PubMed · 17089308
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K H Leitz, J Neu. 2006. [From the North German Arbitration Board].. https://doi.org/10.1055/s-2006-924568
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Anterior spinal artery syndrome is rare in children, especially in neonates. We present a girl with hydrops fetalis and hypothyroidism who developed flaccid paresis of both arms in the neonatal period (around day 25). MRI of the spine performed on day 52 revealed atrophic changes at C5-Th1 without Gd-DTPA-induced enhancement. Nerve conduction studies were also helpful in the diagnosis;in the upper limbs, motor potential was not elicited, while sensory nerve conduction velocity was normal. These clinical and laboratory findings suggested an atypical case of anterior spinal artery syndrome.
A patient who developed isolated brachial diplegia following cardiac surgery is described. The underlying cerebral lesion could not be localized using magnetic resonance imaging (MRI). Evoked potentials disclosed normal findings, while pathological latencies were seen on cortical magnetic stimulation. Their marked improvement over the following year was accompanied by almost complete clinical recovery. The preserved arm reflexes, together with the observed slow firing motor units in electromyography argued against bilateral lesions of the brachial plexus. We attribute the observed diplegia to a medullary lesion at the level of the pyramidal decussation, presumably caused by an intraoperative embolic occlusion of the anterior spinal artery. Cruciate paralysis and man-in-barrel-syndrome (MIBS) both are terms used to describe brachial diplegia; cruciate paralysis when caused by medullary lesions, MIBS when caused either by supratentorial or by medullary lesions. Exclusive use of the term MIBS for bilateral frontal lobe lesions, as in the original description, would provide more clarity in terminology.
Anterior spinal artery syndrome is rare in children. In adults, where it is observed most frequently after resection of thoracoabdominal aortic aneurysms, spinal magnetic resonance imaging is considered the first-line investigation to confirm the clinical diagnosis. A 3-year-old male who presented with this syndrome after palliative cardiac surgery for a complex cardiac malformation associated with aortic coarctation is presented. Clinical diagnosis of anterior horn cell impairment below the L2 level was confirmed by electromyography and F-wave studies. Sparing of dorsal sensory tracts was documented by normal somatosensory-evoked potentials, which confirmed the anterior localization of the lesion. Spinal magnetic resonance imaging performed on day 15 and day 105 after surgery was normal. Neurologic deficits, including flaccid paraplegia, remained stable except for the reappearance of patellar reflexes on day 83. Neurophysiologic conduction studies were consistent with lower motoneuron loss. In this patient, magnetic resonance imaging was less sensitive in demonstrating spinal cord lesion than clinical neurophysiology. Somatosensory-evoked potentials failed to detect the insult. Prevention may therefore require other neurophysiologic monitoring techniques.