Diagnostic Dilemma

Guillain Barre Syndrome

A 4½ years old boy presented with sudden weakness of both lower limbs for 7 days and breathing difficulty for 3 days. Weakness in lower limbs was ascending in nature and now the child was unable to stand and sit. On examination, he had hypotonia, absent deep tendon reflexes, and power of 2/5 in lower limbs and 3/5 in upper limbs. He had a diaphragmatic weakness. Superficial reflexes were also absent. He was suspected to be a case of Guillain Barre Syndrome and given Intravenous Immunoglobulin (IVIg) 2 gm/kg following which his power improved. He did not require any ventilatory support. Over a period of 7 days, his power gradually improved, there was no respiratory problem and the child was able to sit and walk with support. However, his reflexes had still not appeared. His nerve conduction study done was normal.

Why are reflexes absent even though power is better?
Expert Opinion :
Usually, Guillain Barre Syndrome (GBS) is characterized by areflexia, ascending paralysis, and demyelination of the peripheral nerve. However, variants of GBS may present with only areflexia or areflexia and ataxia and even ophthalmoplegia (Miller Fisher Variant) without limb weakness. In a child with GBS, it would be expected that with the recovery of disease and return of power, the reflexes should also improve. However, if there is some amount of sensory deficit (which is not uncommon in GBS), though the power may improve, reflexes may still not come back. These sensory deficits may be subtle and not picked up on nerve conduction studies. A nerve biopsy may show demyelination in such a case. However, recovery usually occurs over a period of time.
Answer Discussion :
Dr akash Sarkate Patil
for DTR affrent fast conducting fibers are heavily myelinated compared to effrent fibers hence even if ncv and power normal , DTR absent
7 months ago
Abhishek Madhura
7 months ago

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