scholarly journals Lack of Torsion in the Type-III Duane Retraction Syndrome (DRS-III) Affected Left Eye During Dix -Hallpike Test

Author(s):  
Ajay Kumar Vats

Abstract Background Duane retraction syndrome (DRS) is a congenital cranial dysinnervation disorder (CCDD) of ocular movements, characterized by deficits in horizontal duction associated with narrowing of palpebral fissure, retraction of eye globe on attempted adduction and occasionally accompanied by upshoot or downshoot of the eye globe. It is caused by congenital absence of sixth cranial nerve, which results in fibrotic changes in the extraocular muscles leading to an abnormal ocular motility--a concept known as CCDD. Depending on whether only abduction/adduction or both are affected, DRS has been classified into three types designated as type-I, type-II and type-III. The torsional movements of the affected eye in DRS have not been reported to be deficient hitherto, which could be due to difficulties in the routine bedside evaluation of such movements. Case Presentation An unusual case of a patient of left unilateral type-III DRS is reported, who presented with a short history of vertigo on getting up from supine to sitting position and on assuming right lateral recumbent position. The diagnostic right Dix-Hallpike test (DHT) revealed upbeating torsional geotropic positioning nystagmus in the normal right eye and upbeating positioning nystagmus without torsional component in the abnormal left eye and this clinical finding was video recorded. Conclusion The observed lack of incyclotorsion of the left eye, affected with DRS-III during right Dix-Hallpike positioning, is primarily due to the absence of initial slow-phase excyclotorsional component. If the slow phase of VOR does not occur, then the fast-phase VOR, which is a refixation saccade, will be lacking too. An anastomosis, either in the lateral wall of the cavernous sinus or within the orbit, between the trochlear nerve and fibers of the oculomotor nerve can lead to simultaneous co-contraction of the inferior and superior oblique muscles. This is the most probable explanation for such finding of asymmetrical absence of torsional component in the left eye affected by DRS-III, during right Dix -Hallpike positioning. Thus, the recording of eye movements (voluntary and involuntary) opened a window into the brain to conceptualize neural and mechanical factors influencing the human eye movements.

Author(s):  
Ayse Gul Kocak Altintas ◽  
Ayse Gul Kocak Altintas

Duane retraction syndrome is the most frequently seen restrictive ocular motility disorders. It is clinically presented with limitation of horizontal movement, variable amounts of upshoots or downshoots and globe retraction combined with narrowing of the palpebral aperture on attempted adduction. An 8-year-old patient presented with severe restriction of abduction, reciprocal upshots or downshoots, and globe retraction combined with the palpebral fissure narrowing of on adduction. After the modified Y splitting of LR and recession of both horizontal rectus operation, all cosmetically disfiguring clinical features disappeared. In this case report modified Y splitting procedure and its long-term efficacy is presented.


2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
A Gurgu ◽  
L Petrescu ◽  
C Vacarescu ◽  
CT Luca ◽  
C Mornos ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Background CRT improves both systolic and diastolic function, thus increasing cardiac output. However, less data is available concerning diastolic dyssynchrony and fusion pacing CRT. The aim of our study was to assess the outcome of LV diastolic asynchrony in a population of fusion pacing CRT without right ventricular (RV)  lead. Methods Prospective data were collected from a cohort of patients (pts) with right atrium/left ventricle leads (RA/LV CRT). Baseline and every 6 months follow-up included standard ETT and classical dyssynchrony parameter measurements. Diastolic dyssynchrony was done by offline speckle-tracking derived TDI timing assesment of the simultaneity of E" and A"  basal septal and lateral wall 4 chamber view. New parameters were introduced: E" and respectively A" time (E"T / A"T) as the time difference between E" (respectively A" ) peaks septal and lateral wall. Exercise tests, drugs optimization and device individual programmimg were systematically performed in order to maintain constant fusion and improve CRT response. Patients were divided in three groups: super-responders (SR), responders (R) and non responders (NR). Results Sixty-two pts (35 male) aged 62 ± 11 y.o. with idiopathic DCM implanted with a RA/LV CRT were analyzed: 34%SR / 61%R / 5%NR. Baseline initial characteristics: QRS 164 ± 18 ms; EF 27 ± 5.2; 29% had type III diastolic dysfunction (DD), 63% type II DD, 8% type I DD. Average follow-up was 45 ± 19 months; mean LVEF at the last follow-up was 37 ± 7.9%. The E"T decreased from 90 ± 20 ms to 25 ± 10 ms in SR with significant LV reverse remodelling (LV end-diastolic volume 193.7 ± 81 vs 243.2 ± 82 ml at baseline, p < 0.0028) and lower LV filling pressures (E/E" 13.2 ± 4.6 vs 11.4 ± 4.5, p =0.0295). DD profile improved in 65% of R with a reduction in E/A ratio (1.46 ± 5.3 vs. 0.82 ± 3.9 at baseline, p= 0.4453). Non-sudden cardiac death occurred in 3 NR pts (2%) with type III DD, severe LA volume and larger E" T /A"T (E"T> 85 msec A"T > 30 msec).  Significant cut off value calculated by ROC curve for LV diastolic dyssynchrony is E"T > 80 ms and A"T of > 25 msec. Conclusions Fusion pacing CRT without RV lead showed a positive outcome; improving LV diastolic dyssynchrony in responders and super-responders patients is obvious. Larger randomized studies are needed to define the role of diastolic asynchronism as a predictor of favorable response in fusion pacing. Abstract Figure. Typical TDI patterns in LV fusion pacing


2010 ◽  
Vol 77 (5) ◽  
pp. 499-502 ◽  
Author(s):  
F Bayrakli ◽  
K Bilguvar ◽  
D Ceyhan ◽  
AG Ercan-Sencicek ◽  
T Cankaya ◽  
...  

2018 ◽  
Vol 10 (1) ◽  
pp. 90-93
Author(s):  
Harsha Pagad ◽  
Pooja Ramnani ◽  
Sumita Karandikar ◽  
Archana Tadwalkar ◽  
Nita Shanbhag ◽  
...  

Background: Duane's retraction Syndrome is a congenital form of strabismus characterized by horizontal eye movement limitation and globe retraction with palpebral fissure narrowing in attempted adduction. It may be associated with co-existing ocular and systemic pathologies. Crocodile tears, or a paradoxical gustatory lacrimal reflex can be either congenital or acquired. The congenital cases typically are associated with Duane’s syndrome in most of the cases.Case: We present in our case report, uncommon case of bilateral Duane’s syndrome with bilateral crocodile tears - both of which can be assumed to be of probably central origin. Though several cases have been reported, there are very few from the Indian subcontinent. Here, we also review the literature of the syndrome with crocodile tears.Conclusion: All cases of Duane’s retraction syndrome warrant a thorough screening for coexisting ocular and systemic abnormalities. Ours is a classic report of bilateral Duane’s syndrome with bilateral congenital crocodile tears, the coexistence of these two anomalies emphasising the aetiology to be anomalous innervation occurring at central level i.e. is brain stem.


2019 ◽  
Vol 121 (5) ◽  
pp. 1865-1878 ◽  
Author(s):  
A. M. Pastor ◽  
P. M. Calvo ◽  
R. R. de la Cruz ◽  
R. Baker ◽  
H. Straka

Computational capability and connectivity are key elements for understanding how central vestibular neurons contribute to gaze-stabilizing eye movements during self-motion. In the well-characterized and segmentally distributed hindbrain oculomotor network of goldfish, we determined afferent and efferent connections along with discharge patterns of descending octaval nucleus (DO) neurons during different eye motions. Based on activity correlated with horizontal eye and head movements, DO neurons were categorized into two complementary groups that either increased discharge during both contraversive (type II) eye (e) and ipsiversive (type I) head (h) movements (eIIhI) or vice versa (eIhII). Matching time courses of slow-phase eye velocity and corresponding firing rates during prolonged visual and head rotation suggested direct causality in generating extraocular motor commands. The axons of the dominant eIIhI subgroup projected either ipsi- or contralaterally and terminated in the abducens nucleus, Area II, and Area I with additional recurrent collaterals of ipsilaterally projecting neurons within the parent nucleus. Distinct feedforward commissural pathways between bilateral DO neurons likely contribute to the generation of eye velocity signals in eIhII cells. The shared contribution of DO and Area II neurons to eye velocity storage likely represents an ancestral condition in goldfish that is clearly at variance with the task separation between mammalian medial vestibular and prepositus hypoglossi neurons. This difference in signal processing between fish and mammals might correlate with a larger repertoire of visuo-vestibular-driven eye movements in the latter species that potentially required a shift in sensitivity and connectivity within the hindbrain-cerebello-oculomotor network. NEW & NOTEWORTHY We describe the structure and function of neurons within the goldfish descending octaval nucleus. Our findings indicate that eye and head velocity signals are processed by vestibular and Area II velocity storage integrator circuitries whereas the velocity-to-position Area I neural integrator generates eye position solely. This ancestral condition differs from that of mammals, in which vestibular neurons generally lack eye position signals that are processed and stored within the nucleus prepositus hypoglossi.


2012 ◽  
Vol 52 (185) ◽  
Author(s):  
U D Shrestha ◽  
S Adhikari

Goldenhar syndrome (GHS) is also known as Oculo-Auriculo-Vertebral (OAV) syndrome or Branchial arch syndrome. Duane retraction syndrome (DRS) is a congenital disorder of ocular motility characterized by limited abduction, adduction or both. It is unilateral in 80% of cases. The important and interesting part of this eight months old child is presence of GHS with DRS. She has bilateral invol-vement, which is seen in only 5-8% of GHS, as compared to high incidence of unilateral involve-ment. This child also had refractive error of + 6.00/ - 1.5 * 180. At four year of age her vision with glass was 6/9. Children with GHS and DRS should have early eye examination done to treat the problem of refractive error. Keywords: Duane retraction syndrome; goldenhar syndrome, refractive error.


2012 ◽  
Vol 59 (3) ◽  
pp. 77-80
Author(s):  
Milorad Ljutica ◽  
Miroslav Stamenkovic ◽  
Dusica Risovic ◽  
Nebojsa Nikitovic ◽  
Branko Stankov

Objective: To describe the results of asymmetric bilateral medial rectus retroposition in unilateral Duane retraction syndrome-this type I. Method: This is a retrospective study involving 18 patients, mean age 11.2 years (3-37 years) with unilateral Duane retraction syndrome?s type who were operated in 2009 up to 2011 year. The aim was to investigate the existence of ocular torticollis, size deviation before and after surgery and presence of amblyopia, anisometropia, type of refractive errors and lateralization. Results: All 18 patients were surgically treated by asymmetric retroposition of internal rectus (for 1-2mm more on the healthy eye). All patients preoperatively de-monstrated ocular torticollis. Size of mean preoperative deviation was +22.2 pD of primary angle PD and +46 pD of secondary angle. Postoperative finding: deviation of> +10 PD is noted to the presence of mild torticollis in one patient. Intermediate follow-up period was 9.8 months. Unilateral Duane?s syndrome is common among members of women (67%) compared to males (33%). Lateralization of Duane?s syndrome is more common in the left eye compared to the right (83% vs. 17%). Amblyopia was present in two patients and anisometropia (11.1%). As for refractive errors: hyperopia was found in 9 patients (50%), hypermetric astigmatism in 7 cases (39%), myopia and 1 (5.5%) and emmetropia (5.5%). None of the patients had no pathological changes in the fundus and anterior segment. Conclusion: Asymmetric bilateral medial rectus retroposition is extremely effective in the treatment of ocular torticollis and esotropia in patients with unilateral Duane retraction syndrome-this type I.


2019 ◽  
Vol 78 (1) ◽  
Author(s):  
Débora Guimarães Resende ◽  
Daniele Foussalussa Nespolo ◽  
Lorrana Luysse dos Anjos Assis ◽  
Breno Resende Rodrigues da Cunha

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