Jacqueline Theis, O.D., F.A.A.O.

Transcription

1 Neuro-Ophthalmological Emergencies Presenting in Primary Care Optometry Describes the symptoms, signs, and management of neuro-ophthalmological emergencies. Signs/Symptoms to be Concerned about (especially if more than one of the following) Diplopia o Recent onset o Pupil-involving Cranial Nerve III Palsy o Multiple Cranial Nerve Palsies Intracranial tumors Meningitis Polyneuropathy Cavernous Sinus Lesion Sudden Vision Loss o Compressive Lesion o Giant Cell Arteritis Anisocoria o Horner Syndrome o Aneurysm Pupil-involving Cranial Nerve III Palsy Ptosis o Horner Syndrome o Cranial Nerve III Palsy Other neurological symptoms o Recent onset headache o Scalp tenderness o Jaw claudication o Weakness/Fatigue o Difficulties swallowing/breathing o Recent weight loss The importance of Taking an adequate History What to Ask Chief complaint: diplopia o Monocular or binocular o Horizontal, diagonal, vertical? o Gaze dependent? All worrisome complaints o Onset sudden or gradual? What were you doing? o Frequency is it getting better, worse, or staying the same? o Duration intermittent or constant? o Timing better at worse or end of the day? o What makes it better? o Has is ever happened before?

2 Other associated symptoms o Pain o Vision changes o Headaches o Tingling limbs, fingers toes o Numbness/weakness facial, extremeties o Nausea/vomiting o Imbalance/vertigo o Photophobia o Hearing loss o Jaw claudication, neck stiffness, temporal artery pain o Recent weight loss o Fever o History of Trauma o History of Vasculopathies (Hypertension, diabetes, cholesterol) o Cancer Previous medical and ocular history Examination Evaluation of afferent visual pathway o Visual Acuity Pinhole Acuity if not 20/20 o Color Vision monocularly Ishihara Red Cap Desaturation o Visual Fields Confrontation Automated Amsler o Pupils Size and reaction in light and dark Near response RAPD checking Evaluation of Efferent Visual Pathway o Ocular Alignment Cover test in primary gaze at distance and near Cover test in 9 fields of gaze o Ocular Motility H pattern assessing for mechanical restrictions +/- nystagmus Ocular Health o External Exam Eyelid symmetry MRD1/MRD2 Palpebral Aperture

3 Exophthalmometry Proptosis? o Slit Lamp/Dilated Fundus Examination Optic Nerve Retina Conditions to be Concerned about Giant Cell Arteritis o Epidemiology o Ocular Manifestations Arteritic Anterior Ischemic Optic Neuropathy - life/sight threatening Sudden vision loss 50% Transient vision loss 30% followed by permanent vision loss Signs o RAPD o Optic disc edema o Retinal Cotton wool spots Central retinal artery occlusion Posterior ischemic optic neuropathy Diplopia/CN palsy o Systemic Symptoms Jaw claudication 48% Neck Pain -17% Headache 57% Scalp Tenderness 20% Weight Loss -40% Anorezia/weight loss 31% Myalgias 28% Malaise 37% Temporal artery tortuosity May be asymptomatic in 20% of cases Absence of these findings does not exclude diagnosis o Lab Work Elevated ESR/CRP High sensitivity 85.7%/97.5% Normal ESR/CRP can occur Abnormal CBC with differential - Thrombocytosis, normocytic anemia, leukocytosis Lower sensitivity <60% Medium-/large-vessel systemic vasculitis affecting elderly (>50yo)

4 T-cell mediated granulomatous inflammation in wall of posterior ciliary artery à ischemia of optic nerve head o Treatment Immediate systemic steroids if suspected oral/iv Aspirin may reduce stroke risk ~81mg orally Horner s Syndrome o Signs/Symptoms Mild Ptosis (2mm) Lower eyelid elevation ( reverse ptosis ) Anhydrosis Anisocoria greater in dim illumination Congenital cases iris heterochromia Lower IOP Stroke, MS, tumor, internal carotid dissection, herpes zoster virus, otitis media, tolusa-hunt syndrome, prolactinoma o Life-threatening complication Carotid Artery Dissection Incidence 3 per 100,000, peak incidence 5 th decade Responsible for up to 20% of strokes in young adults Can be traumatic or spontaneous Other associ signs ipsilateral headache *PAINFUL horner s o Diagnosis (+) Cocaine/Hydroxyamphetamine test o Work Up CBC with Differential CT of chest MRI of brain/neck MRA/CTA of head/neck or carotid Doppler for carotid artery dissection Performed same day, other tests can wait 1-2 days Aneurysm of posterior communicating artery Pupil-Involving CN III palsy o Signs/Symptoms Fixed, dilated, poorly reactive pupils Compressive lesions affect both central somatomotor fibers and peripheral superomedial pupil fibers (usually spared in ischemic lesions) Rule of pupil o Complete CNIII with normal pupil likely ischemic o Incomplete CNII with normal pupil uncertain etiology, 14% will have aneurysm and presents in early phase before pupil is affected o Complete CNIII with pupil likely compressive

5 Anisocoria relevant if >1mm Ptosis Complete palsy limitation of movement in all fields except abduction Superior divisition Ptosis and elevation defect Inferior division Inability to look nasally or inferiorly PAIN o Differential Diagnosis Myasthenia Gravis Thyroid eye disease CPEO Orbital inflammatory pseudotumor Internuclear ophthalmoplegia Skew deviation Parinaud syndrome/dorsal midbrain lesion Giant Cell arteritis *pupil not involved of pupil involving More common Aneurysm (usually) to posterior communicating artery o 9-36% of CNIII palsies o PCA aneurysms present with CNIII 30-60%, and approx. 40% of aneurysms are located at level of PCA, ophthalmic artery and cavernous sinus Factors that increase risk of rupture o Age >50 o Female o Smoker o Hypertension o Alcohol consumption o Family history of intracranial aneurysm or subarachnoid hemorrhage o Aneurysm > 10mm o Polycystic kidney disease Less common Tumor, trauma, congenital, uncal herniation, cavernous sinus lesion, pituitary apoplexy, orbital disease, herpes zoster, leukemia, ischemic o Management CNS imaging gadolinium enhanced MRI and MRA/CTA to rule out mass/aneurysm Pupil involving CN III Pupil sparing CN III in pts less than 50 Incomplete CN III because could be evolving pupilinvolving CNIII palsy

6 o If no imaging, monitor patients >5 days for pupil involvement If additional CN III involvement Prompt neuroimaging (+) Aberrant regeneration Pupil-sparing >3 months without improvement ESR, CRP, platelets Myasthenia Gravis o Life-threatening complication myasthenic crisis Intracranial tumor/inflammation/infection/ischemic infarct (Stroke)/Pseudotumor o Visual field defect o +/- APD o Papilledema o Reduced Visual Acuity o Reduced Color Vision Cavernous Sinus Thrombosis o Signs (cavernous sinus holds CN II, IV, VI and allows for venous drainage Acute onset fever Proptosis Chemosis Ptosis CN III, IV, and or VI nerve palsies Other 50-80% Periorbital edema Headache Lethargy Optic disc edema Venous engorgement Other <50% Reduced VA 2/2 ION, CRA/VO, corneal ulcer Sluggish or dilated pupils Periorbital/corneal sensory loss Infectious, prothrombotic disorder (polycythemia, vasculitis, pregnancy, postpartum, oral contraceptives),trauma, surgery, or compression by tumors of skull base/nasopharnyx o DDx Orbital cellulitis High flow Carotid-cavernous fistula Periorbital edema, ophthalmoplegia, increased IOP, decreased vision

7 Supraorbital bruit Arterialized conjunctival vessels o Tx Treat infection/underlying disease Pituitary Apoplexy o Signs/Symptoms Bitemporal or junctional scotoma field loss Unilateral or bilateral ophthalmoplegia CNIII >CNVI>CNIV palsies o Systemic symptoms Severe headache Neck Stiffness o Life threatening because compression of brainstem/hypothalamus causes reduced less of consciousness, thermoregulation, and cardiorespiratory function Hypofunction of pituitary à hypothyroidism, hyponatremia, hypercortisolism o Epidemiology % of surgically managed pituitary adenomas Males>females ~60% Peak incidence 40s Hemorrhage/infarction of pituitary tumor causing sudden enlargement of pituitary gland Possible precipitating factor Hypotension, malignant hypertension, anticoagulant/dopaminergic agonist treatment o Management MRI High-dose corticosteroid replacement therapy followed by surgery