Jonathan D Trobe MD Professor of Ophthalmology and Neurology University of Michigan EXAMINER DONT LET ME DOWN I DEPEND ON YOU MACHINE YOU ASK TOO MUCH OF ME YOU DO NOT EXPECT MRI TO BE ABNORMAL ID: 1042579
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1. Neurologic Visual NuancesJonathan D. Trobe, MDProfessor of Ophthalmology and NeurologyUniversity of Michigan
2. EXAMINER:“DON”T LET ME DOWN.I DEPEND ON YOU!”
3. MACHINE:“YOU ASK TOO MUCH OF ME.”
4. YOU DO NOT EXPECT MRI TO BE ABNORMAL*Toxic optic neuropathyNutritional optic neuropathyInfiltrative (cancerous) optic neuropathyIndirect optic nerve trauma*At least acutely! Thinning (atrophy) and high T2 signal often appear late
5. EXAMINER:“WHY CAN’T YOU BE MORE SENSITIVE?”
6. MACHINE:“I CANNOT ALWAYS MEET YOUR VERY HIGH EXPECTATIONS!”
7. YOU EXPECT MRI TO BE ABNORMAL, BUT IT IS SOMETIMES NORMAL1. Retrobulbar neuritis2. Hypoxic-ischemic encephalopathy 3. Nonketotic hyperglycemia
8. Hypoxic-ischemic encephalopathy 45 year old man with spontaneous cardiac arrest*Recovers neurologic function but visual acuities hand movements OUEye exam normal*Parmar HA, Trobe JD. J Neuro-Ophthalmol 2016;36:264
9. Hypoxic-ischemic encephalopathyMRI Five Days After Cardiac ArrestDWIFLAIRMRI Four Months After Cardiac Arrest,(Visual Acuity Now 20/25 OU)FLAIR
10. Nonketotic Hyperglycemia68 year old man changes diabetes medication that leads to very poor control (blood sugar > 600 mg/dL)* Develops “intermittent photopsias and distorted vision”Visual acuities 20/50, 20/40; eye exam otherwise normal except…*Taban M, Naugle RI, Lee MS. Arch Ophthalmol 2007;125:845
11. Nonketotic HyperglycemiaNeuropsychologic exam normalMRI and single-photon emission computed tomography (SPECT) normal!Electroencephalogram not performedBlood glucose corrected and patient recovers completely within seven days*Taban M, Naugle RI, Lee MS. Arch Ophthalmol 2007;125:845
12. Nonketotic Hyperglycemia: Another Case With Normal MRI*Freedman KA, Polepalle S. Am J Ophthalmol 2004;137:1122
13. EXAMINER: “WHY CAN’T YOU BE MORE SENSITIVE?”
14. MACHINE: “I’M HERE FOR YOU.YOU JUST DON’T READ MY SIGNALS!”
15. MRI ABNORMALITIES SUBTLE AND OFTEN OVERLOOKED1. Hypoxic-ischemic encephalopathy2. Nonketotic hyperglycemia3. Lateral geniculate body ischemia, vasogenic edema (PRES)4. Occipital cerebritis5. Creutzfeldt-Jakob Disease6. Visual variant Alzheimer Disease
16. Hypoxic-ischemic encephalopathy:Very Mildly Abnormal MRI Overlooked16 year old boy with cardiac arrest in motor vehicle accident*Resuscitated and recovers neurologic function but says “I can’t see” Visual acuity 20/15 OU but visual fields*Margolin E et al J Neuro-Ophthalmol 2007;27:292
17. Hypoxic-ischemic encephalopathy:Very Mildly Abnormal MRI OverlookedFLAIRDWIMRI 11 Days After Resuscitation: Read as “Normal”T2Margolin E et al. J Neuro-Ophthalmol 2007;27:292
18. Hypoxic-ischemic encephalopathy:Abnormal MRI OverlookedVisual field loss persistsMargolin E et al. J Neuro-Ophthalmol 2007;27;292
19. Hypoxic-ischemic encephalopathy:Abnormal MRI Overlooked suscitatiionMargolin E et al J Neuro-Ophthalmol 2007;27:292Repeat MRI 60 Days After ResuscitationMRI 11 Days After Resuscitation
20. Nonketotic Hyperglycemia: Abnormal MRI Misinterpreted4 patients with NKH, blurred vision, and temporary homonymous hemianopias*2 of 4 no previously known diabetesEEG showing abnormalities in ALL: slowing in 2, spikes in 2 *Lavin PJM Neurology 2005;65:616
21. Nonketotic Hyperglycemia: Abnormal MRI MisinterpretedT2Post-contrast T1DWIADCDecreased T2, gyral enhancement, restricted diffusion*Lavin PJM Neurology 2005;65:616
22. Nonketotic Hyperglycemia: Abnormal MRI OverlookedMRI interpreted as focal encephalitis, tumor, or migraine Vision recovers within days of normalizing glucoseRepeat MRIs are normal*Lavin PJM Neurology 2005;65:616
23. Optic Tract Trauma:Abnormal MRI Overlooked45 year old man has closed head injury in motorcycle accident*Regains consciousness and appears neurologically normal, but claims no sight in left hemifieldNeuro-ophthalmology exam shows left homonymous hemianopia but no pupil or fundic abnormalitiesBrain MRI 2 months after accident reported as “normal” so I thought he was malingering!*Kowal KM J Neuro-Ophthalmol 2017;37:17
24. Optic Tract Trauma:Abnormal MRI OverlookedKowal KM J Neuro-Ophthalmol 2017;37:17
25. Where’s the MRI Abnormality?20 year old pre-medical student develops high-altitudinal sicknessEmerges from coma and notes poor vision in right hemifieldVisual acuity normal Right afferent pupil defect Bowtie disc pallor right, temporal pallor leftRight homonymous hemianopia Grabe H et al. J Neuro-Ophthalmol 2012;32:38
26. Where’s the MRI Abnormality?Non-contrast CTMR Contrast VenogramCT: lucencies in both thalami (black arrows),blood products at terminusof internal cerebral veins (white arrow)Non-opacification of both internalcerebral veins (arrow)
27. Where’s the MRI Abnormality?Axial spoiled gradient T1 shows encephalomalacia in region of left lateral geniculate body (arrow)
28. Lateral Geniculate Body PRES:Really?31 year old woman develops binocular blurred vision and headache in 38th week of gestation*Diagnosed with hemolysis, elevated liver enzymes and low platelets (HELLP syndrome) Treatment: anti-hypertensive agents and platelet transfusionsDelivered by C-section of healthy baby, but…*Stem M et al. J Neuro-Ophthalmol 2014;34:372
29. Lateral Geniculate Body PRES:Really?On 1st post-partum day, vision falls to finger counting OUNeuro-ophthalmological exam: bullous retinal detachments OUIn 48 hours, vision recovers to 20/25 OU but retinal detachments unchangedStem M et al. J Neuro-Ophthalmol 2014;34:372
30. Lateral Geniculate Body PRES:Really? Stem M et al. J Neuro-Ophthalmol 2014;34:372High FLAIR signal in lateral geniculate bodies bilaterally; no signal abnormality in optic radiation or cortex!
31. Isolated Lateral Geniculate Body Damage: Subtle MRI AbnormalitiesT1 Post-ContrastShock-induced Lefebvre PR et al. AJNR 2004;25:1544FLAIRIdiopathic microinfarct-induced Mudumbai RC. J Neuro-Ophthalmol 2007;27:169 Extrapontine myelinolysisDonahue, Kardon, ThompsonAJO 1996;119:378Post-contrast T1FLAIRH1N1 InfluenzaBreker DA et al J Neuro-Ophthalmol2015;35:265
32. Occipital Cerebritis:Is MRI Abnormal?15 year old girl gets headache, lassitude, mild nauseaComplains of blurred vision in inferior right hemifield and seeing “whirling ceiling fan” thereNeuro-ophthalmic exam normal except…
33. Occipital Cerebritis:Is MRI Abnormal?T2FLAIRPre-Contrast T1Post-Contrast T1Subtle high FLAIR signal in left occipital region without enhancement
34. Occipital Cerebritis:Is MRI Abnormal?Standard blood tests negativeLumbar puncture normalElectroencephalogram: slowing in left occipital regionRecovers spontaneously after several monthsFollow-up MRI normal
35. Creutzfeldt-Jakob Disease: MRI Normal At Outset66 year old woman reports blurred vision in left inferior hemifield*Ophthalmologic exam is normal except…Brain MRI normal! *Prasad S et al J Neuro-Ophthalmol 2010;30:260
36. Creutzfeldt-Jakob Disease: MRI Normal At OutsetVisual field worsens, “shimmering lights,” “palinopsia”, “impaired depth perception”Neurologic exam: “mildly-impaired tandem gait, copying”Prasad S et al J Neuro-Ophthalmol 2010;30:260
37. Creutzfeldt-Jakob Disease: MRI Normal At OutsetMRI now shows subtle visual cortex abnormalitiesPrasad S et al J Neuro-Ophthalmol 2010;30:260
38. Creutzfeldt-Jakob Disease: MRI Normal At OutsetLP normal (14-3-3 “ambiguous”)VEP and EEG normalFDG PET abnormal in parieto-occipital regionsPrasad S et al J Neuro-Ophthalmol 2010;30:260
39. Creutzfeldt-Jakob Disease: MRI Normal At OutsetPatient loses all vision over 8 weeks, develops myoclonus, impaired arousalDies within 12 weeks of symptom onsetAutopsy: neuronal loss and spongiform vacuolization MOSTLY OCCIPITALPrasad S et al J Neuro-Ophthalmol 2010;30:260
40. “Visual Variant” Alzheimer Disease75 year old man has 3 car crashes, all on his right sideHis wife reports slight cognitive slowing and short-term memory lossHesitant navigation, impaired puzzle-solvingVisual acuity 20/20 OU, otherwise normal ophth exam except…
41. “Visual Variant” Alzheimer Disease Also described by Brazis P, Lee AG, Graff-Radford N, Desai NP, Eggenberger ER. J Neuro-Ophthalmol 2000;20:92
42. Conclusions1. Retrobulbar vision loss may rarely display no MRI abnormalities or very subtle and overlooked abnormalities2. Every segment of the visual pathway may show this phenomenon
43. ConclusionsMRI Abnormalities May Be Normal or Subtle and OverlookedRetrobulbar neuritis not rareHypoxic-ischemic encephalopathy not rare Radiation optic neuropathy not rare Optic tract trauma/ischemia rare Lateral geniculate PRES/ischemia rare Visual cortex cerebritis rareCreutzfeldt-Jakob disease rare Alzheimer disease rareNonketotic hyperglycemia very rare
44. Conclusions3. Faced with strong clinical evidence for persistent retrobulbar visual loss:Review MRI with expertsGet higher-definition MRI with special sequencesRepeat MRI laterDeploy functional imaging: EEG, VEP, fMRI, SPECT, PET
45. Case 130 year old woman with clinical features of new retrobulbar neuritis OSGood quality MRI normal!Patient elects no treatment;vision recovers completely within weeks2 months
46. Case 1How often is good quality (orbit) MRI normal in acute retrobulbar neuritis?
47. Case 1: Retrobulbar neuritisPublished sensitivity (thickening, enhancement, high T2) = 94%-97%*Unpublished U of Michigan study has MRI sensitivity of only 84%!*Kupersmith MJ et al. Brain 2002; 125:812 Hickman SJ et al. J Neurol 2004;251:996 Rizzo JF et al. Ophthalmology 2002;109:1679
48. Case 273 year old woman undergoes 30 Gy x-irradiation for metastatic lung cancer to frontal lobe10 months later, loses all vision OS over several daysNeuro-ophthalmologic exam shows retrobulbar optic neuropathy without chiasmal featuresNeurologic exam otherwise normalMRI performed 2 weeks after acute vision loss is normal
49. Case 22 months later, loses vision OD to finger countingSmall APD OS; normal fundus OD; pale optic disc OSMRI performed at time of vision loss OD shows RIGHT pre-chiasmatic optic nerve enhancement, but LEFT pre-chiasmatic optic nerve still looks normal
50. Case 2Vision does not change17 months after vision loss OS and 15 months after vision loss OD, right optic nerve enhances briskly and left optic nerve now enhances slightly
51. Case 2: Radiation Optic NeuropathyWhat are the imaging abnormalities of radiation optic neuropathy?What is the timing of these abnormalities in relation to vision loss?
52. Case 2: Radiation Optic NeuropathyMRI abnormalitiesEnhancement, thickening, high T2 signalAlways located at optic nerve/chiasm junctionMay be subtle or absent acutely even when vision loss is profoundMay precede vision loss
53. Case 342 year old woman with cardiac arrest after motor vehicle accidentVisual acuity 20/50 OU with bilateral homonymous hemianopiaFive months later, MRI with 5mm slice thickness interpreted as normal
54. Case 3Is this cortical visual loss?Why no MRI abnormalities?
55. Case 3Expert review shows INCREDIBLY SUBTLE gyral enhancement and high FLAIR signalPost-contrast T1FLAIR
56. Case 3: Hypoxic-ischemic encephalopathyMRI abnormalitiesMay be absent at time of profound vision loss May be subtle and overlooked because:radiologists do not consider visual cortex a border zone regionradiologists not familiar with normal appearance of visual cortexCortical thinning (atrophy) typically appears months laterCortical visual loss May be predominant or ONLY neurologic abnormality