Neuro-Ophthalmology
Optic Neuropathies
Differential Diagnosis of Optic neuropathies
Causes of a Swollen Optic Nerve
- Congenital
- Anomalous
elevation
- Hyaline
bodies (drusen)
- Gliotic
dysplasia
- Ocular
disease
- Uveitis
- Hypotony
- Vein
occlusion
- Inflammatory
- Papillitis
- Neuroretinitis
- ?
Papillophlebitis
- Infiltrative
- Lymphoma
- Reticuoendothelial
- Systemic
disease
- Anemia
- Hypoxemia
- Hypertension
- Uremia
- Disc
tumors
- Hemangioma
- Glioma
- Metastatic
- Vascular
- Ischemic
neuropathy
- Arteritis,
cranial
- Arteritis,
collagen
- Juvenile
diabetes
- Proliferative
retinopathies
- Orbital
tumors
- Perioptic
meningioma
- Glioma
- Sheath
"cysts"
- Retrobulbar
mass
- Graves'
disease
- Elevated
intracranial pressure
- Mass
lesion
- Pseudotumor
cerebri
- Hypertension
Typical Clinical Features of Optic
Neuropathy
- Decreased visual acuity
- Decreased color vision
- Visual field defect (central
scotoma, arcuate, altitudinal) not specific of type of optic neuropathy
- Ipsilateral RAPD if
unilateral or asymmetric
- Light-Near dissociation in
bilateral symmetric cases
- Optic
disc edema or atrophy (normal in retrobulbar optic neuropathy)
Compressive Optic Neuropathy
Painless, progressive, gradual vision loss.
Optic disc edema or atrophy, RAPD.
Optociliary shunt vessels (collateral circulation between retina and
choroidal circulation).
Orbital signs: proptosis, chemosis, conjunctival injection.
Causes:
- Intracranial/intraorbital
benign and malignant tumors: meningioma, glioma, craniopharyngioma,
pituitary adenoma, lymphoma, metastasis
- Inflammatory or infectious
disease: mucoceles
- Primary bone disesease:
osteoporosis, fibrous dysplasia
- Vascular: orbital
hemorrhage, carotid artery aneurysm, dolichoectasia of carotid, AVM
- Hydrocephalus
- Iatrogenic: intranasal
balloon catheter, intracranial oxidized callulose hemostat
- Thyroid
ophthalmopathy
Infiltrative/Inflammatory Optic
Neuropathy
- May present with typical features of optic neuropathy
- Causes:
- Optic Neuritis
- Neoplastic:
plasmacytoma, carcinomatous meningitis, leukema, lymphoma, paraneoplastic
disease
- Infectious:
cryptococcus, aspergillus, Lyme disease, tuberculosis, syphilis
- Inflammatory:
Churg-Strauss, Contiguous sinus disease, Sarcoidosis, SLE, Sjogren's
syndrome, relapsing polychondritis, polyarteritis nodosa, inflammatory
bowel disease
- Inflammatory autoimmune
causes respond to steroids.
- Consider: Lumbar puncture,
CBC, syphilis serology, ANA, Lyme titer, CXR, ACE
- See: Work-up of Atypical or unexplained optic
neuropathy
Toxic/Nutritional Optic Neuropathies
- Painless, bilaterally
symmetric, slowly progressive visual loss.
- VF defect: bilateral central
or cecocentral loss.
- Causes of Toxic
Neuropathy
- Common:
- Ethambutol: 6% at
doses of 25mg/kg/day. Less than 15mg/kg/day safer
- Ethanol and Tobacco:
may be B12 and Folate deficiency, cyanide in tobacco.
- Chloramphenicol
- Less common:
- Amniodarone,
Ethylene glycol, 5-Fluoruracil, Glue sniffing, Heavy metals (arsenic,
lead, mercury), Isoniazid, Methanol, Methotrexate, Vincristine
- Causes of Nutritional
Optic Neuropathy
- B6, B12, Folate,
Niacin, Riboflavin, Thiamine (B1)
- Evaluation of presumed
Toxic/Nutritional optic neuropathy:
- MRI of optic nerves
- Serum B12, Folate
- Erythocyte Folate
level
- CBC with diff
- Urine heavy metal
screen (mercury, lead, arsenic)
- Syphilis serology
- Leber's hereditary ON mutational
analysis
Ischemic Optic neuropathy:
Non-Arteritic Anterior Ischemic Optic Neuropathy
Typical Clinical Features
- >40 years old, painless
(pain in 8-12%)
- Unilateral VA or visual
field loss (central, cecocentral, arcuate, altitudinal)
- Optic
disc edema, RAPD
- Small Optic cup
- Underlying vasculopathic risk factors
- Lack of premonitory
symptoms (transient visual loss)
- Visual loss remains static
but may progress
Atypical Clinical Features
(reasons to exclude other causes of optic
neuropathy)
- < 40 years old (AIONY-
AION of the young)
- Bilateral, simultaneous
onset (15% of AION but think Arteritic AION first)
- Visual field not consistant
with optic neuropathy (homonomous defect)
- No acute disc edema or RAPD
- Large Cup
- No vasculopathic risk factors
- Amaurosis
fugax
- Progression of visual loss
beyond 2-4 weeks
- Recurrent episodes in same
eye
- Anterior or posterior
segment inflammation
Conditions associated with
Ischemic Optic Neuropathy (Anterior and Posterior)
- Systemic Vasculopathy
- HTN
- Diabetes
- Arteriosclerosis,
atherosclerosis, ischemic heart disease
- Uncommon:
- Female carrier of
Fabry's dz
- Takayasu's arteritis
- Carotid artery dz:
occlusive dz & carotid dissection
- Thromboangiitis
obliterans
- Vasospasm from
migraine or Raynaud's dz
- Acute blood loss or
hypotension
- Postsurgical: CABG, Lumbar
spine, Abdominal, Radical neck, Mitral valve
- After treatment of
Malignant hypertension
- Hemodialysis
- Nocturnal hypotension
- Phlebotomy
- Cardiac Arrest
- Intra Ocular surgery: CE,
secondary IOL, Retinal surgery
- Ocular: Hyperopia, Optic
disc drusen, papilledema, elevated IOP, Birdshot chorioretinopathy
- Infections: Aspergillus,
Herpes Zoster, Lyme dz, Herpes labalis, Staphylococcal cavernous sinus
thrombosis, syphilis
- Inflammations: connective
tissue dz, Rheumatiod arthritis, SLE, PAN, post viral vasculitis, Behcet's
dz,
- Hematologic: anemia,
leukemia, polycythemia vera, Sickle cell trait, Thrombocytopenic purpura,
Waldenstrom macroglobulinemia
- Embolic
- Medications: Oral
contraceptives, Interferon alpha, sumatriptin, intracarotid carmustine
- Misc.
- Post immunization
- Radiation necrosis
- Smoking
- Trauma
Evaluation
- If typical features present:
- exclude GCA in patients over 50
- no neuroimaging
- no carotid studies
unless there are other signs of carotid disease (Ocular ischemic sydrome)
- If atypical features present:
Treatment
- No proven therapy
- Medical control of
underlying HTN, diabetes, smoking
- Avoid overaggressive
control of blood pressure
- ASA Q day to reduce
morbidity and mortality from stroke, MI
Posterior Ischemic Optic Neuropathy
Arteritic Anterior Ischemic Optic Neuropathy (Giant Cell
Arteritis)
Typical Features
- Age >50 (median 75)
- Acute, often severe vision
loss
- Unilateral or bilateral
(higher incidence of bilateral than NA-AION)
- Pallid swelling of optic
nerve
- Optic atrophy and some
have glaucomatous like cupping
- Constitutional signs/symptoms
- Other
signs
Constutional signs/symptoms
- Headache (4-100%)
- Scalp or temporal artery
tenderness (28-91%)
- Weight loss (16-76%)
- Jaw Claudication (4-67%)
- Anorexia (14-49%)
- Proximal muscle aches or
weakness (28-86%)
- Polymyalgia Rheumatica:
morning stiffness >30 minutes, proximal joint pain
- Fatigue and malaise
(12-97%)
- Leg claudication (2-43%)
- Elevated ESR (usually
>50 by Westergren method)
- Temporal artery biopsy
positive
Other signs of GCA
- Visual Loss
- transient
visual loss
- non-embolic BRAO or CRAO
- combined CRAO and CRVO
- ophthalmic artery
occlusion
- posture related
retinal ischemia / choroidal or retinal ischemia / cotton wool
spots
- general anesthesia
induced ischemic optic neuropathy
- pre-, peri-,
post-chiasmal ischemic visual field defects
- Anterior Segment Ischemia
- episcleritis,
scleritis
- iritis,
conjunctivitis
- glaucoma, uveitic
glaucoma
- transient bilateral
corneal edema
- acute hypotony
- marginal corneal
ulceration
- Autonomic pupil
abnormalities
- tonic pupil
- light-near
dissociation
- Horner's syndrome
- miosis, mydriasis
- Diplopia
- orbital ischemia
- ophthalmoplegia due
to cranial nerve or brainstem ischemia
- INO, nystagmus
- transient diplopia
with or without ptosis
- tranisent oculomotor
synkinesis
- Orbital disease
- orbital pseudotumor
- orbital infarction
- reversible bruit
- Less common features
- large vessel
involvement (eg. subclavian /carotid / axillary / aorta / cerebral arteritis
/ coronary arteritis and MI)
- pain syndromes
(headache, neckache, backache)
- perepherial
neuropathies
- respiratory tract
(cough, hoarsness, tongue ischemia)
- visceral involvement
(eg. renal, liver, small bowel)
- ischemic skin lesions
- Mortality from MI or
mesenteric infarction
ESR
- Normal values:
- Men: age divided by
2
- Women: age + 10
divided by 2
- Alternative etiologies for
elevated ESR
- Infections
- Connective tissue
diseases
- Renal disease (esp.
nephrotic syndrome and uremia)
- Malignant neoplasm
(21% of neg TAB had malignancy in Hedges series1992)
- Diabetes mellitus
- Diffuse disseminated
atheroembolism
- Laboratory evaluation for
markedly elevated ESR (>100 mm / hr) includes:
- CBC with
differential
- BUN / creatinine,
ALP, SPEP, U/A,
- CXR, PPD with
controls
- Stool guaiac
- General physical
exam
CRP
- >2.45 mg /dl was 100%
sensitive for detection of GCA
- Combined with an elevated
ESR, specificity 97%
Temporal Artery Biopsy
- In those of high to moderate
clinical suspicion
- Highest sensitivity and
specificity for any test for GCA
- Positive result justifies
long term steroids
- Negative result saves
significant side effects of steroids
- Take 2-5 cm of
artery
- 14% diagnosed with
GCA based on second biopsy
- False negatives
occur (1-61%) therefore steroids are justified if clinical suspicion high
even with bilaterally negative TAB
- Can be done within a
few weeks of starting steroids
High clinical suspicion for GCA (American
College of Rheumatology 1990)
- Highest sensitivity
criteria:
- Highest specificity
criteria:
- Jaw and / or tongue
claudication
- Visual
abnormalities (e.g. AION, amaurosis, optic atrophy)
- Temporal artery
abnormalities (e.g. decreased pulse, tenderness, nodules)
- 91.% specific and 94%
sensitive with 3 of 5:
1.
Age >50
2.
New localized headache
3.
Temporal artery abnormality (decreased
pulse,tenderness, nodules)
4.
ESR >50
5.
Abnormal temporal artery biopsy (necrotizing arteritis,
multinucleated giant cells)
- Temporal artery biopsy,
unilateral, -bilateral, then occipital artery can be biopsied
- IF all negative, treat as
biopsy negative GCA or look for alternative reason for symptoms and
elevated ESR
- Repeat TAB may be
necessary long after steroids begun to look for reactivation of disease or
to prove absence of inflammation despite rising ESR
Moderate clinical suspicion for GCA
- Constitutional symptoms
absent in 18% of patients
- Check ESR / CRP and obtain
TA biopsy
- If ESR/ CRP elevated and
TAB negative:
- consider TAB of
other side
- If ESR and/or CRP normal
and TAB negative & few systemic symptoms
- If ESR and/or CRP elevated
and bilateral TAB negative
- consider alternate
etiologies of elevated ESR
- consider tapering
steroids
Low clinical suspicion for GCA
- Typical AION in know
vasculopath with no constitutional signs / symptoms
- Investigate alternative
etiologies of elevated ESR
Treatment
- Immediately start
corticosteroids:
- Prednisone 60-100
mg PO QD
- If severe visual loss,
bilateral involvement, monocular, or loss if vision during oral therapy:
- Taper by 10% per week as
long as ESR and symptoms remain stable
- Most can be tapered off
steroids within one year
- Some may require years or
indefinite steroid use
- If ESR rises: interrupt
taper 2-4 weeks
- If symptoms occur despite
stable ESR: might have to go up on steroid
- Side effects of chronic
corticosteroid use
- Cushinghoid
features
- Steroid myopathy
- HTN, DM,
osteoporosis
- compression fractures
(up to 25%)
- psychosis
- fluid retention
requiring diuretics
- cataract, glaucoma
- Other immunosuppressive
agents have little controlled data regarding efficacy
- deflazacort,
imuran, dapsone, cyclosporine, methotrexate, azathioprine
Optic Neuritis
Typical Features (suggesting
demyelination)
- Acute, unilateral vision
loss in young adult (<40 y/o), +RAPD
- Periocular pain (90%) esp
with eye movement
- Normal (65%) or swollen
(35%) optic nerve head
- Eventual visual
improvement over several weeks to normal or near normal VA (90%), may
recover for months
- Some complain of residual
deficits in contrast sens, color vision, stereopsis
Atypical Features:
suggestive of other cause of optic
neuritis
- Bilateral, simultaneous
onset in adult (>50 y/o)
- Lack of pain
- Findings suggestive of
inflammatory process: uveitis, macular exudate or star, retinal infiltrate
- Lack of visual improvement
or worsening after 30 days
- Lack of improvement of at
least one line of VA in three weeks
- Exquisitely steroid
sensitive or dependent
Other causes of optic neuritis
(often evident by history)
- Guillian-Barre synd
- Infections:
- Bacteria: TB,
syphillis, Lyme ,cat scratch, meningitis, mycoplasma, Whipples's dz
- Virus: adenovirus,
rubella, chicken pox, herpes zoster, HIV, infectious mono, measles,
mumps, influenza
- Protozoa:
toxoplasmosis
- Fungi: Aspergillus,
Histoplasmosis
- Parasites:
intraocular nematodes
- Post-vaccination:
smallpox, tetanus, rabies, influenza, BCG
- Focal infection/
inflammation: paranasal sinusitis, mucocele, beesting
- Systemic inflammation:
Behcet's dz, inflammatory bowel dz, Reiter's synd, Sarcoid, SLE
Evaluation of Optic Neuritis
- Work-up based on the Optic
Neuritis Treatment Trial
- MRI to help predict
risk of Multiple Sclerosis and for treatment decision making: See Neuroimaging
- If atypical features present: CXR,
directed lab tests (e.g., FTA-ABS, ANA, serum chemistry, CBC), lumbar
puncture
- Visual Evoked Potentials:
Not used routinely
- may be helpful in
identifying a second site of neurologic involvement (previous optic
neuritis) with no examination findings or history of optic neuritis.
Neuroimaging in Optic Neuritis
- MRI
- periventricular
white matter changes 40-70%
- gadolinium enhancing
lesions 26-37%
- Risk of developing MS in
1-4 years is 30% with optic neuritis and abnormal MRI.
- Beck et al
(1993,1995) 36% developed MS in 2 yrs with 2 or more lesions on MRI. But
only 5% if MRI was normal or 1 lesion.
- Absence of MRI findings
does not protect patient with Optic Neuritis from developing MS
Treatment of Optic Neuritis (ONTT)
- Methylprednisolone sodium
succinate 250mg IV Q 6hr for 3 days
- Followed by: prednisone
1mg/kg/day PO for 11 days
- Reduction in rate of
clinically definite MS during first 2 yrs. especially in patients with MRI
abnormalities.
- Treatment within 2 months
of visual loss might be of benefit (not ONTT)
Risk of Developing Multiple Sclerosis
- 25-35% risk after optic
neuritis
- probably higher for women
than men
Work-up of Atypical or unexplained optic
neuropathy
First:
- MRI of optic nerves
- ESR, CBC with diff,
Syphillis serology, ANA
- CXR, ACE
- LP
Second:
- Gallium Scan if
sardoidosis suspected
- PPD for TB
- Anti-DS DNA, compliment
levels for SLE or other collagen vascular disease
- Leber's hereditary optic
neuropathy mutation blood test
- Heavy metal screeen
- Serum B12 and Folate
- Lyme titer in edemic area
or exposure history
Hereditary Optic Neuropathies
Leber's hereditary optic neuropathy
- Mitochondrial DNA mutation:
11778, 3460, 14484
- Young Males (90%)
- Rapid acute, painless,
unremitting visual loss usually (20/200 to HM)
- Sequential bilateral
(40-50% simultaneous, or second eye days to weeks to months later)
- VF loss: central or
cecocentral scotomas
- Fundus findings during
acute phase: Triad
- Telangiectatic
microangiopathy
- Apparent swelling of
NFL around disc
- no leakage on FFA
- Fundus findings after
visual loss
- attenuated
arterioles
- NFL loss esp.
papulomacular bundle
- temporal optic nerve
pallor
- nonglaucomatous
cupping
- Associated findings rare
- cardiac conduction
defects
- dystonia
- multiple
sclerosis-like clinical pattern
- Treatments unproven
- stop smoking /
cocaine / alcohol reducing metabolic stress
- multivitamins,
folate, B12, thiamine
Dominant Optic Atrophy
- Chromosome 3 (OPA-1
gene)
- Ganglion cell death
- Bilateral temporal pallor
- Painless progressive
bilateral vision loss in a young person. Average VA 20/160
- Chromosome 18 (one family)
Other Hereditary Optic Neuropathies/
Atrophies
- Dominant optic atrophy with
congenital deafness
- Dominant optic atrophy with
progressive hearing loss and ataxia
- Dominant optic atrophy with
ataxia and pes cavus
- Recessive optic atrophy
with progressive hearing loss, spastic quadriplegia, mental deterioration
and death
- Recessive optic atrophy
with juvenile diabetes mellitus, diabetes insipidus and hearing loss
(Wolfram's synd)
- Hereditary optic atrophy
with progressive hearing loss and polyneuropathy
- Hereditary infantile optic
atrophy with pyramidal tract signs, ataxia, mental retardation, urinary
incontinence and pes cavus (Behr's synd)
- Optic atrophy with
hereditary ataxias (Fredreich's ataxia, Marie's ataxia)
- Optic atrophy with
Charcot-Marie-Tooth disease (hereditary sensorimotor neuropathy)
Traumatic Optic Neuropathy
Clinical Features
- History of impact to head,
face, orbit
- Unilateral or bilateral
vision and VF loss
- RAPD, normal optic nerve
most common
- Optic atrophy follows
- Exclude: Open globe,
Traumatic cataract, vit
hemorrhage, RD
Mechanisms
- Compressive or Direct
mechanical injury
- Laceration; Optic
nerve contusion, edema; Avulsion or
transection; Bone fragment or fracture
- Hemorrhage
- Retrobulbar with
increased intraorbital pressure; Subperiosteal hematoma; Optic nerve
sheath hematoma
- Vascular injury
- Vasospasm,
ischemia, infarction
Evaluation
- Neuroimaging: CT to see
fractures, hemorrhage
- Incidence of visible optic
canal fracture does not correlate well with severity of vision loss
- Grades (Cook 1996):
- Better than 20/200,
no posterior orbital fracture
- 20/200 to LP, no
posterior orbital fracture
- NLP or posterior
non-displaced orbital fracture and some remaining vision
- NLP and a displaced
posterior orbital fracture
Treatment
- Canthotomy or cantholysis
if orbit is tense
- Drain subperiosteal
hematoma if present
- Methylprednisolone 30 mg/kg
IV bolus then 5.4mg/kg/hr IV for 48 hours even if NLP
- If vision improves after
48h on solumedrol start rapid oral prednisone taper
- If no clinical response
after 48h or vision worsens during prednisone taper, consider surgical
decompression
Optic Disc Edema with a Macular Star
and Neuroretinitis (ODEMS)
Clinical Features:
- Swelling of optic disc,
peripapillary and macular hard exudates, and often vitreous cells (90%)
- Usually benign,
self-limited process
- Average age: 20-40,
childhood to adult (range: 6-50)
- Unilateral (66%), usually
painless
- Antecedent viral illness
in 50%
- Va 20/20 to LP but
averages 20/40-20/200
- Dischromatopsia, RAPD
- Central or cecocentral
scotoma
- Disc
edema is earliest sign
- Macular Star after disc
edema begins to resolve. Re-examine in 2 weeks.
- More rare signs: cells in
AC, sheathing of peripapillary veins, scleritis, uveitis, central or
branch artery occlusions
Etiology
- Idiopathic (most common- probably
most are viral)
- Infectious (etiologies in Bold
most important)
- Viral: hepatitis B,
herpes simplex, herpes zoster, Epstein-Barr, influenza A, mumps,
coxsackie B
- Bacterial: cat-scratch
disease (bartonella henselae) , tuberculosis, salmonella
- Spirochetes: Lyme
disease, syphilis, leptospirosis
- Parasites and
protozoa: toxoplasmosis, toxocara
- Rare causes
- Papilledema from
increased intracranial pressure
- Optic nerve tumors
- Diffuse unilateral
subacute neuroretinits (DUSN)
- Acute
neuroretinopathy associated with progressive facial hemiatrophy
(Parry-Rhomberg syndrome)
- Vascular causes: AION, BRAO,
CRAO, Hypertension, Diabetes,
polyarteritis nodosa, Eales'
Disease
Work Up
- Screening laboratory
studies:
- RPR, FTA-ABS, Lyme
titer, Bartonella henselae serology, CXR
- If negative: PPD,
Toxoplasma titers, Toxocara
- Consider rare causes
Prognosis
- Usually benign,
self-limited
- Disc edema and peripapillary
retinal detachment resolve in 2-3 months
- Macular star begins to
disappear after one month but may persist up to one year
- Optic atrophy and RPE
changes may occur
- Visual recovery usually
good but significant permanent visual loss reported
- Recurrence rare but more
common with toxoplasmosis
- Not associated with
progression to Multiple Sclerosis:
Treatment
- Idiopathic cases- no
treatment
- Treated identified
infectious agent
- Steroids-role unclear,
perhaps helpful in rare recurrent cases
Amaurosis
- Unilateral vision loss
lasting seconds to minutes but may last up to 1-2 hours
- Vision returns to normal.
Etiology
Same list for Transient Visual Obscurations
- Embolic Causes
- Thrombotic
(platelet), calcific, cholesterol, fat, talc, air, amniotic fluid
- Hypoperfusion
- hypotension,
compression of atherosclerotic carotids, vasospasm (eg. migraine),
orbital mass compressing optic nerve, elevated CSF pressure (transient
visual obscurations), elevated IOP, Giant Cell Arteritis,
C-C fistula
- Anterior Chamber or
vitreous blood
- Corneal surface changes,
corneal dystrophies,
- Disk abnormalities- Optic
disc drusen
- Uhthoff's phenomonon / demylenation
Disc Edema
Grading
Grade 1 : C-shaped halo; <360º of blurred disk margin
Grade 2: 360º blurred disk margin
Grade 3: vessel obscuration at disk edge
Grade 4 : vessel obscuration at center
Grade 5: mushroom shaped elevation- chronic
Differential Diagnosis of bilateral disc edema
see: Optic Neuropathy DDx
see: Etiologies for bilateral disc
edema with normal visual function
- Compressive: optic
nerve sheath meningioma, glioma
- Inflammation: Optic neuritis, Optic disc edema with macular star, uveitis
- Infiltrative:
sarcoid, Lyme, TB, lymphoma, leukemia
- Leber's hereditary optic neuropathy
- Papilledema: Increased intracranial pressure,
IIH
- Pseudopapilledema (disc drusen or anomalous
discs)
- Trauma,
hypotony
- Thyroid
ophthalmopathy
- Vascular- AION,
venous sinus thrombosis, C-C fistula, diabetic swelling,
CRVO
Features of pseudopapilledema
- absent central cup, small
disc diameter
- vessels arise from central
apex of disc
- disc transilluminated with
glow of drusen present
- irregular disc margins and
peripapillary RPE changes
- rare hemorrhages
- no exudates or cotton wool
spots
Bilateral disc edema with normal
visual function
- Hypertensive retinopathy
- Cyanotic congential heart
disease (decreased arterial sats and polycythemia)
- Sleep apnea
- Spinal cord tumors (will
have s/s of myleopathy)
- Guillian-Barre syndrome
- POEMS (perepherial
neuropathy, organomegaly, endocrinopathy, monoclonal
gammopathy, and skin changes)
- Uremia
- Hypoxemia and anemia
- Syphilis, sarcoidosis,
viral mengoencephalitis (causing perineuritis)
Causes for increased
intracranial pressure
- Primary Causes
- Secondary Causes
- Hydrocephalus
- Mass lesions- tumor,
hemorrhage, large infarction, abscess
- Meningitis,
encephalitis
- Subarachnoid
hemorrhage
- Trauma
- AVM
- Intra- or
extra-cranial venous obstruction
- Secondary pseudotumor cerebri syndrome
Work-up for suspected Papilledema
- Consider causes of increased intracranial pressure
- Blood pressure
- CT of brain if hemorrhage
or infarction suspected acutely
- MRI with/ without contrast,
MRA, MRV
- Lumbar puncture following
normal neuroimaging
- accurate opening pressure
- cell count, differential
- glucose, protein,
- cytology, VDRL, other
microbial studies
Idiopathic Intracranial
Hypertension (IIH)
>95% female, >95% Obese, mean age 30 years
Dandy Criteria
Symptoms
- Headache- daily,
holocranial, pulsatile, nausea, no vomiting
- Transient
visual obscurations
- Pulsatile Tinnitus
- diplopia
- Photopsias
- Eye pain
- GVF: enlarged blind spot,
inferior nasal step (nerve fiber bundle loss), arcuate, central loss,
altitudinal
Causes of Secondary Pseudotumor Cerebri
- Nutritional
- Hyper- or
Hypovitaminosis A
- Vitamin D (rickets)
- Multiple vitamin
deficiencies
- TPN
- Drugs
- Nalidixic acid
- Tetracycline
- Minocycline
- Nitrofurantoin
- PCN, ofloxacin,
ciprofloxacin
- Amiodarone
- Lithium
- Phentoin
- Cytosine arabinoside
- Cyclosporine
- Danazol or withdrawl
from
- All-trans retinoic
acid, tretinoin, isotretinoin
- Ketamine
- Steroids, and
withdrawl from steroids
- Depo-provera
- Endocrine and metabolic
dysfunction
- pregnancy and
postpartum
- menarche
- Hyper- or
Hypothyroidism
- Systemic illness
- SLE, Bechets synd,
Cystic Fibrosis
- Hematologic
- Iron deficency anemia
- Pernicious anemia
- Thrombocytopenia and
thrombocytosis
- TTP
- polycythemia
- renal or bone marrow
transplantation
- Malignancies
- Infections
- HIV, Lyme, typhoid
fever,
- Immune
- renal or bone marrrow
transplantation
- Guillan-Barre synd
- Familial pseudotumor
cerebri- AD or AR
Work-up for IIH
- see: work up for papilledema
- If atypical features: MRV
- If signs of venous
occlusive dz: evaluate for hypercoagulable
state and vasculitis
- For men, thin patients, and
younger than 15
- Rule out other causes of
elevated ICP: secondary pseudotumor
- CBC, syphilis serology,
calcium, phosphate, creatinine, electrolytes
- Goldman or Humphrey
- Stereo photos
Treatment
- Monthly for 6-12 months
for VF, stereo disc photos, VA, RAPD until papilledema regressed
- Weight loss, limit fluid
intake, decrease salt intake
- Diamox (can start 250
bid-tid and go up to 4g/day) (teratogen)
- Lasix (start 20 mg bid)
concomitant use in children
- High dose solumedrol if
acute severe vision loss
- Repeat LP's- unpopular
- Lumbar-Peritoneal shunt-
when failure, acute vision loss
- Optic nerve sheath
fenestration- unilateral can help decrease CSF pressure and improve
contralateral eye
Ocular
Misalignment/Diplopia
Monocular
Diplopia
- Refractive error:
astigmatism
- Poorly fitting contact lens
- Cornea: Keratoconus,
Corneal surface abnormalities, Tear film (dry eye), Refractive surgery,
Corneal Transplant
- Lid: chalazion
- Iris: iridotomy /
iridectomy, miotic pupil
- Lens: cataract, subluxation
or dislocation,
- IOL: positioning holes,
decentered
- Retinal abnormalities
Transient
diplopia
Binocular
diplopia
Restrictive
syndromes
Paretic
syndromes
Majority of abnormalities of ocular motility. Includes supranuclear,
perepherial nerve, muscle or neuro-muscular junction diseases
Horizontal
gaze palsy- Causes
- Frontal lobe lesions
- Epileptogenic lesions in the frontal eye fields
- Unilateral parietal lesions
- Bilateral parietal lesions
- Lesions in the corona radiata adjacent to the genu of the internal
capsule
- Hemorrhages deep in a cerebral hemisphere, particularly the
thalamus
- Mesensephalic
- Pontine lesions affecting the abducens nucleus and/or the PPRF
- Selective saccadic palsy
- Paraneoplastic loss of horizontal voluntary eye moment
- Congenital and familial bilateral horizontal gaze palsy
- Pseudo-horizontal gaze palsy with pontine lesions
Internuclear ophthalmoplegia (INO)
INO-Clinical Features
- Unilateral
- Bilateral
- pathology of the medial longitudinal fasciculus (MLF) results in
adducting delay or paresis in ipsilateral eye with attempting
contralateral gaze inducing exodeviation and horizontal diplopia.
- residual adducting ability seen with accommodation
INO-Clinical
Features-Unilateral
- Skew deviation with the higher eye on the side of the lesion
- Vertical gaze-evoked nystagmus and impaired vestibular and pursuit
vertical eye movements
- Ipsilateral down beat nystagmus and contralateral incyclorotatory
nystagmus
- Transient torsional nystagmus (clockwise in left INO,
counterclockwise in right INO)
- Normal vertical saccades
- Exotropia less common
INO-Clinical
Features- Bilateral
- Bilateral adduction paresis or lag with eyes aligned in primary
gaze
- Exotropia, with both eyes deviated laterally
- Vertical gaze-evoked nystagmus and impaired vestibular and pursuit
vertical eye movements
- Impaired vertical gazeholding
- Rostral involvement of MLF
at the level of the midbrain third nerve nucleus results in wide angle
exodeviation: so called "wall-eye bilateral INO"
INO-Etiology
- In General: <50
years old is demyelinating disease, >50 years old is microvascular
compromise from vertebrobasilar insufficency, far less common are AVM's,
neoplasia (mets or glioma), inflammatory diseases.
- Multiple sclerosis
- Brainstem infarction (GCA, SLE, PAN, Sickle cell, Neuro Behcet’s, Eals,
Pyoderma gangrenosum, angiography)
- Brainstem hemorrhage
- Brainstem and fourth ventricular tumors
- Infections
- Head trauma
- Cervical hyperextensive or manipulation injury
- Cancer-related
- Nutritional and metabolic disorder (Wernicke’s encephalopathy,
pernicious anemia, hepatic encephalopathy, maple syrup urine disease,
abetalipoproteinemia, Fabry’s disease, hexosamiminidase A deficiency
- Degenerative disease (progressive supranuclear palsy, familial
spinocerebellar degeneration)
- Arnold Chiari malformation and associated hydrocephalus or
syringobulbia
- Drug intoxications
INO-Evaluation
- Depends on clinical circumstance
- Isolated INO or in association with brainstem signs requires MRI
- ESR if infarct is detected in patient older
than 50
- For a bilateral INO, a work up for drug intoxication, syphilis and
pernicious anemia, LP should be considered.
One-and-a-half Syndrome
One-and-a-half syndrome-Clinical features
- Conjugate gaze palsy to one side and impaired adduction or
abduction on looking to the other side
- Lesion in PPRF or abducens nucleus and the adjacent MLF on the side
of the complete gaze palsy, rostral brainstem, or the cavernous sinus
- Often associated with exotropia of the eye opposite the side of the
lesion
One-and-a-half
Syndrome-Etiology
- Brainstem infarction
- Brainstem hemorrhage
- Multiple sclerosis
- Tumors (primary or metastatic of the brainstem, fourth ventricle,
or cerebellum)
- Postoperatively after the removal of the tumors of the psterior
fossa
- Basilar artery aneurysms or brainstem AV malformation
- Trauma
- Mucormycosis of the cavernous sinus
Vertical
gaze palsy- Causes
- Bilateral lesions of the rostral interstitial nuclei of the MLF
- Bilateral pretectal lesions or midline lesions of the posterior
commissure
- Unilateral lesions of the riMLF
- Unilateral mesancephalic lesions
Myopathies
Primary
overaction syndromes
- Oblique overaction
- Convergence spasm
- Can mimic 6th nerve
palsy. Miosis with increasing esodeviation usually confirms dx. Occasionally
seen with prolonged reading. Psychogenic component and associated with
malingering.
- May respond to
periodic relaxation of accommodation or reading glasses, or may require
cycloplegia
- Superior oblique myokymia
- Repetitive firing of
superior oblique muscle.
- Etiology unknown
- Intermittent
diplopia, vertical or torsional oscillopsia.
- Fine amplitude
vertical and torsional movements best seen with slit lamp.
- Usually responds to
Tegretol.
- Ocular neuromyotonia
- 3rd or 6th nerve.
- In patients with
history of radiation to parasellar lesions.
- Often dectectable by
having patient look in direction of affected muscle and failure of muscle
to relax.
- Treatment: tegretol
- Oculogyric crisis
- seen in phenothiazine
overdose and described in postinfectious parkinsonism
- bilateral tonic
supraduction associated with neck hyperextension, usually without visual
complaints
Binocular
Vertical Diplopia- Causes
- Supranuclear
- Ocular Motor Nerves
- Ophthalmoplegic migraine
- Neuromuscular Junction
- Eye Muscle
- Trauma
- Orbital floor
blowout fracture
- Direct trauma to
extraocular muscles (intramuscular hematoma)
- Congenital
- Tumors
- Metastatic to
muscles or orbit
- Retinal/ optic pathway
- foveal displacement
- Hemifield slip phenomenon
Skew deviations
- Asymmetric involvement of
internuclear connections that control vertical gaze
- May be comitant but more
frequently incomintant, ductions are full
- Hyperdeviation increasing on ipsilateral downgaze
- Deviation does not localize to one muscle or nerve
- Associated posterior fossa signs
- Seen following ischemic,
inflammatory, traumatic and neoplastic involvement of the brain stem in
any location from the pontomedullary junction to the rostral midbrain
- If intermittant suspect
vertebrobasilar TIA or brainstem tumors
Supranuclear monocular elevation paresis
- Double elevator palsy
- Etiology
- Primary inferior rectus restriction
- Primary superior rectus palsy
- Myasthenia
gravis
- Fascicular third nerve lesion
- Pretectal supranuclar lesions
- Congenital or acquired
Vertical
one-and-a-half Syndrome
- Thalamo-mesencephalic infarction
- Vertical upgaze palsy and monocular paresis of downgaze on the side
of the lesion or contralateral to the lesion
- Bilateral mesodiancephalic infarct
- Impairment of all downward rapid eye movements (including the
vestibuloocular reflex) and downward smooth pursuit (nondissociated
downgaze paralysis) associated with monocular paralysis of elevation
- Mesodiencephalic junction and medial thalamus
- Monocular elevation parasis of the right eye with contralateral
paresis of downward gaze
Binocular Horizontal Diplopia
Ocular Myasthenia Gravis
Ocular
myasthenia gravis- Causes
- Weakness improving with rest
- Acetylcholine receptor sites for neuromuscular transmission are
blocked by immune complexes (antibodies are present in 50% with
isolated ocular involvement)
- Drugs causing, unmasking or worsening MG
- Procainamaide
- Quinidine
- Polymixin, aminoglycoside, and monobasic amino
acid antibiotics
- Corticosteroids
- Beta blockers
- Chloroquine
- Lithium
- Phenytoin
- Cisplatin
- Magnesium
Ocular myasthenia-Clinical presentation
- Ptosis
- Cogan’s lid twitch
- Brief overelevation of the upper eyelid with an upward saccade
- Diplopia
- Graves disease occurs in 5% of
myasthenia gravis patients
Ocular
myasthenia-Diagnosis
- Tensilon test (Edrophonium chloride)
- Side effects
- Diaphoresis
- Lacrimation
- Abdominal cramping
- Nausea
- Vomiting
- Salivation
- Atropine sulfate (0.4-0.6 mg) should be
immediately available
- 2 mg of tensilon are injected as a test dose
- Pt is observed for 60 seconds-if the symptoms
disappear or decreases the test is positive
- If no response is elicited the remaining 8 mg
are administered
- Prostigmine
- Neostigmine methylsulfate
- Useful in children and in adults without ptosis
- Allows for a longer observation period
- Positive test produces resolution of signs
within 30-45 minutes
- Dosage = weight (kg) x 1.5 mg / 70
- Sleep test
- Eyes closed for 30 minutes
- Improvement of measurements repeated
immediately
- Ice-pack test
- Neuromuscular transmission improves with cold
- Icepack is placed over closed eyes for 2
minutes
- Improved ptosis occurs with MG
- EMG:
Single fiber EMG showing decremental response
Graves' Disease
See Strabismus notes: Graves Dz
See Oculoplastics notes: Graves Dz
Chronic Progressive External
Ophthalmoplegia (CPEO)
- Mitochondrial abnormality
of the extraocular muscle cells produce slowly progressive weakness.
Deletions of the mitochondrial genome >50%.
- Mitochondria seen as
"ragged red fibers" on histologic examination and inclusion body
abnormalites on electron microscopy
- associated facial
weakness, eyelid closure problems and corneal exposure
- systemic assoications: Kearns-Sayre
syndrome; cardiac conduction defects and potential for heart
block, variable retinal pigmentary changes, hearing impairment. Cardiac
evaluation essential.
Myotonic
Dystrophy
- may be associated with a
mild form of CPEO
- Red and green polychromatic
cataract dots can be seen
- myoneural junction disease-
antibodies against acetylcholine receptors
- A cause of low IOP
Isolated
nerve palsies
3rd Nerve Palsy see
Strabismus notes: 3rd nerve palsy
- peripheral location of
pupillary fibers: rare for mass lesion to spare pupil but common for other
causes to spare pupil
- acute, complete,
isolated, pupil-sparing:
- congenital: most are
birth trauma related, may have pupil involvement or aberrant regeneration
- children <10;
transient post viral ophthalmoplegia, aneurysms rare (follow)
- adolescents and young
adults: demylenating disease (MRI)
- >40 y/o and
vasculopathic (check BP and fasting glucose, recovery 3 months)
- if >70: r/o GCA
(ESR and CRP)
- trauma: check for CC
fistula (orbital bruit, arterialized episcleral vessels, higher IOP,
higher IOP pulse pressure)
- progressive, non-isolated
lesions, pupil involved, painful: aneurysms, MRI with gad, MRA,
angiography, CSF
- incomplete: cavernous
sinus or orbital apex lesions, rarely midbrain (MRI with gad orbits and
brain)
- aberrant regeneration:
1) eyelid elevation or hang-up with adduction or depression. 2) miosis
with elevation 3) persistent vertical gaze limitation. Think trauma or
compression (MRI)
4th Nerve Palsy see
Strabismus notes: Superior Oblique palsy
- Presents with vertical
diplopia and no signs or history of muscle restriction
- Hyperdeviation increases on
contralateral gaze and ipsilateral head tilt
- Maddox rod and pen light in
nine cardinal positions
- Double maddox rod: 3° to 10°
of excyclotorsion
- Bilateral fourth nerve
palsy: > 10°
- Causes
- Congenital cases:
head tilt in old photographs or vertical fusional range of > 3
diopters
- Acquired cases: head
trauma most common cause (often trivial), microvascular disease
- Nuclear involvement:
very rare: AVM, trauma, demyelination.
- At exit from
brainstem: pineal gland lesions (pinealoma, pinealoblastoma, teratoma,
dysgerminoma, choriocarcinoma), often bilateral fourth with other signs
of dorsal midbrain syndrome.
- subarachnoid space/
cavernous sinus lesions: microvascular abnormalities, inflammations
(meningitis), carotid-cavernous fistula, iatrogenic following cutting of
edge of tentorum by neurosurgeon
- Clinical approach:
yield for imaging in isolated fourth palsy is low even in those with
no history of head trauma. Older patients 3 month follow-up.
6th Nerve Palsy
Isolated abduction deficit, slowed ipsilateral saccades.
6th Nerve Palsy- Eiologies
- Brain stem causes:
- inflammation
(postviral, demylenating),
- tumors: pontine or
cerebellar glioma (children) ependymoma, or medulloblastoma
(adolescents/young adults)
- vascular lesions
(elderly)
- metabloic
derangement (vitamin B deficiencies, Wernicke-Korsakoff syndrome).
- Subarachnoid space
- inflammation
(sarcoid),
- infections
(basilar meningitis seen in TB or fungus),
- infiltrative
including lymphoproliferative disorders, compressive lesions arising from
the clivus (meningioma, chordoma, chondrosarcoma, metastatic disease),
- cerebellar pontine
angle lesions (neurilemoma of CN VIII or meningiomas)
- shifts in brain
position from changes in intracranial pressure (acute hydrocephalus, following
lumbar puncture, IIH)
- "petrous
pyramid"lesions
- enlargement of the
inferior petrosal sinus with a direct or dural cavernous sinus fistula
- inflammatory
lesions in the petrous pyramid (Gradenigo syndrome)
- neoplasms.
Congenital 6th nerve palsies
- Duane syndrome-
congenital absence of the 6th nerve.
- Usually unilateral
Lateral rectus is innervated by various branches of the 3rd nerve
resulting in narrowing of the palpebral fissure with attempted adduction.
- Three types; Type
1: limited abduction, Type 2: limited adduction, Type 3: Limited
abduction and adduction. Cross-fixate if adduction intact.
- Möbius syndrome-
bilateral palsy of 6th and 7th nerve and possibly lower cranial nerves,
deformities of hands and feet can also occur. Cross-fixate using
adduction.
6th Nerve Palsy- Work-up
- first determine if it is
isolated 6th.
- If not isolated do MRI
with gadolinium. Significant pain is indication for a work-up.
- Older vasculopathic
patient with isolated 6th nerve palsy: presumptive microvascular insult to
nerve. It should clear within period of 2-3 months. Failure to resolve or
evidence of progression should prompt MRI.
- Children with post viral
inflammatory lesions should also clear.
- Adolescents and young
adults with isolated 6th: demyelinating disease may be cause, MRI if
failure to resolve or any other signs.
- Trauma associated
isolated 6th requires limited work-up by checking 7th and 8th nerves.
- CSF analysis may be
appropriate in setting of negative MRI.
Phakomatoses
Neurofibromatosis type I (See
Pathology : neurofibromatosis)
- Type I (NF-1)- most
common, "perepherial", 1 in 3000-5000, widespread hamartomas of
perepherial nerves and tissues of neural crest origin
- Long arm Chromosome 17
- Criteria for
diagnosis (two or more):
- Six or more
café-au-lait spots >5mm in prepubescent or >15 in postpubescent
- Two or more
neurofibromas or one plexiform neurofibroma
- Freckling in
axillary, inguinal, or other intertriginous area
- Optic nerve glioma
- Two or more Lisch
nodules
- Osseous lesion:
sphenoid bone dysplasia, thinning of long-bone cortex , with or without
pseudoarthrosis
- First degree
relative with NF-1
- Lisch nodules- tan
gelatinous consistency, spindle shaped melanin-containing cells, first
seen between 5-10 yrs.
- Choroidal lesions-
localized collections of melanocytes, do not affect vision.
- Nodular neurofibromas-
- nodular cutaneous,
subcutaneous or fibroma molluscum.
- Can be
papulonodules or pedunculated can occur in orbit rarely.
- Plexiform
neurofibroma- seen in 30%, extensive subcutaneous swelling,
indistinct margins,
- hyperpigmentation
and hypertrichosis can occur. Considerable enlargement over time. 1
- 10% involved the
face, commonly upper eyelid and orbit. S-shaped configuration of upper
eyelid. Glaucoma in same eye in 50%. Temporary relief of ptosis and
disfigurement with debulking surgery.
- Optic Glioma-
- pilocytic
astrocytoma involving optic nerve or chiasim.
- Found in 15% of
those with MRI or CT. Entire optic nerve shows fusiform or cylindrical
enlargement. "Kinking" of optic nerve.
- Mass bright on T1
and dark on T2.
- Differs from optic
nerve sheath meningioma which is seen in higher frequency in NF-1 as
well. Can slow growth spontaneously.
- Often glioma of
optic nerve grows back to Chiasm. Significant morbidity with chiasmal
lesions; bilateral vision loss, hydrocephalus, hypothalamic dysfunction
leading to precocious puberity or hypopituitarism.
- Mortality 50%
(within months of diagnosis).
- Chemotherapy is
being tested. Megadose radiation has been used but not proven to stop
vision loss or preserve life.
- Prominence of corneal
nerves- 20%, represents glial hypertrophy.
Associations: 1) juvenile xanthogranuloma,
2) capillary hemangioma, 3) leukemia, 4) rhabodomyosarcoma, 5) Wilms tumor, 6)
scoliosis, 7) pseudoarthrosis of tibia, 8) hypoplaisa of sphenoid bone causing
ocular pulsation, 9) macrocephaly, 10) aqueductal stenosis, 11) seizures, 12)
minor intellectual defects 12) pheochromocytoma (test blood pressure)
- Glaucoma- 1-2%,
unilateral, more often in cases of concomitant ipsilateral plexiform
neurofibroma and/or congenital iris ectropion.
- Routine follow-up: 1-2
years, test blood pressure
- NF-II: associated
with acoustic neuromas, not associated with glaucoma
The Pupil
- Anatomic Pathways
- Simple Anisocoria
- Midbrain Lesions:
- Parasympathetic Lesions:
Anisocoria greater in light
- Sympathetic Lesions: Horner's syndrome
- Episodic Pupil phenomena
Simple
Anisocoria
- Most common cause of anisocoria
- Varied from day to day
- Greater in dim light
- Dilate normally after cocaine instillation
- No dilation lag
Argyll
Robertson Pupil
- Tertiary syphilis
- Small pupils which are often irregular
- Do not react to light but the near response is normal
- Iris atrophy with transillumination defects
- Dilation is poor after instillation of mydriatics
- Serum RPR, FTA-ABS
Parinaud
Syndrome
Adies Tonic
Pupil
- Sluggish, segmental papillary responses to light
- Better response to near followed by slow redilation
- Post ganglionic parasympathetic pupillomotor damage
- 75% female, 80% unilateral
- Hypersensitive to topical parasympathomimetic pilocarpine
0.05%-0.1%
- Systemic association : HZV, HSV, GCA, syphilis, orbital trauma
- Bilateral association: diabetes, alcoholism, dysautonomia
associated with cancer, amyloidosis
Traumatic
mydriasis
- Damage to the papillary sphincter
- Relative midriasis immediately after injury.
- Becomes midsized and poorly responsive to bright light and dim
illumination
- Notches in the pupillarly margin
- Iris transillumination
Pharmacologic
mydriasis
- Reacts poorly to light and near reflex
- Instill pilocarpine 0.5% or 1.0% (mydriasis with third nerve palsy
or Adie’s syndrome)
Horner's
Syndrome
Horner's syndrome-Clinical Findings
- Ipsilateral mild ptosis (denervation of the Muller’s muscle)
- Upside down ptosis (sympathetic denervation to lower eyelid
retractors
- Apparent enophthalmos
- Anisocoria due to ipsilateral miosis
- Dilation lag
- Decreased accommodative amplitude or accommodative paresis
- Transient ocular hypotony and conjunctival hyperemia
- Ipsilateral facial anhidrosis
- Ipsilateral straight hair in congenital cases
- Heterochromia of the iris (usually congenital but rarely acquired)
Horner’s syndrome- Localizing Signs & Symptoms
First
Order Localization
- Posterolateral hypothalamus, brainstem, lateral column of the
spinal cord
- hypothalamic or brainstem signs or symptoms (contralateral fourth
nerve palsy, diabetes insipidus, disturbed temperature or sleep
regulation, meningeal signs, vertigo, sensory deficits, anhidrosis of the
body.)
Second
order Localization
- Exit (C8 T1 T2) ciliospinal center of Budge, exits the ventral
root, arches over the apex of the lung, ascends into the cervical
sympathetic chain. Synapse in the superior cervical ganglion
- Neck or arm pain
- Anhidrosis involiving the face and neck
- Brachial plexopathy
- Vocal cord paralysis
- Phrenic nerve palsy
Third
order Localization
- Travels with carotid artery into the cavernous sinus, on to the
abducens nerve, travels with the ophthalmic division of the trigeminal
nerve, joins the nasociliary branch of the trigeminal nerve, pass through
the ciliary ganglion, reach the eye as long and short ciliary nerves.
- Ipsilateral headache
- Tearing, facial flushing, rhinorhea
Horner’s
Syndrome- Etiology
First
order- Etiology
- Neoplasm( pituitary, third ventricle, brainstem, spinal cord)
- Infection (syphilis, poliomyelitis, meningitis)
- Demyelination
- Inflammation (sarcoid)
- Trauma
- Ischemia or infarction (Hypothalamic, Wallenberg Syndrome)
Second
order- Etiology
- Neoplasm (Glomus tumors, Breast CA, Sarcomas, Lung CA,
Lymphoreticular, Neurofibroma, Thyroid adenoma)
- Mediastinal or neck lympadenopathy
- Cervicothoracic abnormalities (cervical rib, pachymeningitis,
hypertrophic spinal arthritis, foraminal osteophyte, ruptured
intervertebral disc, thoracic aneurysm
- Neck, brachial plexus, lung trauma or surgery
- Carotid artery dissection (ipsilateral head pain, amaurosis fugax,
dysgeusia)
- Infection or inflammation
Third
order- Etiology
- Caverous sinus lesions
- Headache syndromes
- Inflammatory (cervical lymphadenopathy, otitis media, petrosits,
sphenoid sinus mucocele
- Infections (Herpetic geniculate neuralgia, meningitis, sinusitis
- Neoplasm (Cavernous sinus, cervical node metastasis, metastatic,
orbital)
- Systemic or autonomic (diabetes, Fisher’s syndrome, Shy-Drager)
- Trauma (basilar skull fracture, orbital fracture, radical middle
ear surgery)
Horner’s Syndrome- Congenital
- Obstetric perinatal forceps with trauma to the carotid sympathetic
plexus
- Presumed superior cervical ganglion lesions
- Surgical (thoracic) or obstetric trauma (brachial plexus) to the
preganglionic pathway
Horner’s Syndrome- Pharmacological Localization
- Cocaine 5-10% will dilate a normal pupil 1mm, but no effect on a HS
pupil (inhibits norepinephrine reuptake at the neuromuscular junction)
- Hydroxyamphetamine will dilate pre-ganglionic, post-ganglionic
pupil will not dilate (releases stored norepinephrine from post ganglionic
adrenergic nerve endings at the dialator muscle of the pupil)
- The cocaine test and hydroxyamphetamine test cannot be given on the
same day
Horner’s Syndrome- Indications for Imaging
- Childhood HS without a history of clear trauma
- Non-isolated HS in adults
- Chronic pain, pulmonary or other systemic symptoms or signs
suggesting malignancy, and additional cranial nerve abnormalities.
Horner’s Syndrome- Raeder Paratrigeminal Syndrome
- Middle-aged males
- Horner’s Syndrome
- Unilateral headaches not characteristic of cluster
- No underlying pathology can be identified
Nystagmus and other Ocular Oscillations
Spasmus nutans
- Acquired nystagmus
- Pendular nystagmus, head nodding and abnormal head posture
- Develops within the first year or two of life
- Benign and self-limited lasting months to several years
- Normal ophthalmologic exam and negative MR imaging
- Diagnosis of exclusion
Acquired monocular pendular nystagmus
- Heimann-Bielschowsky phenomenon
- May improve if vision is corrected
- Multiple sclerosis
- Neurosyphilis
- Brainstem infarct
Monocular
downbeat nystagmus
- Acute infarction of the medial thalamus and upper midbrain with
pontocerebellar degeneration
- Dysfunction of the ipsilateral brachium conjunctivum
Bilateral Nystagmus
Bilateral disconjugate oscillations
See Saw
nystagmus
- One eye rises and intorts, the other falls and extorts, the
movements are then reversed
- Pedular
- Represents oscillations involving central otolithic connections,
especially the interstitial nucleus of cajal
- Causes:
- Parasellar masses
- Brainstem and thalamic stroke
- Multiple sclerosis
- Trauma
- Arnold-Chiari malformation
- Hydrocephalus
- Syringobulbia
- Septo-optic dysplasia, retinitis pigmentosa,
and cone degeneration
- Congenital
Convergence-retraction
nystagmus
- Repetitive adducting saccades
- Mesencephalic lesions affecting the pretectal region most commonly
- Part of Parinaud Syndrome
Divergence
nystagmus
- Hindbrain abnormalities
- Associtead with downbeat nystagmus
Repetitive
divergence
- Slow divergent movement followed by rapid return to primary
- Associated with hepatic encephalopathy
Oculomasticatory
myorhythmia
- Pendular vergence oscillations of the eyes associated with
concurrent contraction of the masticatory muscles
- Whipples disease
Bilateral symmetric conjugate oscillations
Pendular
nystagmus
- Motor or congenital sensory
- Binocular visual loss
- Structural lesion
Jerk
nystagmus
- Slow phase with velocity that increases exponentially as the eyes
move in the direction of the slow phase
- Spontaneous or induced
- With vertigo
Horizontal jerk nystagmus with vertigo
- Eye drift in direction parallel to the plane in which the diseased
innner ear canal lies
- Slow component toward the lesion
- Most prominent with fixation is prevented
Periodic
alternating nystagmus
- Primary position jerk nystagmus after 60-120 seconds stops for a
few seconds and then starts beating in the opposite direction
- No vertigo
- Associated with periodic alternating oscillopsia, periodic
alternating gaze, or periodic alternating skew deviation
- Congenital or acquired
- Disease to craniocervical junction
- May be controlled with baclofen
Torsional
nystagmus
- Brainstem or posterior fossa lesions
Upbeat
nystagmus
- Damage to the central projections of the anterior semicircular
canals
- Worse in upgaze, does not increase in lateral gaze
- Damage to the ventral tegmental pathways or medullary disease
- Evaluation:
- MR imaging warranted
- Ophthalmologic and neurologic evaluation
Downbeat
nystagmus
- Greatest when the patient looks down, and can increase in lateral
gaze
- Cervicomedullary junction disease
- Causes include midline medullary lesions, posterior midline
cerebellar lesions, diffuse cerebellar disease
- Deficient drive by the posterior semicircular canals are postulated
as an explanation
- Cerebellar lesions may cause this nystagmus by disinhibition of the
cerebellar effect on the vestibular nuclei
- Convergence may convert downbeat to upbeat nystagmus
- Evaluation
- MR imaging
- Drug levels of anticonvulsants or lithium
- B12, Mg, thiamine
- Alcohol or toluene abuse investigated
- Spinal tap if signs of CNS infection
- With subacute or acute cerebellar signs, a
paraneoplastic process must be considered
Gaze-evoked
nystagmus
- Eye drift to midposition
- Evaluation if not drug induced should include MRI
- Etiology
- Brainstem/cerebellar diease
- Brun’s nystagmus
- Drug-induced
- Anticonvulsants
- Sedatives
- Alcohol
- Physiologic nystagmus
- Rebound nystagmus
- Convergence-induced nystagmus
Saccadic
intrusions
- Interfere with macular fixation of an object
- Inappropriate saccades
- Usually occur in the context of neurological disease
- If no cause is known, MR imaging is warranted
- Classificiation
- Treatment
Square-wave
jerks
- Saccade off the target followed by a corrective saccade 200 msec
after
- Larger than 1 or 2 degrees are pathologic
- Square-wave jerk etiology
- Cerebral or cerebellar lesions
- Progressive supranuclear palsy
- Huntington’s chorea
- Parkinson’s disease
- Wernicke-korsakoff syndrome
- Friedreich’s ataxia
- Lithium or tobacco use
Macrosquare-wave
jerks
- Larger amplitude (20-40 degrees)
- Occasionally present in the vertical plane
- Macrosquare-wave jerk etiology
-
- Multiple sclerosis,
- Cerebellar hemorrhage
- Olivopntocerebellar atrophy
- Multiple systems atropy
- Arnold-Chiari malformation
Macrosaccadic
oscillations
- Oscillations around the fixation angle with intersaccadic intervals
approximately 200 msec
- Cerebellar disease especially midline and underlying nuclei
Saccadic
intrusion-Treatment
- GABA-A agonist
- Benzodiazepines
- Barbiturates
Ocular Flutter
- Continuous saccadic activity in only the horizontal direction
- Related to vascular, neoplastic, paraneoplastic, or immune damage
to pause cells
Opsoclonus
- Continuous saccadic activity in both horizontal and vertical
directions
- Related to vascular, neoplastic, paraneoplastic, or immune damage
to pause cells
Spontaneous eye movements in comatose patients
- Ping Pong gaze
- Cyclic horizontal roving
- Bilateral cerebral damage, rarely posterior
fossa lesion, hepatic, hypoxic, carbon monoxide, drug intoxication
- Repetitive divergence
- Slow deviation out, rapid return to primary
- Metabolic encephalopathy
- Nystagmoid jerk of a single eye
- Vertical, horizontal, or rotatory movements
- Middle or low pontine lesion
- Status epilepticus
- Small amplitude vertical (occasionally
horizontal) movements
- Diffuse encephalopathy (hypoxia)
- Ocular bobbing
- Fast down, slow up
- Pontine lesion, extra-axial posterior fossa
mass, diffuse encephalopathy
- Inverse ocular bobbing (ocular dipping)
- Slow down, fast up
- Anoxia, post status epilepticus (diffuse
encephalopathy)
- Reverse ocular bobbing
- Fast up, slow down
- Diffuse encephalopathy, rarely pontine lesion
- Slow-upward ocular bobbing (converse ocular bobbing, reverse ocular
dipping)
- Slow up, fast down
- Diffuse encephalopathy, rarely pontine lesion
- Pretectal pseudobobbing
- V-pattern down and in
- Pretectal (hydrocephalus)
- Vertical ocular myoclonus
- Pendular, vertical isolated eye movements
- Pontine lesion
Transient Visual Loss
Monocular vs. Binocular
Monocular Transient Visual Loss
Gaze evoked monocular transient
vision loss
- suspect structural lesion
of orbit- orbital imaging
- cavernous hemangioma,
optic nerve sheath meningioma
- Rare causes: osteoma,
meurofibroma, glioma, medial rectus granular myoblastoma, varices, orbital
trauma, metastasis
- One case: pseudotumor
cerebri
Monocular transient vision loss lasting SECONDS
Monocular transient vision loss lasting MINUTES
(Amaurosis Fugax)
See Diagnosis and
Treatments
Causes of Ocular Hypoperfusion
- Carotid Occlusive Disease-
decreased vision with light exposure, postpradial, or change in position
·
Ocular ischemic syndrome: panuveitis,
neovascular glaucoma, rapidly progressive cataract, pain that improves when
lying down
- Venous stasis retinopathy-
(hypotensive retinopathy)
Diagnosis and treatment
of Amaurosis fugax
- Due to ipsilateral
carotid stenosis
- 70-99%-
endarterectomy if good surgical candidate and surgeon mortality &
morbidity less than 2%. Reduces 2 year stroke risk from 26% to 9%. And
reduces fatal strokes from 13.1% to 2.5%
- 30-69%- uncertain
if endarterectomy is beneficial
- 29% or less-
search for cardiac or aortic source of emboli, if none found treat with
ASA and control risk factors
- Age >55 and no
visible emboli
- Due to ocular
hypoperfusion
- decreased retinal
artery pressure
- evaluate and treat
carotid stenosis
- superficial
temporal artery to middle cerebral artery bypass
- PRP or perepherial
retinal cryotherapy to reduce oxygen requirement
- If no thromboembolic
source found
- MRI / MRA-
possible vascular malformation
- Labs: ESR, CBC,
antiphospholipid antibodies, ANA, other studies for collagen vascular
disease and dysproteinemia
- Calcium channel
blockers; verapamil for migraine and ocular ischemic syndrome
Monocular transient vision loss lasting HOURS
- Thromboembolic disease
- Carotid stenosis
- Migraine
Binocular Transient Visual Loss
Binocular transient visual loss lasting
minutes
- Migraine- small area that
grows over 15 minutes
- Migraine plus collagen
vascular disease
- AVM's of occipital lobes
(simulate migraine)- but more often headache restricted to one side of
head and homonomous transient visual field loss.
- Vertebrobasilar TIA
- Embolic disease
Migraine Simulators
- Occipial Lobe AVM
- Internal Carotid artery
dissection
- Acute vitreous or retinal
detachment
- Vertebralbasilar TIA's
- Venous sinus thrombosis
- Meningioma or other mass
occupying tumor
Causes of Hypercoagulable state in
young
- Anti-thrombin III
deficiency
- Factor V Leiden mutation
- Homocysturnia
- Anti-phospholipid
antibodies
- Protein C or S deficiency
Causes of Spontaneous Hyphema
- Intraocular lens
especially iris fixation lens
- vascular anomalies of the
iris (eg. myotonic dystrophy or Sturge-Weber
synd)
- microangioma
- diffuse hemangiomatosis of
childhood
- neoplasms: melanoma or
retinoblastoma
- diseases of blood or
vessels: leukemia, lymphoma, hemophilia, scurvy
- rubeosis iridis
- severe iritis
- fibrovascular membranes
- juvenile xanthogranuloma
- occult trauma
- delayed bleeding after
trauma
- hydro-ophthalmos
- malignant exophthalmos
- histiocytosis X
- post sclerotomy with
cautery