NEURO 101 NEURO SYMPTOMS NEURO SIGNS. Transient Visual Obscurations AMAUROSIS FUGAX WORK-UP FOR AMAUROSIS

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1 NEURO 101 Jill Autry, O.D., R.Ph. Eye Center of Texas, Houston NEURO SYMPTOMS Sudden or gradual vision loss/visual field loss AION, optic neuritis, compressive lesion Transient visual obscurations Papilledema, amaurosis fugax, migraine Pain on eye movement Optic neuritis, sinusitis Diplopia Cranial nerve palsy Myasthenia gravis, multiple sclerosis, thyroid disease Orbital tumor Headache NEURO SIGNS APD (Afferent Pupillary Defect) Visual field defects Ptosis Ocular motility restriction Optic nerve edema/pallor Pupil abnormalities Nystagmus Proptosis Transient Visual Obscurations Unilateral Amaurosis fugax Associated carotid disease Giant cell arteritis Bilateral Vertebrobasilar artery insufficiency Unilateral or Bilateral Papilledema (lasting seconds) Migraine (lasting minutes) AMAUROSIS FUGAX Unilateral vision loss of seconds to minutes to one hour Sectoral or total darkening of vision Ocular exam usually normal May see arteriolar emboli May see signs of Ocular Ischemic Syndrome Retinal hemorrhages NVI NVD NVE WORK-UP FOR AMAUROSIS Carotid doppler CBC with differential HbA1C Lipid profile ECHO Any signs/symptoms of Giant Cell? Order ESR (sed rate) and C-reactive protein 1

2 Levator dehiscence Post-surgical Congenital Myasthenia gravis Horner s Syndrome Third nerve palsy Pseudoptosis Botox induced PTOSIS RAPD Relative Afferent Pupillary Defect Use brightest light possible Make room as dark as possible Optic nerve disease Severe retinal disease CRVO, CRAO, Large RD ANISOCORIA Physiologic Same size in light and dark Usually less than 1mm size difference Look at old photos Small pupil Size difference greater in dark Large pupil Size difference greater in light SMALL PUPIL Horner s Syndrome Argyll Robertson Long standing Adie s Iritis Miotic drop HORNER S SYNDROME Horner s triad Miosis, Ptosis, Anhydrosis Congenital Often has heterochromia Old photos / history Acquired Patient may report increased accommodation 2

3 Mydriatic Testing of Horner s Difficult Way Fail to dilate with cocaine 10% solution HORNER s Fail to dilate with hydroxyamphetamine 1% POSTGANGLIONIC Do testing at least 24 hours apart Mydriatic Testing of Horner s Easy Way If dilates with Iopidine HORNER s Work up whether pre or post ganglionic MRI of Brain and Neck CT chest CBC with differential MRA Others ARGYLL ROBERTSON Light-Near dissociation Little response to light Normal response to near Both pupils eventually affected Neurosyphillis Order RPR, FTA-ABS Other light-near dissociations Therefore, also order MRI LARGE PUPIL Adie s Tonic pupil Torn iris sphincter History of blunt trauma Mydriatic drop Posterior communicating artery aneurysm (PCA) Scopolamine patch ADIE S TONIC PUPIL Usually young female Poor reaction to light Slow constriction to near Slow redilation following near constriction Vermiform movement Constricts to 0.125% pilocarpine May not constrict in initial stage Long standing can result in small pupil 3

4 MONOCULAR VS. BINOCULAR Does diplopia disappear with either eye covered? Monocular diplopia is present with only one eye open. Binocular diplopia disappears with occlusion of either eye. Is second image clear and distinct? Binocular Is second image a ghost image or shadow? Monocular Does pinhole/refraction/artificial tear remove second image? Monocular BINOCULAR DIPLOPIA Horizontal, vertical, or oblique? Horizontal--Lateral Rectus, Medial Rectus Vertical--Inferior or Superior recti, oblique muscles Intermittent vs. Constant Intermittent MG, MS, Thyroid Constant Nerve Palsy, muscle entrapment How long since first noticed? Acute Nerve Palsy Chronic Phoria breakdown, tumor, thyroid, MG Worse in certain gaze? Helps diagnose muscle of concern No increase in certain gaze suggests phoria breakdown BINOCULAR DIPLOPIA Worse at distance or near? Distance Lateral, inferior, or superior recti Near Medial recti or oblique muscles Worse in am or pm? MG worse in pm Phoria breakdown often worse when tired in pm Any recent trauma/surgery to eye/face/head? Face lift, airbag, MVA, retinal surgery Previous episodes? Full EOMs? Check separately and together. OTHER DIAGNOSTIC SIGNS Head tilt/turn III, IV, or VI nerve palsy Large fusional amplitudes Decompensating phoria Tingling/numbness/young female Multiple sclerosis Visual field defect Unilateral-orbit Temporal hemianopsia-chiasm BINOCULAR Cranial nerve palsy III, IV, or VI Phoria breakdown Orbital disease Thyroid Inflammatory/infectious proptosis Multiple sclerosis Myasthenia Gravis Post-operative Post-traumatic CONSTANT DIPLOPIA Cranial nerve palsy III, IV, VI Unilateral orbital disease Post-operative Post-traumatic Aniseikonia 4

5 3 rd NERVE PALSY Diplopia Exotropia and/or hypotropia Ptosis Classic-Down and out presentation May or may not have pupil involvement 3 rd Nerve Pupil Testing Pupil involving Fixed, dilated pupil; minimally reactive to light MRA with MRI Posterior communciating artery aneurysm Pupil Sparing Pupil equal in size to other eye Normal light reaction Ischemic microvascular disease 4 th NERVE PALSY Vertical or oblique diplopia Head tilt towards unaffected side to decrease or eliminate diplopia Trauma most common, then ischemic, demyelinating disease, or congenital Tumor rare Park s 3 Test Hypertropia, worse in opposite gaze, worse in same side head tilt Right, left, right Left, right, left 6 th NERVE PALSY Horizontal diplopia Worse in temporal gaze of affected eye Decreased diplopia in gaze away from affected eye Commonly vasculopathic Also seen with trauma or increased intracranial pressure Uncommon MS, tumor, Giant cell UNILATERAL ORBITAL DISEASE Unilateral proptosis Unilateral visual field defect Unilateral decreased acuity RAPD MRI of brain and orbits Optic nerve gliomas, meningiomas, lypmphomas, cavernous hemangiomas, mucoceles, infection, inflammation UNILATERAL ORBITAL DISEASE Optic nerve gliomas Meningiomas Lymphomas Cavernous hemangiomas Mucoceles Infection (orbital cellulitis) Inflammation (orbital pseudotumor) Thyroid although bilateral, often asymmetric 5

6 INTERMITTENT BINOCULAR DIPLOPIA Phoria breakdown Thyroid eye disease Myasthenia gravis Multiple sclerosis PHORIA BREAKDOWN Intermittent diplopia History of childhood patching/strabismus Long-standing head tilt/turn Approximately equal in all gazes (comitant) Large fusional ranges Full ductions and versions THYROID EYE DISEASE Pseudoptosis Proptosis Lid retraction Intermittent or constant diplopia Inferior rectus-most often affected first Medial rectus Superior rectus Lateral rectus Do CT of orbits rather than MRI for thyroid MYASTHENIA GRAVIS Ptosis Intermittent diplopia Younger women; older men Worse at end of day or with fatigue Ask about difficulty swallowing or breathing Muscle weakness worse at end of day DIAGNOSING MYASTHENIA Check for increased ptosis with fatigue Check orbicularis muscle function MRI of Brain and Orbits Tensilon test Ice-test ( Poor man s Tensilon test ) Acetylcholine receptor antibodies AChR binding antibody; if negative order AChR modulating Thyroid panel EMG and/or single fiber EMG MULTIPLE SCLEROSIS Female > Male years old Intermittent diplopia (usually 4 th nerve) Optic neuritis Nystagmus Tingling or numbness in extremities Uhtoff s sign Worsening vision with increased body temperature Lhermitte s sign Shock-like sensation with neck flexion 6

7 OPTIC NEURITIS Decreased vision over days Unilateral Pain on eye movements Decreased color vision (red cap test) + RAPD Visual field defects vary Swollen disc or retrobulbar MRI of Brain and Orbits with Flair sequencing OPTIC NEURITIS TREATMENT TRIAL (ONTT) Recommends treatment with IV methylprednisolone x 3 days Avoid prednisone orally until AFTER treatment with IV (10-14 days) Hastens visual recovery but not final visual outcome Prolongs time to development of MS Do not use oral steroids alone DIAGNOSING MULTIPLE SCLEROSIS MRI of brain with Flair testing Inspection of CSF for oligoclonal bands Inspection of CSF for increased IgG index VER testing shows increased latency Neurologist NEURORETINITIS Initially appears as optic neuritis May have vitreous cells and retinal white lesions Generally unilateral Over days to weeks, a macular star will form Sometimes partial, sometimes complete Commonly associated with cat-scratch disease or other infectious etiology Arteritic Ischemic Optic Neuropathy AKA Giant Cell Sudden, painless vision loss (CF or worse) Chalky white, swollen ONH + RAPD Sometimes associated 6 th nerve palsy Patient >50 yo, poor health Unilateral progressing to bilateral Headache, jaw claudication, scalp tenderness, muscle/joint aches, weight loss, fever IS IT GIANT CELL? Sed rate Normal values Men age 2 Women age C-Reactive protein Platelets Temporal artery biopsy If positive treat with systemic steroids 7

8 NON-ARTERITIC ISCHEMIC OPTIC NEUROPATHY Sudden, painless loss of vision With or without ONH swelling Unilateral Only occasionally progresses to bilateral + RAPD Visual field defect Usually altitudinal Patients often have vasculopathic conditions Diabetes, hypertension, hyperlipidemia Patients often have a disc at risk VIRAL PAPILLITIS Generally in children Follows recent viral illness Unilateral optic nerve swelling MRI PAPILLEDEMA SIGNS Bilateral ONH swelling caused by increased intracranial pressure Peripapillary swollen NFL Blurring of disc margins Blurring of ONH vasculature Peripapillary flame shaped hemorrhages Enlarged blind spots on VF testing No RAPD PAPILLEDEMA SYMPTOMS Transient obscurations of vision lasting seconds (usually bilateral) Headaches worse upon wakening Diplopia secondary to 6 th nerve palsy Little or no vision loss *unless chronic Color vision intact *unless chronic PSEUDOTUMOR CEREBRI Papilledema Negative MRI of Brain Negative MRV of Brain Increased opening pressure on lumbar puncture Normal CSF composition Obese females (Diamox and weight loss) Pregnancy (Diamox after 20 weeks gestation) Medication induced (remove offending agent) MEDICATION INDUCED PAPILLEDEMA Accutane Steroids Vitamin A Tetracycline Doxycycline Minocycline Amiodarone Isoniazid HRT/BC pills Lithium Nitroglycerin Chemo meds 8

9 PSEUDOPAPILLEDEMA Blurred disc margins No blurring of vasculature Little or no cupping No NFL swelling Can do OCT if have baseline OCT See NFL swelling Can do FA Can see leakage at ONH if papilledema present BURIED NERVE DRUSEN Little or no cupping Vasculature radiates from center of disc See similar appearance in parents or siblings With age, drusen start to appear on surface B-scan shows buried drusen OCT shows blank areas 7 th NERVE PALSY Unilateral facial nerve paralysis Inability to close lid Inability to smile on affected side No diplopia Start Valtrex 1 gram tid or other high dose antiviral Manage corneal exposure Bell s Palsy Diagnosis of exclusion Full facial palsy including forehead Order MRI of brain with special attention to 7 th nerve VISUAL FIELD DEFECTS Unilateral Altitudinal AION Central scotoma Optic neuritis Tumor compression Optic nerve head drusen Varying defects Optic neuritis Tumor compression Optic nerve drusen VISUAL FIELD DEFECTS Bilateral Binasal Compression of both optic nerves; glaucoma Bitemporal hemianopsia Pituitary tumor or other chiasmal lesion Blind spot enlargement Papilledema, ONH drusen, Myelinated NFL VISUAL FIELD DEFECTS Bilateral Homonymous hemianopsia Stroke and trauma Superior quadranopsia Temporal lobe- Pie in the sky tumor more common; also stroke Inferior quadranopsia Parietal lobe- Pie on the floor Stroke more common; tumor less likely Macular sparing Occipital lobe 9

10 HEADACHES Sinus headache Headache often discussed with eye doctors Pressure around eyes Headache worse when bending over or lying down History of seasonal allergies or recent cold Recommend nasal sprays/decongestants HEADACHES Tension headache Most common headache Diffuse pain like a band encircling the head Also have pain at the back of neck and base of skull MIGRAINES Women>Men; 3:1 Generally starts before 20 years of age Often have family history May have nausea and vomiting, fatigue, photophobia Headaches predominantly on same side;may occasionally switch sides Headache triggers -Stress -Chocolate -BC pills -Bright lights -Alcohol -Pregnancy MIGRAINE RELATED AURA Flashing lights, heat waves, jagged objects, tunnel vision, colored spots Lasting 15 to 30 minutes May or may not be accompanied by HA Acephalic migraine History of migraine is common CLUSTER HEADACHES Unilateral Very painful Typically affects men Lasts minutes to hours; typically occurs at same time each day May disappear as easily as they appeared May see ipsilateral tearing, rhinorrhea, Horner s HEADACHES OF CONCERN Associated with any of the following: Scalp tenderness, weight loss, pain with chewing, ONH swelling or pallor, fever, change in behavior, stiff neck No history of headaches More severe headache than usual A headache always in the same location A headache which awakens the patient Aura follows headache 10