appearance of contralateral R1 in a child with VATER syndrome

초록

Introduction Blink reflex has been used to analyze the central or peripheral lesions of the trigeminal or facial nerves in patients with facial palsy or neuralgia. When stimulating the supraorbital nerve, the ipsilateral early response (R1) is derived and then the ipsilateral late response (R2), contralateral R2 is following. We have experienced a rare case of appearance of the contralateral R1 in a child with facial palsy. Case On April, 2013, a two-month-old (one month-old calibration age) girl visited for the right facial palsy. At birth, she had facial palsy, microtia, esophageal atresia with tracheoesophageal fistula imperforated anus, polydactyly, a pattern was consistent with the VATER syndrome. At her fist visit, she showed right facial palsy and relatively more developed left facial muscles. The brain MRI showed only a little slow myelination without malformation. Electrical stimulation therapy (EST) was performed about three months, and nerve conduction study (NCS) on the both facial nerves was performed in August 2013. The NCS on Rt. Facial nerve (nasalis recording) showed no response and the ENOG was 100%. Following NCS showed a regeneration state with 83% of ENOG in May 2015. The NCS conducted in March 2020 (when she was 7 years old) showed 86% of ENOG and decreased amplitude (0.6mV) (Table 1). In Blink reflex study contralateral R1 was found when we stimulated affected side (Table 2).This contralateral R1 would be explained by the following reasons. First, it might occur when there is contraction of the orbicularis oculi muscle on the opposite side of the stimulus. Second, when the location of the stimulus is close to the midline, both sides could be stimulated. Third, it caused by the re-organizing of synapses after nerve damage, creating a direct anatomical connection between trigeminal sensory nucleus and opposite facial nucleus