Rex Ballinger, OD FAAO VAMHCS Baltimore,MD, David Reed, OD FAAO, Havre de Grace, MD
Optic nerve overview:
 The subarachnoid space of the brain is continuous with the optic nerve sheath
 four parts; intraocular, intraorbital, intracanalicular, intracranial
 Intraorbital: ~5 mm in diameter, with fat surround.
 Intracanalicular: lesser wing of the sphenoid, surrounded by muscle cone
 subarachnoid space: (CSF) with intracranial continuity
Case: Doc, the nerve is swelling up!!
Papilledema:
 optic disc swelling secondary to elevated intracranial pressure
 vision usually is well preserved with acute papilledema
 almost always presents as a bilateral phenomenon
 may develop over hours to weeks
 The term is not be used to describe optic disc swelling with underlying infectious, infiltrative, or inflammatory etiologies.
Pathophysiology:
 Disc swelling is result of axoplasmic flow stasis with intra-axonal edema in optic disc area
 cerebrospinal fluid (CSF) pressure increases
 pressure is transmitted to the optic nerve
 optic nerve sheath acts to impede axoplasmic transport
 accumulation of material at the level of the lamina cribrosa
 characteristic swelling of the nerve head
 Papilledema may be absent in cases of prior optic atrophy
Symptoms:
 Headache: worse on awakening, exacerbated by coughing or other Valsalva maneuver.
 Nausea and vomiting: if severe rise in ICP. Mmay be followed by a loss of consciousness, pupillary dilation, and death.
 Visual symptoms often are absent: However: o transient visual obscurations (may be orthostatically related o Blurring of vision o constriction of the visual field o decreased color perception may occur. o Diplopia may be seen occasionally if a sixth nerve palsy is associated. o Visual acuity is preserved except in very advanced disease.
Clinical evaluation:
 Disc hyperemia
 Subtle edema of the nerve fiber layer especially nasal disc
Obscuration of fine peripapillary vessels.
Later obscuration of disc margins
 Small NFL hemorrhages
 Spontaneous venous pulsations, followed by venous congestion/ exudates/CWS
 Peripapillary retinal radial folds (Paton lines). Choroidal folds also may be seen.
 Chronic disease may lead to atrophy.
Etiology:
 Any tumors or space-occupying lesions of the CNS
 Idiopathic intracranial hypertension
 Decreased CSF resorption
 Increased CSF production (tumors)
 Obstruction of the ventricular

 system
Cerebral edema/encephalitis
Craniosynostosis
Management:
 Tailored medical or surgical management
 Specific therapy should be directed to the underlying mass lesion if present.
 Diuretics: acetazolamide useful in selected cases (esp. idiopathic intracranial hypertension).
 Weight reduction .

 Corticosteroids
 Surgical Care: removal of mass lesion
 Lumboperitoneal shunt or ventriculoperitoneal shunt.
 Optic nerve sheath decompression
 Diet: (idiopathic intracranial hypertension)
Pseudotumor Cerebri
Pathophysiology: unclear.
 Presumed resistance to absorption of CSF across the arachnoid villi.
 Other theories: abnormality in cerebral circulation with increase in brain's H20
 increase in ICP transmitted intracranial structures, including the optic nerves
 commonly occurs in women who are overweight
 role of obesity is unclear
 increased intra-abdominal pressure suggested affecting cardiac filling pressures
 leads to impeded venous return from the brain
 untreated: may lead to optic neuropathy.
DDx:
 Pseudopapilledema
 Drusen of the optic nerve heads
 Malignant hypertension
 Bilateral infiltrative/infectious/inflammatory optic neuropathy
 Bilateral anterior ischemic optic neuropathy
 Bilateral optic nerve papillitis
 Bilateral optic nerve tumors (eg, glioma, meningioma)
Work-up
 Neuroimaging
 Ultrasonography
 Lumbar Puncture
Management:
 Monitor: visual acuity, color vision, optic nerve head observation, and perimetry.
 Weight control for patients who are overweight, 6% weight loss has been shown to reduce ICP and papilledema.
 Use of diuretics to control the intracranial pressure: Acetazolamide 1 g/d (500 mg sequel bid) up to 2 g/d
 furosemide if intolerant to acetazolamide (but less effective)
 Corticosteroids: if inflammatory etiology, may supplement acetazolamide
 Optic nerve sheath fenestration
 Lumboperitoneal or ventriculoperitoneal shunt
The case of the hurting nerve
Optic neuritis:
 Inflammation of the optic nerve- pain
 initial episode in multiple sclerosis
 DX is usually made clinically.
.
 15- 20% of MS cases manifest as optic neuritis
 38-50% of MS patients develop optic neuritis at some point
 risk of development of MS after an episode of isolated optic neuritis was 30% at 5year follow-up and 38% at 10-year follow-up.
Other Etiologies:
 Lyme disease  orbital cysticercosis
 Tuberculosis
 Syphilis
 viral agents such as HIV, hepatitis
B virus, herpes virus, and CMV
 paranasal sinus infection




 radiation therapy drugs gluten sensitivity hypereosinophilic syndrome vasculitis (GCA).

Management:
Optic Neuritis Treatment Trial:
 28% and 35% of patients developed recurrence within 5 and 10 years,
Most recover spontaneously
 Intravenous methylprednisolone therapy has shown to increase rates of visual recovery without significant long-term benefit for visual function.
 Corticosteroids: considered for patients who require faster recovery
 Treatment with corticosteroids and/or immunomodulation agents (eg, interferon beta-
1a, interferon beta-1b, glatiramer acetate) higher risk pts. (multiple sclerosis. )
Optic nerve glioma
 cell of origin for optic nerve gliomas is unknown
 classified as grade I astrocytomas (usually not, but may develop malignancy
 meningeal hyperplasia vs perioptic meningioma.
Work-up:
 MRI with gadolinium- imaging of choice
 CT: evaluate erosion or expansion of the optic canal
 Ultrasound: not helpful
Management:
 Varies with size of tumor and pts. Health
 Goal: cure the disorder, relieve symptoms, or improve vision or comfort.
 Surgical removal may cure some optic gliomas
 Partial removal to reduce some of the bulk of the tumor in many cases
 Radiation therapy if tumor extensive and surgery not possible
 Corticosteroids reduce swelling and inflammation during radiation Tx, or if symptoms recur.
The Cases of the swollen optic nerves:
A.
Disc edema
B.
Papilledema:
1.
Definition: bilateral disc edema secondary to increased intracranial pressure
2.
EMERGENCY!!
C.
Differential diagnosis of swollen optic nerves
1.
Ischemia: AION
Sex a.
Most common acute optic neuropathy > 50 years old
2.
Inflammation: optic neuritis
3.
Infiltrative: Sarcoid
4.
Infectious
5.
Compression: anterior orbital lesions
6.
Idiopathic and diabetic papillopathy
D.
65 yo WF Routine Eye Exam: Giant Cell (Temporal) Arteritis:
Characteristic
Age
AAION mean: 70
NAAION mean: 60
F>M F=M
Assoc. Symptoms
Visual Acuity
Optic Nerve
ESR
CRP
HA, scalp tenderness jaw claudication
TVL
< 20/200: 60% pallid edema normal size cup mean= 70m/hr male: age/2 female: age+10/2 high
high CRP & ESR=97% specific for GCA none
> 20/200: 60%
Pallid or hyperemic small cup: “disc at risk” mean=20-40mm/hr normal

FFA
Natural History
Management
Alphagan: 5/day disc and choroidal delay rarely improve fellow eye: 54%-95%
TA biopsy
1-2g. IV methylprednisone x 2-5 days
then 60-100mg oral prednisone x 6-8 weeks
then taper according to ESR/CRP months-year disc delay up 42% improve fellow eye: 12%-19% none proven:
1.
Arteritic Anterior Ischemic Optic Neuropathy: 5%-10% a.
Giant Cell Arteritis b.
American College of Rheumatolgy: need 3 of 5 criteria for diagnosis i.
development of symptoms or findings > 50 years old ii.
new onset, or new localized headache iii.
temporal artery tenderness or decreased pulsation iv.
Westergren ESR > 50mm/hr v.
positive TA biopsy: mononuclear cells or granulomatous inflammation c.
ocular manifestations: i.
ischemic optic neuropathy: anterior: 40%, posterior: 3% ii.
amaurosis fugax: 2%-19% iii.
central retinal artery occlusion: 15% iv.
other: vein occlusion, EOM palsy, retinal ischemia
2.
Non-Arteritic Anterior Ischemic Optic Neuropathy: 90%-95% a.
Risk Factors: i.
HTN: 50% ii.
Diabetes: 25% iii.
Other: smoking, collagen-vascular, hyperlipidemia, chronic renal failure & dialysis, and migraine b.
Pseudo Foster Kennedy Syndrome c.
IONDT: Ischemic Optic Neuropathy Decompression Trial: No benefit d.
NPION: Neuro Protection in Ischemic Optic Neuropathy: Pilot study i.
Alphagan 5 times per day ii.
May have small benefit: larger trial needed
E.
9 yoWF referred for blurry vision in one eye: Cat Scratch Neuroretinitis
1.
Organism: a.
Bartonella henselae: gram (-) rickettsia b.
transmitted: cats or fleas from infected cats
2.
Characteristics: a.
regional, painful lymphadenopathy b.
fever c.
malaise d.
2 weeks after cat scratch
3.
Clinical Findings: a.
Parinaud oculoglandular syndrome: i.
most common ocular manifestation: 7% ii.
granulomatous conjunctivitis iii.
PAN b.
Neuroretinitis: i.
Most common neuro-ocular manifestation ii.
visual loss with optic disc edema iii.
uveitis iv.
retinal edema with exudates: stellate v.
white retinal inflammatory lesions c.
BRAO: rare: retinal vasculitis with focal retinitis
4.
Management: a.
usually self-limited b.
doxycycline 100mg bid PO c.
ciprofloxacin 750 mg bid PO d.
other: trimethoprim-sulfamethoxazole & clindamycin