Neuro-ophthalmology Marek Michalec, MD. http://flylib.com/books/3/283/1/html/2/9%20-%20neuro-ophthalmology_files/c9ff1.png Anisocoria- unequal pupil sizes http://www.cascadilla.com/eyecharts/traditional/images/poster-traditional.jpg https://visionhelp.files.wordpress.com/2011/04/vep-waveform.jpg Neuro-ophthalmology •Study integrating ophthalmology and neurology •Disorders affecting parts of CNS devoted to vision or eye: •Afferent system (visual pathway, incl. optic nerve) •Efferent system (ocular motor control, pupillary function) Part I •Neuro-ophthalmologic •Examination Examination •History •Eye examination (visual acuity, tonometry, anterior segment examination, funduscopic examination) •Perimetry •Color vision, contrast sensitivity, electrophysiology (ERG, VEP) •MRI of brain, •Neurologic examination Visual acuity •Each eye separately •Distance and near vision •Using of corrective lenses, pinhole •Using Snellen chart (20 feet) – normal 20/20 •Count fingers, hand motion, light perception, no light perception http://www.color-blindness.com/wp-content/images/Ishihara-Plate-36-38.jpg http://www.michaelgaigg.com/blog/images/color-vision-test-07.jpg http://jaxairnews.jacksonville.com/sites/default/files/imagecache/superphoto/editorial/images/image s/mdControlled/cms/2011/07/13/856430181.jpg Color vision • • •Each eye separately •Comparison between eyes •Examination: •pseudoisochromatic plates (Ishihara) •100 Hue test (Farnsworth-Munsell) • Farnsworth-Munsell 100 Hue test •Ordering the color tiles as patient sees it http://glimpsejournal.com/public/journals/1/images/2.3-Jameson-Kimberly-A-Human-Potential-for-Tetra chromacy_clip_image006_0000.jpg https://farm4.staticflickr.com/3241/2437831432_b3958e2a34_b.jpg Contrast sensitivity •Examining spatial frequency •Decreased in some optic nerve disorders (typically optic neuritis) https://www.good-lite.com/cw3/assets/product_full/500051_lg.png http://omicsonline.org/JCEOimages/2155-9570-3-254-g003.gif Perimetry •To assess the quality of visual field •Characteristic visual field defect =location of possible intracranial lesions http://elketabalmokadas.files.wordpress.com/2012/11/visual-field-defects-diagram.jpg Perimetry •Automated static perimetry • • • http://firsteyecarewestplano.com/wp-content/uploads/2013/05/Bailey_Plano_AdvTest_7313.jpg http://www.ijo.in/articles/2011/59/7/images/IndianJOphthalmol_2011_59_7_53_73694_u3.jpg Perimetry •Goldmann kinetic perimetry • http://img.medicalexpo.com/images_me/photo-m2/ophthalmic-perimeter-ophthalmic-examination-kinetic-p erimetry-70788-161847.jpg http://www.yamout.co/wp-content/uploads/2012/07/vfgoldmann.gif Electrophysiologic examination •ERG = Electroretinography •Access possible functional pathology of retina (scotopic, photopic and central part) •Flash ERG (activity of bipolar cells as an answer to stimation of photosensitive cells – rods, cones) •Pattern ERG (activity of gaglionar cell as a response to stimulation of cones in macula) •VEP = Visual evoked potentials (responses) •Access the capability of anterior visual pathways –optic nerve •Major use: diagnosis/confirm of optic neuritis Electrophysiologic examination http://www.stormoff.ru/en/data/catalog/1288085865_RETI-compact%20P%20(PVEP,%20PERG,%20Flash%20VEP,% 20ERG,%20on-off%20ERG,%20EOG,%20Glaucoma%20Screening%20via%20PERG).JPG Electroretinography • http://www.intechopen.com/source/html/17262/media/image2.png Visual evoked potentials • http://www.oculist.net/downaton502/prof/ebook/duanes/graphics/figures/v8/1050/004f.gif Multifocal ERG, Multifocal VEP •Mostly experimental use, not standard in clinical medical practice here http://extras.springer.com/2007/978-3-540-32706-6/html/fig/fullres/fig_07_03.jpg Part II •Pathology of Afferent system Afferent system •Retina (cones, rods, bipolar and ganglion cells) •Optic nerve •Optic chiasm •Optic tract •Lateral geniculate body •Optic radiation •Visual cortex (V1 = Brodmann area 17) • http://upload.wikimedia.org/wikipedia/commons/8/84/1543%2CVesalius%27Fabrica%2CVisualSystem%2CV1.jp g Pathologies of Afferent Visual System •Papilledema • •Optic Neuritis • •Optic Neuropathy • •Optic Atrophy Papilledema •Not a disease - sing secondary due to elevated intracranial pressure (ICP) •Unspecific sign •Require immediate diagnosis = increased ICP is a life-threatening situation!!! •60% of cases = increased ICP caused by intracranial tumor!!! •Other possible causes: hydrocephalus, meningitis, encephalitis, brain abscess... Papilledema •Clinical picture •Early •Margins are obscured •Optic cup initially preserved •Hyperemic disc •Acute •Elevation of disc •Radial hemorrhages •Grayish-white exudates •Chronic •Disc edema •Obiterated optic cup • • http://upload.wikimedia.org/wikipedia/commons/1/1a/Papilledema.jpg http://avserver.lib.uthsc.edu:8080/Medicine/eye_exam/PapillededemaWeb.jpg Optic neuritis •Inflammation of the optic nerve •Intraocular – within the globe •Retrobulbar – posteriot to the globe •Usually unilateral •Tendency to repeat •Etiology •Often associated with multiple sclerosis (MS) = demyelinating optic neuritis (20% = first sign of MS) •Other possible inflammatory causes: Lyme disease, syphilis, inflammation from orbit, paranasal sinuses... • Optic neuritis •Symptoms •Sudden vision loss within several hours (mild blurring/light perception) •Central, paracentral scotoma •Retrobulbar/parabulbar pain •Present afferent pupillary defect •Prognosis •depends on underlying disorders •MS = usually good – significant spontaneous improvement (several weeks) •Some permanent disturbances of vision are possible (color vision decreasing, scotoma) • Anterior Ischemic Optic Neuropathy •Etiology •Acute disruption of blood supply (due to vascular changes, infarction) •Symptoms •Sudden unilateral loss of vision •Altitudinal or wedge-shaped visual field defect •Present afferent pupillary defect •Clinical picture •Edema of optic disc •Segmental obscuration of margins (correlation with visual field defect) Anterior ischemic optic neuropathy •2 forms •Benign: Nonarteritic AION •Malign: Arteritic AION • •Arteritic AION •Association with systemic vasculitis (giant cell arteritis) •Diagnosis: sedimentation rate, biopsy of temporal artery •High risk of affection of contralateral (fellow) eye within days/ weeks!!! •Need for immediate therapy with high dose intravenous corticoids!!! • D:\slides\Neuropathies 14.JPG AION forms Arteritic form Non-arteritic form % of cases AION 10 % 90% age 70 years 60 years Sex Female > male Female = male Systemic disease association Giant cell arteritis (Horton disease) idiopathic Prognosis Very rare mild Fellow eye affection often (50-90%) rare (10-20%) Diagnostics: Sedimentation (FW) Very high normal treatment High dosage of systemic corticoids Not available Optic Atrophy •Irreversible loss of axons as a result to damage of optic nerve •Etiology •Primary due to trauma, direct pressure by tumor •Secondary due to affection of optic nerve (optic neuritis...) •Glaucomatous due to glaucomatic damage •Pathogenesis •Ascending - lesion located anterior to the lamina cribrosa •Descending – lesion located posteriot to the lamina cribrosa http://www.reviewofoptometry.com/CMSImagesContent/2011/11/030_RO1111_F1.gif Optic Atrophy •Clinical picture •Total/partial pale optic disc •Well defined / blurred margins •Constricted / reduced retinal vessels •Etiology •Vascular (AION, RAO) •Inflammation (optic neuritis, neuroinfections) •Compressive (orbital/intracranial mass) •Traumatic (avulsion, bone fracture) •Toxic (methyl alcohol, various poisons, cytostatics) •Congenital/hereditary (LHON, Kjer atrophy) •Systemic (hematooncological diseases) • https://c2.staticflickr.com/8/7254/7602735410_622dcf9a80_m.jpg Part III •Pathology of Efferent system Efferent system •1) Cranial neuropathies (III, IV, VI) • •2) Pupillary abnormalities http://static.laramyk.com/wp-content/uploads/2010/05/extraocular-muscles.jpg Eye movement •Ocular motility – produced by extraocular muscles •4 rectus muscles (lateral, medial, superior, inferior) •2 oblique muscles (superior, inferior) • Výsledok vyhľadávania obrázkov pre dopyt extraocular muscles Cranial neuropathies •Signs •Oculomotor nerve palsy •Diplopia •Multiple muscle paralysis •Ptosis •Anisocoria •Trochlear nerve palsy •Vertical diplopia •Abnormal head tilt •Abducens nerve palsy •Horizontal diplopia in the gaze palsy • Cranial neuropathies •Etiology •Ischemic (diabetes, hypertension, hyperlipidemia) •Demyelinating disease (MS) •Compressive (tumor, aneurysm) •Elevated ICP • •Multiple cranial neuropathies = suspect lesion in the posterior orbit or cavenrous sinus region Pupil •Miosis – parasympathetic nervous system •Mydriasis – sympathetic nervous system https://innerpowerdojo.files.wordpress.com/2014/03/pupils-nervous-system-control-e1394551420977.png ?w=604 • http://vmerc.uga.edu/CranialNerves/2/2a-M2-Pup%20constrict-illus/Consensual/2a-M2-PupConstrict-Cons ensual-illus-4.jpg Sympathetic pathway • http://www.ib.cnea.gov.ar/~redneu/2013/BOOKS/Principles%20of%20Neural%20Science%20-%20Kandel/gatewa y.ut.ovid.com/fulltextservice/ct%7B06b9ee1beed594190674f1983457a7dd32af6a0d5a4c9892~53/da7c45ff14.g if.png Pupillary abnormalities •Anisocoria •inequality of pupil size •May be physiologic •Possible accidental discovery •May be isolated / associated with eyelid or ocular motility abnormalities •Diagnosis •Direct shine at pupil •Test near response (miosis with accomodation) •Pupil sizes in light and dark • Horner’s Syndrome •Signs •Miosis (pupil does not dilate in dark) •Ptosis •Pseudo-enophthalmus •Anhidrosis (diminished sweating) •Heterochromia (if congenital) •Etiology •Trauma, internal carotid artery dissection, brain stem strokes, MS, brain tumor, syringomyelia, apical lung tumor, goiter, thyroid carcinoma... • http://upload.wikimedia.org/wikipedia/commons/thumb/9/9d/Example_of_Horner%27s_syndrome_in_a_cat.jp g/800px-Example_of_Horner%27s_syndrome_in_a_cat.jpg https://encrypted-tbn0.gstatic.com/images?q=tbn:ANd9GcQHabT_WUPnITWnnyi7vAI09yVLpzqk_1jkIlY-7N5B1gJ Vz6HF http://amaprod.silverchaircdn.com/data/Journals/OPHTH/6958/esc05003f3.png Adie’s Pupil •Signs •No present / slow miosis to light •Present miosis to accomodation •Pupil is larger with light/near dissociation •Etiology •Inflammation (viral or bacterial infection) •Therapy •Pilocarpine drops, thoracic sympathectomy Thank you for your attention!