Cases reported "Ocular Motility Disorders"

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1/30. Acquired Pseudo-Brown's syndrome immediately following Ahmed valve glaucoma implant.

    A 76-year-old woman noted vertical and horizontal diplopia one day following placement of an Ahmed valve in the superonasal quadrant of her left eye. She was unable to elevate her left eye, especially in adduction. She refused implant removal and strabismus surgery alone failed to satisfactorily resolve her problem. On forced duction testing, the implant became wedged between the globe and orbit superonasally. Subsequent repositioning of the valve resulted in resolution of her motility problem. Implant-orbital disproportion can produce a pseudo-Brown's syndrome. Surgeons are encouraged to perform forced duction testing at the time of glaucoma implant placement to detect and prevent this complication.
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keywords = strabismus
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2/30. A case of paroxysmal tonic upgaze of childhood with ataxia.

    Paroxysmal tonic upgaze of childhood is a rare, distinctive, childhood syndrome that may be associated with ataxia and sometimes strabismus or amblyopia. Neurological examination as well as metabolic studies, electroencephalogram and neuroradiological investigations are normal in these patients. Although it has been considered as an age-related, dopa-sensitive dystonia, the exact pathogenetic mechanism is still unknown. Aggravation of attacks by fatigue, intercurrent infection or vaccination, and possible corticomesencephalic dysmaturation may underlie this abnormality. We report on a sporadic case of paroxysmal tonic upgaze with ataxia in which there was prompt aggravation of symptoms with sleep without response to levodopa treatment. This case suggests a different underlying pathogenetic mechanism from dopaminergic pathways for this syndrome.
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keywords = strabismus
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3/30. Treatment of subtotal medial rectus myectomy complicating functional endoscopic sinus surgery.

    During the past 2 decades, the introduction of functional endoscopic sinus surgery (FESS) has dramatically improved the treatment of sinus disorders. However, a variety of orbital complications have been reported, including optic nerve damage, hemorrhage, infection, compromise of the lacrimal drainage apparatus, and strabismus. At least 10 cases have reported damage to the medial rectus muscle. (1-8) Treatment options for such patients have been limited, especially because most are adults at risk for anterior segment ischemia after transposition of vertical rectus muscles. We describe 2 patients whose medial rectus myectomies were repaired by using nonabsorbable "hang-back" sutures in combination with a botulinum toxin (Botox) injection of the antagonist lateral rectus muscle. Good primary position alignment was achieved in both patients, and one patient was able to regain binocular function. We recommend this surgical approach, especially in patients at increased risk for anterior segment ischemia.
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ranking = 1
keywords = strabismus
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4/30. Active management in patients with ocular manifestations of myasthenia gravis.

    PURPOSE: myasthenia gravis can cause variable strabismus with disabling diplopia and/or poor cosmesis. A retrospective study of a group of patients with myasthenia gravis or 'myasthenia gravis like' syndromes was made. methods: The study group consisted of patients who had undergone botulinum toxin treatment and/or surgery for disabling diplopia, poor cosmesis or both. Surgical treatment was by conventional techniques including recess/resect, posterior fixation, superior oblique tenotomy and adjustable sutures. RESULTS: There were 9 patients in the study group (8 female, 1 male). Age at surgery ranged from 21 to 59 years (mean 46 years). Six were symptom-free following treatment. Two, although symptomatically improved, had occasional diplopia. One patient failed treatment and required an occlusive contact lens. CONCLUSIONS: The ocular manifestations of myasthenia gravis or 'myasthenia gravis like' syndromes may respond to surgery and/or botulinum toxin injection. Active intervention should be considered when deviations become stable. To our knowledge this is the first report of the use of botulinum toxin in such patients.
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ranking = 1
keywords = strabismus
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5/30. 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 = 2
keywords = strabismus
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6/30. Ocular motility in genetically defined autosomal dominant cerebellar ataxia.

    PURPOSE: To describe ocular motility in patients having genetically characterized dominant cerebellar ataxia. DESIGN: Observational case series. METHOD: Nine ataxic adults having the following molecular genetic diagnoses underwent ophthalmic examination and ocular motility recordings: four with spinocerebellar ataxia type 6 (SCA-6), three with SCA-3, one with SCA-1, and one with episodic ataxia type 2 (EA-2). RESULTS: Versions were normal in eight patients. Most were orthotropic, but one with SCA-3 had exotropia at near. The near point of convergence was remote in five patients. Eight of nine patients had horizontal nystagmus evoked by lateral gaze. All patients with SCA-6 had downbeat nystagmus. Downbeat nystagmus was absent in SCA-1, SCA-3, and EA-2. Three patients with SCA-6 and one with EA-2 had symptomatic improvement when treated with acetazolamide. CONCLUSION: patients with genetically defined dominant cerebellar ataxia generally had normal binocular alignment and versions and only mild vergence impairment. Downbeat nystagmus was strongly associated with the SCA-6 mutation, whose associated episodes of dizziness and imbalance may be relieved by acetazolamide.
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ranking = 6.1392581426352
keywords = exotropia
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7/30. Discordant pursuit asymmetry and esotropia in monozygous twins.

    PURPOSE: To report the relationship between infantile esotropia and asymmetry of monocular pursuit tracking. DESIGN: Observational family case series. methods: A twin pair discordant for infantile esotropia was confirmed by dna analysis to be monozygous at 99.95% probability. Digitally sampled electro-oculographic recordings of monocular horizontal pursuit eye movements were performed for the twins and their orthophoric father. RESULTS: Mean directional asymmetry for right and left eyes was 31% and 5% for the esotropic twin, 10% and 8% for the orthophoric twin, and 5% and 6% for the father. The asymmetry was statistically significant only for the deviating right eye of the esotropic twin (P = 0.0217). CONCLUSION: In this family, monocular pursuit asymmetry is probably not a heritable predisposition to infantile esotropia, but a consequence of early strabismus. Discordance of infantile esotropia and pursuit asymmetry in monozygous twins implicates environmental in addition to genetic causes.
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ranking = 1
keywords = strabismus
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8/30. Midbrain syndrome with eye movement disorder: dramatic improvement after cranioplasty.

    BACKGROUND: patients with skull defects sometimes develop neurological deficits, which have been grouped under "the syndrome of the trephined". The deficits are usually nonspecific or nonlocalizing, such as apathy or diffuse headaches. We report, to our knowledge, a first case of severe midbrain syndrome associated with a skull defect. Cranioplasty dramatically resolved the patient's symptoms. A midbrain syndrome represents the main manifestation of the syndrome of the trephined and can be corrected by cranioplasty. CLINICAL PRESENTATION: A 38-year-old man with head trauma and epidural hematoma initially had normal eye motility. He developed a skull defect after infection following cranioplasty. He presented with onset of neurological symptoms one year after head trauma with a skull defect, a small divergent and vertical strabismus, elevation deficit of both eyes, headaches and fatigue. Over several months he developed severe bilateral deficit of adduction, elevation, depression and convergence. He had neuropsychological deficits, fatigue, headaches and impaired coordination. neuroimaging and lumbar puncture did not show evidence of increased intracranial pressure or hydrocephalus. INTERVENTION: Cranioplasty using Palacos was performed one-and-a-half years after trauma. Immediately after surgery, the patient noted remarkable improvement in his symptoms. Headaches and fatigue disappeared within two days. Two weeks after cranioplasty the patient had orthotropia and virtually normal ocular motility. Neurological symptoms completely disappeared. Recovery remained sustained for over 5 years after cranioplasty. CONCLUSION: To our knowledge, this represents the first case of the syndrome of the trephined in which the neurological deficits map primarily to the brainstem and show rapid improvement following cranioplasty. We show that cranioplasty in patients with large skull defects is indicated for more then cosmetic reasons and should be considered even after longer periods following a trauma.
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ranking = 1
keywords = strabismus
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9/30. Botulinum toxin A in the treatment of adherence syndrome.

    The effect of botulinum toxin A is investigated in the treatment of adherence syndrome related to strabismus surgery. An 18-year-old boy with decompensated right congenital fourth nerve palsy underwent ipsilateral superior rectus muscle recession and inferior oblique muscle disinsertion. During the early postoperative period, a right hypotropia with limitation of elevation developed and the result of the forced duction test was positive for passive elevation of the right eye. Botulinum toxin A was injected into the right inferior rectus muscle on the 15th postoperative day and orthophoria was achieved 1 week later. A repeat injection of botulinum toxin A was performed 6 months later. On follow-up 2 1/2 years later, his eyes remained orthophoric with limitation of elevation in the right eye and the patient was satisfied with the result. This case demonstrated that adherence syndrome can be treated successfully by botulinum toxin A injection if it is administered during the acute phase, before the development of fibrous scar tissue.
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ranking = 1
keywords = strabismus
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10/30. Psoriasiform eruption from intramuscular botulinum A toxin.

    Botulinum A toxin is used intramuscularly in the treatment of spastic neuromuscular disorders, strabismus, and laryngeal dystonia. The toxin has recently been reported as being useful for the cosmetic removal of glabellar furrows. The clinical effect of the toxin lasts four months or longer. Systemic side effects are rare and usually transient. We report the case of a psoriasiform eruption temporally related to the injection of botulinum A toxin into the medial rectus muscle to treat an ocular motility disorder. To our knowledge, this is the first case of a psoriasiform dermatitis caused by this agent.
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ranking = 1
keywords = strabismus
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