Cases reported "Nystagmus, Pathologic"

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21/33. Inidcations of the posterior fixation operation in strabismus.

    Retroequatorial fix-tion of a rectus muscle by means of nonabsorbable scleral sutures weakens this muscle in its primary field of action without disturbing the balance with its antagonist in other positions of gaze. This procedure has been employed in patients with dissociated vertical deviations, nystagmus compensation (blockage) syndrome, double-elevator paresis, nonaccommodative convergence excess, and Duane's retraction syndrome. Complications were not encountered, but the operation was ineffective in some patients and reoperation became necessary.
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ranking = 1
keywords = strabismus
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22/33. Leigh's subacute necrotizing encephalomyelopathy manifesting as spasmus nutans.

    Subacute necrotizing encephalomyelopathy (SNE), or Leigh's disease, is an unusual neurologic disorder that is associated with nystagmus, strabismus, and optic atrophy in the majority of cases, and is most often described in infants. The defect currently thought to account for SNE is the presence of an inhibitor factor in the thiamine pathway. We saw a patient whose eye findings initially were believed to represent spasmus nutans, but whose clinical course, computed tomographic findings, and autopsy findings were typical of SNE.
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ranking = 0.25
keywords = strabismus
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23/33. X-linked recessive congenital stationary night blindness, myopia, and tilted discs.

    A family with X-linked recessive congenital stationary night blindness, myopia, and tilted discs has been identified. All affected members have decreased vision, nystagmus, strabismus with decreased binocular function, visual field defects, abnormal fundus appearance with typical fluorescein angiographic findings of tilted disc syndrome, abnormal electroretinograms, and abnormal visual evoked responses to patterned stimuli. Similar clinical evaluation of an obligate carrier revealed no ocular abnormalities.
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ranking = 0.25
keywords = strabismus
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24/33. See-saw nystagmus after strabismus surgery.

    A 7-year-old child underwent craniotomy for a craniopharyngioma. One month later she developed a divergence excess type exotropia and had strabismus surgery 11 months later. The day after strabismus surgery, the parents noted abnormal eye movements that represented see-saw nystagmus. The patient had a small esotropia after surgery, but it was found that stimulated convergence controlled the nystagmus. This case illustrates the dual central and ocular influence on the development of see-saw nystagmus.
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ranking = 1.8066507179873
keywords = exotropia, strabismus
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25/33. Infantile presentation of X linked retinoschisis.

    Five infants who presented with nystagmus and/or strabismus were found to have bilateral highly elevated bullous retinoschisis involving the macula. Haemorrhage was present within the schisis cavity or the vitreous in four patients. The bullous retinoschisis eventually reattached spontaneously leaving pigment demarcation lines. A family history of X linked retinoschisis (XLRS) was known in two of the patients but in the other three subsequent investigation showed other male family members to be affected. It is important to recognise this uncommon presentation of XLRS so that the correct diagnosis is made and appropriate genetic counselling is given. Surgical treatment is not usually indicated and the visual prognosis is better than the initial appearance may suggest.
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ranking = 0.25
keywords = strabismus
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26/33. Eye signs in craniopharyngioma.

    A total of 45 patients with craniopharyngioma are reviewed, with particular reference to the presenting clinical features and neuro-ophthalmological findings. Of these 50 per cent had been referred in the first instance to an eye clinic. More than half the children presented with the clinical picture of raised intracranial pressure, although one-third of these had optic atrophy rather than papilloedema. One-third of the children had a history of concomitant strabismus, and only one patient had a paretic squint. The majority of the adults presented with visual failure and optic atrophy. Bitemporal hemianopia was fairly frequently found (27 per cent of our patients at the time of diagnosis) but was asymmetrical and unpredictable in its evolution. Homonymous hemianopia was relatively common, a presenting feature in II per cent of patients in this study. Full fields were found initially in nine patients (20 per cent), a high incidence compared with pituitary adenomas or suprasellar meningiomas. We consider pleomorphism, that is a distinct change from one type of field defect to another with progress of the disease, to be a characteristic feature of the tumour (as indeed are fluctuations in the clinical state and visual acuity); it was detected in 22 per cent of our patients. Endocrine disorders were common, especially in adults, and mental deterioration was a frequent presenting feature in patients over 30 years of age. Straight x rays of the skull are practically diagnostic of craniopharyngioma in children, but in adults a normal x ray does not exclude the diagnosis.
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ranking = 0.25
keywords = strabismus
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27/33. The ophthalmologic manifestations of the cardio-facio-cutaneous syndrome.

    The cardio-facio-cutaneous (CFC) syndrome is an uncommon multiple congenital anomalies/mental retardation syndrome whose major manifestations are congenital heart defects, relative macrocephaly, stunted growth, ectodermal dysplasia, characteristic facial appearance, and psychomotor developmental delay. All described cases were sporadic and cytogenetically normal. We report three additional patients with this diagnosis. All three patients developed strabismus, requiring extraocular muscle surgery. Two of our patients also had nystagmus. Combining the 18 previously reported cases with our additional 3, 9 of 21 had strabismus, 10 of 21 had ptosis, and 6 of 21 had nystagmus. Ophthalmic craniofacial abnormalities of hypoplastic supraorbital ridges, prominent epicanthal folds, and antimongoloid slant of the palpebral fissures were also common features contributing to the characteristic facies described for this syndrome. The ophthalmologist could aid in the diagnosis and treatment of these patients because of the prominent ophthalmologic symptomatology of the CFC syndrome.
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ranking = 0.5
keywords = strabismus
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28/33. Latent nystagmus. Release and suppression at will.

    PURPOSE. The authors report the cases two unusual patients with infantile convergent strabismus and latent nystagmus. methods. electronystagmography was used. RESULTS. The two patients were able to release and suppress their nystagmus at will. With voluntary effort, the nystagmus became as strong as it was when brought out by occlusion of the squinting eye and, in one of the patients, even stronger. The nystagmus beat toward the fixing eye, and the slow phases had slightly decreasing velocity or were linear. Both patients were able to evoke and to stop the nystagmus in front of visual contours and, one of them, also in the dark. CONCLUSIONS. This phenomenon could be the result of voluntary control of the visual input contributed by the amblyopic eye and/or a direct influence of will on the slow eye movement and fixation systems. A mechanism related to vergence eye movements is less likely. The ability to release and suppress a latent nystagmus at will is unusual and, to the authors' knowledge, has not been described before.
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ranking = 0.25
keywords = strabismus
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29/33. diagnosis of oculocutaneous albinism with molecular analysis.

    PURPOSE: To use molecular analysis to diagnose oculocutaneous albinism in a patient with an atypical clinical presentation. methods: A 34-year-old woman with a history of strabismus and absent cutaneous pigment underwent comprehensive ophthalmic examination, visual-evoked potentials to detect altered optic decussation, and molecular analysis. RESULTS: Examination showed fine nystagmus, iris transillumination, foveal hypoplasia, and corrected visual acuity of 20/25 in each eye. Misrouting of the retinostriate fibers was demonstrated with visual-evoked potentials. Mutations in the tyrosinase gene established the diagnosis of oculocutaneous albinism 1 even though the patient had atypical clinical features. CONCLUSIONS: Molecular analysis can establish the diagnosis of oculocutaneous albinism 1 in the patient with atypical ocular features.
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ranking = 0.25
keywords = strabismus
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30/33. Meitens' syndrome.

    This paper reports on Mietens' syndrome which is characterized by mental retardation, growth failure, flexion contracture of the elbows, dislocation of the radius, abnormally short ulna and radius, bilateral corneal opacity, horizontal and rotational nystagmus, strabismus and a small pointed nose with depressed root. This is the second on the Mietens' syndrome.
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ranking = 0.25
keywords = strabismus
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