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326 Systemic Disorders With Optic Nerve and Retinal FindingsLarry P. Frohman, MDA 35-year-old African-American woman had gradual bilateral painless visual loss over 3 months. When initially seen, the visual acuities were HM OD, NLP OS. The MRI showed diffused enhancement of the optic nerves and lacrimal glands. The evaluation strongly suggested sarcoidosis, with elevated angiot...
327 Chiasmal SyndromesLarry P. Frohman, MDThis 36-year-old woman presented in 1988 with 3 weeks of vertical binocular diplopia. She was a known amblyope OD. Her examination was notable for a right hyperdeviation of 1 PD present in right gaze and a subtle left noncongruous homonymous field defect. She was lost to follow-up, but 5 years later...
328 Chiasmal SyndromesLarry P. Frohman, MDThis 36-year-old woman presented in 1988 with 3 weeks of vertical binocular diplopia. She was a known amblyope OD. Her examination was notable for a right hyperdeviation of 1 PD present in right gaze and a subtle left noncongruous homonymous field defect. She was lost to follow-up, but 5 years later...
329 Systemic Disorders With Optic Nerve and Retinal FindingsLarry P. Frohman, MDSkin rashes occur in about 30 percent of patients with sarcoid. When seen, the rashes offer an accessible site for obtaining histologic material for confirmation of the clinical diagnosis. Pair with 91_68.
330 Neuro-Ophthalmic Imaging-MRIMitchell J. Wolin, MDAxial view of Arnold-Chiari malformation on a patient with downbeat nystagmus. Note the presence of the cerebellar tonsils posterior to the caudal medulla. In addition to downbeat nystagmus, Arnold-Chiari malformations can sometimes lead to increased intracranial pressure and papilledema.
331 Neuro-Ophthalmic Imaging-MRIMitchell J. Wolin, MDSagittal view of Arnold-Chiari malformation on a patient with downbeat nystagmus. The compression of the cervicomedullary junction is clearly depicted in the sagittal view.
332 Ocular Manifestations of Congenital/Inherited DiseasesWilliam Fletcher Hoyt, MDNeurofibromatosis, type 1, is an autosomal dominant phakomatosis characterized by Lisch nodules of the iris (hamartomas) plexiform neurofibromas, café-au-lait spots on the skin, and axillary freckling. Intracranial tumors such as optic pathway gliomas may occur. Disease/Diagnosis: Neurofibromatosis...
333 Ocular Manifestations of Congenital/Inherited DiseasesLarry P. Frohman, MDOn optic nerve CT scan, this patient with neurofibromatosis, type 1, shows the classic railroad-track sign of optic nerve meningioma and the kink sign of optic nerve glioma. Disease/Diagnosis: Neurofibromatosis, Type 1.
334 Isolated Optic Neuritis/NeuropathyJohn A. Charley, MDSeveral Primary Mutations result in Leber's hereditary optic neuropathy, including mitochondrial deletions at positions 11778, 3460, and 14484. Although the 11778 is the most common mutation, the 11484 has the best prognosis for spontaneous recovery. This case exhibits the 3460 mutation.
335 Isolated Optic Neuritis/NeuropathyJohn A. Charley, MDSeveral Primary Mutations result in Leber's hereditary optic neuropathy, including mitochondrial deletions at positions 11778, 3460, and 14484. Although the 11778 is the most common mutation, the 11484 has the best prognosis for spontaneous recovery. This case exhibits the 3460 mutation.
336 Isolated Optic Neuritis/NeuropathyJohn A. Charley, MDSeveral Primary Mutations result in Leber's hereditary optic neuropathy, including mitochondrial deletions at positions 11778, 3460, and 14484. Although the 11778 is the most common mutation, the 11484 has the best prognosis for spontaneous recovery. This case exhibits the 3460 mutation.
337 Isolated Optic Neuritis/NeuropathyJohn A. Charley, MDSeveral Primary Mutations result in Leber's hereditary optic neuropathy, including mitochondrial deletions at positions 11778, 3460, and 14484. Although the 11778 is the most common mutation, the 11484 has the best prognosis for spontaneous recovery. This case exhibits the 3460 mutation.
338 Isolated Optic Neuritis/NeuropathyJohn A. Charley, MDSeveral Primary Mutations result in Leber's hereditary optic neuropathy, including mitochondrial deletions at positions 11778, 3460, and 14484. Although the 11778 is the most common mutation, the 11484 has the best prognosis for spontaneous recovery. This case exhibits the 3460 mutation.
339 Isolated Congenital Optic Disc AnomaliesRoger Turbin, MDShown are the fundi of a - year old child with dominant optic atrophy, 20/200 OU. Pair with 1996_59. Disease/Diagnosis: Congenital optic atrophy.
340 Isolated Congenital Optic Disc AnomaliesRoger Turbin, MDShown are the fundi of a - year old child with dominant optic atrophy, 20/200 OU. Pair with 1996_60. Disease/Diagnosis: Congenital optic atrophy.
341 Neuro-Ophthalmic Vascular DiseaseMark L. Moster, MDNeovascularization of the iris may form in response to an ischemic disease of the retina, such as diabetic retinopathy. Carotid artery occlusion may result in ocular ischemia that may induce neovascularization.This is a dramatic image of iris neovascularization.
342 Neuro-Ophthalmic Imaging-CT ScanMitchell J. Wolin, MDThis is a patient with trauma leading to enucleation, with swelling years later over the implant. This is a presumed chronic abscess between orbit and dura.
343 Ocular Manifestations of Congenital/Inherited DiseasesWilliam Fletcher Hoyt, MDTuberous sclerosis is an autosomal dominant phakomatosis characterized by astrocytic hamartomas of the retina or optic nerve, adenoma sebaceum of the face, periungual fibromas, and astrocytic hamartomas of the brain, with secondary seizures and mental retardation. Disease/Diagnosis: Tuberous Scleros...
344 Ocular Manifestations of Congenital/Inherited DiseasesAnthony C. Arnold, MDNote the retinal/optic nerve head astrocytoma in a 12-year-old boy with tuberous sclerosis. Disease/Diagnosis: Tuberous Sclerosis.
345 Neuro-Ophthalmic Vascular DiseaseMark J. Kupersmith, MDAneurysms of the intracranial circulation may act as mass lesions and compress the afferent of efferent visual pathway. Ophthalmic artery aneurysms may compress the optic nerve and result in an optic neuropathy (ie, visual loss, afferent pupillary defect, optic atrophy). Treatment includes endovascu...
346 Neuro-Ophthalmic Vascular DiseaseMark J. Kupersmith, MDAneurysms of the intracranial circulation may act as mass lesions and compress the afferent of efferent visual pathway. Ophthalmic artery aneurysms may compress the optic nerve and result in an optic neuropathy (ie, visual loss, afferent pupillary defect, optic atrophy). Treatment includes endovascu...
347 Systemic Disorders With Optic Nerve and Retinal FindingsMark J. Kupersmith, MDSarcoidosis is an inflammatory multisystem granulomatous disease that may result in an inflammatory or infiltrative optic neuropathy, papilledema from increased intracranial pressure due to meningeal inflammation or intracranial granuloma, or may present with an optic disc granuloma. Pair with 91_31...
348 Systemic Disorders With Optic Nerve and Retinal FindingsMark J. Kupersmith, MDSarcoidosis is an inflammatory multisystem granulomatous disease that may result in an inflammatory or infiltrative optic neuropathy, papilledema from increased intracranial pressure due to meningeal inflammation or intracranial granuloma, or may present with an optic disc granuloma. Pair with 91_32...
349 Systemic Disorders With Optic Nerve and Retinal FindingsLarry P. Frohman, MDSkin rashes occur in about 30 percent of patients with sarcoid. When seen, the rashes offer an accessible site for obtaining histologic material for confirmation of the clinical diagnosis. Pair with 91_69.
350 Trochlear Motility DisturbancesDon Bienfang, MDThis patient displays a posttraumatic left fourth nerve palsy sustained after having struck her head on the dashboard.
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