Are electrophysiological criteria useful in distinguishing childhood demyelinating neuropathies?

作者：Potulska Chromik Anna; Ryniewicz Barbara; Aragon Gawinska Karolina; Kabzinska Dagmara; Seroka Andrzej; Lipowska Marta; Kaminska Anna M; Kostera Pruszczyk Anna^*

来源：Journal of the Peripheral Nervous System, 2016, 21(1): 22-26.

DOI：10.1111/jns.12152

• Charcot-Marie-Tooth disease
• childhood hereditary neuropathy
• chronic demyelinating inflammatory polyneuropathy
• hereditary neuropathy with liability to pressure palsy
• PMP22

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.

• 出版日期2016-3