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Clinical Case Challenges In Neuro-Optometry I

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1 Clinical Case Challenges In Neuro-Optometry I
Thomas J. Landgraf, O.D., F.A.A.O.

2 “Clinical Case Challenges in Neuro-Optometry”
Thomas Landgraf, O.D., F.A.A.O. Clinical Associate Professor, UMSL College of Optometry

3 My Background Graduate of ICO…Chicago Residency at PCO…Philadelphia
SCO x 15 years…Memphis Now at UMSL College of Optometry In terms of Neuro-Eye… Dr. Lawrence Gray at ICO & PCO

4 My Background At SCO…Chief of Ocular Disease Goals for this lecture
Not an expert Share patient care experiences Share “optometric legal consultant” experiences

5 Resources Journals and Internet Review of Optometry
Review of Ophthalmology Handbook of Ocular Disease Management Clinical Guide To Ophthalmic Drugs

6 Neuro-Optometry Why spend 3 hours on it? Conditions are both:
Vision threatening Life threatening “True” ocular emergencies

7 Case #1: ONH Edema? Always A Tough DDx (Differential Diagnosis) S:
52 yo Caucasian male referred to me Tentative diagnosis of CRVO OS

8 Case #1: ONH Edema? Always a Tough DDx S:
Painless vision loss OS x 2 weeks Prosthetic OD due to trauma No significant medical or ocular conditons Low daily dosage of methadone Nicotine patch

9 Case #1: ONH Edema? Always a Tough DDx O: BVA OS: 20/400
OS pupil round and reactive to light Normal SLX Tonometry 17 mm Hg BP: 280/170 RAS: not done at previous visit

10 Case #1: ONH Edema? Always a Tough DDx O: DFE OS:
Optic nerve head edema Accompanied by flame hemes, exudates, cotton wool spots, and macular edema Normal peripheral retina

11 Case #1: ONH Edema? Always a Tough DDx
A: Malignant Hypertension and Resultant Retinopathy OS P: Immediate referral to medical center For lowering of BP Referral to retinal specialist Level Of Comfort Confirmation

12 Case #1: ONH Edema? Always a Tough DDx Follow-up 4 months later
Current meds: minoxidil, norvasc, coumadin HTN and its complications Noted improved vision But some glare, distortion, “wavy lines” in central vision

13 Case #1: ONH Edema? Always a Tough DDx Follow-up 4 months later
BVA OS: 20/20 BP: 160/85 DFE OS: exudative macular star, healthy ONH (.2/.2), normal peripheral retina

14 Case #1: ONH Edema? Always a Tough DDx Follow-up 4 months later
Resolving Malignant Hypertensive Retinopathy Improved Blood Pressure Educated on compliance

15 Case #1: ONH Edema? Always a Tough DDx Bottom Lines
Primary Care OD’s need to take BP’s Especially on those with retinal vascular disease Consider typically bilateral retinal conditions In monocular patients

16 Case #1: ONH Edema? Always a Tough DDx
Timely diagnosis for malignant HTN Can significantly reduce morbidity and mortality Like Neuro-Eye Disease: sight and life threatening

17 Pseudotumor Cerebrii (PTC)
Background “false brain tumor” Increased intracranial pressure without an intracranial mass Major diagnosis of exclusion: a true intracranial tumor All patients with papilledema must have neuro-imaging studies

18 PTC: Why? Poor CSF absorption
By meninges surrounding brain and spinal cord Increased intra-abdominal pressure From obesity  elevated intrathoracic pressure  decreased venous drainage from the brain

19 PTC: Diagnosis Who? Obese women of childbearing age Secondary
Obstruction to venous drainage: cerebral venous thrombosis Exongenous agents: tetracycline, vitamin A, corticosteroids, BCP’s Medical conditons: lupus, sarcoidosis, anemias, blood dyscrasias

20 PTC: Diagnosis Symptoms
Bad HA’s: frontal, around the eyes, pressure-like, throbbing Transient visual loss Intracranial noises: heartbeat or whooshing sound in ears, tinnitus Vision loss: blur, temporal VF defect

21 PTC: Diagnosis Signs Optic disc edema VA, pupils, EOM’S usually normal
Unilateral, bilateral, asymmetric VA, pupils, EOM’S usually normal VF: blind spot enlargement, inferonasal loss, generalized constriction

22 PTC: Differential Diagnosis
Intracranial mass Meningitis: abrupt onset, fever and chills, stiff neck Bilateral inflammatory optic neuropathy: early and central vision loss, pain on eye movement, retrobulbar

23 PTC: Differential Diagnosis
Pseudopapilledema: optic disc drusen or tilted discs, ultrasound may aid Neuroretinitis: macular exudate, early central vision loss Bilateral ION: older, vascular risk factors, painless, early vision loss

24 PTC: Ancillary Tests Optometric In-Office: VF B scan ultrasound
Photos or optic nerve imaging

25 PTC: Ancillary Tests Neurologist or neuro-eye doc referral
Neuroimaging before lumbar puncture Standard MRI of the brain CT scan with contrast if patient markedly obese

26 Neuroimaging Major Scans Used To Evaluate Neuro-Eye Disease
CT (Computerized tomography) MRI (Magnetic Resonance Imaging)

27 Neuroimaging CT Good to view bony abnormalities, calcifications, acute hemorrhages Valuable to diagnosis of orbital processes Test of choice for thyroid eye disease

28 Neuroimaging MRI Far better at characterizing soft tissues
Preferable for most intracranial processes Not subject to bone artifact Contrast media and special studies can sharpen Gadolinium is a contrast material that can increase signal intensity

29 PTC: Ancillary Tests Lumbar Puncture Required for the diagnosis of PTC
Neurologist, radiologist or ER physician Usually > 200 mm

30 Lumbar Puncture Procedure
Patient positioned on side in fetal position with back fully flexed 18 g needle inserted at L4-L5 interspace Opening pressure measured when needle penetrates subarachnoid space HA is most common complication

31 Lumbar Puncture Opening pressure Normal: 60-80 mm of H20
Borderline elevated: mm of H20

32 Lumbar Puncture CSF evaluation Color
Clear and colorless is normal Cloudy: infection Xanthochromic (yellow): subarachnoid hemorrhage Cell count and differential, cytology, chemical analysis, serologic analysis, microscopy, culture

33 PTC: Management “Comanage” with neurologist
Initial LP  improved signs and sxs VF, DFE, photos or optic nerve imaging every month x 3 months Every 2-3 months thereafter for about a year Individual case variability

34 PTC: Management “Comanage” with neurologist
Other options for some persistent signs and sxs CAI’s : acetazolamide Other diuretics Weight loss HA management

35 PTC: Management Diamox Not just for angle closure
Decreased CSF production up to 50% 1-3 grams qd 500 mg bid, tid, qid Side effects: taste alteration, nausea, fatigue, diarrhea, tingling Not with sulfa allergies, kidney disease

36 PTC: Management Headache management Topamax (topiramate)
Migraine prophylaxis and epilepsy PTC: HA relief and mild inhibition of carbonic anhydrase, also causes weight loss Recently: development of angle closure glaucoma from choroidal expansion

37 PTC: Management For signs and symptoms unresponsive to LP, severe vision loss Corticosteroids Surgery Optic nerve sheath fenestration CSF diversion (shunt)

38 PTC: My Clinical Experience
Relatively rare condition? Not at SCO “Comanagement” turns into MANAGEMENT Optometrists take the time Need to be familiar with ancillary diagnostic tests and treatment options

39 Case #2: Monocular Acute Vision Loss In A Golden Girl
85 yo Caucasian female Cx: acute vision loss OD 2 weeks earlier Earache Sore temporal veins Jaw claudication Past medical hx: non-contributory

40 Case #2: Golden Girl O: BVA +APD OD BP: 150/100 No carotid bruits
LP OD, 20/30 OS +APD OD BP: 150/100 No carotid bruits SLX: NS consistent with 20/30 VA

41 Case #2: Golden Girl O: DFE: pallid swelling of the optic nerve OD
Othewise normal retina and posterior pole OU

42 Case #2: Golden Girl A: P:
Provisional Diagnosis: Giant Cell Arteritis OD P: FLAN: increased arterial filling time OD Choroidal nonfilling defect OD 80 mg Prednisone po daily

43 Case #2: Golden Girl P: R/O all causes of Anterior Ischemic Optic Neuropathy CBC: Elevated monocyte and platelet counts ESR: 44 FTA-ABS and VDRL non-reactive

44 Case #2: Golden Girl One week later….. Right temporal artery biopsy
Ear pain and temporal HA resolved

45 Case #2: Golden Girl Two weeks later….. ESR: 4 Plan: Monitor with ESR
And for prednisone side effects

46 Case #2: Golden Girl Eventually….. VA did not improve OD
But remained stable OS

47 Anterior Ischemic Optic Neuropathy (AION) Arteritic
Giant Cell Arteritis Nomenclature following vision loss Temporal Arteritis

48 AION-artertic Background
Granualomatous vasculitis of medium-sized arteries “True” ocular emergency The Goal: Prevention of contralateral vision loss

49 AION-arteritic Background Contralateral vision loss 2/3 if untreated
Within weeks if untreated

50 AION-arteritic Why Granulomatous vasculitis of temporal artery 
Occlusion of short posterior ciliary arteries (supply anterior optic nerve)  AION-artertic

51 AION-arteritic Diagnosis: Who? Rose Nylen on the Golden Girls
Average age of onset = 70 years Female and Scandanavian Lower incidence rates Tennessee & Israel

52 AION-arteritic Diagnosis: symptoms
Unilateral decreased VA, temporal HA, scalp tenderness VA usually < 20/200 Amaurosis fugax Anorexia, fever, malaise, depression Onset is variable

53 AION-arteritic Clinical Features
Vasculitis of coronary arteries: MI, CHF, angina pectoris Neurologic: peripheral neuropathies, ischemic brain damage Polymyalgia Rheumatica: pain and stiffness of the neck, shoulders, hips

54 AION-arteritic Diagnosis: signs AION-arteritic
Optic nerve edema, hemes, cotton wool spots APD VF defects: central, altitudinal, arcuate

55 AION-arteritic Diagnosis: signs AION-arteritic
Pallor described as chalky white CRAO in up to 10% of patients Disc eventually glaucoma-like with cupping BUT with pallor

56 AION-arteritic Differential Diagnosis Vs. non-arteritic AION Worse VA
Worse VF HA and scalp tenderness Constitutional symptoms Older Worse ESR, CRP, CBC variables

57 AION-arteritic Ancillary Tests Optometric In-Office VF
Optic nerve imaging Photos FLAN considered to check for choroidal perfusion defects

58 AION-arteritic Ancillary Tests Referral
ESR (erythrocyte sedimentation rate) Westergren 15% of GCA patients: normal ESR CRP (C-reactive protein) Elevated in > 91% Also elevated WBC, platelet counts; IgG anticardiolipin antibodies

59 AION-arteritic Ancillary Tests Referral Temporal artery biopsy
Should be performed on all suspects > 95% sensitive, 100% specific Can be done shortly after steroid treatment Inflammatory cells in the muscular walls of the artery

60 AION-arteritic Diagnosis: Summary History and clinical impression
ESR and CRP Confirm with temporal artery biopsy

61 AION-arteritic Management
Referral: neurologist, internist (PCP), rheumatologist For suspects or diagnosed: Systemic steroids

62 AION-arteritic Management Systemic steroids Hospital admission
1-2 gm IV methylprednisone x 2-3 days  mg of oral prednisone: tapered very slowly

63 AION-arteritic Management Systemic steroids Anecdotal vision recovery?
Poor prognosis? No solid support for anti-steroid medications (Rheumatrex aka methotrexate)

64 AION-arteritic My Clinical Experience This is rare?
Most important from an educational perspective Presume the worst if suspect Vs. glaucomatous optic neuropathy Combination of AION + OAG in my patients


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