Blindness In An Elderly Woman

Transcription

1 Blindness In An Elderly Woman A 74 y/o woman with a chief complaint of: a cloud in front of my right eye and I can t t see through it Symptoms began 24 hours prior to examination. Visual loss was painless and confirmed to be monocular by patient s s cross cover testing

2 Blindness There was no associated acute headache, constitutional upset, or other neuro- logical symptoms. The patient wore refractive lens and had experienced no other visual symptoms, specifically no flashes (photopsias) floaters nor visual distortions

3 Blindness PMH: excellent health Hypertension rxed with diuretics Surgeries: Hysterectomy FH: Mother living and well age 92. Father killed age 39.

4 Blindness Review of systems: HEENT: Frequent mild, temporal headaches. Scalp tenderness while combing hair. Pain in front of ears while chewing.

5 Blindness ROS (continued) General: vague sense of malaise for several months; 12 pound weight loss. Lungs: neg Heart: neg Abdominal: Evaluation for postprandial al abdominal pain 3 months ths prior nonrevealing Extremities: migratory muscle and joint pains without stiffness, swelling, s tenderness, or weakness. Neuropsych: one episode of painless isolated horizontal double vision lasting g 40 minutes 2 months prior to eval

6 Blindness Physical Examination HEENT: Soft right carotid bruit Scalp tenderness to tap temporal fossa; Weak temporal pulses. General: Unremarkable Neurol : positive only for visual findings:

7 Blindness Neuro ophthalmologic examination: Normal appearance to inspection VA: 20/150 OD 20/ 25 OS Color: 3/6 HRR plates OD 6/6 OS VF: Full field OS ; Central scotoma with inferior breakout OD Pupils: OD sluggish with an afferent defect; briskly reactive OS Fundus: Disk head swelling with fine peripapillary hemorrhage OD Normal appearing disc OS.

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9 Blindness Laboratory Studies CBC: wbc 7200 with nl dif. Hb 11.4 Platelets: 490,000

10 Blindness Laboratory Studies CMP: unremarkable Serum amylase- nl C-reactive protein (CRP) Erythrocyte sedimentation rate (ESR) 77mm/hour-

11 Blindness Additional Studies Chest Xray: : normal EKG : nonspecific ST wave change MRI : After evaluation treatment was initiated and a diagnostic procedure was performed

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15 Blindness Cortical blindness always produces balanced visual loss

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20 Grand Rounds Hemispheric infarcts will not produce unilateral visual loss because the preserved hemisphere contains signals generated from half of the macula on each eye. Same applies to optic tracts/ radiations

21 Grand Rounds Chiasmal lesions may at times cause monocular visual loss But there has to be a visual field defect in the other eye

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28 Grand Rounds Prechiasmal intracranial monocular visual loss can be due to: Meningioma But tempo is wrong (meningioma( is common but slow growing)

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32 Grand Rounds Prechiasmal intracranial monocular visual loss may be due to: Ophthalmic artery aneurysm is a consideration But A lesion in this location will not result in disc head swelling MRI virtually always reveals lesion

33 Grand Rounds Therefore The location of visual failure lies in the anterior visual pathway: retina or optic nerve The absence of subjective complaints of photopsias and floaters plus the fundic findings of disc swelling exclude the retina.

34 Grand Rounds The optic nerve is the site of injury. Dif Dx: Compressive Infiltrative Inflammatory Ischemic

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36 Could this be optic neuritis?

37 Grand Rounds Picture inflamed ON

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41 Grand Rounds Is this inflammatory? ie.. optic neuritis? Pros: Sudden visual loss Swollen nerve Suggestive VF loss Cons: Age Absence of pain on eye movement Presence of constitutional sx. Absence of signal change on MRI Presence of hemorrhages Preservation of color vision

42 Grand Rounds Is the lesion ischemic? Ischemia takes two forms Central retinal artery occlusion Posterior ciliary vessel occlusion

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45 Grand Rounds Optic Disc infarction- 2 types Arteritic versus nonarteritic Identical in appearance!

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47 Grand Rounds Non arteritic anterior ischemic optic neuropathy ( NAION): Younger population Hypertensive, vasculopathic Opposite optic disc is tight

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50 Grand Rounds Anterior Ischemic Optic Neuropathy (AION) Normal optic cup opposite eye Older age group, > 70y/o Constitutional symptoms, temporal headaches, and particularly jaw claudication!

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52 Grand Rounds Case summary: Older individual Ischemic optic neuropathy Normal optic cup Constitutional symptoms Jaw claudication Transient diplopia Blood work indicates inflammation

53 Blindness DX: GIANT CELL ARTERITIS aka TEMPORAL ARTERITIS

54 Giant Cell Arteritis A chronic granulomatous vasculitis of large and medium sized vessels, etiology unknown, occurring in the elderly. Most common cause of vasculitis in elderly. Occurrence is a medical emergency with potential systemic and ophthalmic complications Prevention of blindness depends on prompt diagnosis and initiation of steroid therapy

55 Giant Cell Arteritis Pathology: affects the cranial branches of arteries originating from the aortic arch Usually associated with marked elevated acute-phase reactants Closely related to polymyalgia rheumatica 10% - 15% of cases involve extracranial vessels and present as transient ischemic attacks particularly amaurosis fugax With AION risk to other eye is high

56 Giant Cell Arteritis Typical Features New onset headache Scalp tenderness Jaw Claudication Polymyalgia Rheumatica Fever, anorexia, weight loss PE: Tender, nodular temporal arteries scalp tenderness, temporalis muscle atrophy ophthalmic signs.

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58 Giant Cell Arteritis Epidemiology 1/5000 people > 50y/o Peak between y/o Women twice as frequently Cyclic pattern of increased incidence every seven years. Frequency increased at higher latitude Black = Hispanic =White

59 Giant Cell Arteritis Laboratory Examination. Erythrocytic sedimentation rate (ESR) = or > 50 mm/hr. NB: 10% are nl Platelets frequently > than 450,000. C Reactive protein ( CRP) most sensitive not affected by erythrocyte number or shape, immunoglobulins renal function or cholesterol.

60 Giant cell arteritis Definitive diagnosis: Temporal artery biopsy

61 Pathology Sebastian R. Alston, M.D. September 19, 2008

62 Giant Cells Elastic lamina Media Temporal Arteritis Fragmented internal elastic lamina Chronic granulomatous inflammation with giant cells No necrosis

63 Temporal Arteritis Giant Cells (arrow) are characteristic but are not always present Intimal Hyperplasia Necrosis not usually present

64 Giant-Cell Arteritis of the Temporal Artery Weyand C and Goronzy J. N Engl J Med 2003;349:

65 Temporal Arteritis Giant cells may be marker of more aggressive course (e.g. blindness) If active inflammation is not present, temporal arteritis is indistinguishable from arteriosclerosis Skip lesions occur Step through vessel Immunohistochemistry Treatment may cause differences in morphology

66 References Armstrong AT, Tyler WB, Wood GC, Harrington TM, Clinical importance of giant cells in temporal arteritis.. J Clin Pathol (2008) 61: Cox M, Gilks B, Healed or quiescent temporal arteritis versus senescent changes in temporal artery biopsy specimens. Pathology (2001) 33: (Abstract) Font RL, Prabhakaran VC, Histological parameters in recognizing steroid-treated treated temporal arteritis: : an analysis of 35 cases. Br J Ophthalmol (2007) 91: Gooi P, Brownstein S, Rawlings N, Temporal arteritis: : a dilemma in clinical and pathological diagnosis. Can J Ophthalmol (2008) 43: Poller DN, Van Wyk Q, Jeffrey MJ, The importance of skip lesions in temporal arteritis.. J Clin Path (2000) 53: Weyand CM and Goronzy JJ, Medium- and large-vessel vasculitis.. N Engl J Med (2003) 349:

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68 Giant cell arteritis American College of Rheumatology Age onset > 50y/o New headache Temporal artery abnormality ESR > 50 Abnormal artery biopsy

69 Giant Cell Arteritis Pathogenesis Unknown adventitial antigen attracts T cells through vasovasorum. Interferon gamma macrophage differentiation and migration.

70 Giant Cell Arteritis Pathogenesis Adventitia: Cytokines Media: Metalloproteins Intima: : Nitric Oxide Synthase 2 + repair mechanisms = intimal luminal hyperplasia/ degradation of internal elastic lamina.

71 Giant Cell Arteritis Granulomatous infiltrate: 50 % Nonspecific lymph infiltrate: 50% Biopsy > 1.5 cm due to skip lesions Absent giant cells doesn t negate dx Start steroids immediately (2 week window)