CHRONIC INFLAMMATORY DEMYELINATING POLYRADICULONEUROPATHY IN CHILDREN

Objectives: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired neuropathy. It is important to consider this diagnosis in children. The authors reported two patients with age of onset in the five year-old and the second in 15 year-old. Both have a slowly progressive course and were first diagnosed as Charcot Marie Tooth disease. As there was no family history, nor siblings or cousins affected, the diagnosis of CIDP was considered since it is potentially treatable.

Methods: Evidence of demyelination was confirmed by electrophysiological study in the two patients. In the younger patient, it was a predominantly sensory neuropathy and in the elder it was a mixed sensorimotor neuropathy. The CSF protein is elevated in one patient and acellular in both. Both patients received intraveinous immunoglobulin (IVIg), 0.4g/kg body weight daily for five days without side effects. They received after pulsed high-dose of methylprednisolone followed by prednisolone 1mg/kg/day.

Results: A favourable response was seen within four weeks with a good long-term out-come two years later with no relapses and the neurological examination was completely normal for the elder and minimal disturbance of big toe position sense in the younger one.

Conclusion: In Tunisia, Charcot Marie Tooth disease is more frequent than CIDP and neurologists are more prone to consider a diagnosis of hereditary neuropathy than acquired neuropathy. However, when there was no familiar history and the clinical course was relapsing or chronic progressive or suacute monophasic, a CIDP must be considered and the treatment by IVIg and steroids given with a good long-term outcome.