The abnormal nucleus as a cause of congenital facial palsy

Abstract

Background-Congenital facial palsy (CFP) is clinically defined as facial palsy present at birth. It is associated with considerable disfigurement and causes functional and emotional problems for the aVected child. The aetiology of the majority of cases however, remains elusive. Aims-To investigate the role of a neuroanatomical abnormality as a cause of unilateral CFP. Methods-Magnetic resonance imaging (MRI) scans were performed on 21 patients with unilateral CFP. Fifteen patients had unilateral CFP only; six suVered from syndromes which can include unilateral CFP. Results-Of the 15 patients with unilateral CFP only, four (27%) had an abnormal nucleus or an abnormal weighting of this area on the MRI scan, compared to one (17%) of the remaining six patients. Conclusion-Developmental abnormalities of the facial nucleus itself constitute an important, and previously ignored, cause of monosymptomatic unilateral CFP.

Facial Nerve Innervation and Facial Palsies

“…It is in close proximity to the eighth nerve (CNVIII, vestibulocochlear) and its fi bers pass around the nuclei of the sixth nerve (CNVI, abducens) and the superior salivatory nerve. Pontine lesions can be associated with a number of syndromes that are characterized by a spectrum of involvement of these nerves (Jemec et al 2000 ) . Examples include Moebius syndrome (congenital VIth and VIIth cranial nerve palsies) and pseudobulbar palsy (bilateral facial paralysis and other cranial nerve de fi cits, a hyperreactive gag re fl ex and hyperre fl exia associated with hypertension).…”

Congenital Isolated Facial Palsy Associated With Cleft Lip and Palate

“…Moebius syndrome is hypoplasia of the V, VI, VII and VIII cranial nerves/nuclei associated clubbed feet, missing fingers or toes, abnormal chest-wall and strabismus. [5] Sign of trauma such as behind ear ecchymosis and hemoperitoneum can predict the traumatic causes, and it have good prognosis where improvement occurs within a few weeks unless the nerve is completely torn [6] The disordered of congenital facial nerve agenesis has poor prognoses comparing to whom are traumatic. [7] Cleft lip and cleft palate are considered the most common congenital defects involving craniofacial structure with incidence around 2,55/1000 newborn, developing in the early embryonic stages between the 4th and 8th week of intrauterine life.…”

Rare Association of Bilateral Anotia with Congenital Facial Palsy

“…Other causes include, intrauterine posture, intrapartum compression, and familial and congenital aplasia of the nucleus; the last being most frequently reported for bilateral cases. There are a number of syndromes which encompass CFP as part of their symptoms, including the cardiofacial, Moebius, Poland's, and Goldenhaar's syndrome 1,7 . Some cases of CFP have been a ributed to agenesis of the petrous por on of the temporal bone, with resul ng agenesis of the facial and auditory nerves, the external ear and the mastoid region 7 .…”

“…There are a number of syndromes which encompass CFP as part of their symptoms, including the cardiofacial, Moebius, Poland's, and Goldenhaar's syndrome 1,7 . Some cases of CFP have been a ributed to agenesis of the petrous por on of the temporal bone, with resul ng agenesis of the facial and auditory nerves, the external ear and the mastoid region 7 . CFP may cause mul ple problems in newborn like diffi culty in nursing, eye closure, development of speech, expression of emo ons, mas ca on.…”