Thirteen children with chronic inflammatory demyelinating polyneuropathy monitored between 1975 and 2005 are reported from Centre hospitalier universitaire Sainte-Justine, Montreal, Canada. Age at diagnosis varied between 3 and 14 years; 4 were female and 9 male. All presented with symmetrical muscle weakness, mainly in the lower limbs. One had asymmetrical upper limb weakness. Symptoms evolved over 1 week to 1 year, and diagnosis was confirmed after 4 weeks of progression or relapsing course. Sensory symptoms, hypoesthesia and painful paresthesias, occurred in 54% (7/13) of patients at diagnosis. Two patients had diplopia. A respiratory tract viral infection preceded onset in 23% (3/13). Disability varied between minor and severe, 2 to 5 on the Rankin score. Diagnosis was confirmed by delayed nerve conduction, elevated protein >35 mg/dL and leukocytes <10/mm3 in the CSF, and nerve biopsy in 5 patients (38%), showing inflammation and segmental demyelination. Evolution was polyphasic in 10 (77%) patients, with 1 to 10 relapses over a follow-up of 6 months to 5 years. Patients treated before 2000 (7/13) received prednisone with good response. Some required repeat courses at relapse and some received azathioprine or cyclophosphamide in addition. After 2000, 3 of 4 patients received intravenous immunoglobulin with an excellent initial response. All required repeat treatments for relapses, including add-on therapies in 2. Two recent patients are refractory, but their disabilities are only minimal to minor. [1]

COMMENT. Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired progressive symmetrical proximal weakness with areflexia, associated with paresthesias or ataxia. Presentation in children is more subacute than chronic, with numerous relapses. Prognosis is usually favorable with complete functional recovery. In the above study, most had relapses requiring repeat courses of prednisone and add-on therapies, and some cases were refractory to treatment.

In a report of 39 patients with CIDP treated with either high-dose, intermittent IV methylprednisolone (IV MP), IV immunoglobulin (IVIg) or oral prednisone or cyclosporine, 81 to 88% showed improvement in muscle strength, and these treatments were equally effective. Weight gain and cushingoid features were less frequent in patients treated with IVMP (19% affected) than in those receiving oral prednisone (58%). IVMP as initial and maintenance therapy is recommended in CIDP patients with weakness or disability [2]; Ped Neur Briefs March 2005; 19:18).

Mitochondrial neurogastrointestinal encephalomyopathy in an 18-year old female presented with symptoms suggestive of relapsing CIDP, but the gastrointestinal symptoms and lactic acidosis were not compatible. [3]

Noninvasive ventilation used in treatment of respiratory failure in children with neuromuscular disease results in a reduction of symptoms, hospitalizations, and health care costs, without adverse effects on quality of life. Fourteen patients were followed for 6 to 84 months (median 30 mo). Daytime sleepiness and headache, and sleep quality improved, despite disease progression. [4]