"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
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Bent Spine syndrome MRI: T2 images
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Wasting & Fibrosis (T-spine): Symmetric, Severe (Arrows)
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Wasting & Fibrosis (L-spine): Asymmetric (Arrow)
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