Two Novel CHN1 Mutations in 2 Families With Duane Retraction Syndrome

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Two novel CHN1 mutations in 2 families with Duane retraction syndrome.

OBJECTIVE To determine the genetic cause of Duane retraction syndrome (DRS) in 2 families segregating DRS as a dominant trait. METHODS Members of 2 unrelated pedigrees were enrolled in a genetic study. Linkage analysis was performed on the CHN1 locus. Probands and family members were screened for CHN1 mutations. RESULTS The 6 affected individuals in the 2 pedigrees have DRS. Both pedigrees ...

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Duane retraction syndrome: MRI features in two cases.

PURPOSE Neuroimaging findings in Duane's retraction syndrome (DRS), through magnetic resonance imaging (MRI), suggest that aplasia of the abducens nerve (VI) can be responsible for several forms of DRS. METHODS Brain MRI was performed in two children of 2.5 and 7 years of age with left sided DRS type II and right sided DRS type I, respectively. 3D T2 weighted images through the brainstem were...

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Duane retraction syndrome in a Nigerian child

We report a case of a four year old Nigerian girl who presented to the paediatric unit of our eye clinic with complaints of a squint on looking to the right side and reduction in the size of the right eye when looking to the left. On examination, she had right exotropia in the primary position of gaze. There was limitation of abduction and widening of the palpebral fissure of the right eye on r...

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Properties of saccades in Duane retraction syndrome.

PURPOSE To improve understanding of the binocular control of saccades by making high-resolution eye movement recordings in patients with unilateral Duane retraction syndrome (DRS) type I (marked by congenital absence of the sixth cranial nerve). METHODS Binocular eye movements were recorded in four patients in binocular viewing conditions during a saccade task. RESULTS Affected-side gaze sh...

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Novel SLC9A6 mutations in two families with Christianson syndrome.

To the Editor : Mutations in SLC9A6 are associated with Christianson syndrome, a syndromic form of X-linked intellectual disability characterized by severe intellectual disability, acquired microcephaly, seizures and ataxia (1, 2). Only nine mutations and two genomic aberrations involving SLC9A6 have been reported to date (1–7). Here, we report on two German families with novel SLC9A6 mutations...

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ژورنال

عنوان ژورنال: Archives of Ophthalmology

سال: 2011

ISSN: 0003-9950

DOI: 10.1001/archophthalmol.2011.84