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PEDIATRICS Vol. 114 No. 2 August 2004, pp. 427-434

Neonatal Loss of Motor Function in Human Spina Bifida Aperta

Deborah A. Sival, MD, PhD^*,, Tiemen W. van Weerden, MD, PhD, Johan S.H. Vles, MD, PhD, Albert Timmer, MD, PhD^||, Wilfred F.A. den Dunnen, MD, PhD^||, A.L. Staal-Schreinemachers, MD, Eelco W. Hoving, MD, PhD^¶, Krystyne M. Sollie, MD^#, Vivianne J.M. Kranen-Mastenbroek, MD, PhD, Pieter J.J. Sauer, MD, PhD^* and Oebele F. Brouwer, MD, PhD

^* Departments ofPediatrics
Neurology
^|| Pathology
^¶ Neurosurgery
^# Obstetrics, University Hospital Groningen, Groningen, Netherlands
Department of Neurology, University Hospital Maastricht, Maastricht, Netherlands

Objective. In neonates with spina bifida aperta (SBA), leg movements innervated by spinal segments located caudal to the meningomyelocele are transiently present. This study in neonates with SBA aimed to determine whether the presence of leg movements indicates functional integrity of neuronal innervation and whether these leg movements disappear as a result of dysfunction of upper motor neurons (axons originating cranial to the meningomyelocele) and/or of lower motor neurons (located caudal to the meningomyelocele).

Methods. Leg movements were investigated in neonates with SBA at postnatal day 1 (n = 18) and day 7 (n = 10). Upper and lower motor neuron dysfunction was assessed by neurologic examination (n = 18; disinhibition or inhibition of reflexes, respectively) and by electromyography (n = 12; absence or presence of denervation potentials, respectively).

Results. Movements, related to spinal segments caudal to the meningomyelocele, were present in all neonates at postnatal day 1. At day 1, leg movements were associated with signs of both upper (10 of 18) and lower (17 of 18) motor neuron dysfunction caudal to the meningomyelocele. In 7 of 10 neonates restudied after the first postnatal week, leg movements had disappeared. The absence of leg movements coincided with loss of relevant reflexes, which had been present at day 1, indicating progression of lower motor neuron dysfunction.

Conclusions. We conclude that the presence of neonatal leg movements does not indicate integrity of functional lower motor neuron innervation by spinal segments caudal to the meningomyelocele. Present observations could explain why fetal surgery at the level of the meningomyelocele does not prevent loss of leg movements.

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Key Words: spina bifida • motor neuron damage • leg movement • denervation • electromyography • meningomyelocele

Abbreviations: SBA, spina bifida aperta • MMC, meningomyelocele • LMN, lower motor neuron • UMN, upper motor neuron • EMG, electromyography

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Received for publication Jul 7, 2003; Accepted Dec 12, 2003.

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