+ Site Statistics
References:
52,654,530
Abstracts:
29,560,856
PMIDs:
28,072,755
+ Search Articles
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ PDF Full Text
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Translate
+ Recently Requested

A case of Moebius syndrome--electrophysiological studies of facial nerve and brainstem



A case of Moebius syndrome--electrophysiological studies of facial nerve and brainstem



Rinsho Shinkeigaku 31(11): 1192-1196



A five-year old boy was the product of a 40 week pregnancy by vertex presentation complicated only by threatened abortion at approximately 8 weeks gestation. Apgar score was 5 after one minute. At birth he was noted to have a generalized hypotonia associated with facial diplegia, small mandible, weak suck and swallow reflexes. Admission examination revealed small mandible, mask-like facial expression and mild mental retardation. Cranial nerve examination showed bilateral blepharoptosis and facial nerve palsies. Pupil reflexes were normal, but corneal reflexes were impaired bilaterally. Diplopia due to the left abducens nerve palsy was suggested. There was no atrophy of the tongue. Motor tone, strength, and deep tendon reflexes were normal. A normal 46 XY karyotype was present. The other clinical and laboratory findings were normal. MRI of the brain was unremarkable. The characteristics of electrophysiological studies were summarized as follows: 1) Auditory brainstem evoked responses demonstrated waveforms IV-V were abnormal because their amplitudes were less than 30% of wave I bilaterally. 2) Somatosensory evoked potentials documented by central conduction times from cervical region to sensory cortex were prolonged on both sides. 3) Facial nerve conduction velocity was calculated by evoked EMGs of the mentalis muscle electrically stimulated at two distal points over the marginal mandibular branch. MCV of the left side was reduced (34.2 m/sec). 4) The amplitude of the facial muscle potentials evoked by facial nerve stimulation was reduced on both sides. 5) Blink reflex responses documented by the latency difference of R1 responses between the two sides were prolonged.

(PDF emailed within 1 workday: $29.90)

Accession: 039031006

Download citation: RISBibTeXText

PMID: 1813186


Related references

Moebius syndrome with central hypoventilation and brainstem calcification a case report. European Journal of Pediatrics 150(8): 582-583, 1991

Congenital facial palsy and emotion processing: The case of moebius syndrome. Genes, Brain, and Behavior 2019: E12548-E12548, 2019

Case of bilateral congenital lack of abduction with facial paresis (Moebius syndrome). Wiener Klinische Wochenschrift 69(30): 541-542, 1957

Animation of facial palsy in Moebius syndrome by means of a circumoral sling: case report. Journal of Maxillofacial Surgery 4(4): 214-216, 1976

Autonomic Responses to Emotional Stimuli in Children Affected by Facial Palsy: The Case of Moebius Syndrome. Neural Plasticity 2019: 7253768, 2019

Cross-facial nerve grafting as an adjunct to hypoglossal-facial nerve crossover in reanimation of early facial paralysis: clinical and electrophysiological evaluation. Annals of Plastic Surgery 46(3): 301-307, 2001

Reproducibility of normal facial motor nerve conduction studies and their relevance in the electrophysiological assessment of peripheral facial paralysis. Neurophysiologie Clinique 27(4): 300-308, 1997

Facial mimicry is not necessary to recognize emotion: Facial expression recognition by people with Moebius syndrome. Social Neuroscience 5(2): 241-251, 2010

Brainstem dysgenesis: beyond Moebius syndrome. Revista de Neurologia 66(7): 241-250, 2018

Pathogenetic considerations on a rare case of Moebius' syndrome (facial diplegia with bilateral external ophthalmoplegia and congenital "relaxatio" of the left hemidiaphragm). Rivista di Clinica Pediatrica 66: 1-20, 1960

Differences in glial, synaptic and motoneuron responses in the facial nucleus of the rat brainstem following facial nerve resection and nerve suture reanastomosis. European Archives Of Oto-Rhino-Laryngology. 251(7): 410-417, 1994

+ - Reconstruction of the intratemporal facial nerve using interposition nerve graft: time course of recovery in facial movement and electrophysiological findings. Acta Oto-Laryngologica. Supplementum 2008(559): 85-90, 2008

On the site of transcranial magnetic stimulation of the facial nerve: electrophysiological observations in two patients after transection of the facial nerve during neuroma removal. Neurosurgery 36(2): 346-349, 1995

How Deleterious Is Facial Nerve Dissection for the Facial Nerve in Parotid Surgery: An Electrophysiological Evaluation. Journal of Craniofacial Surgery 28(1): 56-60, 2016

Facial animation in children with Moebius and Moebius-like syndromes. Journal of Pediatric Surgery 44(11): 2236-2242, 2010