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Cureus Feb 2023Moebius syndrome (MBS) is a rare congenital cranial nerve disorder characterized by unilateral, bilateral symmetrical, or asymmetrical facial (VII) and abducens (VI)... (Review)
Review
Moebius syndrome (MBS) is a rare congenital cranial nerve disorder characterized by unilateral, bilateral symmetrical, or asymmetrical facial (VII) and abducens (VI) nerve palsies. Genetics and rhombencephalon vascular disturbances from intrauterine environmental exposures have been attributed to its development. It can present with various orofacial abnormalities. Although the diagnosis is purely clinical, certain characteristic features are present in the brain's images. With no cure, it is essential to devise management on a personalized basis. We discuss etiology, presentation, diagnostic approaches, and effective management in the existing literature. This comprehensive review examines the clinic-pathological aspects of Moebius syndrome. The authors employed the PUBMED base index to identify pertinent literature and reference it according to research keywords. Findings suggest the most popular etiology is the theory of intrauterine vascular disruption to the brainstem during embryogenesis, followed by the genetic hypothesis. Intrauterine environmental exposures have been implicated as potential risk factors. Facial and abducens nerve palsies are the most common presenting features. However, clinical manifestations of lower cranial nerves (IX, X, XI, XII) may be present with orthopedic anomalies and intellectual deficiencies. The diagnosis is clinical with minimal defined diagnostic criteria. Characteristic radiological manifestations involving the brainstem and cerebellum can be observed in imaging studies. With no definitive treatment options, a multidisciplinary approach is employed to provide supportive care. Despite radiological manifestations, Moebius syndrome is diagnosed clinically. Although incurable, a multidisciplinary approach, with personalized rehabilitative measures, can manage physical and psychological deficits; however, standard guidelines need to be established.
PubMed: 36960250
DOI: 10.7759/cureus.35187 -
Italian Journal of Pediatrics Jun 2016Moebius syndrome (MBS) is rare disease characterized by nonprogressive congenital uni- or bi-lateral facial (i. e. VII cranial nerve) and abducens (i. e. VI cranial...
BACKGROUND
Moebius syndrome (MBS) is rare disease characterized by nonprogressive congenital uni- or bi-lateral facial (i. e. VII cranial nerve) and abducens (i. e. VI cranial nerve) palsy. Although the neurological and ophthalmological findings are quite well-known, data concerning the attendant functional difficulties and their changes over time are seldom addressed. In this study we attempt to estimate the prevalence of clinical and functional data in an Italian cohort affected by MBS.
METHODS
The study included 50 children, 21 males and 29 females, aged 1 month to 14 years. The patients entered into a multidisciplinary diagnostic and follow-up protocol that had the specific purpose of detecting clinical and developmental deficits related to MBS.
RESULTS
Involvement of the VII cranial nerve (total/partial, bilateral or unilateral) was present in 96 % of patients, and of the VI nerve in 85 %. Two patients were without impairment of the VII nerve and seven patients had no involvement of the VI nerve and were thus classified as Moebius-like because of the involvement of other CNs. Additional affected CNs were numbers III-IV in 16 %, V in 11 %, VIII and X each in 8 %, the XI in 6 %, the IX, most often partially, in 22 %, and the XII in 48 % of cases. Their development was characterized by global delay at one year of age, motor, emotional and speech difficulties at two years of age, a trend toward normalization at three years of age but with weakness in hand-eye coordination, and achieving average results at five years of age. Overall 90 % of children had a normal developmental quotient whereas only 10 % manifested cognitive deficits.
CONCLUSION
Early rehabilitation may enhance the recovery of normal function, particularly in vulnerable areas of development. It is possible that early intervention that integrates sensory and visual information with emotional difficulties can improve the prognosis of the child with MBS.
Topics: Abducens Nerve; Adolescent; Child; Child, Preschool; Cohort Studies; Disability Evaluation; Early Medical Intervention; Facial Nerve; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Italy; Male; Mobius Syndrome; Neuropsychological Tests; Rare Diseases; Risk Assessment; Treatment Outcome
PubMed: 27260152
DOI: 10.1186/s13052-016-0256-5 -
The Kurume Medical Journal Mar 2022An ossified left petroclinoid ligament was found during routine dissection of the skull base in an adult male cadaver. The petroclinoid ligament is clinically and... (Review)
Review
An ossified left petroclinoid ligament was found during routine dissection of the skull base in an adult male cadaver. The petroclinoid ligament is clinically and surgically important given its anatomical relationships to cranial nerves III, V, and VI, so its ossification is a risk factor for injuries due to trauma, increased intracranial pressure, and vascular and tumor compression resulting in abducens and oculomotor palsies. The causes of petroclinoid ligament ossification are yet to be completely elucidated although several reports have associated them with age-related and physiological processes. Assessing the integrity of the petroclinoid ligament is important during skull base surgical interventions to avoid postoperative complications. Therefore, this paper reviews the petroclinoid ligament and its variation, the ossified petroclinoid ligament.
Topics: Abducens Nerve; Adult; Cadaver; Dissection; Humans; Ligaments; Male; Osteogenesis
PubMed: 35095019
DOI: 10.2739/kurumemedj.MS671007 -
Clinical Case Reports Dec 2022Moebius Syndrome is a rare multifactorial condition defined by congenital complete or partial VII and VIth cranial nerves palsy and other physical abnormalities. We...
Moebius Syndrome is a rare multifactorial condition defined by congenital complete or partial VII and VIth cranial nerves palsy and other physical abnormalities. We present the case of a 3 months old infant with Moebius sequence and breathing and eating difficulties, managed by tracheostomy and laryngoplasty.
PubMed: 36523389
DOI: 10.1002/ccr3.6715 -
Child Neurology Open 2023Moebius Syndrome, is a rare, non-progressive congenital neuropathological syndrome characterized primarily by the underdevelopment of the facial (CN VII) and abducens... (Review)
Review
Moebius Syndrome, is a rare, non-progressive congenital neuropathological syndrome characterized primarily by the underdevelopment of the facial (CN VII) and abducens nerve (CN VI). Other features of Moebius Syndrome include facial nerve paresis, ophthalmoplegias, orthodontic deficiencies (including crowded dentition, swollen and hyperplastic gingiva, dental calculus, etc.), musculoskeletal abnormalities, and impaired mental function. Due to the rarity of the disorder, very few case studies have been reported in the literature. This article summarizes the significant features of the disease according to commonalities in reported cases, along with several newly recognized features cited in recent literature. We have explored the different diagnostic criteria and the newly recognized imaging modalities that may be used. Understandably, the condition detrimentally affects a patient's quality of life; thus, treatment measures have also been outlined. This study aims to provide updated literature on Moebius Syndrome MBS and improve understanding of the condition.
PubMed: 37868706
DOI: 10.1177/2329048X231205405