the neuro-ophthalmology of headache charles e. maxner md, frcpc departments of medicine (neurology)...

The Neuro-Ophthalmology of

Headache

Charles E. Maxner MD, FRCPCDepartments 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


HistoryJohn 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


• 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


Sir Hubert Airy’s Artistry (1870)


IHS ICHD-2 Code 1.2Migraine with Aura

• Positive >Negative Scotomata

• Often hemianopic• Buildup and march• 20-30 minute duration• Subsequent headache


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

Cortical• Déjà vu• Jamais vu• Micropsia• Macropsia• Dyschromatopsia


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..”


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?


Headache and CBF

Spreading oligemia during

migraine aura:Adapted from Lauritzen


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


Serotonin System and Sterile Inflammation


Migraine Aura: Cause?• Biochemical:Magnesium• Neuro-transmitter: Serotonin• Visual cortex: Aspects of Visual Input

• Electrical: “Migraine Generator”


Acute Treatment of Migraine: The Triptans


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


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


• 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 manifestationsLacrimationEyelid EdemaConjunctival InjectionHorner syndromeBenign episodic unilateral pupillary dilation

• IHS Section 3 (3.1-3.4)

Trigeminal Autonomic Cephalgias

• 3.1 Cluster HeadacheEpisodicChronic

• 3.2 Paroxysmal HemicraniaEpisodicChronic (CPH)

• 3.3 SUNCTShort-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)






• 2:1• >5• 2-30 minutes• Required for

diagnosis• At least one• At least one• Yes• At least one• Yes• No


Features of TACs (SUNCT)






• 1:2• 3-200• 5 seconds to 4 minutes• No• Both

• No• No• No• No• No


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”

From Tom Carlow

J Neuro-Ophthalmol 2002; 22:215-221

• Neuroimaging suggests an inflammatory process

• Trigeminovascular activation: Sterile inflammation: Demyelination

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

!!

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: 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

the neuro-ophthalmology of headache charles e. maxner md, frcpc departments of medicine (neurology)...

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