OPTIC NEUROPATHIES Optic Neuritis vs AION. Jacqueline M.S. Winterkorn, Ph.D., M.D.

Transcription

1 OPTIC NEUROPATHIES Optic Neuritis vs AION Jacqueline M.S. Winterkorn, Ph.D., M.D.

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3 OPTIC NEUROPATHIES Inflammatory Optic Neuritis Ischemic Optic Neuropathy Compressive Optic Neuropathy Traumatic Optic Neuropathy Glaucoma Hereditary Optic Neuropathy Toxic-Nutritional Optic Neuropathy Autoimmune Optic Neuropathy

4 OPTIC NERVE VS MACULAR DISEASE Optic Nerve Macula Pain often never Description dark areas distortion Brightness reduced normal VA variable decreased RAPD obvious absent or small Color reduced normal VF NFL defect normal or central Photostress normal delayed Amsler scotoma metamorphopsia VER large delay small delay

5 DEFINING OPTIC NEUROPATHY Signs Localizing to Optic Nerve Decreased Visual Acuity Visual Field defect reflecting nerve fiber layer altitudinal respecting horizontal meridian arcuate cecocentral Relative afferent pupillary defect

6 Swinging Flashlight Test Left RAPD

7 OPTIC NEUROPATHIES Compare Inflammatory Optic Neuritis and Ischemic Optic Neuropathy

8 HISTORY A 32 year old woman experienced headache and left eye pain on eye movement for 2 days and a gradual clouding of vision in her left eye. The clouding progressed over 4-5 days until the patient no longer was able to count fingers with that eye. The right eye seemed normal. She had no prior medical history and took no medications. She admitted to feeling fatigued after drinking hot beverages. She recalled a few months of lower extremity tingling starting 2 months after a MVA 3 years earlier.

9 Exam OD OS VA 20/20- HM AOHRR Color 5 1/2 of 6 0/6 Visual Fields Pupils: Motility Fundus Left RAPD.9 log units full pink disc, sharp margins c/d.4 ou

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14 OPTIC DISC SWELLING Papillitis - 35% Optic Neuritis Swollen disc Visual loss Anterior Optic Neuritis Retrobulbar Neuritis - 65% Optic Neuritis Normal appearing disc The patient sees nothing and the doctor sees nothing Papilledema High ICP Bilateral swelling of discs Vision usually normal

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17 TYPICAL DEMYELINATING OPTIC NEURITIS 32 year old woman Prodrome of pain inflammation at annulus of Zinn 95% report pain Progression of visual loss up to 10 days Color vision loss out of proportion to VA Relative afferent pupillary defect Disc appears normal at first Recovery over 2-6 months

18 DEMOGRAPHICS OF OPTIC NEURITIS Occurs between ages 15 and 45 Women:Men = 4:1 Visual Acuity loss from minimal to NLP 4% of patients have NLP Association with Multiple Sclerosis 50% patients with MS have optic neuritis 90% patients with MS have abnormal VER 20% MS, optic neuritis is presenting symptom

19 ASSOCIATION OF OPTIC NEURITIS Prior Studies AND MS Perkin and Rose -10 years after ON: 30% clinical diagnosis MS Rizzo and Lessell - 15 years after ON 74% of women have clinically defined MS 34% of men have MS 2-5% per year develop MS

20 ASSOCIATION OF OPTIC NEURITIS AND MS ON Study Group Data Risk of clinical MS 5 years 30% 10 Years 38% 12 years 40% MRI lesion [1] correlated with risk 5 years: Nl MRI 16%; abnormal MRI 51% 10 years: 22% 56%

21 ON Study Group Data Factors Lowering risk of cdms Male No pain Disc swelling, heme, exudates Macular edema Normal MRI

22 Differential Diagnosis of Optic Neuritis Demyelinating Optic Neuritis Papillophlebitis Neuroretinitis AION

23 CAUSES OF INFLAMMATORY OPTIC NEURITIS Demyelination [MS] Postviral Granulomatous Inflammation Sarcoid Syphilis Collagen Vascular Lupus Autoimmune

24 Importance of Early Diagnosis of MS ONTT Beck, RW, ONSG. The ONTT. Arch Ophthalmol 1988; 106: ONSG. The clinical profile of acute ON. Experience of the ONTT. Arch Ophthalmol 1991; Beck, RW et al. A randomized, controlled trial of corticosteroids in treatment of acute ON. NEJM 1992; 326: CHAMPS Jacobs LD et al. The effect of intramuscular interferon beta 1a treatment on the rate of development of clinically definite MS. NEJM 2000; 343:898

25 ONTT Optic Neuritis Treatment Trial 457 patients with acute Optic Neuritis mean age 32 77% women Randomized to 3 treatment arms Oral prednisone [1mg/kg for 14 days] IV methylprednisolone [250mg qid x 3 days] followed by 11 days of prednisone Oral placebo x 14 days Beck et al Arch Ophth :331-2

26 ONTT Optic Neuritis Treatment Trial All had neurologic & neuro-ophthalmic exams MRI Blood tests [glucose, ANA, FTA-ABS] Followed 1 year to determine visual outcome Followed 2+ years to study effect of treatment on development of clinically defined MS Now 12 years follow

27 ONTT Optic Neuritis Treatment Trial Methylprednisolone hastened recovery [acuity, contrast sensitivity, color,vf] by about 2-3 weeks compared with prednisone and placebo. No difference in final visual outcome. Methylprednisolone-treated patients had 50% fewer attacks up to 2 years. Oral prednisone-treated had increased risk of recurrent optic neuritis. Contraindicated.

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30 Presence of one [or more] 3mm diameter white matter lesion on MRI increases risk of developing MS.

31 CHAMPS Controlled High-Risk Avonex[interferon -1a] MS Study Avonex IM after first attack [with 2 MRI lesions] Optic Neuritis 50% brain stem-cerebellar syndrome 28% spinal cord syndrome 22% At 3 years, clinically definite MS developed 35% of Avonex group 50% of placebo group (P<0.001) Avonex-treated had 57% fewer MRI lesions Suggest early intervention

32 HISTORY A 55 year old man noted blurred vision in his left eye one morning when he tried to read the NYTimes. His vision neither improved nor worsened since then. He denies any pain or headache. He had perfect vision until he started wearing reading glasses 15 years ago. He is in excellent health except his internist started him on a beta blocker one month ago for labile hypertension.

33 AION Blurred Vision Visual Field Loss Usually Sudden Onset Painless or mild ache Always disc edema disc at risk

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36 DEFINITION OF AION Anterior Ischemic Optic Neuropathy Infarction of Optic Nerve Head Occlusion of Posterior Ciliary Arteries Not Embolic Can be sign of Temporal Arteritis

37 Blood Supply to the Optic Nerve Spalton et al, Atlas of Clinical Ophthalmology, 1984

38 PATHOPHYSIOLOGY OF AION Crowded disc at risk Small vessel disease - poor perfusion Axons swell and compress neighbors Axons die, K leaks, Ca++ enters Prostaglandin and phospholipase cycles Cascade of disc infarction Occurs only once - crowding resolves Fellow eye at risk

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40 PSEUDO FOSTER KENNEDY Foster Kennedy Syndrome Optic atrophy in poorly seeing eye; papilledema in eye with good vision subfrontal mass compressing Pseudo Foster Kennedy Syndrome Bilateral sequential AION with poor vision in both eyes

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42 SYSTEMIC CONDITIONS ASSOCIATED WITH AION No prior diagnosis 50% Hypertension 35-50% Diabetes 10-25% Smoking 20% Myocardial Infarction.. 14% Hypercholesterolemia 11% Hypercoagulable states. Rare

43 SYSTEMIC RISK FACTORS FOR AION Small Vessel Disease especially hypertension 40% patients with AION treated for hypertension Is the risk factor high blood pressure or low blood pressure? Overtreated hypertension Poor autoregulation luxury perfusion

44 Luxury Perfusion after AION

45 24-Hour Blood Pressure Diurnal curves of AION and Control subjects were parallel and close most of the day. In early morning, AION patients exhibited slower, more fluctuating rise in BP compared with Controls who had a rapid steady rise in BP from nadir to day levels.

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47 DEMOGRAPHICS OF AION Common in patients age per 100,000 No gender preference [55% male] 95% white small cup/disc ratio

48 IONDT TREATMENT FOR AION Ischemic Optic Neuropathy Decompression Trial Vision was worse after nerve sheath fenestration Aspirin - prophylaxis for second eye Gingko biloba and Trental Sinemet - Johnson Neuroprotection? Steroids - for Arteritic AION

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50 SPECIAL OCCURRENCES OF AION SURGERY - Spinal, coronary and GI ION can be bilateral ION can be anterior or posterior Systemic risk factors hypotension - shock hypoperfusion anemia low blood volume hemodilution Renal Dialysis - uremic optic neuropathy Amioderone Toxicity Giant Cell [Temporal] Arteritis

51 An independent 78 yr old woman complains to internist about neck stiffness for several weeks. Aleve prescribed without relief. She calls to report that she has headaches and a sore scalp. Elavil is begun She develops jaw pain on chewing and is referred to dentist for TMJ. The night before the dental exam, she experiences several episodes of TVL and then notes blurred vision in her left eye. By the next morning she is NLP OD and 20/400 OS.

52 Jonathan Hutchinson

53 Headache TEMPORAL ARTERITIS SYMPTOMS Jaw Claudication Weight Loss Malaise Polymyalgia Rheumatica Scalp tenderness Transient visual obscurations

54 TEMPORAL ARTERITIS SIGNS Tender Thickened Temporal artery AION Bilateral HM or NLP Chalky white disc Choroidal ischemia CRAO

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58 Temporal Arteritis Increases with age: 1/1000 over 80. TA patients older than naaion patients. AION most common cause visual loss. Worse visual outcome than naaion. 75% bilateral within days.

59 AGE: naaion vs TA

60 Visual Acuity: naaion vs TA

61 WORK-UP FOR TEMPORAL ARTERITIS WESR -over 50 C-Reactive Protein - over 3.5 CBC - anemia Platelets - thrombocytosis TA biopsy - within 7-10 days

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63 PATHOLOGY OF TA Interruption of internal elastic lamina Inflammatory Infiltrate Multinucleated giant cells Lumen occluded Skip lesions

64 TREATMENT FOR Arteritic AION Trendelenberg positioning Immediate IV Solumedrol 250 qid Followed by Prednisone 1+ mg/kg/day Continue high dose for a month If WESR low, taper over 12 months If refractory, MTX or Dapsone ASA Ulcer precautions and Ca++ supplements

65 Optic Neuritis or Anterior Ischemic Optic Neuropathy Overlap makes diagnosis difficult

66 HISTORY A 44-year old man noted blurred vision in his right eye accompanied by a mild right-sided ache. When he covered the left eye, he could not read. His vision has not improved and may even be slightly worsening. He is now seeing flashes and sparks in his left eye every time he hears a loud noise. He is in good health, except for pain in his knee.

67 EXAM OD OS VA 20/20-20/20 AOHRR 4/6 6/6 VF inferior altitudinal full Pupils 1.2 log units Right RAPD Motility full OU Fundus swollen disc c/d.1

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69 PHOSPHENES in OPTIC NEUROPATHY Visual-Auditory Synesthesia Auditory - Induced Visual Phosphenes Seen in Optic Neuritis Seen with AION

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72 DIAGNOSIS BY OUTCOME He had pain. He had synesthesia. He had inferior altitudinal defect. He had disc at risk. Disc swelling was appreciated. Developed optic atrophy. Acuity and VF did not improve. AION.

73 When to Treat for MS Treat early Neurodegeneration progresses without symptoms Defer immunomodulatory treatment in monosymptomatic ON, if normal MRI optic disc swelling or retinal edema no pain

74 Review Questions

75 A 28-year-old woman with ocular pain, decreased visual acuity, and color vision loss is most likely also to have a. a swollen disc. b. an afferent pupillary defect. c. an altitudinal visual field defect. d. a small cup/disc ratio in the fellow eye.

76 The results of the ONTT indicated that A. Oral Prednisone was associated with more rapid recovery. B. Placebo and Methylprednisolone groups had fewer attacks of optic neuritis over the next 2 years than the Prednisone group. C. In general, the Placebo group had poorer recovery of vision than the Prednisone group. D. In general, the Prednisone group had poorer recovery of vision than the Methylprednisolone

77 The CHAMPS study showed that A. Avonex speeded recovery from brainstem symptoms B. the Avonex group were more likely to get another attack within 3 years. C. the Avonex group had better vision after Optic Neuritis than the Placebo group. D. the Avonex group developed fewer new lesions on the MRI.

78 Which statement is not true: In Temporal Arteritis A. AION is the most common visual sign. B. Jaw claudication is caused by ischemia. C. The C-reactive protein is almost always elevated. D. A positive biopsy must show giant cells. E. The patient may complain of sore throat and hoarseness.

79 The appearance of the optic discs in this patient [atrophy OD and swelling OS] is most commonly caused by a. Anterior Ischemic Optic Neuropathy. b. Optic Neuritis in multiple sclerosis. c. Sub frontal Meningioma. d. Pseudotumor cerebri. e. Optic Nerve glioma.

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82 Visual Field Defects in Non-Arteritic Ischemic Optic Neuropathy eyes in 34 patients

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88 When to Neuroimage or It s not glaucoma Loss of central vision unlikely in glaucoma consider dominant optic atrophy Rapidly progressive visual loss POAG is slowly progressive arteritic AION causes cupping Asymmetry or unilateral optic neuropathy Large RAPD is non-glaucomatous Hemianopic VF loss Neuroretinal rim pallor

89 Which statement is not true: In Temporal Arteritis A. AION is the most common visual sign. B. Jaw claudication is caused by ischemia. C. The C-reactive protein is almost always elevated. D. A positive biopsy must show giant cells. E. The patient may complain of sore throat and hoarseness.

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97 When to Neuroimage or It s not glaucoma Loss of central vision unlikely in glaucoma consider dominant optic atrophy Rapidly progressive visual loss POAG is slowly progressive arteritic AION causes cupping Asymmetry or unilateral optic neuropathy Large RAPD is non-glaucomatous Hemianopic VF loss Neuroretinal rim pallor

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99 Differential Diagnosis of Optic Neuritis Demyelinating Optic Neuritis Papillophlebitis Neuroretinitis AION

100 CAUSES OF INFLAMMATORY OPTIC NEURITIS Demyelination/degeneration [MS] Postviral Granulomatous Inflammation Sarcoid Syphilis Collagen Vascular Lupus Autoimmune

101 Optic Neuritis first indication of MS Which patients at highest risk for MS

102 Post viral optic neuritis

103 DEFINING OPTIC NEUROPATHY Signs Localizing as Optic Nerve disorder Decreased Visual Acuity Visual Field defect reflecting nerve fiber layer altitudinal respecting horizontal meridian arcuate cecocentral Relative afferent pupillary defect

104 Avonex Mechanism of action Side Effects flu, skin rx, neutralizing Abs Other Drugs Avonex plus Antigren - trafficking