Cases reported "Nystagmus, Congenital"

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1/2. 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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keywords = rod-cone dystrophy, rod-cone, dystrophy
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2/2. 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 = 0.24994645029323
keywords = rod-cone dystrophy, rod-cone, dystrophy
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