Neuroophthalmological Emergencies

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Neuro-ophthalmological Emergencies

• Luis A. Serrano,M.D.

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Case 1

• 62 yr. old female
• May,1996 developed bilateral ptosis and
  generalized weakness.
• Tensilon test.
• March,1997 thymectomy.
• Treated with Prednisone, IV immuneglobulin and
  Cellcept.
• Lid crutches for ptosis.

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• May,2006 decreased vision left eye
• June, 2006 uneventful cataract surgery L eye
• July 25,2006 headache
• Ct scan negative.
• RA , deceased C3/C4
• August 11, 2006 ptosis LUL
• MRI/MRA, spinal tap - negative

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Differential Diagnosis

• Are new signs due to Myasthenia
• Could this be Temporal arteritis
• Tolosa Hunt syndrome
• Diabetic third nerve palsy

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Aneurysm

• Cause 13-30 of oculomotor nerve palsies.
• 35 - 40 of aneurysms at this location
  eventually will cause a third nerve palsy.
• Almost 90 of unruptured aneurysms presenting
  with 3rd nerve palsy are gt 4mm in diameter.

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Aneurysms

• 90 of patients develop symptoms when they
  rupture.
• 70-80 mortality rate.
• 30 of patients do not reach the hospital.
• 33 of patients die within six months.
• 15 of patients die during the next decade.

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Literature

• Non visualization of a Large Cerebral Aneurysm
  despite high resolution MRA Turtz ,A. et al
  Journal of Neurosurgery 294-295, 1995.
• Negative MRI vs. Real Disease. Weinberg, D.
  Surv Oph 312, Jan-Feb, 1996.
• Third nerve palsies and angiography. Keane, J.
  Arch. Neurol, Vol 48 pg. 470, 1991.

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Case 2

• 67yr. old hypertensive male
• Chief complaint - sudden painless visual loss in
  the right eye.
• Flu like symptoms and headaches one week prior to
  event.

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Eye Exam

• Acuity O.D. CF O.S. 20/30
• Ext. exam - normal
• Pupils - R afferent defect
• Extraocular movements - full

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Clinical Course

• Diagnosis Ischemic Optic Neuropathy
• Sed Rate( Westergreen) 90mm/hr
• Prednisone 60mg. daily
• Referred for temporal artery biopsy.
• One week later lost vision in the left eye.

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Clinical Course

• Hospitalized PRMC.
• Solumedrol IV given for 5 days.
• Temporal artery biopsy

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Giant Cell Arteritis (Temporal Arteritis, Cranial
Arteritis)

• Systemic necrotizing vasculitis.
• Affects primarily cranial branches of arteries
  that originate from the arch of the aorta.
• Common in U.S. and Europe.
• Incidence - 0.49- 23.3/100,000 over 50
• Rare under 50
• Mean age of onset is 70 years.

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Systemic Manifestations

• Headache most common symptom
• Constitutional symptoms
• Alterations mental status
• Scalp tenderness
• Jaw claudication
• Respiratory tract symptoms
• Cardiovascular symptoms

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Neurological Manifestations

• Occur in 30 of patients with GCA.
• Peripheral neuropathies
• Mononeuropathies or polyneuropathies.
• Stroke
• Cerebral, cerebellar, brainstem

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Neuro-Ophthalmological Manifestations

• Visual loss
• Transient
• Permanent
• 10-60 of patients

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Visual Loss in Giant Cell Arteritis

• Posterior Ischemic Optic Neuropathy
• Homonimous Visual Field Defects
• Cortical Blindness.
• Visual Hallucinations.
• Ocular Ischemic syndrome

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Diplopia in Giant Cell Arteritis

• Craneal nerve palsies

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Diplopia In Giant Cell Arteritis

• Brainstem Ischemia
• INO, Skew, One and a Half Syndrome

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Laboratory Studies

• Normochromic normocytic anemia
• Thrombocytosis
• High Sed Rate 90 of patients with biopsy
  proven GCA.
• C reactive protein odds of positive biopsy
  increase when CRP is above 2.45mg/dl.

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Diagnosis

• Suspect the condition in any elderly patient with
  visual loss or diplopia even when the patient has
  no constitutional or systemic symptoms.
• CBC, Sed Rate, C-reactive protein
• Temporal artery biopsy.

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Treatment

• Goal
• prevent visual loss in opposite eye
• prevent ischemic damage in other organs
• If diagnosis is suspected start steroids.
• Dont wait for biopsy.
• Steroids
• 1.5 2.0mg/Kg/ day
• 1 gm Solumedrol IV for 3 days

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Treatment

• Maintain on high dose oral steoids until sed rate
  decrease and symptoms improve.(2- 4 weeks)
• Start tapering 10 of total daily dose /1-2
  weeks.
• Monitor symptoms, Sed rate and CRP before
  lowering dose.

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Prognosis

• GCA runs a self limited course that lasts 1-2
  years.
• May remain active for 5 -14 yrs.
• Prognosis for life is good
• Prognosis for vision loss is poor.
• 58 of patients have mayor complications of
  steroids.

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Case 3

• 62 yr. old male
• Chief complaint diplopia of 4 days duration.
• 5 days prior to his visit he woke up with R
  frontal headaches not relieved wirh Tylenol.
• Saw his general practitioner Rx. Demerol

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• Next day pain recurred.
• Went to emergency room spasms in vessels of the
  eye
• Ct scan head - normal. Non specific increased
  density in area of sella.
• Dx. Migraine Rx. HD45 Nubain IM D/C home
• Following day diplopia
• Again to E.R. Decadron 4mg q 6 hrs

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Neuro-oph exam

• Visual acuity O.D. 20/30 O.S. 20/40
• External exam mild ptosis RUL
• Pupils 4 mm RTL3 no RAPD
• Fundus assymetric glaucomatous cupping.
• Intraocular pressure O.D. 20 O.S. 28mm Hg.
• Motility mild weakness of RSR.

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Visual Fields
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Pituitary Apoplexy

• Occurs in 5-10 of untreated patients
• Acute swelling and infarction within a
  preexisting pituitary macroadenoma
• Precipitating factors anticoagulation,head
  trauma, surgery, treatment with radiation or
  bromocriptine

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Pituitary Apoplexy

• Symptoms - headaches, visual loss,
  ophthalmoplegia.
• Frequent misdiagnosis migraine,SAH
• Complications blindness, hyperpyrexic coma

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Treatment

• IV steroids
• Immediate referral to neurosurgeon.

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David,N. Pituitary Apoplexy goes to the Bar
Litigation for Delayed Diagnosis, Deficient
Vision and Death. J. Neuro-Ophthalmol
200626128-133

• No insurance, no care.
• Large suprasellar tumors may produce no obvious
  endocrine or neurologic symptoms.
• Misdiagnosis of glaucoma and migraine
• False negative CT finding
• Impotence failed to trigger an intracraneal
  evaluation.

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Increased Intracraneal Pressure
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Case 4

• 25 yr. history of congenital cataracts and
  hydrocephalus. Shunted at age 2.
• May 23, 2001 - Visual acuity O.D. 20/70 O.S.
  20/40
• June 8, 2001 - headaches
• June 20, 2001- visual loss
• July 11, 2001 - NLP optic atrophy

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Shunt Complications

• Shunt failures
• gt70 of patients have been operated twice
• Shunt failure rate 40 first year and 50
  second year
• Infection 5 -10
• Pulmonary hypertension and embolism
• Shunt nephritis
• Slit ventricles

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Slit Ventricles

• Present in CT Scan of 3-70 of infants
• 3-5 develop symptoms of increased intracraneal
  pressure
• Occurs between one and five years after shunt
  placement
• Etiology poorly understood

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Symptoms of Shunt Malfunction

• Irritability
• Poor feeding
• Lethargy
• Headaches
• Vomiting
• Fever

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Neuro-ophthalmic Complications of Shunt Placement

• Blindness - optic canal
• Chiasmatic compression - suprasellar cistern
• Hemianopsia - optic tract
• Six nerve palsy - floor 4th ventricle
• Dorsal midbrain syndrome - post commissure
• Internuclear ophthalmoplegia - midbrain parenchyma

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• Shunt failure without ventricular proclaimed by
  ophthalmic findings. Katz, D. J. Neurosurg.
  Vol. 81, Nov. 1994.
• Neuro-ophthalmic complications of raised
  intracraneal pressure, hydrocephalus, and shunt
  malfunction. Chou, S. Neurosurgery Clinics of
  North America, Oct. 1999, Vol. 10 No. 4.
  P587-607.
• Neuro-ophthalmogical symptoms in children
  treated for internal hydrocephalus. Tzkov, C.
  Pediatric Neurosurg 1991-92217-320

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• Bilateral visual loss and disc edema in
  15-year-old girl. Newman, N. Survey of
  Ophthalmology. Vol. 38, No. 4. January-February
  1994.
• Visual loss as the manifestating symptom of
  ventriculoperitoneal shunt malfunction. Lee, A.
  AJO, July 1996, Vol. 122, No. 1, P127-129
• Permanent visual loss after shunt malfunction
  Arroyo,H. Neurology 3525-29,1985

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Predicting shunt failure on the basis of
clinical symptoms and signs in children. Garton,
H. J. Neurosurg 94202-210, 2001

• Ruling out malfunction remains problematic
• Early failure (lt5 months) - 26
• Symptoms irritability, nausea and vomiting
• Signs - LOC, papilledema, erythema around shunt,
  and peritonitis
• Late failure (gt9 months) - 12
• Symptoms - loss of milestones and decreased
  level of consciousness