Chronic inflammatory demyelinating polyneuropathy associated with contactin-1 antibodies in a child

A previously healthy 2-year and 9-month old boy was brought to the emergency department for a 6-day history of weakness in the legs and frequent falls, rendering him unable to walk 1 day before admission. He did not have pain, dysphagia, bladder dysfunction, or sensory symptoms. There was no history of trauma, but he developed diarrhea 3 days before symptom onset. Family history was negative for consanguinity or neurologic diseases. At examination, he had bilateral leg weakness requiring substantial aid to walk a few steps and was unable to stand up from the floor. He had absent tendon reflexes in the lower extremities and flexor plantar responses. Strength and reflexes in upper extremities and the rest of the examination were normal. CSF showed a protein concentration of 125 mg/dL (NR: 15–45), with normal white blood cell count and glucose concentration. Blood cell count and chemistry were normal, and stool culture was negative. Nerve conduction studies (NCSs) and EMG showed decreased amplitudes in both peroneal nerves (table e-1, links.lww.com/NXI/A131). The patient was treated with IV immunoglobulins (IVIg) 2 g/kg administered in 3 days. During the next 2 weeks, there was mild improvement in motor strength as he was able to walk and stand up with support (the Guillain-Barré syndrome disability scale [GBSds]1 score remained 3), and he was discharged home. Two weeks later (4 weeks after symptom onset), he was brought back for worsening weakness in the legs and n...
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: Guillain-Barre syndrome, Chronic inflammatory demyelinating polyneuropathy, All Pediatric, EMG Clinical/Scientific Notes Source Type: research