Are electrophysiological criteria useful in distinguishing childhood demyelinating neuropathies?

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 analys...

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Bibliographic Details
Published in:Journal of the peripheral nervous system 2016-03, Vol.21 (1), p.22-26
Main Authors: Potulska-Chromik, Anna, Ryniewicz, Barbara, Aragon-Gawinska, Karolina, Kabzinska, Dagmara, Seroka, Andrzej, Lipowska, Marta, Kaminska, Anna M., Kostera-Pruszczyk, Anna
Format: Article
Language:English
Subjects:
Adolescent
Charcot-Marie-Tooth disease
Charcot-Marie-Tooth Disease - diagnosis
Child
Child, Preschool
childhood hereditary neuropathy
chronic demyelinating inflammatory polyneuropathy
Diagnosis, Differential
Electrodiagnosis - methods
Electrophysiology - methods
Female
hereditary neuropathy with liability to pressure palsy
Hereditary Sensory and Motor Neuropathy - diagnosis
Humans
Male
Neural Conduction
PMP22
Polyradiculoneuropathy, Chronic Inflammatory Demyelinating - diagnosis
Retrospective Studies
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Summary: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.
ISSN:1085-9489
1529-8027
DOI:10.1111/jns.12152