Cases reported "Nystagmus, Congenital"

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1/13. Periodic alternating nystagmus and congenital nystagmus: similarities in possibly inherited cases.

    In this paper, 2 patients, a daughter with periodic alternating nystagmus and a mother with congenital fixation nystagmus, are presented, and the similarities of the two disorders are discussed, not only in the eye movements, but also in their underlying abnormalities. The literature was reviewed, and the link between periodic alternating and congenital fixation nystagmus, which had been suggested based on circumstantial evidence, may be confirmed by our cases. The possible evidence of inheritance suggests that the two disorders share common abnormalities in visual processing.
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keywords = visual
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2/13. Needle aspiration and endodiathermy treatment of epithelial inclusion cyst of the iris.

    PURPOSE: To report a case of recurrent epithelial inclusion cyst of the iris treated with needle aspiration and endodiathermy. methods: Case report. A 27-year-old man with a history of congenital cataracts, congenital nystagmus, cataract surgery in each eye at 3 years of age, and a long-standing iris epithelial cyst presented with decreased vision of 20/400 in the better seeing left eye. The reduced vision was diagnosed secondary to growth of the iris inclusion cyst over the pupil. RESULTS: The iris cyst, which occluded the chamber angle and pupil, was treated with aspiration, separation of the cyst wall from the cornea, and endodiathermy. Twelve months after cyst surgery, no evidence existed of epithelial cyst regrowth. The visual function was significantly improved, although Snellen visual acuity was unchanged at 20/400. CONCLUSION: The minimal invasiveness of needle aspiration and diathermy can result in a satisfactory outcome without undertaking more extensive and invasive surgical treatments for epithelial inclusion cysts of the anterior chamber.
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keywords = visual
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3/13. Laser in situ keratomileusis in an eye with congenital nystagmus.

    A 38-year-old man with congenital nystagmus had laser in situ keratomileusis (LASIK) to correct a spherical equivalent of -11.0 diopters (D) in the right eye and -10.0 D in the left eye. Baseline uncorrected visual acuity (UCVA) was 20/400 in both eyes, and best spectacle-corrected visual acuity was 20/60 in the right eye and 8/20 in the left. The procedure was performed using the Aesculap Meditec MEL 60 excimer laser system and a semicircular suction ring device. Six months after LASIK, the patient had a UCVA of 20/60 in the right eye and 8/20 in the left eye, with no improvement with spectacle correction. Laser in situ keratomileusis can be performed with a semicircular suction ring device to correct refractive errors in patients with congenital nystagmus, but more cases have to be performed to confirm that the device is safe and suitable for these patients.
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ranking = 2
keywords = visual
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4/13. A girl without a chiasm: electrophysiologic and MRI evidence for the absence of crossing optic nerve fibers in a girl with a congenital nystagmus.

    An otherwise healthy 15-year-old girl with a congenital nystagmus was evaluated at our department using visual evoked potential recording and magnetic resonance imaging. She appears to have the unique isolated inborn absence of the optic chiasm, described only once before in two unrelated girls. Unlike these previously described cases, our patient does not seem to display a see-saw nystagmus.
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ranking = 1
keywords = visual
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5/13. Clinical and oculographic response to Dexedrine in a patient with rod-cone dystrophy, exotropia, and congenital aperiodic alternating nystagmus.

    PURPOSE: We report a child with retinal dystrophy and congenital (a)periodic alternating nystagmus (APAN) who responded immediately with improved visual function and electrooculographic parameters after taking the psychopharmacologic stimulant Dexedrine Spansule (Glaxo-Smith Kline, NC, USA) as part of treatment for his attention Deficit Disorder. DESIGN: Interventional case report. methods: General ophthalmic, ocular motor and sensorimotor examinations and ocular motility recordings were performed before and after administration of the drug Dexedrine Spansule. RESULTS: The patient's binocular visual acuity improved only at 1.5 after medicine hours from 20/63 to 20/50, his exotropic deviation decreased from 25 to 10 prism diopters, his stereopsis increased from none to 800 sec/arc and ocular motility recordings showed increased foveation periods and more and lengthened APAN transition/null zones. CONCLUSION: For unexplained reasons the stimulant Dexedrine "paradoxically" improved the nystagmus, binocular function and visual acuity in this patient with retinal dystrophy and congenital nystagmus. This observation may be the basis for investigation of a new pharmacological treatment approach to patients with congenital nystagmus or strabismus.
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ranking = 3
keywords = visual
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6/13. Onset of oscillopsia after visual maturation in patients with congenital nystagmus.

    PURPOSE: To describe the clinical and oculographic characteristics of a cohort of five patients with congenital nystagmus (CN) and late-onset oscillopsia caused by a coincidental decline in other visual and/or ocular motor functions. DESIGN: Retrospective, observational, case series. PARTICIPANTS: Five visually mature patients with CN and recent-onset oscillopsia were evaluated clinically and with motility recordings. INTERVENTION: Eye movement analysis was performed off-line by computer analysis of digitized data. Nystagmus was analyzed for null-zone characteristics, waveforms, frequency, amplitudes, and slow-phase drift velocity during foveation. Surgical and medical treatment of associated ocular conditions in four of five patients. MAIN OUTCOME MEASURES: Presence of symptomatic oscillopsia and average time during foveation periods of slow-phase drift velocity less than 10 degrees /second. RESULTS: One of the five patients had associated rod-cone dystrophy, and another had recurrence of childhood head posturing with return of an eccentric null zone. The remaining three patients had decompensated strabismus associated with their oscillopsia. All five patients complained of oscillopsia in primary position that was relieved in the four who received treatment. Treatment included prismatic correction in one patient and surgery in three. Recordings in primary position after treatment showed increased duration during foveation periods of slow-phase drift velocity less than 10 degrees /second and an overall decreased intensity (amplitude/frequency) of the nystagmus. CONCLUSIONS: Symptomatic oscillopsia in patients with CN is unusual. This visually disturbing symptom can be precipitated by new or changing associated visual sensory conditions (e.g., decompensating strabismus, retinal degeneration). If the associated conditions can be treated, then accompanying oscillopsia may be relieved.
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ranking = 8
keywords = visual
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7/13. Clinical and oculographic response to Tenuate Dospan (diethylpropionate) in a patient with congenital nystagmus.

    PURPOSE: We report a female adult with congenital nystagmus who responded with improved visual function and oculographic parameters after taking the anorexic diet drug Tenuate Dospan (diethylproprionate; Watson laboratories, Inc., Corona, california). methods: Observational case report. Clinical ophthalmic examination and ocular motility recordings were performed before and after administration of the drug Tenuate Dospan. RESULTS: The binocular visual acuity of the patient improved from 20/70 to 20/50, her exotropic deviation decreased from 12 to 4 prism diopters, her stereopsis increased from none to 200 seconds/arc, and her ocular motility recordings showed increased foveation periods and a broadened null zone. CONCLUSION: For unexplained reasons, the anorexic stimulant Tenuate Dospan "paradoxically" improved the nystagmus and binocular function in this patient with congenital nystagmus. This observation may be the basis for investigation of a new pharmacological treatment approach to patients with congenital nystagmus or strabismus.
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ranking = 2
keywords = visual
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8/13. Laser in situ keratomileusis in a patient with congenital nystagmus.

    We report the results of laser in situ keratomileusis (LASIK) to correct myopia in a 47-year-old woman with congenital motor nystagmus and myopia. The patient had simultaneous bilateral LASIK using the Alcon-Summit-Autonomous LADARVision excimer laser. Her uncorrected visual acuity (UCVA) preoperatively was 20/600 in the right eye and 20/800 in the left eye; best corrected visual acuity was 20/40 in both eyes. Twelve months after bilateral LASIK, with an enhancement procedure in both eyes at 4 months, UCVA was 20/40 in both eyes. corneal topography showed well-centered ablation zones.
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ranking = 2
keywords = visual
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9/13. Capsular block syndrome associated with horizontal jerk nystagmus.

    A 78-year-old cataract patient with horizontal jerk nystagmus had phacoemulsification and intraocular lens (IOL) implantation in the capsular bag with continuous curvilinear capsulorhexis. One week postoperatively, the posterior capsule ballooned posteriorly, the anterior capsule opening was sealed to the IOL optic, and a transparent liquefied substance accumulated between the lens optic and the posterior capsule. The best corrected visual acuity was 0.6 with a myopic shift compared with the refraction after the first day. A neodymium:YAG laser posterior capsulotomy was performed, and the capsular block syndrome (CBS) resolved. The results confirm the experimental model proposed by Zacharias suggesting that saccadic eye movements contribute to CBS under certain anatomic conditions.
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ranking = 1
keywords = visual
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10/13. Ocular findings in cerebro-oculo-facial-skeletal syndrome (Pena-Shokeir-II syndrome).

    PURPOSE: To report the ocular findings in cerebro-oculo-facial-skeletal syndrome or Pena-Shokeir-II syndrome. methods: Case report. RESULTS: A five-month-old male infant presented with bilateral posterior polar cataract, microphthalmos, nystagmus, and marked non-glaucomatous optic nerve atrophy. Systemic abnormalities such as microcephaly, micrognathia, flexion contractures of the elbows and knees, hypotonic musculature, and failure to thrive, with pronounced statomotor retardation, led to the diagnosis of cerebro-oculo-facial-skeletal syndrome or Pena-Shokeir-II syndrome. cataract surgery did not improve the poor visual performance. CONCLUSIONS: Cerebro-oculo-facial-skeletal syndrome (Pena-Shokeir-II syndrome) should be included in the differential diagnosis of bilateral microphthalmos, congenital cataract, nystagmus, and pronounced optic nerve atrophy, and cataract surgery does not markedly improve vision.
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keywords = visual
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