Cases reported "Strabismus"

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1/46. strabismus fixus divergens and associated craniofacial anomalies.

    A case of congenital strabismus fixus divergens is described. Other anomalies were vertically oval corneas, high mixed astigmatism, amblyopia, horizontal nystagmus, microcephaly, maxillary hypoplasia, micrognathia, low set large ears, and hyperostosis frontalis interna.
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2/46. Spontaneous reversal of nystagmus in the dark.

    AIM: To report five children with horizontal jerk nystagmus in whom eye movement recordings in the dark revealed a spontaneous reversal in the direction of the nystagmus beat. Three patients were blind in one eye and were diagnosed as having a manifest latent nystagmus (MLN), and two patients had strabismus and congenital nystagmus (CN). methods: eye movements were recorded using DC electro-oculography with simultaneous video recording, including infrared recording in total darkness. RESULTS: Four patients had decelerating velocity slow phase jerk nystagmus when recorded under natural lighting conditions; the fifth case had accelerating velocity and linear slow phase jerk nystagmus. Under absolute darkness, nystagmus reversed in direction of beat with a mixture of linear and decelerating velocity slow phase waveforms. One child with unilateral anophthalmos could wilfully reverse the beat direction of his nystagmus by trying to look with his blind eye in the light and dark. CONCLUSIONS: These observations support the theory that LN/MLN beat direction is determined by the "presumed" viewing eye and may be consciously controlled. The spontaneous reversal of beat direction in the dark suggests eye dominance is predetermined. Eye movement recordings identified mixed nystagmus waveforms indicating that CN (accelerating velocity slow phases) and LN/MLN (linear/decelerating velocity slow phases) coexist in these subjects.
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ranking = 13
keywords = nystagmus
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3/46. Two types of foveation strategy in 'latent' nystagmus: fixation, visual acuity and stability.

    The authors studied the foveation dynamics of two individuals with latent/manifest latent nystagmus (LMLN) to test the hypothesis that oscillopsia suppression and good visual acuity require periods of accurate target foveation at low slip velocities. Congenital nystagmus (CN) waveforms contain post-saccadic foveation periods; the LMLN waveform does not and yet allows for both oscillopsia suppression and good acuity. During fixation with both eyes open, there were intervals when the eyes were still and correctly aligned; at other times, there was esotropia and nystagmus with slow-phase velocities less than /- 4 deg/sec and each fast phase pointed the fovea of the fixating eye at the target. However, cover of either eye produced LN and a different strategy was employed: the fast phases carried the fixating eye past the target and the fovea subsequently reacquired it during the slowest parts of the slow phases. The authors confirmed this in both subjects, whose high acuities were made possible by foveation occuring during the low-velocity portions of their slow phases. A nystagmus foveation function (NFF), originally developed for CN, was calculated for both LN and MLN intervals of fixation and it was found to track visual acuity less accurately for individuals with high acuity. Individuals with LMLN exhibit two different foveation strategies: during low-amplitude LMLN, the target is foveated immediately after the fast phases; and during high-amplitude LMLN, target foveation occurs towards the end of the slow phases. Therefore, the saccadic system can be used to create retinal error rather than eliminate it if this strategy is beneficial. Individuals with LMLN foveated targets with the same eye-position and -velocity accuracy as those with CN and the NFF provides a rough estimate of acuity in both. Current calibration methods for both infrared and search-coil techniques need to be altered for subjects with LMLN.
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keywords = nystagmus
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4/46. Ocular tilt reaction due to a mesencephalic lesion in juvenile polyarteritis nodosa.

    PURPOSE: To describe a case of ocular tilt reaction caused by vasculitic lesions in the midbrain in a child with polyarteritis nodosa. DESIGN: Observational case report. methods: A 5-year-old girl with a chronic illness developed diplopia associated with a left head tilt, right hypertropia, torsional nystagmus, slowed vertical saccades and poor convergence. Fundoscopic examination demonstrated conjugate leftward torsion of the eyes consistent with a sustained ocular tilt reaction.Renal angiography confirmed polyarteritis nodosa and cerebral magnetic resonance imaging demonstrated mesencephalic pathology. CONCLUSIONS: polyarteritis nodosa is a difficult condition to diagnose in a child and can cause brainstem lesions. This rare case of ocular tilt reaction of midbrain origin highlights that a sustained head tilt in a child can be due to brainstem pathology, rather than a fourth nerve palsy.
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keywords = nystagmus
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5/46. Uncommon ocular changes in Down's syndrome (mongolism).

    A one-year-old White female mongoloid child was seen with typical palpebral fissures, esotropia, myopic astigmatism, and nystagmus. Additional uncommon ocular findings included ganglionic neuroretinal hypoplasia (hypoplasia of the optic nerve) and bilateral congenital impatency of the nasolacrimal duct. An active search for these ocular changes is recommended in cases of Down's syndrome.
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6/46. Eight eye muscle surgical treatment in a patient with acquired nystagmus and strabismus: a case report.

    The purpose of this paper is to outline the clinical benefits and electrophysiological changes after extraocular muscle surgery for acquired nystagmus and strabismus in an adult patient. a 41-year-old Caucasian female suffered from oscillopsia, bilateral cranial nerve IV palsies, and vertical nystagmus. Medical therapy was ineffective. She underwent bilateral, eight muscle surgical procedure which included: tenectomies of the superior oblique OU, myectomies of the inferior oblique OU, recessions of the medial rectus muscles OU (with OD on a post-operatively adjustable suture), and differential recessions of the superior rectus muscles OU. This provided marked improvement in her symptoms. These results suggest that surgery for acquired nystagmus can affect an improvement in the characteristics of the nystagmus that result in visual complaints. Combining "nystagmus surgery" plus strabismus surgery may be a viable option in some adult patients with acquired nystagmus who are unresponsive to medical therapy.
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ranking = 10
keywords = nystagmus
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7/46. arnold-chiari malformation.

    arnold-chiari malformation is a congenital malformation of the skull near the foramen magnum in which the cerebellum and the medulla are caudally displaced. This herniation of the brainstem causes down-beat nystagmus and oscillopsia, the most commonly presenting sign and symptom, respectively. Differential diagnoses for the arnold-chiari malformation include, but are not limited to, demyelinating disease, tumor, and vascular disorders. Symptoms will generally worsen with time and may even be brought on during exercise or valsalva maneuvers. A correct diagnosis can lead to timely surgical intervention which can improve the quality of eye movements. Treatment generally involves the surgical decompression of the surrounding spinal tissue.
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ranking = 1
keywords = nystagmus
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8/46. Skew deviation: a precursor to basilar artery thrombosis.

    The aim of this study was to describe skew deviation and vertical nystagmus as the initial signs for basilar artery thrombosis, a life-threatening disease. A 51-year-old woman complained of vertical diplopia for more than 20 h. A computed tomography of the brain was normal, but subsequently the patient developed additional symptoms including nausea, ventilation problems (dyspnoea) and somnolence. Neuro-ophthalmological evaluation revealed a skew deviation and a vertical nystagmus. magnetic resonance imaging allowed the diagnosis of basilar artery occlusion. An emergency intervention with cerebral catheter angiography and local intra-arterial thrombolysis was performed. Total recanalization of the basilar artery was achieved resulting in a complete neurological recovery, including the skew deviation and nystagmus. This rare case of skew deviation associated with basilar artery occlusion was a diagnostic challenge and highlights adequate differential diagnosis. Skew deviation is an important clinical sign. In this patient it was the key to a correct diagnosis enabling an immediate and successful intervention.
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ranking = 3
keywords = nystagmus
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9/46. Chiari 1 malformation presenting as strabismus.

    INTRODUCTION: strabismus has been previously reported as a rare presenting feature of the Type 1 Chiari malformation. case reports: We report a case series of twelve patients with Chiari 1 malformations with either strabismus or diplopia as part of their initial presentation. Ten patients had diplopia at the time of presentation, while 2 young children (ages 2 and 6) presented with esotropia without complaints of diplopia. Of the 10 patients with diplopia, 7 were constantly or frequently tropic while 3 had symptomatic phorias. One or more unusual features of the strabismus led to further investigations and the diagnosis of Chiari in these patients. The most common oculomotor disturbance was a comitant esotropia. Most patients were managed with prism glasses. One patient had strabismus surgery as primary treatment with early orthotropia. Three patients underwent neurosurgical decompression, with minimal improvement of their strabismus; one of these underwent subsequent successful strabismus surgery. CONCLUSIONS: Chiari 1 malformation may present with strabismus or diplopia as the major finding. Associated neurological features may be nonspecific (e.g., headache), subtle (e.g., gaze-evoked nystagmus), or delayed. Although neurosurgery may be required in some cases, primary strabismus management (surgical or prismatic correction) can be successful, particularly when strabismus is the lone (or sole specific) finding.
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ranking = 1
keywords = nystagmus
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10/46. Mietens-Weber syndrome: two new patients and a review.

    In 1966, Mietens and Weber reported four out of six siblings from a consanguineous couple with growth failure, dislocation of the head of the radii, bilateral flexion contracture of the elbows, short ulnae and radii, bilateral corneal opacities, horizontal and rotational nystagmus, strabismus, small, pointed nose and mild to moderate mental retardation. Since then, only three other cases have been reported. We report on two new cases, a pair of female twins aged 9 years. The patients were born after an uneventful, normal pregnancy, to young and non-consanguineous parents. After birth, physical findings included horizontal nystagmus and dislocation of both elbows because of abnormally short radii and ulnae in both twins. Further clinical examinations showed moderate psychomotor delay with marked language compromise. Karyotypes were normal in both girls. A review of the literature reveals that the Mietens-Weber syndrome is an uncommon disorder with a probable autosomal recessive pattern of inheritance. To our best knowledge, including the two cases reported here, only nine cases have been observed so far. The finding of congenital nystagmus and radii dislocation in a patient with mental retardation is probably nonrandom and is highly suggestive of Mietens-Weber syndrome.
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ranking = 3
keywords = nystagmus
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