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Abstract
Discussion Forum (0)
Fat fraction and Cross-sectional area analysis of the sciatic nerve in Charcot‑Marie‑Tooth disease type 1A patients using MRI
Poster No: 1142

Presenter: Hye Mi Kwon
Institution:

Introduction: CMT type 1A (CMT1A) results from duplication of the peripheral myelin protein 22 (PMP22) gene on chromosome 17. CMT1A is a demyelinating neuropathy, that typically demonstrates nerve hypertrophy as an increased cross-sectional area (CSA) on imaging studies compared with normal controls. Our purpose in this study was to assess the fat fraction (FF) and CSA of the sciatic nerve in CMT1A patients using Dixon-based proton density fat quantification MRI and to elucidate its potential association with clinical parameters.

Methods: Thigh MRIs of 18 CMT1A patients and 18 age- and sex-matched volunteers enrolled. Analyses for FF and CSA of the sciatic nerve were performed at three levels (proximal, middle, distal). CSA and FF were compared between the two groups and among the different levels within each group. The relationship between the MRI parameters and clinical data were assessed in the CMT1A patients.

Results: The CMT1A patients showed significantly higher FF at level 3 (p = 0.0217) and significantly larger CSA at all three levels compared with the control participants (p < 0.0001). Comparisons among levels showed significantly higher FF for levels 2 and 3 than for level 1 and significantly larger CSA for level 2 compared with level 1 in CMT1A patients. CSA at level 3 correlated positively with the CMT neuropathy score version 2 (CMTNSv2).

Conclusions: In conclusion, the sciatic nerve FF of CMT1A patients was significantly higher at level 3 compared with both the controls and the measurements taken on more proximal levels. Our result showing a distal tendency for increased FF in CMT1A patients could suggest a distal predominance of increased interfascicular fat. Sciatic nerve CSA at level 3 correlated significantly and positively with CMTNSv2, suggesting its potential value as an imaging marker for clinical severity.

References: No
References 1:
References 2:
References 3:
References 4:

Grant Support:

Keywords: Charcot-Marie-Tooth disease, cross sectional area, Intraepineurial fat quantification, CMT1A, sciatic nerve
Fat fraction and Cross-sectional area analysis of the sciatic nerve in Charcot‑Marie‑Tooth disease type 1A patients using MRI
Poster No: 1142

Presenter: Hye Mi Kwon
Institution:

Introduction: CMT type 1A (CMT1A) results from duplication of the peripheral myelin protein 22 (PMP22) gene on chromosome 17. CMT1A is a demyelinating neuropathy, that typically demonstrates nerve hypertrophy as an increased cross-sectional area (CSA) on imaging studies compared with normal controls. Our purpose in this study was to assess the fat fraction (FF) and CSA of the sciatic nerve in CMT1A patients using Dixon-based proton density fat quantification MRI and to elucidate its potential association with clinical parameters.

Methods: Thigh MRIs of 18 CMT1A patients and 18 age- and sex-matched volunteers enrolled. Analyses for FF and CSA of the sciatic nerve were performed at three levels (proximal, middle, distal). CSA and FF were compared between the two groups and among the different levels within each group. The relationship between the MRI parameters and clinical data were assessed in the CMT1A patients.

Results: The CMT1A patients showed significantly higher FF at level 3 (p = 0.0217) and significantly larger CSA at all three levels compared with the control participants (p < 0.0001). Comparisons among levels showed significantly higher FF for levels 2 and 3 than for level 1 and significantly larger CSA for level 2 compared with level 1 in CMT1A patients. CSA at level 3 correlated positively with the CMT neuropathy score version 2 (CMTNSv2).

Conclusions: In conclusion, the sciatic nerve FF of CMT1A patients was significantly higher at level 3 compared with both the controls and the measurements taken on more proximal levels. Our result showing a distal tendency for increased FF in CMT1A patients could suggest a distal predominance of increased interfascicular fat. Sciatic nerve CSA at level 3 correlated significantly and positively with CMTNSv2, suggesting its potential value as an imaging marker for clinical severity.

References: No
References 1:
References 2:
References 3:
References 4:

Grant Support:

Keywords: Charcot-Marie-Tooth disease, cross sectional area, Intraepineurial fat quantification, CMT1A, sciatic nerve
Fat fraction and Cross-sectional area analysis of the sciatic nerve in Charcot‑Marie‑Tooth disease type 1A patients using MRI
Dr. Hyemi Kwon
Dr. Hyemi Kwon
PNS 2022 Annual Meeting eLibrary. Kwon H. 04/14/2022; 355995; 1142
user
Dr. Hyemi Kwon
Abstract
Discussion Forum (0)
Fat fraction and Cross-sectional area analysis of the sciatic nerve in Charcot‑Marie‑Tooth disease type 1A patients using MRI
Poster No: 1142

Presenter: Hye Mi Kwon
Institution:

Introduction: CMT type 1A (CMT1A) results from duplication of the peripheral myelin protein 22 (PMP22) gene on chromosome 17. CMT1A is a demyelinating neuropathy, that typically demonstrates nerve hypertrophy as an increased cross-sectional area (CSA) on imaging studies compared with normal controls. Our purpose in this study was to assess the fat fraction (FF) and CSA of the sciatic nerve in CMT1A patients using Dixon-based proton density fat quantification MRI and to elucidate its potential association with clinical parameters.

Methods: Thigh MRIs of 18 CMT1A patients and 18 age- and sex-matched volunteers enrolled. Analyses for FF and CSA of the sciatic nerve were performed at three levels (proximal, middle, distal). CSA and FF were compared between the two groups and among the different levels within each group. The relationship between the MRI parameters and clinical data were assessed in the CMT1A patients.

Results: The CMT1A patients showed significantly higher FF at level 3 (p = 0.0217) and significantly larger CSA at all three levels compared with the control participants (p < 0.0001). Comparisons among levels showed significantly higher FF for levels 2 and 3 than for level 1 and significantly larger CSA for level 2 compared with level 1 in CMT1A patients. CSA at level 3 correlated positively with the CMT neuropathy score version 2 (CMTNSv2).

Conclusions: In conclusion, the sciatic nerve FF of CMT1A patients was significantly higher at level 3 compared with both the controls and the measurements taken on more proximal levels. Our result showing a distal tendency for increased FF in CMT1A patients could suggest a distal predominance of increased interfascicular fat. Sciatic nerve CSA at level 3 correlated significantly and positively with CMTNSv2, suggesting its potential value as an imaging marker for clinical severity.

References: No
References 1:
References 2:
References 3:
References 4:

Grant Support:

Keywords: Charcot-Marie-Tooth disease, cross sectional area, Intraepineurial fat quantification, CMT1A, sciatic nerve
Fat fraction and Cross-sectional area analysis of the sciatic nerve in Charcot‑Marie‑Tooth disease type 1A patients using MRI
Poster No: 1142

Presenter: Hye Mi Kwon
Institution:

Introduction: CMT type 1A (CMT1A) results from duplication of the peripheral myelin protein 22 (PMP22) gene on chromosome 17. CMT1A is a demyelinating neuropathy, that typically demonstrates nerve hypertrophy as an increased cross-sectional area (CSA) on imaging studies compared with normal controls. Our purpose in this study was to assess the fat fraction (FF) and CSA of the sciatic nerve in CMT1A patients using Dixon-based proton density fat quantification MRI and to elucidate its potential association with clinical parameters.

Methods: Thigh MRIs of 18 CMT1A patients and 18 age- and sex-matched volunteers enrolled. Analyses for FF and CSA of the sciatic nerve were performed at three levels (proximal, middle, distal). CSA and FF were compared between the two groups and among the different levels within each group. The relationship between the MRI parameters and clinical data were assessed in the CMT1A patients.

Results: The CMT1A patients showed significantly higher FF at level 3 (p = 0.0217) and significantly larger CSA at all three levels compared with the control participants (p < 0.0001). Comparisons among levels showed significantly higher FF for levels 2 and 3 than for level 1 and significantly larger CSA for level 2 compared with level 1 in CMT1A patients. CSA at level 3 correlated positively with the CMT neuropathy score version 2 (CMTNSv2).

Conclusions: In conclusion, the sciatic nerve FF of CMT1A patients was significantly higher at level 3 compared with both the controls and the measurements taken on more proximal levels. Our result showing a distal tendency for increased FF in CMT1A patients could suggest a distal predominance of increased interfascicular fat. Sciatic nerve CSA at level 3 correlated significantly and positively with CMTNSv2, suggesting its potential value as an imaging marker for clinical severity.

References: No
References 1:
References 2:
References 3:
References 4:

Grant Support:

Keywords: Charcot-Marie-Tooth disease, cross sectional area, Intraepineurial fat quantification, CMT1A, sciatic nerve

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