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In 1958, Kearns and Sayre described a multisystem entity, now known as Kearns-Sayre syndrome (KSS). The syndrome is defined as exhibiting a triad of thus far unexplained degenerative conditions: progressive external ophthalmoplegia, retinal pigmentary degeneration, and heart block. Commonly accompanying findings include cerebellar dysfunction and CSF protein levels above 100 mg/dl. Symptoms usually appear in early childhood, but the onset has been seen occasionally in young adults. KSS is a mitochondrial disorder that occurs rarely; the actual incidence is unknown. Ocular findings consist of bilateral ptosis, chronic progressive external ophthalmoplegia, and pigmentary retinopathy. Corneal clouding and optic neuritis are infrequent. We herein report a classic case of Kearns Sayre syndrome and discuss the findings.
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PMID:Kearns-Sayre syndrome: a case report and review. 163 60

The tendinous origins and insertions of the extraocular muscles were studied embryologically by macroscopic and microscopic methods. It is concluded from this investigation that these tendons of origin and insertion arise from mesenchymal tissue similar to that of their respective muscles. These tendon-muscle groups have developed from superior and inferior mesenchymal complexes. The origins of the extraocular muscles are attached to the periorbita by an interlocking of the tendinous and muscular fibers, which allows for mobility of the extraocular muscles in all extreme directions of gaze and also results in a strong mechanical mooring for these muscles. Avulsion at the origins of the extraocular muscles following severe traction or trauma is rare. The additional origin of the superior and medial rectus muscles to the dura of the optic nerve explains the pain that may occur on movement of the eye in optic neuritis. Optic nerve compression and thyroid myopathy is explained by mucopolysaccharide and inflammatory cell infiltration of the muscular interdigitations that extend up to the site of origin of the rectus muscles. Findings of this investigation suggest that the association of ptosis and superior rectus muscle underaction may be due to a persistence of fibrous tissue that has endured from embryologic development between the superior rectus and levator palpebrae superioris muscles. Superior oblique tendon sheath syndrome is explained by embryologic strands remaining between the tendon of the superior oblique muscle and the trochlea. The insertions of the rectus muscles extend from the equator of the eye to the limbus early on in development. By processes of differential degeneration between the sclera and the rectus tendon, posterior recession of the tendon from the limbus, and contemporaneous growth of the anterior segment of the eye, these tendons reach their adult location only between the ages of 18 months and 2 years. In strabismus surgery, measurements for muscle adjustments should be assessed from the limbus rather than from the sites of insertion of these tendons. In the series of patients with esotropia, no mechanical abnormalities were noted in relationship to the insertions of the medial or lateral recti muscles. Furthermore, no correlation was found between the site of insertion of the medial rectus muscle and the degree of esotropia.
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PMID:The origins and insertions of the extraocular muscles: development, histologic features, and clinical significance. 359 Apr 78

Reports of cases of primary and secondary syphilis are increasing in the United States, particularly in urban areas and among homosexual men. While primary syphilis poses little diagnostic difficulty, many physicians are unfamiliar with the multisystem nature of secondary lues. Patients who have secondary syphilis commonly present with systemic signs, skin rash, mucous membrane lesions and generalized adenopathy. Less commonly, secondary syphilis may occur as acute meningitis, sensorineural hearing loss, iritis, anterior uveitis, optic neuritis, Bell's palsy, gastropathy, proctitis, hepatitis, pulmonary infiltration, nephrotic syndrome, glomerulonephritis, periostitis, tenosynovitis and polyarthritis. The diagnosis of secondary syphilis is easily confirmed. Its various manifestations are readily treated with penicillin and, if treated early, are entirely reversible. Two recent cases of secondary syphilis, one presenting as nephrotic syndrome and one as chorioretinitis and ptosis, illustrate the usual and unusual features of this common infection.
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PMID:Secondary syphilis: uncommon manifestations of a common disease. 670 90

In seven patients with intracranial meningioma whose presenting signs and symptoms were ophthalmologic the underlying problem was initially misdiagnosed. Three patients had sphenoidal meningiomas with compression of the anterior visual pathways, but the initial diagnoses were acute optic neuritis, chronic optic neuritis and glaucoma. Two other patients had large frontal meningiomas causing in one case unilateral pain and swelling of the upper lid plus ptosis and hypotropia, and in the other case bilateral frontal morning headaches and intermittent blurring of vision in one eye; they were thought to have a frontal lobe osteoma and migraine respectively. A sixth patient had a large parietal meningioma causing unilateral papilledema in an eye with a corneal graft; the papilledema was not initially recognized because of severe astigmatism in that eye. The last patient had an occipital meningioma that had caused a fixed homonymous field defect and many years of "classic migraine".
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PMID:Meningioma and the ophthalmologist: diagnostic pitfalls. 719 8

A 59-year-old woman was referred to our clinic with sudden visual loss in her right eye after she was treated with 40 mg/day of oral prednisolone for 2 weeks under the diagnosis of idiopathic optic neuritis. At that time, computerized tomography (CT) of the brain showed no evidence of optic nerve or brain pathology. However, there was progressive diminution of right visual acuity associated with a limitation of adduction and abduction in the right eye. On magnetic resonance imaging and repeated CT, a malignant lesion was suggested, and was confirmed as an Aspergillus fungus colony by histopathologic examination. Postoperatively, she was treated with intravenous administration of amphotericin B for 13 weeks. However, her condition continued to deteriorate. She developed ptosis and total ophthalmoplegia in the right eye and blindness in both eyes. After discharge, she was given itraconazole for 20 weeks. She has shown no recovery of visual acuity or extraocular motion during a two-year follow-up period. The clinical features of our case suggest that early diagnosis in a case of aspergilloma presenting with visual loss is difficult and that a high index of suspicion, repeated radiological examination and adequate biopsy may be required for diagnosis.
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PMID:Aspergillosis presenting as an optic neuritis. 1254 51

We report our findings for a patient with orbital apex syndrome associated with herpes zoster ophthalmicus. Our patient was initially admitted to a neighborhood hospital because of nausea and loss of appetite of 10 days' duration. The day after hospitalization, she developed skin vesicles along the first division of the trigeminal nerve, with severe lid swelling and conjunctival injection. On suspicion of meningoencephalitis caused by varicella zoster virus, antiviral therapy with vidarabine and betamethasone was started. Seventeen days later, complete ptosis and ophthalmoplegia developed in the right eye. The light reflex in the right eye was absent and anisocoria was present, with the right pupil larger than the left. Fat-suppressed enhanced T1-weighted magnetic resonance images showed high intensity areas in the muscle cone, cavernous sinus, and orbital optic nerve sheath. Our patient was diagnosed with orbital apex syndrome, and because of skin vesicles in the first division of the trigeminal nerve, the orbital apex syndrome was considered to be caused by herpes zoster ophthalmicus. After the patient was transferred to our hospital, prednisolone 60 mg and vidarabine antiviral therapy was started, and fever and headaches disappeared five days later. The ophthalmoplegia and optic neuritis, but not the anisocoria, gradually resolved during tapering of oral therapy. From the clinical findings and course, the cause of the orbital apex syndrome was most likely invasion of the orbital apex and cavernous sinus by the herpes virus through the trigeminal nerve ganglia.
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PMID:Orbital apex syndrome associated with herpes zoster ophthalmicus. 2214 Mar 5

Pregnancy is often associated with ocular changes which may be more commonly transient but occasionally, permanent. The ocular effects of pregnancy may be physiological or pathological or may be modifications of pre-existing conditions. Physiological changes include increased pigmentation around the cheeks, ptosis, changes in cornea and refractive status,decreased intraocular pressure. These usually resolve post partum. Pre-existing diseases such as Graves disease, Retinitis pigmentosa, optic neuritis, should be monitored due to their remission or relapses in pregnancy. There may be worsening of Diabetic retinopathy, and Central serous chorio-retinopathy with increased risk of Retinal detachment. Conditions like Glaucoma and Non infectious uveal inflammatory disorders may even improve transiently. Pre-eclampsia and eclampsia could result in hypertensive retinopathy, exudative retinal detachment and cortical blindness. Neuro-ophthalmological disorders such as venous sinus thrombosis, benign intracranial hypertension, pituitary adenoma, meningioma and optic neuritis should be kept in mind as differential diagnosis in pregnant women presenting with visual acuity loss, visual field loss, persistent headaches or oculomotor palsies. Use of ophthalmic drugs can affect fetal health during pregnancy.
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PMID:Ocular changes in pregnancy. 2234 13

Idiopathic orbital inflammatory syndrome (IOIS) is a nonspecific inflammation of orbital tissue. As it is a diagnosis of exclusion, systemic testing and, at times biopsy, is utilized to rule out other inflammatory etiologies. Since some inflammatory etiologies that masquerade as typical IOIS can be vision or life threatening, it is important to consider these diagnoses. Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune process that can affect the eye and visual system in 20% of individuals. In this idiopathic process, the deposition of pathogenic autoantibodies and immune complexes damage tissues and cells. Some common ocular manifestations of SLE include keratoconjunctivitis sicca, periocular skin lesions, orbital inflammation, retinal hemorrhages and vasculitis, retinal vaso-occlusive disease, iritis, scleritis, optic neuritis and optic neuropathy. One rare clinical entity in the SLE spectrum is panniculitis, also known as lupus erythematosus profundus (LEP), which is a nodular inflammation of adipose tissue. Panniculitis involving orbital structures as the primary presenting symptom of SLE is quite unusual and has only rarely been previously reported in the literature and has not been reported presenting as IOIS. This uncommon presentation can make the diagnosis more difficult. We describe a patient who had presented with ptosis evolving to orbital inflammation, which was consistent with IOIS by laboratory and histologic examinations. The patient later developed extensive panniculitis and a final diagnosis of LEP was made.
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PMID:Lupus erythematosus profundus masquerading as idiopathic orbital inflammatory syndrome. 2255 72

Introduction. Leptomeningeal carcinomatosis occurs in about 5% of cancer patients. Ocular involvement is a common clinical manifestation and often the presenting clinical feature. Materials and Methods. We report the case of a 52-year old lady with optic neuritis as isolated manifestation of neoplastic meningitis and a review of ocular involvement in neoplastic meningitis. Ocular symptoms were the presenting clinical feature in 34 patients (83%) out of 41 included in our review, the unique manifestation of meningeal carcinomatosis in 3 patients (7%). Visual loss was the presenting clinical manifestation in 17 patients (50%) and was the most common ocular symptom (70%). Other ocular signs were diplopia, ptosis, papilledema, anisocoria, exophthalmos, orbital pain, scotomas, hemianopsia, and nystagmus. Associated clinical symptoms were headache, altered consciousness, meningism, limb weakness, ataxia, dizziness, seizures, and other cranial nerves involvement. All patients except five underwent CSF examination which was normal in 1 patient, pleocytosis was found in 11 patients, increased protein levels were observed in 16 patients, and decreased glucose levels were found in 8 patients. Cytology was positive in 29 patients (76%). Conclusion. Meningeal carcinomatosis should be considered in patients with ocular symptoms even in the absence of other suggestive clinical symptoms.
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PMID:Optic neuritis as isolated manifestation of leptomeningeal carcinomatosis: a case report and systematic review of ocular manifestations of neoplastic meningitis. 2422 6

A 68-year-old man presented with periorbital pain with progressive decrease in visual acuity of the right eye. The patient began to experience these symptoms 6 months ago, but they had become significantly worse in the last month. On examination, the patient had ptosis, proptosis, and impaired movement of the right eye. His medical history was notable for peripheral T-cell lymphoma. During chemotherapy, he had developed pulmonary aspergillosis, which resolved with antifungal medication. The magnetic resonance imaging scan, which was taken because of orbital symptoms, was suggestive of optic neuritis. For 6 months, his visual symptoms deteriorated and improved with intermittent steroid treatment. However, the patient began to experience worsening pain and visual disturbance for the month leading up to the referral visit and could not recognize light in the involved eye. A repeat magnetic resonance imaging study revealed a diffuse involvement of right orbital apex. Subsequent to this finding, an endoscopic biopsy was performed. The pathologic examination confirmed the diagnosis of invasive aspergillosis. Orbital pain, ptosis, and limitation of eye movements improved with antifungal medication, and the patient was able to recognize hand movements.
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PMID:Orbital apex syndrome caused by invasive aspergillosis. 2462 69


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