Key result
A selective pattern of fatty infiltration on lower limb muscle MRI identified LGMD2A with a sensitivity of 76.00% and a specificity of 90.48%.
Why the study?
Does muscle MRI of the lower limbs accurately evaluate fatty infiltration and correlate with disease progression in patients with LGMD2A?
Case-Control (n=53)
Does muscle MRI of the lower limbs accurately evaluate fatty infiltration and correlate with disease progression in patients with LGMD2A?
Upper leg muscle MRI identifies a selective pattern of fatty infiltration that is highly specific for diagnosing LGMD2A and correlates well with disease progression.
May aid LGMD2A diagnosis via selective MRI pattern; leaves open prospective validation before clinical adoption.
INTRODUCTION: Limb-girdle muscular dystrophy type 2A (LGMD2A) is characterized by progressive wasting of muscles for which the disease-monitoring tools are still deficient. METHODS: We performed muscle MRI of the lower limbs in 32 LGMD2A patients and 21 controls. The modified Mercuri scale was employed to evaluate the degree of fatty infiltration. RESULTS: Severe fatty infiltration in the long head of biceps femoris (modified Mercuri scale 3.99) and sparing of extensor digitorum longus (modified Mercuri scale 0.17) were observed. The sensitivity and specificity of this pattern in diagnostic testing was 76.00% and 90.48%, respectively. A comprehensive clinical and MRI evaluation revealed that progressive fatty infiltration in the upper leg correlated well with disease progression, but neither calf involvement nor muscle strength deterioration showed a good correlation. DISCUSSION: The selective involvement pattern is potentially useful for LGMD2A diagnosis. Upper leg muscle MRI is a sensitive evaluation method for monitoring disease progression. Muscle Nerve 58: 536-541, 2018.
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Feng et al. (2018) conducted a case-control in Limb-girdle muscular dystrophy type 2A (LGMD2A) (n=53). Muscle MRI of the lower limbs vs. Controls was evaluated on Diagnostic sensitivity and specificity of selective fatty infiltration pattern. A selective pattern of fatty infiltration on lower limb muscle MRI identified LGMD2A with a sensitivity of 76.00% and a specificity of 90.48%.
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