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Basilar Migraines and Retinal Migraines P1: KWW/KKL P2: KWW/HCN QC: KWW/FLX T1: KWW GRBT050-62 Olesen- 2057G GRBT050-Olesen-v6.cls August 3, 2005 19:44 ••Chapter 62 ◗ Basilar Migraines and Retinal Migraines Marcelo Bigal and K. Michael A. Welsh Although the second edition of the International Classifica- graine is a symptom profile that suggests posterior fossa in- tion of Headache Disorders (1), preserved the basic classi- volvement. fication of migraine without aura, the classification of mi- Basilar-type migraine was first brought to attention by graine with aura and it subtypes, as well as rarer forms of Bickerstaff (6), after seeing a teenager whose aura symp- migraine, were considerably restructured. Given the range toms were long lasting, bilateral, and very prominent. of disorders that present with features of migraine, a sys- Shortly after, the same clinician saw an elderly man, with tematic approach to the less common disorders that are aura symptoms that rapidly progressed into coma and part of the migraine spectrum is essential to good clini- death. Autopsy showed thrombosis in the basilar artery. cal management and to research (2,3). In this chapter we After collecting data on 34 patients, Bickerstaff postulated review two of these headaches, basilar-type migraine, a that “some prodromal symptoms ... are not in the ter- migraine with aura subtype, and retinal migraine, a rarer ritory of the internal carotid, but of the basilar artery. form of migraine. The premonitory symptoms are different and reflect brain- stem dysfunction. Symptoms consist of bilateral loss or disturbance of vision, ataxia, dysarthria, vertigo, tinnitus, BASILAR-TYPE MIGRAINE and bilateral peripheral dysesthesias, followed by occipi- tal headache. The syndrome is commonest in adolescent IHS Code and Diagnosis girls” (6,7). Studies on the pathophysiology of basilar migraine are 1.2.6 Basilar-type migraine scarce. For years this headache was thought to originate from a transient disturbance in the vertebrobasilar circula- WHO Code and Diagnosis tion (6–8). Almost two decades ago, neurophysiology stud- ies using electroencephalogram suggested the neurophysi- G43.103 ologic basis of basilar-type migraine, by detecting a typical photoconvulsive response (9). Other studies focused on the Short Description vascular mechanism of basilar-type migraine. La Spina et al., in 1996 (10), documented a basilar-type migraine at- Migraine with aura symptoms clearly originating from the tack using transcranial Doppler (TCD), electroencephalog- brainstem and/or from both hemispheres simultaneously raphy (EEG), and single-photon emission computed affected, but no motor weakness. tomography (SPECT). In the aura phase, the patient had bilateral blindness and ataxia. Doppler ultrasound re- Previously Used Terms vealed reduced mean flow velocity of the posterior cerebral arteries, EEG showed posterior slow activity, and SPECT Basilar migraine documented hypoperfusion in the right parietal and oc- Basilar artery migraine cipital regions. During the headache phase with normal Bickerstaff migraine neurologic examination, TCD showed an increase in the Basilar-type migraine (1.2.6) is a new term, replacing basi- mean flow velocity of both posterior cerebral arteries and lar migraine (4). The change is intended to remove the the EEG revealed increased in occipital slow activity.When implication that the basilar artery (or its territory) is in- headache subsided, EEG slow activity resolved and the volved (5). The distinguishing feature of basilar-type mi- TCD findings were normal. 589 P1: KWW/KKL P2: KWW/HCN QC: KWW/FLX T1: KWW GRBT050-62 Olesen- 2057G GRBT050-Olesen-v6.cls August 3, 2005 19:44 590 The Migraines Current migraine theories consider migraine as a state expand to involve both visual fields, leading at times to of neuronal hyperexcitability, and that neuronal events temporary blindness that may precede other symptoms mediate both the aura and headache phases of migraine (17). Alterations in consciousness may be present, es- (11–13), although studies specifically investigating basilar- pecially in younger subjects (18). Basilar-type migraine type migraine are still lacking. These findings support with prominent alteration of consciousness is often called transient focal reduction of cerebral blood flow during confusional migraine, a term not adopted by the second the aura phase. It remains to be determined if the vas- edition of the International Classification of Headache cular changes are the cause or, as in other forms of Disorders (1). migraine with aura, the consequence of neuronal dysfunc- Occipital headache appears more frequent in basilar- tion. To date, mutations in CACNA1A, encoding a neu- type migraine than in typical migraine with aura (19). ronal calcium channel subunit, and ATP1A2, encoding a Headache is most frequently throbbing. In one case series, catalytic subunit of a sodium-potassium-ATPase, have not nausea was present in most patients (83%). Two studies been found in subjects with basilar-type migraine (14). showed that alteration of consciousness (45% and 75%), vertigo (41%, 63%), bilateral visual disturbances (48%, Classification 86%), bilateral sensory changes (14%, 61%), and ataxia (17%, 63%) are common symptoms in basilar-type mi- In classifying basilar-type migraine, the following criteria graine (16,19). are required (1): A. At least two attacks fulfilling criteria B through E. Differential Diagnosis B. Fully reversible visual and/or sensory and/or speech The diagnosis of basilar-type migraine should be consid- aura but no motor weakness. ered in patients with paroxysmal brainstem disturbances. C. Tw oormore fully reversible aura symptoms of the fol- Adolescents with family history seldom require further in- lowing types: vestigation, but in this age range basilar-type migraine may 1. Dysarthria be difficult to differentiate from complex partial seizures 2. Vertigo or from postepileptic state. Aura symptoms are often fol- 3. Tinnitus lowed by severe, throbbing occipital headache and vomit- 4. Decreased hearing ing. Usually infrequent, they can last for 1 to 3 days. Rarely, 5. Double vision attacks may be complicated by cardiac arrhythmias and 6. Ataxia brainstem stroke (20,21). 7. Decreased level of consciousness The differential diagnosis, besides occipital lobe 8. Simultaneous bilateral visual symptoms in both the epilepsy, includes posterior fossa tumor or malforma- temporal and nasal field of both eyes tion, urea cycle defects, and mitochondrial disorders (21). 9. Simultaneous bilateral paresthesias When vertigo is prominent, occurring at times alone, other D. Headache that meets criteria B through D for migraine causes of vertigo should be considered, including M´eni`ere without aura (1.1) begins during the aura or follows disease. If weakness is present, familial or sporadic hemi- aura within 60 minutes plegic migraine should be considered. E. Not attributed to another disorder In basilar-type migraine first presenting in persons over It is important to note that if distinct motor weakness the age of 50, vascular pathologies should be excluded. In is present, the diagnosis should be hemiplegic migraine such cases, magnetic resonance imaging and angiography even when “basilar symptoms” are present (hemiplegic should be performed (22). migraine sufferers have basilar-type symptoms in 60% of cases) (15). Treatment Clinical Features No acceptable “standard of treatment” for migraine pro- phylaxis currently exists for patients who have basilar-type Basilar-type migraine affects all age groups with the artery migraine (23). Largely anecdotal, some reports sug- usual female predominance, but is most common among gest this disorder may be aggravated by treatment with β- teenage girls (16,17). A distinguishing feature of basilar- blockers to the point of precipitating ischemic strokes (24). type migraine is the bilateral nature and posterior fossa Many specialists recommend a calcium-channel blocker origin of many of the associated symptoms (16), differen- such as verapamil; flunarizine (10 mg per day), which is tiating it from typical migraine. Visual aura, if present, is not available in the United States, may be a useful alterna- usually followed by one of the distinguishing symptoms of tive. Antiepileptic drugs (divalproex sodium, topiramate) this disorder. A hemianopic field disturbance can rapidly have also been recommended. P1: KWW/KKL P2: KWW/HCN QC: KWW/FLX T1: KWW GRBT050-62 Olesen- 2057G GRBT050-Olesen-v6.cls August 3, 2005 19:44 Basilar Migraines and Retinal Migraines 591 Acute management must be approached with caution; A family history of migraine may be present in approx- nonsteroidal anti-inflammatory drugs or combination imately 25% of probands with retinal migraine (a simi- analgesics should first be tried. Restricting use of triptans lar rate is found in other forms of migraine) (27,29). The in basilar-type and hemiplegic migraine is based on the pathogenesis of this disorder remains to be determined. concept that neurologic symptoms associated with basilar- Neuronal spreading depression has been documented in type migraine may be caused by vasoconstriction, con- the retina of chicken and other animals (30,31). Given the sequently increasing the risk of brain infarction. How- monocular nature of retinal migraine, a prechiasmatic ori- ever, Klapper et al. treated 13 patients with basilar-type gin may be inferred (32). Some authors explain retinal migraine or migraine with prominent aura with triptans; migraine by the involvement of the posterior ciliary vas- all responded positively
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