A clinical and electrodiagnostic, retrospective study of 43 children with acute inflammatory demyelinating polyradiculopathy (AIDP) is reported from the Service de Neuropediatrie, Hopital de Bicetre, France. Age of onset ranged from one to 18 years, less than 3 years in 35%. An antecedent infection occurred within 2 months of onset in 74%. Presenting symptoms included pain (47%), weakness (23%), ataxia (15%), and sensory signs (5%). When the neurological impairment had ascended and reached a plateau, weakness was noted in 100%, pain in 63%, ataxia (23%), and sensory signs (23%). Cranial nerve paralyses involved the VIIth in 35%, and extraocular muscles in 16%. Autonomic dysfunction was present in 26%, and 28% required assisted ventilation. All except 2 recovered completely. Motor and sensory nerve conduction studies showed signs of demyelination during the first week in 90%, and the diagnosis of AIDP was confirmed in the 2nd week in all patients. A reduced compound muscle action potential was the earliest abnormality (83%), and motor conduction was abnormal in only 50%, during week 1. The outcome was better in children than in adults. [1]
COMMENT. Based on electrophysiologic criteria, the diagnosis of AIDP may be suspected in the first week and confirmed in the second week of the illness. The clinical manifestations in the acute phase are similar in children and adults, but the outcome of AIDP in children is better. More than one-third of children affected are under 3 years of age. Severe limb or back pain, weakness, and ataxia are the most prominent initial symptoms.
In contrast to the acute form of inflammatory demyelinating polyneuropathy (IDP), the chronic form presents with lower extremity weakness and difficulty in walking in 85%. The majority have a poor long-term prognosis, with frequent relapses and residual weakness (Progress in Pediatric Neurology III, PNB Publ, 1997;pp360-362).
Correlation between cytomegalovirus infection and IgM anti-MAG/SGPG antibody-associated neuropathy [2] is reported from Dokkyo University, Tochigi, Japan. CMV infection may induce the IgM anti-myelin-associated glycoprotein antibody found in some patients with chronic polyneuropathy.