Ocular Motor Dysfunction in Parry–Romberg Syndrome: Four Cases

Prescott, Christina; Hasbani, M; Levada, Andrew J.; Silbert, Jonathan E.; Winterkorn, Jacqueline M.S.; Lesser, Robert L.

doi:10.3928/01913913-20111129-02

Cited by 8 publications

(9 citation statements)

References 13 publications

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“… 1 , 28 , 79 Ocular motility disturbances may also be observed, due to restrictive myopathy (in part inflammatory) 97 and oculomotor palsies. 98 Occasionally, intraorbital fat atrophy has been reported by local imaging (echography or MRI), or histological analysis (when strabismus surgery). 75 , 99 …”

Section: Discussionmentioning

confidence: 99%

Neurological Manifestations in Parry–Romberg Syndrome

et al. 2015

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Parry–Romberg syndrome (PRS) is a variant of morphea usually characterized by a slowly progressive course. Clinical and radiological involvement of the central nervous system may be observed in PRS.We describe 2 patients with PRS and neurological symptoms (one with trigeminal neuralgia associated with deafness, and the second with hemifacial pain associated with migraine without aura) in conjunction with abnormal cerebral MRI including white matter T2 hyperintensities and enhancement with gadolinium. Despite the absence of specific immunosuppressive treatments, both patients have presented stable imaging during follow-up without any clinical neurologic progression. We have performed a large review of the medical literature on patients with PRS and neurological involvement (total of 129 patients)Central nervous system involvement is frequent among PRS patients and is inconsistently associated with clinical abnormalities. These various neurological manifestations include seizures, headaches, movement disorders, neuropsychological symptoms, and focal symptoms. Cerebral MRI may reveal frequent abnormalities, which can be bilateral or more often homolateral to the skin lesions, localized or so widespread so as to involve the whole hemisphere: T2 hyperintensities, mostly in the subcortical white matter, gadolinium enhancement, brain atrophy, and calcifications. These radiological lesions do not usually progress over time. Steroids or immunosuppressive treatments are controversial since it remains unclear to what extent they are beneficial and there is often no neurological progression.

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Section: Discussionmentioning

confidence: 99%

Neurological Manifestations in Parry–Romberg Syndrome

et al. 2015

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“…Ten percent to 35% of cases report ophthalmologic findings, most commonly enophthalmos, eyelid and orbit degeneration, optic neuropathy, ophthalmoplegia, and pupillary dysfunction . Visual disturbances include decreased acuity and diplopia, which can be secondary to tissue degeneration, ocular motor nerve dysfunction, or extraocular muscle fibrosis . Serious ocular manifestations of PRS include scleral melt, panuveitis, hypotonia, phthisis, optic neuropathy, retinal vasculitis, macular edema, central retinal artery occlusion, retinal detachment, and sectional chorioretinal atrophy.…”

Section: Discussionmentioning

confidence: 99%

New Ophthalmoplegia and Recurrent Ptosis After Ptosis Repair Surgery

Kostina

Lesser

2018

JAMA Ophthalmol

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A man in his 60s with a history of surgery to repair right upper eyelid ptosis 1 year prior presented to a neuro-ophthalmology clinic with limited movement of the right eye of 1 year's duration and worsening recurrent ptosis of the same eye. Results of previous single-fiber electromyography and serologic testing for acetylcholine receptor-binding antibodies were negative for myasthenia gravis. His ocular history included amblyopia and nonarteritic ischemic optic neuropathy of the left eye. His medical history included obstructive sleep apnea, coronary artery disease, coronary artery bypass surgery, and prostate cancer, the last in remission for the past 4 years.Visual acuities were 20/30 OD and hand motions OS. The patient had an Adie pupil in his right eye and a relative afferent pupillary defect in the left eye. The right eye demonstrated total ophthalmoplegia, and no deficit was found in the left (Figure 1). Exophthalmometer measurements demonstrated enophthalmos of the right eye associated with facial asymmetry. Margin reflex distance was 0 mm in the right eye and 4 mm in the left. In-office ice and sleep testing did not improve the ptosis or ophthalmoplegia in the right eye. The remainder of his examination was remarkable for punctate epithelial erosions of the right eye and optic atrophy of the left.

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“…1 In the past 10 years, there have been several reports of patients with PHA who exhibit various ocular findings. 2–4…”

Section: Introductionmentioning

confidence: 99%

Progressive hemifacial atrophy with characteristic ocular manifestations in a Chinese patient with a CRB1 mutation

Liu

Wang

et al. 2020

J Int Med Res

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Progressive hemifacial atrophy (PHA) is mainly characterized by asymmetrical atrophy of craniofacial tissue; however, 10% to 30% of patients with PHA exhibit ocular manifestations. Here, we describe abnormal ocular findings in a Chinese patient with PHA. The patient was a 29-year-old Chinese man. Characteristic ocular findings in his affected eye included keratic precipitate, corneal endothelial degeneration, fundus tessellation, pupillary dilation, direct light reflex loss, and visual evoked potential alteration. Whole exosome sequencing revealed that the patient harbored a mutation in the CRB1 gene; this gene has been associated with various retinal dystrophies. During 10 years of follow-up, the patient’s ocular status remained stable. To the best of our knowledge, this is the first report of ocular manifestations of PHA in a Chinese patient, and the first report of a CRB1 mutation in a patient with PHA; these findings may inform future research regarding PHA.

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Ocular Motor Dysfunction in Parry–Romberg Syndrome: Four Cases

Cited by 8 publications

References 13 publications

Neurological Manifestations in Parry–Romberg Syndrome

Neurological Manifestations in Parry–Romberg Syndrome

New Ophthalmoplegia and Recurrent Ptosis After Ptosis Repair Surgery

Progressive hemifacial atrophy with characteristic ocular manifestations in a Chinese patient with a CRB1 mutation

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