The Importance of Flexion MRI in Hirayama Disease with Special Reference to Laminodural Space Measurements

D.K. Bourah et. al., AJNR May 2018

Background

  • First described 1959
  • “juvenile muscular atrophy” of distal upper limb extremity
    or “monomelic amyotrophy.”

Background

  • Insidious onset asymmetric wasting distal upper ext
  • sparing brachioradialis
  • C8-T1
  • 2nd-3rd decades (15-25 years old)
  • M > F
  • RUE > LUE

Materials and Methods

  • January 2014 to July 2017, Northeast India
  • Young adults weakness/wasting hand/forearm
  • motor axonopathy on nerve-conduction studies

Included subjects

  • Other etiologies for weakness
  • AS of C-spine
  • Prior trauma or fixation
  • Inability to adequately flex neck

Excluded subjects

45 subjects

Materials and Methods

  • 1.5T Magnetom Avanto (Siemens)
  • Imaged c-spine in neutral and flexed position
    • Flexion imaging with body coil
    • 30-40°
    • Post-gad sag and axial in flexed position

MR protocol

Materials and Methods

  • Cord flattening, T2 signal, atrophy
  • Max forward shift of posterior dura (laminodural space)
  • AP + TV diameter of cord in neutral and flexed
  • AP diameter of canal in neutral and flexed

Image analysis

Results

  • 44 male, 1 female
  • 14 - 42 years at presentation, mean 23 years
  • 89% unilateral, 11% bilateral
  • 100% hand, 60% forearm, 4% arm muscles

Subjects

Results

  • localized lower cervical cord atrophy: 60%
    • < 2 vertebral heights in 42%, 2-3 in 11%, > 3 in 7%
    • Max mean atrophy at C6-7
    • asymmetric flattening in 69%

Imaging findings

Results

  • T2 cord signal abnormality: 36%
    • Anterior horns: 33%
      • Unilateral: 24%
      • Bilateral: 9%

Imaging findings

Results

Imaging findings

fig. 3

  • Loss of dural attachment, forward shifting of posterior dura, enhancing prominent posterior epidural space: 100%

Results

Imaging findings

fig 1

Results

Imaging findings

fig 2

Results

  • Max LDS: 3 - 10mm, mean 6mm
  • Posterior epidural flow voids: 47%
  • Most had increased LDS at both C & T spine
  • Diameter of bony canal not sig changed
  • At max compression site, cord flattened in AP dimension by avg of 0.7mm
  • Mean decrease in AP/TR ratio of 0.12

Imaging findings

Results

Imaging findings

Results

figs. 4, 5, 6

Discussion

Etiology...

  • Repeated/sustained flexion leads to necrosis of anterior horn cells 2/2 chronic changes in ASA territory microvasculature
  • Differential growth of vert column and cord -> abutment of cord -> ASA ischemia
  • ? atopy/immunologic contribution
  • Posterior epidural venous engorgement 2/2 impaired venous return from neg pressure in LDS

Discussion

Imaging important in diagnosis

  • Neutral MRI may show atrophy, T2 signal, asymmetric flattening
  • Flexion MRI shows classic findings
    • Posterior dural detachment -> widened LDS
    • Enh of engorged posterior epidural venous plexus
    • Compression of cord

Discussion

What criteria to use?

  • Lehman et al 2013: Sens similar for neutral and flexion (70% vs 71%), but neutral findings subtle

Discussion

What criteria to use

Lehman et al

Discussion

What criteria to use?

  • Chen et al 2004: Loss of attachment 93% sensitive
  • Lai et al 2011: LDS increase in 46% healthy subjects
    • Proposed BOTH:
      • increased LDS/canal diameter ratio
      • decreased AP/TR cord diameter ratio
    • But only 3 patients 

Discussion

What criteria to use?

  • Current study applied these proposed criteria to 45 patients
    • Mean LDS/canal ratio increase: 0.46
    • Mean AP/TR cord diameter decrease: 0.12

Limitations

  • No control group to determine cutoff for diagnosing HD
  • Uniform neck flexion angle not achieved in all patients

Thoughts

  • Ratios of LDS/canal & AP/TV seems like overkill
    • Why not just loss of attachment and decreased AP cord diameter in flexion?
  • Flexion images clearly helpful
  • Comparison with asymptomatic controls would be helpful

Conclusions

  • Important to recognize
    • Self-limiting disease if early intervention
      • (conservative or surgical)
    • High index of suspicion in young person with insidious onset hand/forearm weakness
    • Flexion imaging useful for diagnosis as findings can be missed on neutral images

Hirayama disease

By Jason Hostetter

Hirayama disease

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