Neuro-OphthalmicEmergencies

Raed Behbehani , MD FRCSC

What is an emergency ?

•Vision threatening ?

•Life threatening ?

•Recognition.

•Proper investigations/imaging study.

•Appropriate referral.

Painful Diplopia

Case•A 53 year old patient with acute

diplopia.

•Previous episodes few years ago , which lasted two months and recovered.

•Diabetes, and hyprlipedemia.

•Visual acuity : 20/20 OU.

•Pupils : Equally reactive , pupils equal in size.

Case

Pupil-Sparing Third Nerve Palsy

• Diabetes, hypertension, hyperlipedemia, smoking, high hematocrit.

• Pupils is spared.

• Pupil involvement reported only in 14%-32% , but anisocoria (difference in pupil size) is less than 1 mm (relative-sparing).

• Improve within 4-12 weeks (defer neuro-imaging).

Case•78 year old man with acute diplopia,

and headache.

•Diabetes, hypertension, atrial tachycardia.

•Prior history of tight feeling around the eye with 20 seconds of diplopia.

•No history of jaw claudication or transient visual loss.

Pupil-involving 3rd Nerve Palsy

•Pupil involvement indicates compression of the pupillary fibers.

•Posterior communicating artery aneurysm, or mass.

•Appropriate neuro-imaging is (MRI/MRA, MRI/CTA, Angiogram is the gold standard for aneurysm detection).

Risk of Aneurysm and “Rule of Pupil”Ophthalmopleg

iaPupil Aneurysm Risk

Complete/Partial

Complete 86%-100%

Partial Spared 30%

Complete Spared very low

If signs of sub-arachnoid hemorrhage present (headache, photophobia, nausea) “rule of pupil” does not apply.

Painful Ptosis and Anisocoria

Case• A 67 year old man presents with pain in his right eye

for 5 days.

• Hypertension and ischemic heart disease on treatment.

• No double vision.

• VA : 20/30 OU.

• Mild nuclear sclerosis cataracts.

• Fundus: normal.

Case

Evaluation of Horner’s

•Misois, and ptosis (upper and lower lid).

•Dilatation lag, anisocoria worse in dark.

•Cocaine test.

•Hydroxyamphetamine (not used much).

•Iopidine.

•MRI/MRA of the head/neck/upper chest CT.

Oculo-sympathetic Pathway

Acute Horner’s Syndrome

•Painful Horner’s syndrome is a neurologic emergency.

•Although can be seen in many types of headaches (Cluster, Migraine etc).

•Rule out ICA dissection.

•MRI/MRA of the head/neck/upper mediastinum is indicated.

Horner’s Syndrome (MRI)

ICA dissection

•Goal is to prevent secondary neurologic deficit (stroke).

•Obsevation , Anti-coagulation, or stent implantation.

•Referral for a neurovasculr specialist.

Acute vision loss in an elderly patient

Case• A 68 year old patient with sudden loss of

vision in the right eye.

• History of episodes transient loss of vision.

• Diabetes for 30 years.

• Feeling unwell lately with, and loss of appetite, malaise and myalgias.

• Visual acuity: Count finger right , 20/30 left.

• Right RAPD.

Case

Case

Cord-like STA

Case Investigations

•ESR = 86

•CRP positive.

•Platelets elevated ( 560).

•Mildly anemic.

Arteritic Ischemic Optic Neuropathy • New onset of headache (temporal) , acute or

transient loss of vision, jaw claudication, weight loss, fever, and myalgias.

• Age usually over 60.

• Occult GCA ( No systemic symptoms, transient diplopia or transient visual loss).

• A true neuro-ophthalmic emergency (54-95% second eye involvement if untreated) !

• Giant cell arteritis (systemic vasculitis, Aortitis in 20% consider PET/MRA).

GCA

Central retinal artery occlusion Branch-retinal artery occlusion

Posterior Ischemic Optic Neuropathy

(PION)• Both the retina and optic nerve look

normal.

• PION is relatively common in Giant Cell arteritis.

• Flourescin angiogram can show choroidal hypoperfusion.

• Involvement of 2 circulations (systemic vasculitis), retinal artery occlusion and AION indicate giant cell arteritis.

GCA

Diplopia (transient,

constant)Ophtha

lmoplegia

AAION Management

• Stat ESR , CRP and CBC (platelets).

• ESR can be normal in 15-20% of cases.

• CRP is more sensitive and specific.

• CRP and CBC have 97% sensitivity and specificity.

• Start high dose systemic steroids (IV or Oral) immediately upon suspicioun ( AAION can develop in fellow eye within days if untreated !)

• Arrange for temporal artery biopsy within 2 weeks , while patient is on steroids.

QuickTime™ and a decompressor

are needed to see this picture.

Video

TAB

GCA Treatment

•Systemic steroids for a at least 1-2 years.

•Titrate dose according to laboratory indices (CRP,ESR) and symptoms.

•Manage diabetes and osetoporosis.

•Collaboration with rheumatologist.

“Worst Headache”

Case

• 52-year-old, morbidly obese man presents with severe headache (worst in his life).

• Ischemic cardiac disease and angioplasty, COPD, hypertension, and NIDDM.

• On examination: complete right ptosiswith unreactive mid-dilated right pupil، left partial ophthalmoplegia with V1 hypesthesia.

Case

Visual Fields

Visual Field Defects in Chiasmal Syndrome

MRI

Pituitary mass with high signal on T1

Pituitary Apoplexy• “Worst headache in my life”.

• Visual loss, and/or ophthalmoplegia ( uni- or bilateral).

• Patients usually present 2 weeks after ictus.

• > 80% did not have history of pituitary tumor

• Ophthalmoplegia (extension to cavernous sinus with cranial nerve involvement).

• Life threatening (hypotension, shock) because of hypo-pituitarism, and low cortisol levels, and diabetes insipidus.

Case

•A 50 year old with blurred vision and headache for the last 2 weeks.

•Medical History : Diabetes for 5 years.

•Smoker 15 years.

•No prior Surgeries

Case

•Conscious and oriented.

•Visual acuity : 20/20 OU

•Pupils : PERL no RAPD.

•Normal anterior Segment .

•Normal ocular motility.

Case

Case

•CT and MRI/MRV - normal.

•Blood pressure 220/150 !

Malignant Hypertesnion

•Accelerated hypertension with target organ damage.

•Papilledema must be present for diagnosis !

•Dysfunction of cerebral blood flow autoregultaion causing cerebral edema.

•Pre-eclampsia .

•Encephalopathy can be present.

Proptosis in immunocompromised

or diabetic patient

Case •60 year old man with myelodysplastic

disorder on chemotherapy.

•Proptosis, fever, and dyspnea .

•Periorbital swelling and erythema, which got worse over 3 days.

•Visual acuity : 20/20 Both eyes.

•Normal pupils, ocular motility and fundus examination.

Case

CT

Mucormycosis• Vascular thrombosis, tissue necrosis, and fungal

dissemination.

• The mortality rate is as high as 90%.

• Diabetic ketoacidosis , immunosuppressed, organ transplant patients, steroid use, and desferrioxamine.

• Other fungal organisms: Aspergillus.

• Pain and ophthalmoplegia.

• CT of the orbit/paranasal sinuses/cavernous sinus or MRI of the orbit with fat suppression.

Mucor• Immediate biopsy (ENT/Orbit) , with

debridement.

• Orbital exenteration is not always needed.

• Correct any metabolic acidosis to reduce unbound iron (critical for the proliferation of mucor)

• Local delivery of amphotericin B with indwelling catheters.

• Systemic antifungal (IV liposomal encapsulated Amph B less nephrotoxic +- posaconazole).

• Boost immunity (correct neutropenia).

Mucor

• Medical therapy and surgical debridement increase the survival rate (78%) compared to medical management alone (57.5%).

Mucormycosis

Mucor

Non-septate hyphae with branching at 90 degrees.

Summary• Pupil involvement in 3rd nerve palsy suggests

compressive lesion (aneuurysm), get and MRI/MRA or MRI/CTA.

• Always rule out ICA dissection in acute Horner’s syndrome.

• Always rule out GCA as the etiology for ophthalmoplegia or visual loss in >60 year patients.

• In acute severe headache with ophthalmoplegia with multiple CN involvement think of pituitary apoplexy.

• Proptosis and eye redness in diabetic/immunospressed patients can be due to life-threatening fungal infection.