I saw this 24 yrs old young man about three months ago.  He was referred to me for ‘tremulousness’ of both hands below the wrist, progressive since two years.  Progressively unable to write, hold small objects.  He also noticed that the tremors became more when he bends his neck to do fine jobs, say threading a needle.  He describes this tremors as ‘twitching’ of the digits and some parts of the palm.  There has been no h/o neck trauma, neck pain, radicular pain, incontinence, double vision, impaired hearing, exposure to heavy metals, lead and pesticides.  No abnormal or diminished sensation in any part.  No giddiness, LOC, convulsions or abdominal pain.

o/e

Wasting and fasciculations of small muscles of both hands.  Polyminimyoclonus of the digits.  The patient demonstrated the increase of the latter by flexing the neck to the maximum.  DTRs of the lower limbs were brisk.   Plantars were flexor.  Sensory exam was normal.   HEENT and Cranial nerves were normal.  No neurocutaneous markers were seen.

These are the MRI pictures both during normal and flexion of the neck ( my neuroradiology colleague Dr K S Murugan should be complimented). It shows a dynamic compression of the cervical spinal cord during flexion of the neck.   CVJ is normal.  ENMG of the lumbricals showed denervation & fibrillation potentials.  Nerve conduction study of ULs normal.

Advised decompressive surgery (references available since this is the first of such disease I am seeing). Patient not to be seen after that !!

This is Hirayama’s disease (Juvenile Muscular Atrophy with Dynamic Cervical cord compression)  Probably produces anterior horn cell dysfunction and damage on the long run of the cervical cord due to the dynamic compression.  Several surgical procedures including anterior cervical decompression available (anecdotal)

Comments from the members are invited..

G N Lakshminarayan (neurologist), K S Mururan (neuroradiologist)