Intraepineurial fat quantification and cross-sectional area analysis of the sciatic nerve using MRI in Charcot-Marie-Tooth disease type 1A patients

Kim, Hyun Su; Lee, Ji Hyun; Yoon, Young Cheol; Jae, Min; Nam, Soo Hyun; Kwon, Hye Mi; Kim, Seonwoo; Won, Hojeong; Choi, Byung‐Ok

doi:10.1038/s41598-021-00819-0

Cited by 5 publications

(1 citation statement)

References 36 publications

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“…Прогресування хвороби Шарко -Марі -Тута безпосередньо пов'язане з атрофією м'язів та накопиченням жиру. Такі відхилення можна оцінити за допомогою магнітно-резонансної томографії для вимірювання на-копичення жиру в м'язах нижніх кінцівок [10,11] або в ендоневральному просторі сідничного нерва [12]. Нещодавно було запропоновано зв'язати щільність інтраепідермальних нервових волокон із тяжкістю захворювання, оскільки біопсія шкіри пацієнтів із ІA підтипом показала зменшення кількості та високий рівень денервованих клітин Меркеля [13].…”

Section: практикуючому неврологуunclassified

Neural amyotrophy of Charcot-Marie-Tooth (clinical case)

Trishchynska,

Dubynetska

2023

INJ

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The article describes the prevalence of inherited neuromuscular disease, specifically neural amyotrophy, or Charcot-Marie-Tooth disease, its key clinical and neurological features, promising blood biomarkers for future diagnosis and therapeutic strategy assessment, along with current treatment methods under investigation, taking into account gene therapy. A clinical case is presented describing Charcot-Marie-Tooth disease type IA in a young man, which was confirmed by molecular genetic testing. The study of diagnostic criteria for nosologies of this spectrum is relevant, taking into account the rarity of the disease and certain difficulties in making a diagnosis.

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Section: практикуючому неврологуunclassified

Neural amyotrophy of Charcot-Marie-Tooth (clinical case)

Trishchynska,

Dubynetska

2023

INJ

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A neuromuscular clinician's guide to magnetic resonance neurography

Pitman,

Fayad,

Ahlawat

2024

Muscle and Nerve

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Magnetic resonance neurography (MRN) is increasingly used in clinical practice for the evaluation of patients with a wide spectrum of peripheral nerve disorders. This review article discusses the technical aspects of MRN highlighting the core sequences performed for clinical care. A robust, high‐resolution, heavily T2‐weighted fluid‐sensitive sequence performed on a 3.0 Tesla magnet system remains the main workhorse MRN sequence. In specific clinical scenarios, adjunct techniques such as diffusion‐weighted imaging can be added to a protocol for disease characterization. In addition, gadolinium‐based contrast material can also be administered for the purposes of image optimization (suppress adjacent vascular signal) and disease characterization. Technical modifications to field of view and planes of imaging can be made based on the clinical question and discussion with the radiologist(s). On fluid‐sensitive MRN sequences, a normal peripheral nerve exhibits iso‐ to minimally hyperintense signal relative to skeletal muscle with a predictable trajectory, preserved “fascicular” architecture, and tapered caliber from proximal to distal. Peripheral nerve abnormalities on MRN include alterations in signal, caliber, architecture, diffusion characteristics as well as enhancement and provide information regarding the underlying etiology. Although some MRN findings including nerve hyperintensity and long‐segmental enlargement are nonspecific, there are certain diagnoses that can be made with high certainty based on imaging including benign peripheral nerve tumors, high‐grade peripheral nerve injury, and intraneural ganglia. The purpose of this article is to familiarize a neuromuscular clinician with fundamentals of MRN acquisition and interpretation to facilitate communication with the neuromuscular radiologist and optimize patient care.

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Magnetic resonance imaging-based lower limb muscle evaluation in Charcot-Marie-Tooth disease type 1A patients and its correlation with clinical data

Kim

Lee

et al. 2022

Sci Rep

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We aimed to derive comprehensive MRI parameters that reflect intramuscular fat infiltration severity for designated lower extremity levels, based on semiquantitative analyses in Charcot-Marie-Tooth disease type 1A (CMT1A) patients. We reviewed lower extremity MRIs of 116 CMT1A patients. Intramuscular fat infiltration grading using the Mercuri scale was performed for the non-dominant lower extremity at three levels (proximal, mid, and distal) for the thigh and at two levels (proximal and distal) for the lower leg. Based on MRI results, the following parameters were calculated for each level and for entire muscles: fat infiltration proportion (FIP), significant fat infiltration proportion (SigFIP), and severe fat infiltration proportion (SevFIP). The relationships between the MRI parameters and clinical data were evaluated using Spearman’s correlation analysis. FIP, SigFIP, and SevFIP measured for entire muscles significantly correlated with Charcot-Marie-Tooth Neuropathy Score (p < 0.001), functional disability scale (p < 0.001), 10-m walk test time (p = 0.0003, 0.0010, and 0.0011), and disease duration (p < 0.001). Similar correlations were demonstrated for FIP, SigFIP, and SevFIP acquired from the lower leg. Our MRI parameters obtained through semiquantitative analyses of muscles significantly correlated with clinical parameters in CMT1A patients, suggesting their potential applicability as imaging markers for clinical severity.

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Intraepineurial fat quantification and cross-sectional area analysis of the sciatic nerve using MRI in Charcot-Marie-Tooth disease type 1A patients

Cited by 5 publications

References 36 publications

Neural amyotrophy of Charcot-Marie-Tooth (clinical case)

Neural amyotrophy of Charcot-Marie-Tooth (clinical case)

A neuromuscular clinician's guide to magnetic resonance neurography

Magnetic resonance imaging-based lower limb muscle evaluation in Charcot-Marie-Tooth disease type 1A patients and its correlation with clinical data

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