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Neuro-Ophthalmology Celia H. Chang MD Department of Neurology MIND Institute University of California, Davis, Health System [email protected] Lecture Content Disorders of optic nerve and retina Chiasmal and retrochiasmal disorders Pupil disorders Motility disorders 2 Question #1 A 65-year-old woman has abrupt onset of visual loss in the right eye and weakness in the left arm and leg. There is some tingling of the left hand and left corner of her mouth. She is most likely to have a plaque in which blood vessel: A. Anterior cerebral artery B. Basilar artery C. Internal carotid artery D. Middle cerebral artery E. Posterior cerebral artery Question #2 A 35 year old woman woke with double vision. She reports that it gets better if she covers one eye. On exam: when looking to the left, her left eye has nystagmus to the left and her right eye does not move past the midline. She is able to look right without difficulty. She has normal vertical eye movements and is able to converge. The rest of her neurologic exam is normal. The most likely diagnosis is: A. Cavernous sinus thrombosis B. Midbrain lacune C. Multiple sclerosis D. Myasthenia gravis E. Thyroid ophthalmopathy Question #3 A 23 year old woman has the following visual field deficits. Where is the lesion? A. Optic radiation in the temporal lobe (Meyer's loop) B. Optic nerve at the chiasm C. Optic radiation in the parietal lobe D. Lateral geniculate nucleus E. Calcarine cortex of the occipital lobe Question #4 A 60 year old man is unable to report seeing a comb and a fork at the same time even when the 2 objects are held next to each other. He also has poor coordination of hand and eye movements, and difficult tracking objects visually. What is the most likely diagnosis: A. Holmes-Adie syndrome B. Balint syndrome C. Gerstmann syndrome D. Parinaud’s syndrome E. Malingering Question #5 A 54 year old man has double vision. On exam, the left eye is deviated down and out. He also has ptosis and mydriasis. What is the most likely diagnosis: A. Myasthenia gravis B. Guillain-Barré syndrome C. Posterior communicating artery aneurysm D. Kearns-Sayre syndrome E. Horner syndrome Disorders of Optic Nerve and Retina Drusen Optic neuritis Papilledema Retinal emboli Retinal infarcts 8 9 dralbertlin.com/resources/_wsb_326x326_OpticNerveNml01.jpg www.google.com/imgres?imgurl=http://webeye.ophth.uiowa.edu/eyeforum/atlas/photos/OND2.jpg&imgrefurl=http://webe 10 ye.ophth.uiowa.edu Optic Nerve Drusen Refractile bodies Frequently present at the nasal margin. Visual field defects are common in patients Afferent pupillary defect in asymmetric cases 11 12 www.eyes2health.com/images/optic%20neuritis.jpg 13 www.uiowa.edu/~c064s01/nr122%20copy.jpg 14 www.eyeweb.org/atlas/images/papilledema.jpg 15 www.kellogg.umich.edu/theeyeshaveit/acquired/images/papilledema.jpg Pseudotumor Cerebri Symptoms Headache Papilledema Stabismus Evaluate Brain MRI with MRA and MRV Lumbar puncture with opening pressure 16 Treatment 17 en.wikipedia.org/wiki/File:Gray881.png 18 http://www.insightseyecare.net/EyeEducation/retinaNeuronalLayers.jpg 19 img.medscape.com/pi/emed/ckb/radiology/336139-417524-5003.jpg This refractile, yellow fleck is a platelet- fibrin-cholesterol retinal embolus called a Hollenhorst plaque 20 www.google.com/imgres?imgurl=http://www.kellogg.umich.edu 21 uuhsc.utah.edu/moraneyecenter/opatharch/images/retina/22030.jpg Chiasmal and Retrochiasmal Disorders 22 23 en.wikipedia.org/wiki/Visual_system 24 fc.units.it/ppb/neurobiol/Neuroscienze%20per%20tutti/vispath.gif 25 www.jeffmann.net/NeuroGuidemaps/visualfields.gif 26 www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cm&part=A3543 Autonomic Control of Pupils Target Sympathetic Parasympathetic Pupil dilator Alpha 1 contracts M 3 relaxes muscle • Mydriasis • Miosis Ciliary muscle Beta 2 relaxes M 3 contracts • Long range • Short range focus focus 27 Pupil Disorders Afferent pupillary defect (APD) Horner Tonic Third nerve palsy 28 Marcus-Gunn Pupil or Afferent Pupillary Defect Optic neuritis Ischemic optic neuropathies Glaucoma Traumatic optic neuropathy Radiation optic nerve damage Miscellaneous optic neuropathies, such as Leber's optic neuropathy 29 Ischemic retinal disease Marcus-Gunn Pupil or Afferent Pupillary Defect Retinal detachmentif the macula or at least two quadrants of retina Severe macular degeneration Not with cataract 30 Argyll Robertson Pupil Constrict when the patient focuses on a near object (they “accommodate”) Do NOT constrict when exposed to bright light Neurosyphilis 31 32 www.ajnr.org/cgi/content/full/23/6/929/F1 Horner’s Syndrome Ptosis (superior tarsal muscle or Müller's muscle) Miosis Anhydrosis Dilation lag Enophthalmos Iris hypopigmentation in congenital Horner’s due to interference with melanin pigmentation Lesion of cervical or thoracic sympathetic chain Pancoast tumor (apical lung tumor) 33 34 www.jeffmann.net/NeuroGuidemaps/Horners.gif Testing in Horner’s Syndrome Cocaine test Blocks the reuptake of norepinephrine Normal pupil dilates Horner’s: no dilation Apraclonidine (alpha antagonist) reversal of miosis on affected side Paredrine test Hydroxyamphetamine causes neurotransmitter vesicle release if 3rd order neuron is intact which results in mydriasis 35 Holmes-Adie Syndrome or Ross’s Syndrome Mydriasis Loss of deep tendon reflexes Diaphoresis Accommodative paresis Photophobia Ciliary and spinal ganglia 1 /8% pilocarpine test 36 37 img.medscape.com/pi/emed/ckb/ophthalmology/1189694-1198462-92.jpg Third Nerve Palsy Down and out (infraducted and abducted) Ptosis (levator palpebrae some bilateral innervation) Mydriasis (iris sphincter from ipsilateral Edinger-Westphal subnucleus) Posterior communicating artery aneurysm 38 39 http://www.nature.com/eye/journal/v20/n10/images/6702376f10.jpg 40 www.netterimages.com/images/vpv/000/000/006/6994-0550x0475.jpg Motility Disorders Abnormal movement Nystagmus Other Cranial nerve III, IV, VI paresis Parinaud’s Supranuclear gaze palsy Internuclear ophthalmoplegia One and a half syndrome 41 Nystagmus Periodic rhythmic ocular oscillation of the eyes Equal amplitude and velocity (pendular nystagmus) Slow initiating phase and a fast corrective phase (jerk nystagmus) Horizontal nystagmus Vertical nystagmus Rotary Congenital nystagmus 42 Acquired nystagmus Other Abnormal Eye Movements Ocular bobbing Ocular flutter Opsoclonus 43 44 img.medscape.com/pi/emed/ckb/clinical_procedures/79926-1412901-1189799-1585126.jpg 45 www.pedseye.com/img/hyp_ex_02.jpg 46 legacy.revoptom.com/handbook/images/62a.jpg Paresis of Individual Ocular Muscles Graves disease Orbit pathology Cavernous sinus pathology Myasthenia gravis Guillain-Barré syndrome Mitochondrial disease Myopathies 47 48 webhard.gnu.ac.kr/WebLink/limbh/data/fig/no_parinaud.jpg Parinaud’s or Dorsal Midbrain Syndrome Paralysis of voluntary upgaze Accommodative paresis Convergenceretraction nystagmus Eyelid retraction (Collier’s sign) Conjugate down gaze in the primary position 49 Pineal mass Progressive Supranuclear Palsy Gait and balance problems Difficulty with downgaze Mood and behavior problems Depression and apathy Dementia 50 51 www.hmc.org.qa/qmj/qmj_nov_2006/images/Image_QMJ_2006/Fig12_63.jpg 52 http://www.google.com/imgres?imgurl=http://umed.med.utah.edu/ Internuclear Ophthalmoplegia Lack of conjugate adduction in the ipsilateral MLF Accommodation intact Multiple sclerosis Other pontine lesions such as CVA 53 54 http://www.google.com/imgres?imgurl=http://umed.med.utah.edu Where pathway 55 What pathway http://en.wikipedia.org/wiki/File:Ventral-dorsal_streams.svg Cortical Vision Disorders Prosopagnosia Balint syndrome Inability to Triad of: recognize familiar Simultagnosia faces Optic ataxia Visual apraxia Right occipitotemporal Bilateral parietal lobe lobe lesions 56 References/Resources www.eyeweb.org emedicine.medscape.com/ophthalmology umed.med.utah.edu Basics in neuro-ophthalmology by Kathleen B. Digre, MD Using the ophthalmoscope and viewing the optic disc by Kathleen B. Digre, MD www.jeffmann.net/Neuroguidemaps uuhsc.utah.edu www.kellogg.umich.edu www.richmondeye.com 57 Questions and Answers 58.
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