The Neuro-Ophthalmology of Headache

1
The Neuro-Ophthalmology ofHeadache

• 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 lighta violent pain supervened in the
  right temple, then all in the head and neck
• Hippocrates

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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

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Visual Disturbances of Migraine

• Sir Hubert Airy (1871) Published On a distinct
  form of transient hemianopia coining the term
  teichopsia (Greek teichosfortification and
  opsiaseeing)
• X. Galezowski(1882) ophthalmic megrim in 3
  migraineurs with CRAO
• C.M. Fisher(1952) Migrainous amaurosis fugax

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Visual Disturbances of Migraine

• Sir Hubert Airys Artistry (1870)

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Visual Disturbances of Migraine

• IHS ICHD-2 Code 1.2
• Migraine with Aura
• Positive gtNegative Scotomata
• Often hemianopic
• Buildup and march
• 20-30 minute duration
• Subsequent headache

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Visual Disturbances of Migraine
Adapted from Hupp, Kline, Corbett Surv
Ophthalmology 1989 33 221-236
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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

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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 Leãos spreading
  depression..

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Visual Disturbances of Migraine
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Visual Disturbances of Migraine

• Migraine Aura
• Cerebral blood flow studiesOlesen 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?

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

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Visual Disturbances of Migraine
Serotonin System and Sterile Inflammation
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Visual Disturbances of Migraine

• Migraine Aura Cause?
• BiochemicalMagnesium
• Neuro-transmitter Serotonin
• Visual cortex Aspects of Visual Input
• Electrical Migraine Generator

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Visual Disturbances of Migraine
Acute Treatment of Migraine The Triptans
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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

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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)
• NegativegtPositive symptoms
• Qualitatively different from amaurosis fugax
• Vascular spasm Arteriolar vs Venular
• Headache variable

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Visual Disturbances of Migraine

• Carroll D. Retinal migraine. Headache 1970
  109-13.
• Winterkorn J. et al Treatment of vasospastic
  amaurosis fugax with calcium channel blockers.
  NEJM 1993 329396-8.
• Ammache Z. Idiopathic stabbing headache
  associated with monocular visual loss. Arch
  Neurol 2000 57745-6.

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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)

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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

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Features of TACs (Cluster)

• Gender, FM
• Attack frequency/day
• Duration
• Response to indomethacin
• Conjunctival injection, lacrimation
• Nasal congestion,rhinorrhea
• Eyelid edema
• Forehead/facial sweating
• Horner syndrome
• Restlessness, agitation

• 13
• lt 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
17357-369
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Cluster Headache
PET assessment of rCBF in Triggered Cluster
Adapted from May, Goadsby et al Queen Square,
London
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Features of TACs (Paroxysmal Hemicrania)

• Gender, FM
• Attack frequency/day
• Duration
• Response to indomethacin
• Conjunctival injection, lacrimation
• Nasal congestion,rhinorrhea
• Eyelid edema
• Forehead/facial sweating
• Horner syndrome
• Restlessness, agitation

• 21
• gt5
• 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
17357-369
26
Features of TACs (SUNCT)

• Gender, FM
• Attack frequency/day
• Duration
• Response to indomethacin
• Conjunctival injection, lacrimation
• Nasal congestion,rhinorrhea
• Eyelid edema
• Forehead/facial sweating
• Horner syndrome
• Restlessness, agitation

• 12
• 3-200
• 5 seconds to 4 minutes
• No
• Both
• No
• No
• No
• No
• No

Adapted from Friedman. Ophth Clin N Am 2004
17357-369
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

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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 1590-94
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
• WomengtMen
• Associated with cervical
• spondylosis and whiplash
• Relief with local anesthetic

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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
22215-221
32
Migraine and Stroke

• True migrainous infarction
• WomengtMen (BCP, Smoking)
• Co-morbidities (MVP, PFO, Carotid Dissection,
  Anti-Phospholipid antibodies)
• CADASIL, MELAS
• MRI white matter hyperintensies seen in
  cerebellar area

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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
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Further Reading

• Rapoport A, Edmeads J. MigraineThe Evolution of
  Our Knowledge. Arch Neurol 2000 571221-1223.
• 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 17 357-369.
• 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