Cases reported "Nystagmus, Pathologic"

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11/33. Ocular manifestations in Fukuyama type congenital muscular dystrophy.

    We describe a child with Fukuyama type congenital muscular dystrophy (FCMD) who had several ocular manifestations since birth. These included high myopia, strabismus, nystagmus and optic atrophy of both eyes. Later he developed retinal detachment of both eyes. Our survey of 33 cases with FCMD revealed that high myopia and optic atrophy are common in FCMD. retinal detachment was reported in two cases in addition to the present one and was considered to be of developmental origin. The association of congenital muscular dystrophy with brain changes and ocular anomalies were found in FCMD, muscle, eye and brain disease (MEB) and walker-warburg syndrome (WWS). Ocular manifestations in FCMD were, in general, less severe than those in WWS or MEB. Our study suggests that FCMD, MEB and WWS are developmental abnormalities with a continual spectrum of disease severity.
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ranking = 1
keywords = strabismus
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12/33. Treatment of manifest latent nystagmus.

    Eight patients with manifest latent nystagmus, as noted by ocular movement recordings, were examined for nystagmus reduction after surgical or optical treatment. Seven of the patients had strabismus. Five patients underwent strabismus surgery, had no tropia postoperatively, and the manifest latent nystagmus converted to latent nystagmus. Four of these five patients subsequently showed improvement in binocular visual acuity. Three patients received optical treatment; one had accommodative esotropia and, with appropriate spectacle correction, the manifest latent nystagmus was converted to latent nystagmus with improved vision. In the other two patients the manifest latent nystagmus lessened after correction with appropriate spectacles; binocular visual acuity of one of these patients improved. The possibility of converting manifest latent nystagmus to latent nystagmus by strabismus surgery is a reasonable surgical goal. In patients with manifest latent nystagmus and strabismus, surgical or optical alignment of the eyes decreases the nystagmus intensity and may also improve binocular visual acuity.
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ranking = 4
keywords = strabismus
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13/33. Congenital adduction palsy and synergistic divergence: a clinical and electro-oculographic study.

    We studied two patients with a peculiar congenital disturbance of ocular motility in which the horizontal movements of the left eye were always opposite the normal expected direction. The common features were: (1) congenital monocular adduction palsy and exotropia of the left eye; (2) simultaneous abduction of both eyes (divergence) on attempted dextroversion; (3) ocular torticollis, head turned to the right; and (4) inverse nystagmus of the left eye, occurring spontaneously as well as during optokinetic and vestibular testing. Clinical and electrooculographic findings suggested a close relationship to Duane's retraction syndrome and supported the concept that innervational mechanisms were responsible for the phenomenon.
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ranking = 1.2266028719492
keywords = exotropia
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14/33. Paradoxic pupillary phenomena. A review of patients with pupillary constriction to darkness.

    The presence of pupillary constriction to darkness is said to be a useful indicator of retinal disease. However, it is also associated with optic nerve disease. The phenomenon has been reported in patients with congenital stationary nightblindness, congenital achromatopsia, bilateral optic neuritis, and dominant optic atrophy. We have observed this response in additional disorders, including anomalies of the optic nerve development, congenital nystagmus, and a variety of diseases affecting the retina. Notably, four of our patients with strabismus and amblyopia, but without apparent retinal or optic nerve disease, have also demonstrated this paradoxic pupil response. While the mechanism for pupillary constriction to darkness remains unclear, the finding of this response in patients without retinal or optic nerve abnormalities questions its value as a localizing sign.
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ranking = 1
keywords = strabismus
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15/33. Overgrowth, congenital hypotonia, nystagmus, strabismus, and mental retardation: variant of dominantly inherited Sotos sequence?

    We report on 2 patients with macrocephaly, strabismus, esotropia, nystagmus, hypotonia, developmental delay, excessive size, unusual facial appearance, and improvement with age. Many of these abnormalities are present in Sotos sequence. The mothers of both patients share some characteristics with their children. These patients may represent an autosomal dominant form of Sotos sequence.
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ranking = 5
keywords = strabismus
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16/33. Acquired esotropia as initial manifestation of arnold-chiari malformation.

    A 13-year-old patient originally presented with a divergence palsy and gaze-evoked nystagmus. Over a short period of time, the esotropia became increasingly comitant and was successfully treated with strabismus surgery. Three years later, she developed downbeat nystagmus. An arnold-chiari malformation could only be demonstrated using nuclear magnetic resonance imaging (MRI). Subsequent neurosurgical decompression resulted in resolution of the downbeat nystagmus with maintenance of single binocularity.
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ranking = 1
keywords = strabismus
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17/33. Modification of the Kestenbaum operation for correction of nystagmic torticollis and improvement of visual acuity with the use of convergence.

    In five patients with congenital nystagmus and compensatory head turn we performed a modified Kestenbaum operation intended to correct the compensatory head turn and to improve visual acuity. All patients had nystagmus without strabismus and fully developed binocular function. The nystagmus decreased but did not cease completely during the lateral gaze, and the patients adopted compensatory head turn positions to improve their visual acuity. The near acuity was better than the distance and the head turn decreased on near fixation. In such cases the original Kestenbaum procedure transfers the neutral zone to the primary position and corrects the head turn, but does not improve the visual acuity because a considerable amount of nystagmus is still present. Our aim was to perform an asymmetric Kestenbaum operation with a greater amount of surgery to the eye that turns out and a lesser amount to the eye that turns in, thus creating a postoperative divergence of the eyes. The result of compensating for this divergence (through stimulation of the fusional reserve) blocks the residual nystagmus of the neutral zone and, consequently, improves visual acuity. The results obtained from performing this modified Kestenbaum operation in five patients were very satisfactory. The compensatory head turn was corrected in all patients and remained so during the follow-up period. In four cases, the postoperative visual acuity in the neutral zone was better than before. There was no improvement in one case.
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ranking = 1
keywords = strabismus
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18/33. Normalization of binocular VERs after early onset visual deprivation in man.

    Visually evoked responses (VERs) were elicited by a reversing checkerboard target from a patient who suffered early onset bilateral deprivation of form vision as a result of a high refractive error, large amplitude strabismus, and congenital nystagmus. Monocular and binocular steady-state VERs were abnormal in amplitude and wave form even when a correction for the patient's large refractive error (compound hyperopic astigmatism) was worn. Although monocular VERs could not be normalized, a normal wave form and amplitude were restored to the binocular VER by the addition of horizontal prisms to the patient's ophthalmic prescription. The initially degraded binocular VER gradually acquired a normal morphology and amplitude as the magnitude of compensating base-out prisms in the patient's habitual ophthalmic prescription was systematically increased. The relationship between the binocular VER amplitude and the correcting prisms derived by the method described in this paper was subsequently used to arrive at a practical clinical solution for the patient's unusual and debilitating visual symptoms. This electrophysiological evaluation of binocular function at the cortical level proved to be a very useful diagnostic procedure with prognostic value; standard clinic procedures were ineffective in elaborating the patient's sensory and oculomotor disorders. The theoretical and practical implications of managing patients with a history of early onset visual deprivation are discussed.
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ranking = 1
keywords = strabismus
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19/33. Internuclear ophthalmoplegia associated with ipsilateral downbeat nystagmus and contralateral incyclorotatory nystagmus.

    A case of left internuclear ophthalmoplegia combined with downbeat nystagmus and right incyclorotatory nystagmus on left downward gaze, left exotropia and skew deviation is presented. All signs subsided completely within about 3 months after the onset of the disease. The etiology was possibly of vascular origin. The potential site of the lesion causing these oculomotor signs is discussed.
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ranking = 1.2266028719492
keywords = exotropia
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20/33. Atypical sensory nystagmus and its surgical management.

    Two patients with oculocutaneous albinism who had presumed sensory-defect nystagmus also showed the features of congenital motor-defect nystagmus. Both patients used an abnormal head posture to block the nystagmus, and each had coexisting strabismus. Modern surgical techniques simultaneously shifted the null point of the nystagmus to the primary position and corrected the strabismus.
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ranking = 2
keywords = strabismus
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