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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 -
Endocrine Aug 2021Hypophysitis is a rare pituitary inflammatory disorder classified in different ways. Immunoglobulin G4-related disease (IgG4-RD), also a rare disease is a systemic... (Review)
Review
Hypophysitis is a rare pituitary inflammatory disorder classified in different ways. Immunoglobulin G4-related disease (IgG4-RD), also a rare disease is a systemic fibro-inflammatory condition characterized by infiltration of tissue with IgG4-positive plasma cells; however prevalence of both of them probably is underestimated. In this paper, we present an Iranian patient with biopsy-proven IgG4-related hypophysitis and then review the clinical characteristics, laboratory, imaging, pathologic findings and therapeutic management as well as prognosis of 115 published cases of hypophysitis secondary to IgG4-related disease.
Topics: Autoimmune Hypophysitis; Humans; Hypophysitis; Iran; Pituitary Diseases; Pituitary Gland
PubMed: 33837927
DOI: 10.1007/s12020-021-02714-0 -
Journal of Binocular Vision and Ocular... 2020Recent reports confirm innervational compartments of select rectus extraocular muscles as well as the superior oblique. Histopathological and orbital imaging studies... (Review)
Review
Recent reports confirm innervational compartments of select rectus extraocular muscles as well as the superior oblique. Histopathological and orbital imaging studies demonstrate well defined compartmental innervation of the horizontal rectus muscles with less differentiation in the vertical rectus muscles. Acquired vertical misalignment not associated with cyclovertical muscle dysfunction has been associated with horizontal rectus muscle compartment dysfunction. Pattern and other forms of strabismus have been associated with segmental or compartmental abnormal innervation of the extraocular muscles. Taking advantage of segmental function and innervation, selective weakening and strengthening procedures have been used to treat patients with incomitant near/distance disparities, incomitant vertical and torsional strabismus, and patients with A- and V-pattern strabismus.
Topics: Abducens Nerve Diseases; Compartment Syndromes; Humans; Oculomotor Muscles; Strabismus
PubMed: 32463321
DOI: 10.1080/2576117X.2020.1760683 -
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 -
Seminars in Ultrasound, CT, and MR Oct 2022The abducens, or the sixth cranial nerve, is purely motor and runs a long course from the brainstem to the lateral rectus. Travels with the inferior petrosal sinus...
The abducens, or the sixth cranial nerve, is purely motor and runs a long course from the brainstem to the lateral rectus. Travels with the inferior petrosal sinus through the Dorello's canal before entering the cavernous sinus. Based on the location of an abnormality, other neurologic structures may be involved with the disturbs related to this nerve. This article aims to review the abducens nerve anatomy and demonstrates the imaging aspect of the diseases that most commonly affect it.
Topics: Abducens Nerve; Cavernous Sinus; Humans
PubMed: 36116854
DOI: 10.1053/j.sult.2022.04.008 -
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