Muscle magnetic resonance imaging in spinal muscular atrophy type 3: Selective and progressive involvement

Hacer Durmus1, Ravza Yilmaz2, Yesim Gulsen-Parman1

  • 1Department of Neurology, Istanbul Faculty of Medicine, Istanbul University, Millet Caddesi, Capa, 34390, Istanbul, Turkey.

Muscle & Nerve
|August 21, 2016
PubMed
Abstract

Insights

Magnetic resonance imaging (MRI) in spinal muscular atrophy type 3b (SMA3b) reveals distinct muscle involvement patterns. MRI can track disease progression, showing more severe changes in certain muscles over time.

Area of Science:

  • Neurology
  • Medical Imaging
  • Genetics

Background:

  • Spinal muscular atrophy type 3b (SMA3b) is a neuromuscular disorder.
  • Understanding disease progression and muscle involvement is crucial for patient management.

Purpose of the Study:

  • To identify magnetic resonance imaging (MRI) signs of selective muscle involvement in SMA3b.
  • To assess MRI-based disease progression in SMA3b patients.

Main Methods:

  • Twenty-five genetically confirmed SMA3b patients underwent 1.5-Tesla MRI.
  • Muscle involvement patterns and disease progression were analyzed.

Main Results:

  • MRI revealed more severe involvement in iliopsoas and triceps brachii muscles compared to gluteus maximus and biceps brachii, respectively.
  • Quadriceps femoris muscles were severely affected; deltoid, adductor longus, hamstrings, gracilis, sartorius, and rectus abdominis were preserved.
  • A positive correlation was found between MRI changes and disease duration for gluteus maximus and triceps brachii. Follow-up MRIs indicated disease progression.

Conclusions:

  • This study confirms and refines the pattern of selective muscle involvement in SMA3b using MRI.
  • MRI is a valuable tool for monitoring progressive muscle changes in SMA3b.