Case No 8: Severe Axonal Sensory - Motor Neuropathy.

History and condition before starting G – Therapy :

According to his neurophysician Swapnil was apparently normal till 5 ½ years of age when he had a febrile illness for 4 - 5 days and subsequently weakness of all 4 limbs were noticed thereafter. Initially he recovered to some extent. But after 3-4 months, progressive weakness and deterioration in functional activities was reported by their parents. He was diagnosed as Polyneuropathy and subsequently he received a course of steroids without any further improvements.(In fact deteriorated neurologically) There was a history of paresthesia in lower limbs with bilateral facial weakness with neck muscles and truncal weakness. Superficial reflexes were absent with down going plantars. There was history of consanguineous parents and a sibling was also affected with the similar illness. His neurophysician was suspecting some metabolic defect which he found difficult to treat.

On investigation, his Nerve Conduction Studies done on 4th June 1999 & 13th Oct. 1999 revealed evidence of a severe Generalized Sensori – Motor neuropathy. His ESR was also raised, normal CSF study.Unremarkable normal biopsy.

He reported here on 3/8/99 with chief complaint of inability to walk and stand independently and generalized weakness including wasting of whole body.

On examination mild hypotonia was found in his lower limbs with bilateral pes - cavovarus deformity. Functionally, he was independently coming to the sitting position from lying position. His independent mode of ambulation was crawling only. Grip strength was fair on both sides with wasting of both thenar and hypothenar eminence, Bilateral facial weakness was also observed.

Improvements noted after taking G – Therapy for 8 months :

1. He is now independently coming to standing position with a wide based stance and can walk independently also with a clumsy wide based gait.
2. Independent kneel standing and half kneeling is also possible now.
3. Grip strength is also better now.
4. There is also increase in the calf - girth.
5. He can independently manage his few activities of daily living tasks like feeding. dressing and undressing etc.
6. His NCV studies done on(16/6/2000) is suggestive of presence of right median Nerve Motor response though delayed in velocity. (Previous to this no response was observed).
7. According to his neurophysician who had seen him after a gap of 1 year found that his power in upper limbs is slightly better and he can now walk without support which was not possible earlier.