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51 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.
52 Motility DisturbancesLarry P. Frohman, MDA 27-year-old healthy man presented for neuro-ophthalmologic evaluation for recurrent vertical binocular diplopia. In 1982, he had a minor blow to the head, and 12 days later he sustained vertical binocular diplopia, worse on downgaze, without ever having diurnal variation. His sense of taste was al...
53 Motility DisturbancesLarry P. Frohman, MDA 27-year-old healthy man presented for neuro-ophthalmologic evaluation for recurrent vertical binocular diplopia. In 1982, he had a minor blow to the head, and 12 days later he sustained vertical binocular diplopia, worse on downgaze, without ever having diurnal variation. His sense of taste was al...
54 Motility DisturbancesLarry P. Frohman, MDA 27-year-old healthy man presented for neuro-ophthalmologic evaluation for recurrent vertical binocular diplopia. In 1982, he had a minor blow to the head, and 12 days later he sustained vertical binocular diplopia, worse on downgaze, without ever having diurnal variation. His sense of taste was al...
55 Motility DisturbancesLarry P. Frohman, MDA 27-year-old healthy man presented for neuro-ophthalmologic evaluation for recurrent vertical binocular diplopia. In 1982, he had a minor blow to the head, and 12 days later he sustained vertical binocular diplopia, worse on downgaze, without ever having diurnal variation. His sense of taste was al...
56 Systemic Disorders With Optic Nerve and Retinal FindingsLarry P. Frohman, MDCogan's syndrome is a vasculitic-autoimmune process with critical ocular features. In its original classical form it consists of nonluetic interstitial keratitis, with audiovestibular dysfunction, often with pain. More recently, it has been pointed out that there are nonclassical forms as well, part...
57 Neuro-Ophthalmic Vascular DiseaseMitchell J. Wolin, MDCarotid cavernous fistulas (CCFs) are connections between the arterial blood flow from the carotid artery system and the cavernous sinus. CCFs may be direct high-flow fistulas or indirect low-flow fistulas. Most direct CCFs are due to trauma. Enlargement of the superior ophthalmic vein may be demons...
58 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.
59 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.
60 Ocular Manifestations of Congenital/Inherited DiseasesSteven Galetta, MDThis patient is a 40-year-old man with a history of abetalipoproteinemia (Bassen-Kornweig syndrome), diagnosed at age 9. Neurologic complications have included ataxia, retinal degeneration, peripheral neuropathy, progressive leg weakness, dysarthria, and intermittent bladder incontinence. On his neu...
61 Ocular Manifestations of Congenital/Inherited DiseasesSteven Galetta, MDThis patient is a 40-year-old man with a history of abetalipoproteinemia (Bassen-Kornweig syndrome), diagnosed at age 9. Neurologic complications have included ataxia, retinal degeneration, peripheral neuropathy, progressive leg weakness, dysarthria, and intermittent bladder incontinence. On his neu...
62 Ocular Manifestations of Congenital/Inherited DiseasesSteven Galetta, MDThis patient is a 40-year-old man with a history of abetalipoproteinemia (Bassen-Kornweig syndrome), diagnosed at age 9. Neurologic complications have included ataxia, retinal degeneration, peripheral neuropathy, progressive leg weakness, dysarthria, and intermittent bladder incontinence. On his neu...
63 Motility DisturbancesLarry P. Frohman, MDA 27-year-old healthy man presented for neuro-ophthalmologic evaluation for recurrent vertical binocular diplopia. In 1982, he had a minor blow to the head, and 12 days later he sustained vertical binocular diplopia, worse on downgaze, without ever having diurnal variation. His sense of taste was al...
64 Motility DisturbancesLarry P. Frohman, MDA 27-year-old healthy man presented for neuro-ophthalmologic evaluation for recurrent vertical binocular diplopia. In 1982, he had a minor blow to the head, and 12 days later he sustained vertical binocular diplopia, worse on downgaze, without ever having diurnal variation. His sense of taste was al...
65 Isolated Optic Neuritis/NeuropathyMichael Wall, MDThis 36-year-old man noticed blurry vision in his right eye when attempting to sight a gun. He also reported bifrontal headaches responsive to aspirin. Acuity was 20/50 OD, 20/13 OS. His right eye could be refracted to 20/20 with a +2.50 sphere. A 0.3 right relative afferent pupillary defect was pre...
66 Isolated Congenital Optic Disc AnomaliesJoel M. Weinstein, MDThis 8-year-old boy presented with a 2-week history of decreased vision in the right eye. He had undergone a normal MRI and CSF examination, including intracranial pressure, before neuro-ophthalmologic assessment. The fundus photographs and fluorescein angiograms show subretinal neovascularization a...
67 Ocular Manifestations of Congenital/Inherited DiseasesSteven Galetta, MDThis patient is a 40-year-old man with a history of abetalipoproteinemia (Bassen-Kornweig syndrome), diagnosed at age 9. Neurologic complications have included ataxia, retinal degeneration, peripheral neuropathy, progressive leg weakness, dysarthria, and intermittent bladder incontinence. On his neu...
68 Ocular Manifestations of Congenital/Inherited DiseasesSteven Galetta, MDThis patient is a 40-year-old man with a history of abetalipoproteinemia (Bassen-Kornweig syndrome), diagnosed at age 9. Neurologic complications have included ataxia, retinal degeneration, peripheral neuropathy, progressive leg weakness, dysarthria, and intermittent bladder incontinence. On his neu...
69 Ocular Manifestations of Congenital/Inherited DiseasesSteven Galetta, MDThis patient is a 40-year-old man with a history of abetalipoproteinemia (Bassen-Kornweig syndrome), diagnosed at age 9. Neurologic complications have included ataxia, retinal degeneration, peripheral neuropathy, progressive leg weakness, dysarthria, and intermittent bladder incontinence. On his neu...
70 Neuro-Ophthalmic Consequences of TherapyJoel M. Weinstein, MDThe patient is a 38-year-old woman with oligodendroglioma of the left cerebral hemisphere. The patient received intracarotid BCNU and developed BCNU retinopathy, optic neuropathy, and partial ophthalmoplegia. Image 92_21is from 1 month later and demonstrates hemorrhagic swelling of the disc, retinal...
71 Isolated Congenital Optic Disc AnomaliesAnthony C. Arnold, MDThis patient has known pseudoxanthoma elasticum (an uncommon elastic tissue disorder characterized by plaque-like skin folds [plucked chicken skin] and degeneration of collagen fibers involving multiple systems, including the GI tract and heart), angioid streaks, and optic disc drusen.
72 Ocular Manifestations of Congenital/Inherited DiseasesWilliam Fletcher Hoyt, MDImage shows a patient with the Laurence-Moon-Biedl syndrome, an autosomal recessive syndrome characterized by obesity, mental retardation, retinitis pigmentosa (see Image 91_07), polydactyly, and hypogonadism. Pair with 91_07.
73 Chiasmal SyndromesMitchell J. Wolin, MDThe patient is a 60-year-old woman with a chief complaint of decreased vision. In 1986 she was diagnosed with a poorly differentiated breast cancer in her left breast. She underwent mastectomy, and all nodes were negative. She did well until 1991, when she was found to have a chest wall mass. This m...
74 Chiasmal SyndromesMitchell J. Wolin, MDThe patient is a 60-year-old woman with a chief complaint of decreased vision. In 1986 she was diagnosed with a poorly differentiated breast cancer in her left breast. She underwent mastectomy, and all nodes were negative. She did well until 1991, when she was found to have a chest wall mass. This m...
75 Chiasmal SyndromesMitchell J. Wolin, MDThe patient is a 60-year-old woman with a chief complaint of decreased vision. In 1986 she was diagnosed with a poorly differentiated breast cancer in her left breast. She underwent mastectomy, and all nodes were negative. She did well until 1991, when she was found to have a chest wall mass. This m...
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