Case No 13: G. B. Syndrome

NAME : - DIPTI
12 YEARS OLD FEMALE
DIAGNOSIS : G. B. SYNDROME

Dipti reported here a chief complaints of inability to stand and walk independently. According to the neurophysician, she was apparently asymptomatic till six years of age when she presented with signs of paresthesia and numbness in all 4 limbs with rapid onset of difficulty in walking and difficulty in getting up from squatting position. This went on progressing initially and she became almost totally paraplegic with retention of urine too. She also had severe weakness in the hands. There was no dysphagia or dysarthria. No cranial nerve deficit or facial asymmetry or bulbar weakness was found. There was grade 3 to 4 – power in the upper limbs and grade 1 to 2–power at the hips and distally at the toes. There was mild reduced sensation for pinprick distally, but vibration and position sense was preserved. The deep reflexes were brisk except for absent ankle jerks and plantars were down going.

Her CSF and CT spine was normal. The EMG/NCV study done on 12.1.1995 was suggestive of pure motor axonal lesion. She was diagnosed as axonal variant of G. B. Syndrome. Subsequently she was started on vitamins and supportive therapy.

Her NCV study done on 2nd July 1999 was suggestive of motor neuropathy in right common peroneals. Thus her NCV studies were not showing any improvements in this period of 4 ½ years and clinically also she did not show any improvements.

On Examination, mild to moderate hypotonicity was found in the lower limbs. Sitting from supine position was possible only form prone line and side line. Her mode of movement was crawling ( with dragging of lower limbs) and supported walking with walkers. Grip strength was also reduced. She had episodes of breathlessness.

Improvements noted with 4 months of Neuro G –Therapy :

• Started coming to sitting position from supine line too.
• While walking with walkers, started lifting her limbs forward (no dragging of limbs).
• Started maintaining bridging position.
• Marginal improvement in the grip strength is also noted.
• Increase in the speed of supported walking is also found.