Quantifying iron deposition in the cerebellar subtype of multiple system atrophy and spinocerebellar ataxia type 6 by quantitative susceptibility mapping

• Published in: Journal of the neurological sciences Vol. 407; p. 116525
• Main Authors: Sugiyama, Atsuhiko, Sato, Noriko, Kimura, Yukio, Fujii, Hiroyuki, Maikusa, Norihide, Shigemoto, Yoko, Suzuki, Fumio, Morimoto, Emiko, Koide, Kyosuke, Takahashi, Yuji, Matsuda, Hiroshi, Kuwabara, Satoshi
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 15-12-2019
• Subjects: Dentate nucleus; MSA-C; QSM; SCA6; Substantia nigra; MSA-C; QSM; SCA6; Dentate nucleus; Substantia nigra
• ISSN: 0022-510X; 1878-5883
• DOI: 10.1016/j.jns.2019.116525

We used quantitative susceptibility mapping (QSM) to assess the brain iron deposition in 28 patients with the cerebellar subtype of multiple system atrophy (MSA-C), nine patients with spinocerebellar ataxia type 6 (SCA6), and 23 healthy controls. Two reviewers independently measured the mean QSM values in brain structures including the putamen, globus pallidus, caudate nucleus, red nucleus, substantia nigra, and cerebellar dentate nucleus. A receiver operating characteristics (ROC) analysis was performed to assess the diagnostic usefulness of the QSM measurements. The QSM values in the substantia nigra were significantly higher in the MSA-C group compared to the HC group (p = .007). The QSM values in the cerebellar dentate nucleus were significantly higher in MSA-C than those in the SCA6 and HC groups (p < .001), and significantly lower in the SCA6 patients compared to the HCs (p = .027). The QSM values in the cerebellar dentate nucleus were correlated with disease duration in MSA-C, but inversely correlated with disease duration in SCA6. In the ROC analysis, the QSM values in the cerebellar dentate nucleus showed excellent accuracy for differentiating MSA-C from SCA6 (area under curve [AUC], 0.925), and good accuracy for differentiating MSA-C from healthy controls (AUC 0.834). QSM can identify increased susceptibility of the substantia nigra and cerebellar dentate nucleus in MSA-C patients. These results suggest that an increase in iron accumulation in the cerebellar dentate nucleus may be secondary to the neurodegeneration associated with MSA-C.
•We used QSM to assess the brain iron deposition in MSA-C, SCA6, and HC.•The QSM values in the dentate nucleus were higher in MSA-C than those in the others.•The QSM values in the substantia nigra were higher in MSA-C than those in HC.•The QSM value in the dentate nucleus were correlated with disease duration in MSA-C.