title: Are electrophysiological criteria useful in distinguishing childhood demyelinating neuropathies?
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.
authors.affiliation: Department of Neurology, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Anna
authors.initials: A
authors.lastname: Potulska-Chromik
authors.affiliation: Department of Neurology, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Barbara
authors.initials: B
authors.lastname: Ryniewicz
authors.affiliation: Department of Neurology, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Karolina
authors.initials: K
authors.lastname: Aragon-Gawinska
authors.affiliation: Neuromuscular Unit, Polish Academy of Sciences, Warsaw, Poland.
authors.forename: Dagmara
authors.initials: D
authors.lastname: Kabzinska
authors.affiliation: Department of Neurology, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Andrzej
authors.initials: A
authors.lastname: Seroka
authors.affiliation: Department of Neurology, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Marta
authors.initials: M
authors.lastname: Lipowska
authors.affiliation: Department of Neurology, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Anna M
authors.initials: AM
authors.lastname: Kaminska
authors.affiliation: Department of Neurology, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Anna
authors.initials: A
authors.lastname: Kostera-Pruszczyk
mesh_terms: D000293:Adolescent; D002607:Charcot-Marie-Tooth Disease / Q000175:diagnosis*; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D004568:Electrodiagnosis / Q000379:methods*; D004594:Electrophysiology / Q000379:methods; D005260:Female; D015417:Hereditary Sensory and Motor Neuropathy / Q000175:diagnosis; D006801:Humans; D008297:Male; D009431:Neural Conduction; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000175:diagnosis*; D012189:Retrospective Studies
keywords: Charcot-Marie-Tooth disease; PMP22; childhood hereditary neuropathy; chronic demyelinating inflammatory polyneuropathy; hereditary neuropathy with liability to pressure palsy