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IMAGES IN NEUROLOGY
Year : 2021  |  Volume : 24  |  Issue : 6  |  Page : 929
 

Moebius syndrome


Department of Neurology, Sheri Kashmir Institute of Medical Sciences, Jammu and Kashmir, India

Date of Submission06-Mar-2021
Date of Acceptance31-Mar-2021
Date of Web Publication20-Aug-2021

Correspondence Address:
Waseem Dar
Department of Neurology, Sheri Kashmir Institute of Medical Sciences, Jammu and Kashmir - 190 011
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aian.AIAN_182_21

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How to cite this article:
Yaqoob A, Dar W, Raina A, Chandra A, Khawaja Z, Bukhari I, Ganie H, Wani M, Asimi R. Moebius syndrome. Ann Indian Acad Neurol 2021;24:929

How to cite this URL:
Yaqoob A, Dar W, Raina A, Chandra A, Khawaja Z, Bukhari I, Ganie H, Wani M, Asimi R. Moebius syndrome. Ann Indian Acad Neurol [serial online] 2021 [cited 2022 Jul 7];24:929. Available from: https://www.annalsofian.org/text.asp?2021/24/6/929/324151




Moebius syndrome presents as congenital, nonprogressive unilateral or bilateral facial and horizontal gaze palsy.[1],[2] A 16-year-old male presented to our center with restricted eye movements with turning of head sideways to look at, inability to blow out the cheeks, and accumulation of food between teeth and cheeks since childhood. There was no history of drooling and he didn't report any recurrent childhood ear infections. On examination, patient had gaze-evoked nystagmus, horizontal gaze restriction with bilateral medial and lateral rectus palsy with preserved vertical movements. There was micrognathia, bilateral facial palsy (predominant lower face involvement), tongue atrophy, and tongue fasciculations. Intelligence quotient of the patient was normal. MRI of the brain revealed brainstem hypoplasia and straightening of the floor of the fourth ventricle [Figure 1].
Figure 1: (a) Tongue atrophy. (b) Restriction of right lateral gaze (c) Restriction of left lateral gaze (d) Brainstem atrophy (e) Flattening of the floor of 4th Ventricle at the level of the inferior colliculus. (f) Inability to puff out cheeks suggestive of bilateral facial palsy

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The pathogenesis and etiology of the Moebius sequence appeared to be multifactorial. It is postulated to be due to vascular disruption in the brain during prenatal development leading to hypoplasia or agenesis of the cranial nerve nuclei during fetal development. Sporadic mutations in PLXND1 and REV3L genes have also been identified in a number of patients and confirmed to cause a constellation of findings consistent with Moebius syndrome when introduced in animal models. In rare cases, familial patterns have been reported. Most likely, Moebius syndrome is multifactorial, which means that both genetic and environmental factors play some causative role.

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   References Top

1.
Henderson JL. The congenital facial diplegia syndrome: Clinical features, pathology and aetiology: A review of sixty-one cases. Brain 1939;62:381-403.  Back to cited text no. 1
    
2.
Ouanounou S, Saigal G, Birchansky S. Mobius syndrome. AJNR Am J Neuroradiol 2005;26:430-2.  Back to cited text no. 2
    


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