12 year old female girl 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.
