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 lighta 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 Airys Artistry (1870)

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

Visual Disturbances of Migraine

Adapted from Hupp, Kline, Corbett: Surv Ophthalmology 1989; 33: 221-236

Visual Phenomena of Migraine

Positive
Fortification spectra Blurred vision Heat waves Phosphenes Fragmented cracked glass Distortion

Negative

Homonymous hemianopia Tunnel Vision Cortical blindness TMB

Dj vu Jamais vu Micropsia Macropsia Dyschromatopsia

Cortical

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 Leo (1944) P. Milner(1958): ..attention should be drawn to the striking similarity between the time courses of scintillating scotomas and Leos 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?
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)
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)

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 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)

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 At least one Yes At least one Yes No

Adapted from Friedman. Ophth Clin N Am 2004; 17:357-369

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

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

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

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

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International Headache Society

Further Reading

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