The Neuro-Ophthalmology of
Headache
Charles E. Maxner MD, FRCPC
Departments of Medicine (Neurology) and Ophthalmology
Dalhousie University, Halifax, NS
Objectives
• Focus on the Primary Headache Disorders
affecting the visual system
• Review Migraine with Aura with emphasis on the
aura
• Review the concepts of Acephalgic Migraine and
Retinal Migraine
• Review the TAC disorders (Trigeminal Autonomic
Cephalgias)
• Briefly outline several interesting “headache”
syndromes
Visual Disturbances of Migraine
History
“He seemed to see something shimmering
before him like a light…a violent pain
supervened in the right temple, then all in
the head and neck…”
Hippocrates
Visual Disturbances of Migraine
History
John Fothergill (Quaker Physician)
“…it begins with..a singular kind of
glimmering in the sight, objects swiftly
changing their apparent position, and
surrounded with luminous angles like those
of a fortification.”
Reported by R.H. Fox 1919
Visual Disturbances of Migraine
• Sir Hubert Airy (1871): Published “On a distinct
form of transient hemianopia” coining the term
“teichopsia” (Greek: teichos=fortification and
opsia=seeing)
• X. Galezowski(1882): “ophthalmic megrim” in 3
migraineurs with CRAO
• C.M. Fisher(1952): Migrainous amaurosis fugax
Visual Disturbances of Migraine
Sir Hubert Airy’s Artistry (1870)
Visual Disturbances of Migraine
IHS ICHD-2 Code 1.2
Migraine with Aura
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Positive >Negative Scotomata
Often hemianopic
Buildup and march
20-30 minute duration
Subsequent headache
Visual Disturbances of Migraine
Adapted from Hupp, Kline, Corbett:
Surv Ophthalmology 1989; 33: 221-236
Visual Phenomena of Migraine
Positive
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Fortification spectra
Blurred vision
Heat waves
Phosphenes
Fragmented “cracked
glass”
• Distortion
Negative
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Homonymous hemianopia
Tunnel Vision
Cortical blindness
TMB
Cortical
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Déjà vu
Jamais vu
Micropsia
Macropsia
Dyschromatopsia
Visual Disturbances of Migraine
Migraine Aura
• K. Lashley calculated rate of progression of
migraine scotoma as 3mm/min over cortex (1941)
• Spreading cortical depression (3mm/min) of Leão
(1944)
• P. Milner(1958): “..attention should be drawn to
the striking similarity between the time courses of
scintillating scotomas and Leão’s spreading
depression..”
Visual Disturbances of Migraine
Visual Disturbances of Migraine
Migraine Aura
• Cerebral blood flow studies:Olesen and Lauritzen
• Spreading hypoperfusion 2mm/min
• Appeared before migraine symptoms and
continued into headache phase
• Occasional preceding phase of hyperemia
• CBF above ischemic range
• Perfusion changes did not respect vascular
territories
Epiphenomenon?
Visual Disturbances of Migraine
Headache and CBF
Spreading oligemia
during migraine
aura:
Adapted from Lauritzen
Visual Disturbances of Migraine
Migraine Aura: fMRI in Acute Attacks
• Visual aura associated with decremental blood
flow changes (30%)
• Mean transit time increased (30%)
• No DWI change observed with aura
• Areas of occipital cortex contralateral to reported
VF disturbance are non-responsive to standard
visual stimuli during migraine visual aura
• These areas correlate with area of decreased flow
on PWI
Visual Disturbances of Migraine
Serotonin System and Sterile Inflammation
Visual Disturbances of Migraine
Migraine Aura: Cause?
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Biochemical:Magnesium
Neuro-transmitter: Serotonin
Visual cortex: Aspects of Visual Input
Electrical: “Migraine Generator”
Visual Disturbances of Migraine
Acute Treatment of Migraine: The Triptans
Visual Disturbances of Migraine
Acephalgic Migraine
• Typical aura without headache (IHS 1.2.3)
• Episodic migrainous neurologic dysfunction of the
type associated with the “classic” form of
migraine but without headache
• Personal or family history of migraine common
• Normal examination
Visual Disturbances of Migraine
Ocular or Retinal Migraine (IHS1.4)
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Cause of TMB
Retinal or ciliary circulation
True monocular visual loss
Complete or incomplete loss
Transient or permanent (i.e. CRAO,
BRAO, ION, CRVO, CSR)
Negative>Positive symptoms
Qualitatively different from amaurosis fugax
Vascular spasm: Arteriolar vs Venular
Headache variable
Visual Disturbances of Migraine
• Carroll D. Retinal migraine. Headache 1970;
10:9-13.
• Winterkorn J. et al Treatment of vasospastic
amaurosis fugax with calcium channel blockers.
NEJM 1993; 329:396-8.
• Ammache Z. Idiopathic stabbing headache
associated with monocular visual loss. Arch
Neurol 2000; 57:745-6.
Trigeminal Autonomic Cephalgias
• Unilateral Pain in the Ophthalmic Division of
the Trigeminal nerve
• Autonomic manifestations
Lacrimation
Eyelid Edema
Conjunctival Injection
Horner syndrome
Benign episodic unilateral pupillary dilation
• IHS Section 3 (3.1-3.4)
Trigeminal Autonomic Cephalgias
• 3.1 Cluster Headache
Episodic
Chronic
• 3.2 Paroxysmal Hemicrania
Episodic
Chronic (CPH)
• 3.3 SUNCT
Short-lasting Unilateral Neuralgiform headache attacks
with Conjunctival injection and Tearing
• 3.4 Probable of 3.1 to 3.3
Features of TACs (Cluster)
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Gender, F:M
Attack frequency/day
Duration
Response to indomethacin
Conjunctival injection,
lacrimation
Nasal congestion,rhinorrhea
Eyelid edema
Forehead/facial sweating
Horner syndrome
Restlessness, agitation
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1:3
<8
15-180 minutes
Sometimes
At least one
At least one
Yes
At least one
Yes
Yes
Adapted from Friedman. Ophth Clin N Am 2004; 17:357-369
Cluster Headache
PET assessment of rCBF in Triggered Cluster
Adapted from May, Goadsby et al; Queen Square, London
Features of TACs
(Paroxysmal Hemicrania)
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Gender, F:M
Attack frequency/day
Duration
Response to indomethacin
Conjunctival injection,
lacrimation
Nasal congestion,rhinorrhea
Eyelid edema
Forehead/facial sweating
Horner syndrome
Restlessness, agitation
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2:1
>5
2-30 minutes
Required for diagnosis
At least one
At least one
Yes
At least one
Yes
No
Adapted from Friedman. Ophth Clin N Am 2004; 17:357-369
Features of TACs (SUNCT)
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Gender, F:M
Attack frequency/day
Duration
Response to indomethacin
Conjunctival injection,
lacrimation
Nasal congestion,rhinorrhea
Eyelid edema
Forehead/facial sweating
Horner syndrome
Restlessness, agitation
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1:2
3-200
5 seconds to 4 minutes
No
Both
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No
No
No
No
No
Adapted from Friedman. Ophth Clin N Am 2004; 17:357-369
Ice-Pick-Like Headache
• IHS 4.1 Primary Stabbing Headache
• “Needle-in-the-eye” syndrome
• Sharp jabbing pain in orbit, temple, parietal and
occasionally occipital area
• Seconds duration, may have afterburn
• Episodes: rare to multiple per day
• Most often in migraineurs
• Non-steroidal prophylaxis
Photo-Oculodynia Syndrome
• Chronic eye pain with no evidence of damage or
inflammation
• Light sensitive
• Foreign body sensation
• Dry eyes
• Blepharospasm
• Preceeded by minor ocular trauma
• Sympathetically mediated
Fine and Digre. J Neuro-Ophthalmol 1995; 15:90-94
Greater Occipital Neuralgia
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Occipital area pain that radiates to eye
Aggravated by postural and neck movements
Reproduceed by pressing on occipital nerves
Pain in eyebrow, orbit, and temple
Women>Men
Associated with cervical
spondylosis and whiplash
• Relief with local anesthetic
Ophthalmoplegic “Migraine”
• No longer a “migraine” disorder
• Considered a Cranial Neuralgia (IHS 13.17)
• At least 2 attacks of migraine headache associated with
paresis of one or more CN (CN III more frequent than IV
or VI)
• Pain ipsilateral to paresis
• CN palsy accompanies headache or follows it within 4
days
• No MRI lesions except within the nerve
• Rare; Onset in childhood
• Ophthalmoplegia may be permanent and aberrant
regeneration is rare
Ophthalmoplegic “Migraine”
• Neuroimaging suggests an inflammatory process
• Trigeminovascular activation: Sterile inflammation:
Demyelination
From Tom Carlow
J Neuro-Ophthalmol 2002; 22:215-221
Migraine and Stroke
• True migrainous infarction
• Women>Men (BCP, Smoking)
• Co-morbidities (MVP, PFO, Carotid
Dissection, Anti-Phospholipid antibodies)
• CADASIL, MELAS
• MRI white matter hyperintensies seen in
cerebellar area
Secondary Headache Disorders
with Neuro-Ophthalmic Features
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Carotid dissection
PCA aneurysm
Giant Cell Arteritis
Pituitary Apoplexy
IIH (Pseudotumor
Cerebri)
• H. Zoster (V1)
• Brain tumour
• Tolosa-Hunt
Syndrome
• Inflammatory Orbital
Pseudotumour
• Optic Neuritis
• Occipital lobe CVA
International Headache Society
Web
Address
!!
International Headache Society
Further Reading
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Rapoport A, Edmeads J. Migraine:The Evolution of Our Knowledge. Arch
Neurol 2000; 57:1221-1223.
Corbett J.J. Neuro-Ophthalmic Complications of Migraine and Cluster
Headaches. Neurologic Clinics 1983; 1: 973-995.
Hupp S.L., Kline L., Corbett J.J. Visual Disturbances of Migraine. Survey of
Ophthalmology 1989; 33: 221-236.
Friedman D.I. The eye and headache. Ophthalmol Clin N Am 2004; 17: 357369.
Lance J.W., Goadsby P.J. Mechanism and Management of HeadacheSeventh Edition. 2005; Elseveier-Butterworth-Heinemann Publishers
Purdy R.A., Rapoport A.M., Sheftell F., Tepper J. Advanced Therapy of
Headache: 2nd Edition. 2005; B.C. Decker Inc