The Tolosa-Hunt syndrome.
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Biomedical subjects
Publications and source records attributed to L B Kline.
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OBJECTIVE: To report a patient with agenesis of the right internal carotid artery associated with ipsilateral, congenital Homer's syndrome. DESIGN: Case report. METHODS: A 30-year-old woman, with a past history of migraine headaches, underwent neuro-ophthalmologic and neuroradiologic evaluation for transient visual obscurations and congenital Horner's syndrome. RESULTS: A right, third-order neuron Horner's syndrome was confirmed with 1% hydroxyamphetamine topical drops. Cranial magnetic resonance imaging revealed an absent right internal carotid artery flow void, computed tomography demonstrated absence of the right carotid canal, and cerebral angiography confirmed absence of the right internal carotid artery. No atheromatous lesions were found and the results of coagulation studies were normal. CONCLUSIONS: Agenesis of the internal carotid artery is a rare cause of congenital Horner's syndrome. The cause of transient visual blurring in the current patient remains unproven.
This is a report of the use of endoscopic sinus surgery in the management of three patients diagnosed with rhino-orbital or rhino-orbito-cerebral mucormycosis. A retrospective review was performed of the clinical examinations and imaging studies of three patients who underwent endoscopic sinus surgery as part of their therapy for mucormycosis. In addition to endoscopic surgery, all patients had aggressive control of underlying risk factors (diabetes mellitus, immunosuppression) and prolonged intravenous amphotericin B therapy. All three patients survived and avoided orbital exenteration. In selected patients with rhino-orbito-cerebral mucormycosis, endoscopic techniques can play a valuable role in diagnosis and management.
PURPOSE: The purpose of the study is to report the occurrence of optic neuropathy after stereotactic radiosurgery for perichiasmal tumors. METHODS: Records of four patients with visual deterioration after stereotactic radiosurgery were reviewed, including clinical findings, neuroimaging results, and treatment methods. RESULTS: Optic neuropathy developed 7 to 30 months after gamma knife radiosurgery. All patients experienced an abrupt change in visual function. Clinical findings indicated anterior visual pathway involvement. Patterns of field loss included nerve fiber bundle and homonymous hemianopic defects. Gadolinium-enhanced magnetic resonance imaging (MRI) showed swelling and enhancement of the affected portion of the visual apparatus in three patients. Systemic corticosteroids were administered in all patients and one partially recovered. One patient also received hyperbaric oxygen without improvement. CONCLUSIONS: Although rare, optic neuropathy may follow radiosurgery to lesions near the visual pathways. Careful dose planning guided by MRI with restriction of the maximal dose to the visual pathways to less than 8 Gy will likely reduce the incidence of this complication.
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We report a patient with pretectal eyelid lag without retraction. MRI showed high signal abnormality dorsal to the right red nucleus in a region thought responsible for premotor eyelid control. The dissociation of eyelid lag from eyelid retraction in this patient would suggest that these lid signs may have separate neural mechanisms or pathways. We propose that the lesion interrupts inhibitory connections between the vertical gaze center and the central caudal nucleus.
OBJECTIVE: Diplopia is not frequently associated with Arnold-Chiari I malformation. We reviewed our cases of Arnold-Chiari I malformation in which acquired esotropia with diplopia was the main neuro-ophthalmologic finding early in the clinical course. MATERIALS AND METHODS: Five patients were studied, all female, ranging in age from 17 to 36 years, who were treated by the neuro-ophthalmology service of urban teaching hospitals. Eye movement recordings using magnetic search coil technique were performed in two patients. RESULTS: All patients reported onset of horizontal diplopia due to acquired esotropia as an initial manifestation of the Arnold-Chiari I malformation. All had full abduction of each eye. In addition, all five had gaze-evoked nystagmus, two skew deviations, and one bilateral internuclear ophthalmoparesis. Oculography in two patients showed normal abducting saccadic peak velocities. This supports divergence palsy as a mechanism of acquired esotropia and provides evidence against subtle sixth nerve palsy in these patients. Four patients underwent neurosurgical decompression of their Chiari malformations, and neuro-ophthalmologic signs and symptoms improved in all. CONCLUSIONS: Acquired esotropia, often in association with other eye movement abnormalities, may be an early sign of Arnold-Chiari I malformation. This quantitative study indicates that divergence palsy is the cause of esotropia in some patients. Neurosurgical suboccipital and upper cervical decompression may lead to improvement or resolution of diplopia.
Most documented cases of radiation-induced optic neuropathy are unilateral and occur more than 1 year after radiotherapy to the sellar region. We describe a patient with bilateral radiation optic neuropathy 3 months following the completion of radiotherapy. MRI 13 months after the onset of visual failure showed bilateral optic atrophy with residual gadolinium enhancement.
Cranial mononeuropathies, particularly ophthalmoplegia and facial palsy, are common entities in the diabetic population. Simultaneous multiple cranial neuropathies due to diabetes are much less common, however. We present three patients with this entity.
OBJECTIVE: Study of clinical features of ischemic optic neuropathy (ION) developing as a complication of multilevel lumbar spine surgery. DESIGN: Review of all cases of ION that developed within 2 weeks of spine surgery at two academic institutions from 1990 to 1992, and a review of adequately reported cases of ION after other non-ophthalmic procedures. RESULTS: Four new cases are reported in patients who ranged in age from 41 to 65 years. All four had undergone uneventful but prolonged (8 to 9 hours) spine surgery, during which blood pressure was deliberately maintained between 85 and 100 mm Hg systolic and 45 to 65 mm Hg diastolic to reduce bleeding. Hemoglobin values fell 30 to 78 g/L during surgery. Arteriosclerotic risk factors, including systemic hypertension, diabetes, coronary artery disease, and smoking, were present in three cases. There was no evidence of orbital soft-tissue injury, retinal artery occlusion, or other neurologic deficits. The combination of hypotension and anemia has been noted in most of the 30 previously well-documented cases of ION after other non-ophthalmic procedures. CONCLUSIONS: Multilevel lumbar laminectomy should be added to the list of procedures that may produce ION as an isolated complication. Deliberate hypotension maintained for long operative periods in patients with arteriosclerotic risk factors may be the cause.
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BACKGROUND: Neuroretinitis is a distinct clinical entity consisting of moderate to severe visual loss, optic nerve head edema, macular exudate in a stellate pattern, and variable vitreous inflammation. Although the etiology is usually postviral or idiopathic, an acute infectious cause occasionally is demonstrated. METHODS: Five juvenile or young adult patients with neuroretinitis are presented with serologic evidence of Toxoplasma gondii infection. Four of the five patients were treated with systemic antibiotics and corticosteroids; one patient was not treated. RESULTS: With a mean follow-up period of 50 months, visual acuity returned to 20/25 or better in four patients, with one patient regaining visual acuity of 20/60. Two patients had one or more recurrent episodes of neuroretinitis, distinguishing toxoplasmosis from idiopathic neuroretinitis, which is usually a monophasic illness. CONCLUSION: Toxoplasmosis infection is a rare, but potentially treatable, form of neuroretinitis and should be included in the differential diagnosis of "Leber's idiopathic stellate retinopathy."
A 45-year-old woman developed opsoclonus, myoclonus, and severe truncal and gait ataxia. Serum and CSF contained IgG antibodies that appear to be identical to "anti-Ri" antibodies associated with paraneoplastic opsoclonus and ataxia. The patient had a fluctuating course with exacerbations that responded well to corticosteroids and later to cyclophosphamide. Her anti-Ri antibody titer has declined significantly but still remains high. After more than 3 years of follow-up, no neoplasm has been detected.
A 59-year-old male heart transplant recipient experienced progressive visual loss following routine cataract surgery with intraocular lens implantation. Cranial magnetic resonance (MR) imaging suggested and brain biopsy confirmed the cause to be progressive multifocal leukoencephalopathy (PML). The patient died 2 months following cataract surgery. This case illustrates that visual failure may be the initial manifestation of PML, and the ophthalmologist should be aware of this central demyelinating disorder when dealing with immunocompromised patients.
A 28-year old man presented with decreased vision and a bitemporal hemianopia. Magnetic resonance (MR) imaging demonstrated what appeared to be an enlarged optic chiasm and, on T2-weighted images, hypersignal extending along the optic tracts. This was felt to represent an intrinsic chiasmal mass, most likely a chiasmal glioma. Biopsy specimens from the first craniotomy were nondiagnostic. With continued visual failure, the patient underwent a second operation, and the correct diagnosis of craniopharyngioma was established. This article emphasizes the difficulties encountered with neuroradiologic evaluation and histopathologic study of craniopharyngioma.
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