1. The Neuro-Ophthalmology of Headache
Charles E. Maxner MD, FRCPC
Departments of Medicine (Neurology) and Ophthalmology
Dalhousie University, Halifax, NS

2. 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

3. 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

4. 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

5. 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

6. Visual Disturbances of Migraine
Sir Hubert Airy’s Artistry (1870)

7. Visual Disturbances of Migraine
IHS ICHD-2 Code 1.2
Migraine with Aura
Positive >Negative Scotomata
Often hemianopic
Buildup and march
20-30 minute duration
Subsequent headache

8. Visual Disturbances of Migraine
Adapted from Hupp, Kline, Corbett:
Surv Ophthalmology 1989; 33:

9. Visual Phenomena of Migraine
Positive
Fortification spectra
Blurred vision
Heat waves
Phosphenes
Fragmented “cracked glass”
Distortion
Negative
Homonymous hemianopia
Tunnel Vision
Cortical blindness
TMB
Cortical
Déjà vu
Jamais vu
Micropsia
Macropsia
Dyschromatopsia

10. 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..”

11. Visual Disturbances of Migraine

12. 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?

13. Visual Disturbances of Migraine
Headache and CBF
Spreading oligemia during migraine aura:
Adapted from Lauritzen

14. 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

15. Visual Disturbances of Migraine
Serotonin System and Sterile Inflammation

16. Visual Disturbances of Migraine
Migraine Aura: Cause?
Biochemical:Magnesium
Neuro-transmitter: Serotonin
Visual cortex: Aspects of Visual Input
Electrical: “Migraine Generator”

17. Visual Disturbances of Migraine
Acute Treatment of Migraine: The Triptans

18. 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

19. Visual Disturbances of Migraine
Ocular or Retinal Migraine (IHS1.4)
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

20. 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.

21. 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 ( )

22. Trigeminal Autonomic Cephalgias
3.1 Cluster Headache
Episodic
Chronic
3.2 Paroxysmal Hemicrania
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

23. Features of TACs (Cluster)
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
1:3
< 8
minutes
Sometimes
At least one
Yes
Adapted from Friedman. Ophth Clin N Am 2004; 17:

24. Cluster Headache PET assessment of rCBF in Triggered Cluster
Adapted from May, Goadsby et al; Queen Square, London

25. Features of TACs (Paroxysmal Hemicrania)
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
2:1
>5
2-30 minutes
Required for diagnosis
At least one
Yes No
Adapted from Friedman. Ophth Clin N Am 2004; 17:

26. Features of TACs (SUNCT)
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
1:2
3-200
5 seconds to 4 minutes No
Both
Adapted from Friedman. Ophth Clin N Am 2004; 17:

27. 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

28. 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

29. Greater Occipital Neuralgia
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

30. 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

31. Ophthalmoplegic “Migraine”
Neuroimaging suggests an inflammatory process
Trigeminovascular activation: Sterile inflammation: Demyelination
From Tom Carlow
J Neuro-Ophthalmol 2002; 22:

32. 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

33. Secondary Headache Disorders with Neuro-Ophthalmic Features
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

34. International Headache Society
Web Address !!

35. International Headache Society

36. Further Reading
Rapoport A, Edmeads J. Migraine:The Evolution of Our Knowledge. Arch Neurol 2000; 57:
Corbett J.J. Neuro-Ophthalmic Complications of Migraine and Cluster Headaches. Neurologic Clinics 1983; 1:
Hupp S.L., Kline L., Corbett J.J. Visual Disturbances of Migraine. Survey of Ophthalmology 1989; 33:
Friedman D.I. The eye and headache. Ophthalmol Clin N Am 2004; 17:
Lance J.W., Goadsby P.J. Mechanism and Management of Headache-Seventh 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