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Hirayama jc

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journal club on hirayama disease

journal club on hirayama disease

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    Hirayama jc Hirayama jc Presentation Transcript

    • Bilaterally symmetric form of hirayama disese
      • Sunil Pradhan et al
      • Institute of human behavior and allied sciences, New delhi.
      • Neurology,vol.72,number 24,june16,2009.
    • background
      • Hirayama disease (benign juvenile brachial spinal muscular atrophy, benign juvenile muscular atrophy of distal upper extremity, monomelic amyotrophy).
      • Pure motor focal amyotrophy in distribution of c7,8,T1 spinal segments
      • Sporadic, Men, second and early third decade.
      • Muscular weakness and wasting of hand and forearm
      • Oblique amyotrophy.
      • Insidious onset, steadily progressive for 1-3 yrs..stable stage.
      • Dynamic cord compression during neck flexion.
      • Juvenile asymmetric segmental spinal muscular atrophy(JASSMA)
      • Describe bilaterally symmetric form of hirayama disease.
    • Materials and methods
      • 106 (m-103,f-3)
      • 14-32 yrs
      • Two academic centers
      • 1992-2008
      • Patients registry
      • 11 patients with symmetic form
      • Clinical, edx, mri evaluation normal and neck flexion.
    • criteria
      • Onset in teen and early 20s.
      • Symmetric /asymmetric muscle atrophy in c7,c8,t1 myotome.
      • No sensory symptoms/signs
      • Relative sparing of brachioradialis
      • Tremulousness of fingers in outstretched hands
      • Cold paresis
      • Unilaterality ….replaced by MRI findigs
    • MRI criteria
      • Flattening of spinal cord against c5-6 vertebral bodies
      • Forward movement of posterior cervical duramater,
      • reduction in size of posterior cervical subarachnoid space.
      • Contrast enhancing crescent shaped posterior cervical epidural pace.
    • EMG
      • 1 st dorsalinterossei,APB,EDC,brachioradialis,biceps,vastus lateralis,tibialis anterior .
      • Denervation potentials( positive sharp waves and otentials)during rest state were compared in same muscle in two sides.
      • Reinervation potentials (large,wide polyphasic potentials)studied in mild contraction in each muscle(3 x3)
      • 10 sweeps of 100 msec duration-frozen –percent of polyphasic potentials to number of MUP and compared.
    • NCS
      • CMAP of median,ulnar,radial nerve were used to document symmetry of disease .
      • < 20% difference in CMAP of APB,ADM,EDC taken as symmetrical.
      • SNC to rule out axonal forms of polyneuropathy
      • All findings were compared with existing knowledge of the disease
    • results
      • 11/106 symmetrical
      • All male
      • Age 18-24(20.27)
      • Started at mean 17.8 yrs progresed for 1-3 yrs (9),3-4 yrs (2)
      • 9 immunised for polio, none had h/o polio.
      • 6 had onset in winter months
      • None had preceding febrile illness
      • All had weakness and wasting in c5,6 t1 myotomes
      • 6 had partial brachioradialis wasting s/o c6 myotome
      • Unilateral onset in 9 patients (R 6, L 3),bilateral in two.
      • Autonomic dysfunction in all, (excessive sweating in 7,cold hands in 8,hair loss in 5)
      • All had fasciculation at rest ,mini polymyoclonus in outstretched hands.
      • 7 had brisk DTR in lower limbs
    • mri
      • Neutral position-Symmetric cord atrophy in 9, T2 hyper intensity in anterior lateral aspect of lower cervical spinal cord in 7.
      • Neck flexion-band like cord flattening in all (symmetric in 7,asymmetric in 4)crescent shaped enhancing epidural in all.
    • EDX
      • All had N SNAPs
      • All had n emg in lower limbs
      • All clinicallly symmetric form had < 20% difference in CMAPs.
      • Quantitative asessment of percentage of acute denervation and chronic renervation potentials showed nearly symmetric involvement between right and left
    • discussion
      • In this series of 106 patients nearly 10% showed symmetric involvement of both upperlimbs.
      • VS a form of ALS ‘brachial amyotrophic diplegia’ ‘flail arm syndrome’ ‘man in barrel syndrome’-older age, predominant c5-6 involvement, overt fasciculations, gradual appearance of UMN or bulbar signs,avg survival of 5 yrs,no dynamic MRI changes with neck flexion.
      • Vs postpolio atrophy-no h/o polio,symmetrical and MRI.
      • Short length of cervical dural canal that cannot compensate for flexion related increased length of vertebral canal. dural canal becomes tight during neck flexion-anterior displacement of posterior dural wall and spinal cord, spinal cord gets flattened against c5-6 vertebral body,cresent shaped posterior epidural space with prominent venous plexus.
      • Neck flexion related anatomic changes cause mechanical and hemodynamic stress on anterior horn cells in c7,8,T1
      • Severe form of hirayama disease
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      • Thank you
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