2025Article
- Two distinct degenerative types of nigrostriatal dopaminergic neuron in the early stage of parkinsonian disorders.Clinical parkinsonism & related disorders · 2024Article
- Structural alterations of spinocerebellar ataxias type 3: from pre-symptomatic to symptomatic stage.European radiology · 2023Article
- Is Generalized and Segmental Dystonia Accompanied by Impairments in the Dopaminergic System?Frontiers in neurology · 2021Article
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
purposeMachado-Joseph disease (MJD/SCA3) is the most frequent spinocerebellar ataxia worldwide. Pathological studies revealed less melanin-containing dopaminergic neurons in the substantia nigra (SN) pars compacta in MJD/SCA3 patients. The purpose of this study was to quantify the damage in the SN reported for MJD/SCA3 patients in vivo using neuromelanin MR imaging.
methodsWe retrospectively reviewed the clinical information and MR imaging in sixteen MJD/SCA3 patients and fourteen healthy controls. High-resolution T1-WI of the SN were acquired using a 3-T MR system. The neuromelanin-related contrast (NRC), which was defined as the number of pixels of high-signal-intensity areas on T1-WI in the SN, was calculated semi-automatically. The NRC values were compared between MJD/SCA3 patients and normal controls.
resultsThe NRC values were significantly lower in MJD/SCA3 patients than in healthy controls. In MJD/SCA3 patients, a significant negative correlation existed between disease durations and NRC values. No significant difference in the NRC values was revealed between the MJD/SCA3 patients with and without parkinsonism.
conclusionNeuromelanin in the SN may decrease at the early stage of the disease and continue to decrease over time with the disease duration in MJD/SCA3 patients. The NRC may be a useful biological index for monitoring MJD/SCA3.
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