1.! Yes I do. 2.! No I don t. COPE Approved: COPE # PD! " !! What is electrodiagnostics testing? !! Visual Pathway Basic Understanding !!
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1 1.! Yes I do 2.! No I don t Nathan Lighthizer, O.D., F.A.A.O Assistant Professor, NSUOCO Chief of Specialty Care Clinics Chief of Electrodiagnostics Clinic COPE Approved: COPE # 3132-PD #$ #$! " 1.! Monthly basis 2.! Quarterly basis 3.! Yearly basis 4.! Never %& %& %& %&! " # $!! What is electrodiagnostics testing?!! Visual Pathway Basic Understanding!! ERG "! Full field flash "! Pattern "! Focal!! EOG!! VER!! Clinical Cases!! Upstream Photoreceptors Mid-retinal layers Ganglion cell layer NFL/Optic Nerve Optic Chiasm Optic Tract!! Many different types of ERG s "! Full field flash ERG "! Pattern ERG "! Focal ERG "! Multifocal ERG!! Downstream LGN Visual Cortex $&
2 1.! Photoreceptors 2.! RPE layer 3.! Ganglion cell layer 4.! Nerve fiber layer & optic nerve.! Entire visual pathway &' &' &' &' &'! " # $ % From Kaufman and Alm, Adler's Physiology of the Eye, 23!! Full field flash ERG "! Measures the summed response of all photoreceptors (rods & cones)* "! 2 main components of the clinically recorded ERG 1.! Negative A-wave!! photoreceptors 2.! Positive B-wave!! mid-retinal layers **ganglion cells do not contribute**!! Procedure "! Dilate "! Dark adapt for 3 minutes "! Hook up the wires!! Ear clip ground electrode!! Forehead electrode reference electrode!! CL electrode measuring electrode Step 1 Step 2!! steps/tests in the full field flash ERG Scotopic ERG 1.! Dark-adapted dim flash (rod response) Photopic ERG 2.! Dark-adapted bright flash (maximal rod-cone response still mostly rods) 3.! Dark-adapted oscillatory potentials 4.! Light-adapted bright flash (single-flash cone response).! Light-adapted flicker (cone flicker response) Darkadapted (Scotopic) ERG Step 3 %&
3 !! Pattern ERG "! Measures the inner retinal layers!! Indicates diseases of the inner retina/ganglion cell layer/optic nerve Step 4 Step!! VER & ERG are most often done before perg would be done "! Normal brain/orbit imaging, normal full-field ERG, abnormal VER -> pattern ERG may be indicated!! NOT dilated need best correction*!! Pattern ERG "! Placement of electrodes!! Ear clip ground electrode!! Contralateral ear clip reference electrode!! CL electrode measuring electrode!! Assessment of the RPE!! Arden ratio Light peak Dark Trough Normal Arden ratio > 1.8 Abnormal Arden ratio < 1.6!! Don t have to be dilated!! Long, boring procedure for both patient and clinician "! Electrode placement!! Ground electrode Ear!! Measuring electrodes!! Each of the 4 canthi "! 3 minute test!! minutes pre-adaptation!! 1 minutes in the dark!! 1 minutes in the light #&
4 !! Takes about 4 minutes "! Pt s hate it!! Only used when relevant diagnostically "! Not very often "! Best s disease!! Normal ERG & Abnormal EOG!! AKA Visually Evoked Potential (VEP) "! Flash vs. Pattern!! Measures the entire visual pathway "! From cornea to occipital lobe!! 3 electrodes "! Ground -> earlobe "! Reference -> forehead "! Measuring -> occipital lobe!! 1 above inion!! NOT dilated need best correction!! Very useful in: "! Unexplained vision loss "! Malingering "! Psychogenic vision loss "! MS/optic neuritis!! However, usually will not point to a specific diagnosis "! Just tells you there is a problem somewhere in the visual pathway!! 37 yof!! Referred in for electrodiagnostics testing due to RPE changes within central macula OU!! Pt notes mildly decreased vision for past decade or so that seems to have worsened significantly in past year!! Significant sensitivity to lights '&
5 !! BCVA "! OD > 2/3- "! OS > 2/4+!! Color vision "! Severe color vision deficit OD "! Moderate color vision deficit OS!! Anterior segment, IOP s, and peripheral retinal exam unremarkable OU 1.! Full field flash ERG 2.! Pattern ERG 3.! EOG 4.! VER %& %& %& %&! " # $ (&
6 !! Full field flash ERG "! Normal rod response OU "! Severely reduced cone response OD "! Moderately reduced cone response OS!! Diagnosis: "! Cone Dystrophy OD>OS!! Treatment: "! Not a whole lot can be done "! Sun protection to help with photophobia "! Genetic testing!! Sporadic & autosomal dominant inheritance!! Onset "! 1 st 3 rd decade of life!! Classic Triad "! Decreased central vision "! Decreased color vision "! Photophobia!! Also "! Central visual field defects!! Fundus: "! Fairly normal looking "! Macular granularity/pigment mottling "! Bulls eye macular lesion "! Geographic atrophy of the macula!! DDx: "! Congenital color blindness/rod monochromatism "! ARMD "! Stargardts "! Chloroquine/Hydroxychloroquine maculopathy!! Tx: "! Tinted glasses/sunglasses "! Genetic counseling "! Low vision aids!! Prognosis: "! 2/3 2/4 "! Worse if rods become involved "! Age of patient!! 21 yom!! Family history of RP "! Grandfather blind at 2 years old "! Cousin with Usher s syndrome!! Decreased peripheral vision!! Decreased detail at night!! Discharged from military due to vision!! Tour in Baghdad )&
7 !! BCVA "! OD -> 2/4-2 "! OS -> 2/3-1!! Anterior segment & IOP s normal OU 1.! Full field flash ERG 2.! Pattern ERG 3.! EOG 4.! VER %& %& %& %&! " # $!! Full field flash ERG "! Flat-lined rod & cone responses to all stimuli OD & OS *&
8 !! Diagnosis: "! Retinitis Pigmentosa (RP)!! Treatment: "! Not a whole lot can be done "! Genetic testing "! Vitamin A???!! Autosomal recessive, autosomal dominant, X-linked inheritance, sporadic "! Age of onset, rate of progression & amount of eventual visual loss are often related to the inheritance!! Hits the rods first "! Decreased night vision & peripheral vision!! Eventually gets the cones!! Classic Triad "! Bone Spicules in the mid-periphery/periphery "! Arterial attenuation "! Waxy disc pallor!! Also "! CME & PSC s late!! Diagnosis: "! Clinical picture "! ERG!! DDx: "! Stationary rod-cone/cone-rod dysfunction "! CAR/MAR "! Drug toxicity (Phenothiazine)!! Tx: "! No cure available "! Vitamin A Palmitate 1, IU???? "! DHA??? "! Gene therapy "! Cell transplantation!! Prognosis "! Extremely variable!! Upstream Photoreceptors 1.! Normal 2.! Abnormal 3.! Impossible to know for certain Mid-retinal layers Ganglion cell layer NFL/Optic Nerve Optic Chiasm Optic Tract!! Downstream LGN $% $% $% Visual Cortex! " #!&
9 !! 18 yof!! H/O hot grease splattering in her eyes and face while working at a restaurant.!! Pt reports can t see out of right eye since!! Also reports been having really bad HA s since accident as well. 1/1 pain!! Pt reports no medical concerns!! BCVA "! OD -> 2/1- to 2/2 "! OS -> 2/2-!! (-) APD!! Color vision: "! Too blurry to determine any colors OD!! Cornea -> clear!! AC -> D&Q!! Lens -> clear 1.! Full field flash ERG 2.! Pattern ERG 3.! EOG 4.! VER VER OD %& %& %& %&! " # $ VER OS +&
10 !! Pattern VER "! Normal responses to all check sizes down to 2/3 stimulus OD & OS!! Dissociated prism test "! 12 p.d. BO in front of the R eye "! 6 p.d. BU in front of the L eye!! Diagnosis: "! Malingering "! Psychogenic Vision Loss!! Treatment: "! Pt education "! Behavioral/Psych Consult!! 23 year old Male!! Referred in for VER & ERG!! Entering VA s "! OD -> Count 2 feet "! OS -> 2/3!! Pupils, EOM s, Confrontational fields normal OU $,&
11 1.! Toxic/nutritional optic neuropathy 2.! Cone dystrophy 3.! Glaucoma 4.! Malingering/pt is lying.! Leber s hereditary optic neuropathy 6.! Brain tumor / compressive optic neuropathy '( '( '( '( '( '(! " # $ % &!! Upstream Photoreceptors Mid-retinal layers Ganglion cell layer NFL/Optic Nerve Optic Chiasm Optic Tract!! Downstream LGN Visual Cortex 1.! Toxic/nutritional optic neuropathy 2.! Cone dystrophy 3.! Glaucoma 4.! Malingering/pt is lying.! Leber s hereditary optic neuropathy 6.! Brain tumor / compressive optic neuropathy '( '( '( '( '( '(! " # $ % & $$&
12 !! Diagnosis: "! Leber s Hereditary Optic Neuropathy (LHON) OU!! Plan: "! Blood drawn & sent to the NEI for genetic testing as part of the EyeGene program "! Low vision referral at NSU!! 1-3 year old males!! Mitochondrial DNA mutation from the mother!! Symptoms: "! Rapidly, progressive painless vision loss in one eye, and then the other!! Signs: "! Optic nerve head pallor "! Telengiectatic vessels around the nerve!! Treatment: "! Low vision "! Idebenone (Catena)!! Upstream Photoreceptors Mid-retinal layers Ganglion cell layer NFL/Optic Nerve Optic Chiasm Optic Tract!! Downstream LGN Visual Cortex VER 1.! Malingering 2.! Psychogenic Vision Loss 3.! Unexplained decreased vision 4.! Gross assessment of VA in a non-verbal patient.! MS/Optic Neuritis FLASH ERG 1.! RP & its variants 2.! Cone dystrophies & Rod monochromat 3.! Symptoms: "! Night blindness "! Restricted peripheral fields "! Color vision deficits EOG 1.! Best s disease! Going to do ERG first $%&
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