Affiliation:
1. Departments ofPediatrics
2. Neurology
3. Department of Neurology, University Hospital Maastricht, Maastricht, Netherlands
4. Pathology
5. Neurosurgery
6. Obstetrics, University Hospital Groningen, Groningen, Netherlands
Abstract
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.
Publisher
American Academy of Pediatrics (AAP)
Subject
Pediatrics, Perinatology and Child Health
Reference40 articles.
1. O'Rahilly R, Muller F. Bidirectional closure of the rostral neuropore in the human embryo. Am J Anat.1989;184:259–268
2. Van Allen MI, Kalousek DK, Chernoff GF, et al. Evidence for multi-site closure of the neural tube in humans. Am J Med Genet.1993;47:723–743
3. Golden JA, Chernoff GF. Multiple sites of anterior neural tube closure in humans: evidence from anterior neural tube defects (anencephaly). Pediatrics.1995;95:506–510
4. Sival DA, Begeer JH, Staal-Schreinemachers AL, Vos-Niel JME, Beekhuis JR, Prechtl HFR. Perinatal motor behaviour and neurological outcome in spina bifida aperta. Early Hum Dev.1997;50:27–38
5. Hirose S, Farmer DL, Albanese CT. Fetal surgery for myelomeningocele. Curr Opin Obstet Gynecol.2001;13:215–222
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