Neuromuscular

Home, Search, Index, Links, Pathology, Molecules, Syndromes,
Muscle, NMJ, Nerve, Spinal, Ataxia, Antibody & Biopsy, Patient Info

Paraspinous Muscle Weakness 4

Vesalius
      Vesalius




MRI: Paraspinous Muscle Atrophy

"Bent Spine" Syndrome 2

  • Luke 13:11
      And behold, there was a woman who had a spirit of infirmity eighteen years,
        and was bent over and could in no way raise herself up
  • Etiology: Family history positive in up to 50%
  • Epidemiology: Females > Males
  • Clinical
    • Onset age: 4th to 7th decade; Idiopathic disorders usually > 50 years
    • Paraspinous muscles
    • Limb muscles
      • Normal strength
      • Fatigue with repetitive movements
    • Course: Slowly progressive
    • Spinal: Upper thoracic kyphosis
  • Laboratory
    • Muscle CT & MRI
      • Paraspinous muscles: Hypodensity; Fat infiltration
    • EMG
      • Paraspinous: Myopathic 75%
      • Limb: Myopathic 50%
    • Serum CK
      • Normal (75%)
      • Mildly high in minority: Up to 5x elevated
    • Muscle biopsy
      • Paraspinous muscles
        • Variable muscle fiber size
        • Endomysial fibrosis
        • Little necrosis or regeneration
        • Internal architecture: Disorganized
      • Limb muscles: Minor or Non-diagnostic changes
  • Differential diagnosis: Other causes of paraspinous muscle weakness
Bent Spine syndrome
MRI: T2 images

Wasting & Fibrosis (T-spine): Symmetric, Severe (Arrows)

Wasting & Fibrosis (L-spine): Asymmetric (Arrow)

Scoliosis: Adolescent Idiopathic


Scoliosis & Axial Myopathy 3

  Lady bird late, drosophila, homolog of, 1 (LBX1) ; Chromosome 10q24.31; Dominant
Return to Neuromuscular home page

References
1. J Neurol Sci 2000;176:70-74
2. Muscle Nerve 2002;25:189-193, J Neurol 2002;249:730-734, J Neurol Neurosurg Psychiatry 2002;73:1-7
3. Am J Med Genet A 2014;164A:2074-2078
4. Muscle Nerve 2015 Apr 21, Brain 2015 Dec 14, Neurol Clin Pract 2018;8:240-248
5. Am J Human Genet 2015; Online July
6. Rev Neurol (Paris) 2022;178:398-399

6/24/2022