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Case
No 6: Motor Neuropathy with Seizure Disorder with scholastic backwardness.
Jayendra reported here with chief complaints of inability
to walk effectively and unable to jump and manage stairs independently
with generalized muscle wasting with stammering.
His birth history was suggestive of full term normal
delivery. There was history of neonatal septicaemia in the second
week of birth. All the early developmental milestones were achieved
late (walking - 2½ years ; speech – 3 years). At 3 ½ years he had
his first seizure. On investigation his EEG was found to be abnormal
and he was started on carbamezapine. Repeat EEGs at 2-3 years intervals
were always abnormal. He stopped attending school in between and
his behavioural pattern was changed – became recluse and stopped
communicating with others. At 14 years of age he had a sudden onset
of inability to walk while travelling in a train. He recovered gradually
from that episode but has developed gait abnormality (high steppage
gait with mild pelvic tilt) since then. On investigation his EMG/NCV
study was suggestive of severe sensory motor neuropathy in lower
limbs of a mixed demyelinating and axonal degeneration type. Normal
CT Scan was found.
On examination, mild intrinsic stiffness was found
in the lower limbs with bilateral foot drop deformity with pes-cavus.
He was walking with a broad based high steppage gait with dragging
tendency of lower limbs. Jumping and running was not possible. Walking
with sleepers was difficult. He was independent in his activities
of daily living. Wasting of both extremities (distal more than proximal
) was also found. He was having limited social interaction with
disturbed sleep.
Improvements noted with 1 years, 5
months of G – Therapy :
- Can walk with sleepers now which was difficult before.
- Clawing of toes has reduced.
- Better active dorsi – flexion of both ankles now.
- Better balance in single limb standing position now.
- His recent EMG/NCV study is suggestive of increase in the amplitudes
of both sural nerves.
- Better sleep now.
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