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Radiological hints for differentiation of cerebellar multiple system atrophy from spinocerebellar ataxia

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Patients (Table 1)

The age of onset and the age at MRI examination were significantly younger in patients with SCA than in those with MSA-C. There was no significant difference in sex distribution between these two disease entities.

The CAG repeat lengths and the presence of the HCBS in different subtypes of SCA and MSA-C (Table 2)

Table 2 The presence of HCBS in different subtypes of SCA and MSA-C.

All SCA patients in this study have pathologically expanded alleles in different genes. However, among the patients with MSA, none have an intermediate or expanded SCA alleles (SCA1, 2, 3, 6, 7, or 17).

Among the 286 patients with MSA-C, 249 (87.1%) had a pontine HCBS on T2WI MRI at the first brain MRI examination. Of the 684 study subjects (398 with SCA and 286 with MSA-C), 107 patients had a follow -up brain MRI / MRS and six MSA-C patients demonstrated novel HCBS later with disease evolution. The overall prevalence of pontine HCBS was 89.16% in patients with MSA-C and 7.29% in patients with SCA, (among them, 68.97% was found to be SCA2).

Correlation between parameters

The CAG repeat lengths were negatively correlated with the age of onset in SCA patients (p <0.001). Disease duration was significantly and positively correlated with SARA scores (p <0.001), and negatively correlated with cerebellar NAA / Cr (p = 0.040) , cerebellar Cho / Cr (p <0.001), vermis NAA / Cr (p = 0.005), and vermis Cho / Cr (p = 0.017). The longer the disease duration, the higher the SARA scores, and the lower the MRS ratios Because disease duration significantly correlated with MRS parameters and SARA scores, we opted to evaluate the differences in these parameters based on different disease durations.

The differences of parameters between patients of MSA-C and SCA with different disease duration (Table 3)

Table 3 The differences of parameters between patients of MSA-C and SCA with different disease durations.

For patients with a disease duration within 1 year, despite their comparable severity of clinical symptoms, the prevalence of HCBS was significantly higher in patients with MSA-C than in those with SCA. Significantly lower cerebellar NAA / Cr, Cho / Cr, and vermis NAA / Cr, Cho / Cr, and higher SARA scores were also found in patients with MSA-C.

Using receiver operating characteristic curve (ROC) analysis, cerebellar NAA / Cr had the greatest AUC in differentiating MSA-C from SCA. The cutoff value of cerebellar NAA / Cr was 0.79 for raising an index of suspicion of MSA-C with a sensitivity of approximately 89.9%, a specificity of 88.0%, and an AUC of 0.939.

For patients with a similar disease duration in the bracket of 2–3 years, patients with MSA-C deteriorated faster with the need for walking assistance earlier in some of them6and the prevalence of pontine HCBS was significantly higher in patients with MSA-C than in those with SCA. Significantly lower cerebellar NAA / Cr, Cho / Cr, and vermis NAA / Cr, Cho / Cr, and higher SARA scores were found in patients with MSA-C.

Using ROC curve analysis, cerebellar NAA / Cr had the best AUC differentiating MSA-C from SCA. The cutoff of cerebellar NAA / Cr was 0.75 for raising an index of suspicion of MSA-C with a sensitivity of approximately 86.9%, a specificity of 81.1%, and an AUC of 0.865.

For patients with a disease duration in the bracket of 4–5 years, patients with MSA-C had mostly been in a wheelchair6The prevalence of HCBS was significantly higher in patients with MSA-C than in those with SCA. Significantly lower cerebellar NAA / Cr, Cho / Cr, and vermis NAA / Cr, Cho / Cr, and higher SARA scores were also found in patients with MSA-C.

Using ROC curve analysis, cerebellar NAA / Cr had the greatest AUC for differentiating MSA from SCA. The cutoff of cerebellar NAA / Cr was 0.72 for raising an index of suspicion of MSA with a sensitivity of approximately 90.3%, a specificity of approximately 84.2% , and an AUC of 0.917.

For patients with a disease duration in the bracket of 6–8 years, MSA-C patients had all been in a bedridden state6The prevalence of HCBS was significantly higher in patients with MSA-C than in those with SCA. Significantly lower cerebellar NAA / Cr, Cho / Cr, and vermis NAA / Cr, Cho / Cr, and higher SARA scores were also found in patients with MSA-C.

Using ROC curve analysis, cerebellar NAA / Cr and Cho / Cr had similar AUCs in differentiating MSA from SCA. The cutoff of cerebellar NAA / Cr was 0.64 for raising an index of suspicion of MSA with a sensitivity of approximately 83.9%, a specificity of 87.6%, and an AUC of 0.905. The cutoff of cerebellar Cho / Cr was 0.54 for raising an index of suspicion of MSA with a sensitivity of approximately 80.7%, a specificity of approximately 92.8%, and an AUC of 0.913.

We only had 30 patients with a disease duration longer than 8 years in the MSA cohort. Although the disease durations were significantly longer in SCA patients, further subgroup analysis was not possible given the limited number of MSA-C cases with a disease duration longer than 8 years. The prevalence of HCBS was significantly higher in patients with MSA-C than in those with SCA. Significantly lower cerebellar NAA / Cr, Cho / Cr, and vermis NAA / Cr, Cho / Cr, and higher SARA scores were also found in patients with MSA-C.

Using ROC curve analysis, cerebellar NAA / Cr and Cho / Cr had similar AUCs in differentiating MSA from SCA. The cutoff of cerebellar NAA / Cr was 0.64 for raising an index of suspicion of MSA with a sensitivity of approximately 86.2%, a specificity of 87.0%, and an AUC of 0.908. The cutoff of cerebellar Cho / Cr was 0.56 for raising an index of suspicion of MSA with a sensitivity of approximately 87.5%, a specificity of approximately 83.3%, and an AUC of 0.904.

Differentiation of MSA-C from SCA in patients without HCBS in the initial stages of disease (Table 4)

Table 4 The differences of parameters between HCBS negative patients with MSA-C and SCA with a disease duration within 1 year.

We found that pontine HCBS could mostly (89.16%) be detected during follow-up MRI studies in patients with MSA-C. However, discerning MSA-C from SCA in patients without pontine HCBS in the initial stages of disease is both challenging and important with a disease duration of <1 year, we had 15 MSA-C and 46 SCA patients without HCBS in their MRI. Using ROC analysis, a significantly higher AUC of cerebellar NAA / Cr than cerebellar Cho / Cr was found for differentiating MSA-C from SCA. The cutoff for cerebellar NAA / Cr in these patients to raise an index of suspicion of MSA-C was 0.79. The sensitivity, specificity, positive predictive value (PPV) and negative predictive value (PPV) NPV) for MSA-C in patients without HCBS but having cerebellar NAA / Cr ≤ 0.79 were 73.3%, 90.0%, 71.0%, and 91.0%, respectively.

Case demonstration (Fig. 1)

Figure 1
figure 1

Axial FLAIR images at pons level and cerebellar MRS of patients with MSA or SCAs.

The cerebellar MRS of patients diagnosed as MSA-C and SCAs with a duration less than 1 year and without HCBS were demonstrated in Fig. 1. The SARA scores were 4, 9.5, 2, 4, 4 and cerebellar NAA / Cr were 0.69, 0.9, 0.87, 1.02, and 1.02 for MSA-C, SCA1, SCA2, SCA3, and SCA6, respectively.

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