1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-6-86-b 10.15844/pedneurbriefs-6-11-10 Neuropathies Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant HMSN I: Early Diagnosis http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 11 1992 01 07 2016 6 11 86 87 Copyright: © 1992 The Author(s) 1992 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Hereditary motor sensory neuropathy type I in childhood

The value of nerve conduction velocities in the detection of hereditary motor sensory neuropathy type I (HMSN I) in children at risk was determined in 36 children under 10 years of age at the University of Western Ontario, London, Ontario, Canada.

 Hereditary Motor Sensory Neuropathy Type I Pes Planus Myelin Glycoprotein

The value of nerve conduction velocities in the detection of hereditary motor sensory neuropathy type I (HMSN I) in children at risk was determined in 36 children under 10 years of age at the University of Western Ontario, London, Ontario, Canada. Clinical signs and slowed motor nerve conduction velocities were found in 17 of the 36 children who had 1 parent with HMSN I. Four children had slowed conduction velocities at 1 year of age or less. Clinical signs were subtle and included pes planus, distal foot wasting, weakness of ankle eversion and dorsiflexion, and areflexia. In all but 1 of the 17 affected patients the motor nerve conduction velocities were less than 40 m/s. Sensory potentials were abnormal in 7 children with HMSN I at ages 6-7 years. [1]

COMMENT. These data show that HMSN I can be detected in early childhood. Even at 1 year of age or less the motor conduction velocity is significantly slowed. Abnormal physical findings are found in all children who have slow conduction velocities. The signs may be subtle and not accompanied by disability. Pes planus was the most common foot abnormality in this series. Nerve hypertrophy was uncommon.

Dr. Sghirlanzoni et al., Milan, Italy, report two siblings with HMSN III (Dejerine-Sottas disease) whose parents were both affected with autosomal dominant axonal HMSN II. This family and others cited show the existence of an HMSN III phenotype resulting from the homozygous expressions of HMSN I and II genes. [2]

A 4-year-old child with severe hypertrophic peripheral neuropathy had antibodies to myelin glycoprotein of peripheral nerve [3]. This anti-Po glycoprotein activity may have a role in pathogenesis of the neuropathy.

 Feasby TE Hahn AF Bolton CF Brown WF Koopman WJ Hereditary motor sensory neuropathy type I in childhood J Neurol Neurosurg Psychiatry 1992 Oct 55 10 895 897 10.1136/jnnp.55.10.895 1331333 Sghirlanzoni A Pareyson D Balestrini MR Bellone E Berta E Ciano C HMSN III phenotype due to homozygous expression of a dominant HMSN II gene Neurology 1992 Nov 42 11 2201 2204 10.1212/WNL.42.11.2201 1436537 Jelloun-Dellagi SB Dellagi K Burger D Ben Younes-Chennoufi A Hentati FF Steck A Childhood peripheral neuropathy with autoantibodies to myelin glycoprotein P0 Ann Neurol 1992 Nov 32 5 700 702 10.1002/ana.410320515 1280408