Objective:

Background:

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) presents with similarly in adult and pediatric populations. A rare phenotype that consists of distal predominant is distal acquired demyelinating symmetric neuropathy (DADS). In adult patients there is association with anti-MAG antibodies that has not been established in the pediatric population. The first line treatment for DADS in adults is commonly prednisone followed by IVIG or other immunomodulatory therapies. In the pediatric population, the benefits chronic corticosteroids use must be balanced with the adverse effects on bone development and stage of pubertal development.

Design/Methods:

NA

Results:

Described are two cases of DADS who presented typically. Demyelinating polyradiculoneuropathy was confirmed by EMG/NCS. Case 1 is a 15-year-old male who presented with 2 months of symptoms and had inadequate response to IVIG after 1 year of treatment. The patient was unable to play piano and ambulated using 4-point cane.  There was atrophy of the distal limbs. Repeat EMG/NCS demonstrated demyelinating polyneuropathy. There was improvement in upper extremity strength and moderate improvement in the lower extremity with the addition of corticosteroids and the patient resumed piano playing and ambulates independently. Case 2 is a 5-year-old female who presented with 1 week of mild distal weakness of the upper and lower extremities. Family declined to treat given mild symptoms. Eight weeks after onset, the patient could not hold utensils or walk without assistance. Weakness recovered rapidly with IVIG. After 24 months of treatment, the patient is in clinical remission.

Conclusions:

DADS neuropathy presents in the pediatric population as a distinct phenotype from classic CIDP. Prompt diagnosis and initiation of treatment may prevent morbidity from muscle atrophy. EMG/NCS is useful for detecting active demyelination. Second-line treatments should be started in pediatric patients who do to improve after IVIG.