1/31. Solitary fibrous tumour of the orbit. Report of a new case.PURPOSE: Solitary fibrous tumour (SFT) of the orbit is a very rare lesion that may be misdiagnosed as fibrous histiocytoma, haemangiopericytoma, or other orbital tumour. We studied a case of SFT of the orbit in a 35-year-old woman. methods: The patient, with a 1-year history, reported a 'pressure' sensation behind her right eye, but no pain, vision changes, or other associated symptoms. Ocular examination was normal. magnetic resonance imaging (MRI) showed a well-circumscribed medial mass in the right orbit. The tumour was excised. RESULTS: The tumour was a cellular spindle-cell neoplasm with a storiform pattern. The tumour cells were spindle-shaped with bland nuclei and rare mitoses. Immunostaining was positive for vimentin and CD34, but negative for cytokeratin (AE1-AE3), EMA, desmin, smooth muscle actin, S-100, CD31, CD45 and bcl-2. CONCLUSION: SFT can infrequently involve the orbit. The tumour must be removed entirely to avoid recurrences. Careful and continued follow-up is important because orbital recurrence may occur several years after the excision of the primary tumour.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/31. Marcus Gunn jaw winking with trigemino-abducens synkinesis.Congenital ocular aberrant innervation syndromes are a complex group of disorders involving abnormal miswiring of the extraocular muscles. This case report describes a child with both a right Marcus Gunn jaw winking phenomenon and a right trigemino-abducens synkinesis, which has not previously been reported in the literature. Clinically, this child presented with an intermittent elevation of the right eyelid and/or an intermittent right exotropia when opening her mouth while sucking or chewing. This case suggests the primary abnormality in this patient may be abnormal development of the trigeminal nerve resulting in the eyelid abnormalities and strabismus.- - - - - - - - - - ranking = 5.9701115918693keywords = complex (Clic here for more details about this article) |
3/31. Congenital mitochondrial cytopathy and chronic progressive external ophthalmoplegia.BACKGROUND: Chronic Progressive External ophthalmoplegia (CPEO) encompasses different conditions having in common a slowly progressive external and general ophthalmoplegia. The discovery of CPEO is suggestive of mitochondrial cytopathy, but this is not necessarily so. CASE REPORT: We report here a case, presenting at age 9 months, characterized by bilateral blepharoptosis and partial third nerve oculomotor deficiency, with no nystagmus. Mitochondrial cytopathy was suspected on cranial MRI and confirmed by muscle biopsy. Enzyme studies revealed a defect on the complex I respiratory chain. This case is unique in that the symptoms completely resolved under a Ketogen diet.- - - - - - - - - - ranking = 5.9701115918693keywords = complex (Clic here for more details about this article) |
4/31. Respiratory chain complex-I defect mimicking myasthenia.In a 67-year-old woman with ptosis, double vision, dysphagia, ambiguous Tensilon tests, normal acetylcholine-receptor antibodies, normal thymus, and repeatedly abnormal responses to low-frequency repetitive stimulation, ocular myasthenia was suspected. Pyridostigmin was ineffective, but corticosteroids improved the abnormalities. Despite this therapy, lower-limb weakness developed. Reevaluation disclosed abnormal increase of serum lactate during slight exercise, myogenic electromyography, ragged-red fibers, reduced oxidative enzyme staining and abnormally shaped and structured mitochondria on muscle biopsy, and a respiratory chain complex-I defect on biochemical investigation of the muscle homogenate. Respiratory chain disorder due to complex-I defect with abnormal decremental response to low-frequency repetitive stimulation was diagnosed. It is concluded that respiratory chain disorders due to a complex-I defect may mimic ocular myasthenia clinically, electrophysiologically, and even therapeutically.- - - - - - - - - - ranking = 41.790781143085keywords = complex (Clic here for more details about this article) |
5/31. diplopia and involuntary eye closure in spontaneous cerebellar hemorrhage.Spontaneous cerebellar hemorrhage is of difficult clinical diagnosis. The causes can be varied, but the hemorrhage is most often associated with hypertensive cardiovascular disease. The neurological symptomatology is complex and often misleading. The diagnosis is mainly dependent of familiarity of the eye signs seen in this disease. Among these, the spontaneous unilateral eye closure is presented as an additional striking manifestation. The displacement of the brain stem by the hematoma is frequently associated with a seventh nerve palsy on the side of the hemorrhage. The patient in an effort to obviate the diplopia caused by the gaze dissociations and extraocular motor palsies, has only the option to close the eye on the noninvolved side of the face, and thus the eye remaining open is on the side of the cerebellar hematoma. This paper presents reports of two patients with these symptoms.- - - - - - - - - - ranking = 5.9701115918693keywords = complex (Clic here for more details about this article) |
6/31. Congenital fibrosis syndrome associated with central nervous system abnormalities.BACKGROUND: Congenital fibrosis of extraocular muscles (CFEOM) is a complex strabismus syndrome that typically occurs in isolation and results from dysfunction of all or part of cranial nerves III (CNIII) and IV (CNIV) and/or the muscles that these nerves innervate. Only a few patients with CFEOM and additional central nervous system malformations have been reported. We describe four additional patients with CFEOM associated with central nervous system (CNS) abnormalities. methods: Four patients who presented with congenital restriction of eye movements in association with neurological abnormalities underwent complete ophthalmological examination including electroretinography (ERG) and eye movement recordings. Neurological examinations, neuroradiological studies, muscle histology, chromosomal and genetic linkage analysis were performed. RESULTS: Clinical examination and forced duction testing confirmed that all four patients met criteria for CFEOM; all had congenital restrictive ophthalmoplegia primarily affecting extraocular muscles innervated by the oculomotor nerve. Two brothers had CFEOM and Marcus Gunn jaw winking. In each of the four cases, CFEOM occurred in association with one or several neuroradiological findings, including agenesis of the corpus callosum, colpocephaly, hypoplasia of the cerebellar vermis, expansion of the ventricular system, pachygyria, encephalocele and/or hydrancephaly. CONCLUSIONS: We present four cases of CFEOM in association with CNS malformations that confirm that CFEOM can be part of a more complex neurological dysfunction and provide further support to a neurogenic aetiology for this disorder. We also describe for the first time the coexistence of CFEOM and Marcus Gunn jaw winking in two siblings. This suggests a genetic mechanism. Aberrant innervation supports primary developmental abnormality of cranial nerves in CFEOM.- - - - - - - - - - ranking = 11.940223183739keywords = complex (Clic here for more details about this article) |
7/31. Reverse ptosis-induced corneal steepening and decreased vision after LASIK surgery.PURPOSE: To report a patient with a past history of LASIK who had decreased vision and induced corneal steepening after lower eyelid ptosis. Surgical correction of lower eyelid ptosis decreased the corneal steepening and improved visual acuity. methods: Interventional case report. RESULTS: A 37-year-old woman had a history of bilateral LASIK, childhood strabismus surgery, and multiple surgeries to release scarring and improve motility in her left eye. Last surgery to release scar tissue resulted in reverse ptosis (lower eyelid ptosis) and decreased visual acuity from induced corneal steepening. Correction of lower eyelid ptosis by reinsertion of the retractor complex resulted in decreasing corneal steepening, improved visual acuity, and good anatomic position of the lower eyelid. CONCLUSION: Lower eyelid ptosis may induce corneal steepening and decreased vision after LASIK. Surgical correction of ptosis can decrease the extent of steepening and improve visual acuity.- - - - - - - - - - ranking = 5.9701115918693keywords = complex (Clic here for more details about this article) |
8/31. Congenital oculo-bulbar palsy.A girl developed progressive weakness of bulbar and ocular muscles starting before the age of two years. electromyography revealed a widespread subclinical myopathy. An intercostal muscle biopsy showed complex abnormalities including occasional neurofilamentous accumulations and honeycomb-like membranous material in terminal axons. Endplates were small and some secondary synaptic clefts were abnormally deep. acetylcholine receptors extended unusually deeply into the clefts of the junctional folds. Muscle fibres showed subsarcolemmal vacuolation at some places. This form of congenital oculo-bulbar palsy does not appear to have been described previously.- - - - - - - - - - ranking = 5.9701115918693keywords = complex (Clic here for more details about this article) |
9/31. Clinical heterogeneity in familial congenital ptosis: analysis of fourteen cases in one family over five generations.This report describes a proband and his family consisting of 14 patients affected with simple congenital ptosis. pedigree suggests an autosomal dominant pattern with 70-90% penetrance. The family includes patients with both unilateral and bilateral involvement, and cases with and/or without complex synkinesia. Left unilateral preponderance is striking, and pedigree analysis suggests the possibility of a modifier gene determining laterality. This study represents the first report of a large family with congenital autosomal dominant simple ptosis which demonstrates the full clinical spectrum of this condition.- - - - - - - - - - ranking = 5.9701115918693keywords = complex (Clic here for more details about this article) |
10/31. Ptosis as an initial manifestation of orbital lymphoma: a case report.Ptosis on its own is an infrequent initial manifestation of orbital lymphoma. Orbital lymphoma usually presents as a palpable mass with proptosis, diplopia, and conjunctival ("salmon-pink") swelling. We report here a 62-year-old female patient who presented with right eye ptosis. The initial imaging study showed an indistinct enlargement of the superior rectus-levator muscle complex. After 3-4 months, ptosis and upward gazing movement were further restricted. The imaging study revealed a definite soft-tissue mass in the superior orbit surrounding the superior rectus-levator muscle complex. A tumor biopsy through anterior orbitotomy revealed a large diffuse B-cell lymphoma. With the experience of this case, we suggest that orbital lymphoma should be included in the differential diagnosis of ptosis accompanied by impairment of levator muscle function.- - - - - - - - - - ranking = 11.940223183739keywords = complex (Clic here for more details about this article) |
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