Clinical Case Challenges In Neuro-Optometry I - PowerPoint PPT Presentation

Clinical Case Challenges In Neuro-Optometry I

Thomas J. Landgraf, O.D., F.A.A.O.

Thomas Landgraf, O.D., F.A.A.O.

Clinical Associate Professor, UMSL College of Optometry

• 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

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

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

• Why spend 3 hours on it?
• Conditions are both:
• Vision threatening
• Life threatening
• “True” ocular emergencies

• Always A Tough DDx (Differential Diagnosis)
• S:
• 52 yo Caucasian male referred to me
• Tentative diagnosis of CRVO OS

• 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

• 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

• 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

• 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

• 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

• 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

• Always a Tough DDx
• Follow-up 4 months later
• Resolving Malignant Hypertensive Retinopathy
• Improved Blood Pressure
• Educated on compliance

• 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

• Always a Tough DDx
• Timely diagnosis for malignant HTN
• Can significantly reduce morbidity and mortality
• Like Neuro-Eye Disease: sight and life threatening

• 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

• 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

• 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

• 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

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

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

• 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

• Optometric In-Office:
• VF
• B scan ultrasound
• Photos or optic nerve imaging

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

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

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

• 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

• Lumbar Puncture
• Required for the diagnosis of PTC
• Neurologist, radiologist or ER physician
• Usually > 200 mm

• 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

• Opening pressure
• Normal: 60-80 mm of H20
• Borderline elevated: 180-210 mm of H20

• 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

• “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

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

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

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

• 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

• S:
• 85 yo Caucasian female
• Cx: acute vision loss OD
• 2 weeks earlier
• Earache
• Sore temporal veins
• Jaw claudication
• Past medical hx: non-contributory

• O:
• BVA
• LP OD, 20/30 OS
• +APD OD
• BP: 150/100
• No carotid bruits
• SLX: NS consistent with 20/30 VA

• O:
• DFE: pallid swelling of the optic nerve OD
• Othewise normal retina and posterior pole OU

• A:
• Provisional Diagnosis: Giant Cell Arteritis OD
• P:
• FLAN:
• increased arterial filling time OD
• Choroidal nonfilling defect OD
• 80 mg Prednisone po daily

• P: R/O all causes of Anterior Ischemic Optic Neuropathy
• CBC:
• Elevated monocyte and platelet counts
• ESR: 44
• FTA-ABS and VDRL non-reactive

• One week later…..
• Right temporal artery biopsy
• Ear pain and temporal HA resolved

• Two weeks later…..
• ESR: 4
• Plan:
• Monitor with ESR
• And for prednisone side effects

• Eventually…..
• VA did not improve OD
• But remained stable OS

• Or…..
• Giant Cell Arteritis
• Nomenclature following vision loss
• Temporal Arteritis

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

• Background
• Contralateral vision loss
• 2/3 if untreated
• Within weeks if untreated

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

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

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

• 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

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

• 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

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

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

• 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

• 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

• Diagnosis: Summary
• History and clinical impression
• ESR and CRP
• Confirm with temporal artery biopsy

• Management
• Referral: neurologist, internist (PCP), rheumatologist
• For suspects or diagnosed:
• Systemic steroids

• Management
• Systemic steroids
• Hospital admission
• 1-2 gm IV methylprednisone x 2-3 days
•  60-100 mg of oral prednisone: tapered very slowly

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

• 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