Are electrophysiological criteria useful in distinguishing childhood demyelinating neuropathies?

J Peripher Nerv Syst. 2016 Mar;21(1):22-6. doi: 10.1111/jns.12152.

Abstract

Childhood chronic inflammatory demyelinating polyneuropathy (CIDP) needs to be differentiated from hereditary neuropathy. We aimed to validate existing CIDP nerve conduction study (NCS) criteria in a group of children with demyelinating neuropathies of chronic or subacute onset. Retrospective analysis of clinical and NCS results in 18 children with CIDP, 7 with hereditary neuropathy with pressure palsy (HNPP), and 24 with Charcot-Marie-Tooth 1a (CMT1a). AAN and EFNS electrodiagnostic CIDP criteria were fulfilled in 17 of 18 CIDP, 3 of 7 HNPP, and 23 of 24 CMT1a patients. A distal compound muscle action potential (dCMAP) of >9 ms was observed in 14 of 18 CIDP patients but not in any patients with HNPP. Abnormal median/normal sural SNAP (AMNS) and a 10 m/s difference between conduction velocities (CV) of two corresponding nerves were not observed in any CMT1a patients. NCS in CMT1a, HNPP, and CIDP reflect demyelination. dCMAP duration, sensory AMNS, and a 10 m/s CV difference parameter are most useful in the differential diagnosis of pediatric CIDP.

Keywords: Charcot-Marie-Tooth disease; PMP22; childhood hereditary neuropathy; chronic demyelinating inflammatory polyneuropathy; hereditary neuropathy with liability to pressure palsy.

MeSH terms

  • Adolescent
  • Charcot-Marie-Tooth Disease / diagnosis*
  • Child
  • Child, Preschool
  • Diagnosis, Differential
  • Electrodiagnosis / methods*
  • Electrophysiology / methods
  • Female
  • Hereditary Sensory and Motor Neuropathy / diagnosis
  • Humans
  • Male
  • Neural Conduction
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / diagnosis*
  • Retrospective Studies