Migraine and vertebrobasilar dissection: Case reports

J Stroke Cerebravasc Dis 1994;4:36-41 ©"1994 National Stroke Association

Migraine and Vertebrobasilar Dissection: Case Reports 1M. A. Yenari, M.D., 2G. K. Steinberg, M.D., Ph.D., and 3M. P. Marks, M.D.

We report two patients with chronic migraine headaches who developed vertebrobasilar arterial dissections. In both cases, there was a change in both the character and location of the headaches, which prompted further evaluation with magnetic resonance imaging and angiography, which led to the diagnosis. Other possible risk factors for dissection in these patients included hypertension, exercise, and chiropractic manipulation. Both patients were treated with anticoagulation and recovered with little to no neurologic deficit. A review of the literature relating migraine and arterial dissection suggests that there may be an association. Although more extensive controlled studies are needed to demonstrate the significance of this association, we suggest that the diagnosis of cervicocranial arterial dissection be strongly considered in migraine patients who develop an abrupt change in the nature of their headaches. Key Words: Migraine-Arterial dissection-Cerebrovascular disease-Vertebrobasilar artery.

Migraine headaches are often considered a relatively benign entity; however, they can be associated with cerebrovascular ischemia and infarction. It has been estimated that 4-20% of strokes among young patients are due to migraine (1-3) . Migraine-related stroke has been attributed to cerebral vasospasm with subsequent arterial occlusion, platelet hyperaggregation, and vessel wall edema (1). We present two cases of migraine with neurologic deficit associated with angiographically documented dissection in the vertebrobasilar vasculature. A review of the literature relating migraine and dissection is presented.

Case Reports Case 1 A 40-year-old right-handed woman presented to an emergency room with headache and subsequent From the Departments of INeurology and Neurological Sciences, 2Neurosurgery,and 3Radiology,Stanford University Medical Center, Stanford, CA, U.S.A. Address correspondence and reprint requests to Dr. G. K Steinberg at Department of Neurosurgery, Stanford University Medical Center, Room S006, 300 Pasteur Road, Stanford, CA 95305, U.S.A. 36

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right-sided weakness and sensory loss involving the face, arm, and leg. She reported suffering from migraine headaches since childhood. Her headaches were characterized by a right-sided throbbing pain preceded by an aura of flashing lights throught her visual field. Her headaches were frequently associated with nausea and vomiting and occurred one to two times a month, lasted about 10-12 h, and readily responded to bedrest and an occasional aspirin. She had been treated in the past with ergots and methysergide but discontinued the drugs herself because she felt the aspirin was sufficient. Several weeks prior to her presentation, the patient began an exercise regimen of running approximately four miles a day. She reported that she had not been running regularly for several years prior to that. Two weeks after beginning her exercise program, she began to suffer from severe suboccipital headaches, which were different from her typical migraine. This headache began suddenly without her usual aura and was severe at the onset. Approximately 2 weeks after the headaches began, she developed transient rightsided hemiparesis and hemisensory loss associated with speech difficulty, hearing impairment, and blurred vision. She was brought to an emergency room. Her symptoms completely resolved after 2 h. Computed tomography (CT) of the head was nega-

MIGRAINE AND VERTEBROBASIlAR DISSECTION

-

A

... " B

Figure 1. A: AxialTl-weighted MR scan attheleoel ofthepons demonstrates highsignal in thewallofthebasilar artery (curved arrow).

Thisisconsistent with subintimal hemorrhage from a basilar artery dissection. B: Leftvertebral artery angiogram in thelateral projection. Pseudoaneurysm wasnoted attheCl leuel (curued arrow). Thiswasbetter seen onunsubtracted view. High-grade stenosis wasseen in the basilar artery (small arrows) consistent with dissection.

tive. A lumbar puncture was performed, which revealed 56 red blood cells but no xanthochromia and with normal glucose and protein. Magnetic resonance imaging (MRI) demonstrated a small region of ischemia in the medial left pons, and there was also suggestion of a subintimal hemorrhage in the basilar artery consistent with dissection (Fig. lA). MR angiography demonstrated slowed flow in the basilar artery. She was admitted for angiographic studies. Her medical history was otherwise unremarkable. She had no history of hypertension, smoking, collagen-vascular disease, sexually transmitted diseases, illicit drug use, or trauma. She denied any history of chiropractic manipulation. She did report use of oral contraceptive pills approximately 15 years prior to her admission, but she had discontinued them because they had made her migraines worse. There was a family history of migraine, with her father and brother suffering similar headaches. There was no family history of cerebrovascular disease. Her physical examination on admission was entirelywithin normal limits and she was neurologically intact. Laboratory studies including general chemistries, complete blood count, platelet count, erythrocyte sedimentation rate, anticardiolipin antibody, protein C and S, anti-thrombin Ill, fibrinogen, and prothrombin time/partial thromboplastin time were

all within normal limits. Transesophageal echocardiography revealed only a small patent ductus arteriosus. She underwent four-vessel angiography, which demonstrated a pseudoaneurysm of the vertebral artery. The distal vertebral artery was irregular, and the basilar artery was stenotic with poor distal filling secondary to a dissection (Fig. IB). She was subsequently begun on oral anticoagulation with low-dose coumadin and discharged home on a therapeutic dose. She returned to full-time emloyment taking an occasional analgesic for occipital pain but has not had any migraine headaches or any neurologic deficit.

Case 2 A 42-year-old right-handed man with a long history of cluster-migraine presented to an emergency room with severe neck pain, right-sided hemiparesis and hemisensory loss followed by eventual respiratory arrest. He had suffered from headaches since his early 20s, describing his headaches as right-sided, nonthrobbing, boring orbital pain radiating to the occiput. They were often associated with a rightsided conjunctivitis and rhinorrhea. Some of his headaches were preceded by an aura of flashing ] STROKE CEREBROVASC DIS, VOL. 4, NO.1, 1994

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M A. YENARI IT AL.

A

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Figure 2. A: Composite offourT2-weighted axial MR scans through thelower brainsiem. Scans extend from thecervicomedullary junction (upper leftcorner) up totheupper medulla (lower right corner). High signal representative ofan infarct is noted on theleftsideofthe medulla atallthescanleuels. B: Oblique viewofleftvertebral angiogram demonstates a pseudoaneurysm attheC2leve/(curved arrow). In addition, there is irregularity of the distal vertebral and proximal basilar artery.

lights. These symptoms would occur approximately every other year and persist for several days to a month, remitting spontaneously. He had been followed by a local neurologist and was well-controlled on a regimen of methysergide and lithium. Approximately 3 months prior to admission, he suffered a particularly severe bout of cluster headaches. In addition, he developed a new left-sided occipital headache, which was exacerbated with neck turning. A local chiropractor performed neck manipulations, but a week later the pain worsened and the patient complained of vertigo and right-sided sensory loss. He then developed a right hemiparesis and subsequently had a respiratory arrest. His wife began cardiopulmonary resuscitation, and he was rushed to the emergency room, where he was intubated and admitted to the ICU. CT, MRI, and EEG were reportedly negative. Lumbar puncture revealed 42 red blood cells without xanthochromia, no white blood cells, and normal glucose and protein. He was treated with corticosteroids and regained some neurologic function but still had some residual right-sided weakness and sensory loss. He was transferred to this institution for further evaluation. He had a history of hypertension, which was wellcontrolled on medications, and Grave's disease for which he had been treated with 1311in 1985. He was euthyroid on thyroid replacement. He was a nonsmoker and nondrinker. He denied any use of illicit drugs. There was no history of trauma. His medications at the time of admission included synthroid, 38 ] STROKE CEREBROVASC DIS, VOL. 4, NO.1, 1994

propranolol, dexamethasone, ranitidine, and clonidine. His mother suffered from common migraines, but there was no history of cluster headaches or cerebrovascular disease. Pupillary findings and extraocular movements were normal, but he had mild left-sided facial numbness and weakness with a diminished left-sided gag reflex. He also had right-sided hemiparesis and right hemisensory loss involving the arm and leg. Deep tendon reflexes were brisk on the right with an extensor plantar reflex on the same side. Coordination was also normal. His physical examination was otherwise within normal limits at the time of admission. Laboratory studies including blood chemistries, complete blood count, platelet count, erythrocyte sedimentation rate, prothrombin time/partial thromboplastin time, protein C and 5, antithrombin Ill, antinuclear antibody, rapid plasma reagin, and fluorescent treponemal antibody were all normal. Repeat head MRI and MR angiography revealed a left medullary infarct (Fig. 2A). . The patient underwent four-vessel angiography, which demonstrated a left vertebral artery dissection. There was a pseudoaneurysm at the C2level (Fig. 2B). There was also a right vertebral pseudoaneurysm suggesting an old dissection. He was anticoagulated with heparin, then switched to low-dose coumadin. At the time of discharge, he required the assistance of a walker. Three months later, he had made complete

MIGRAINE AND VERTEBROBASllAR DISSECTION

neurologic recovery except for subjective diplopia on far right lateral gaze and slight altered right-sided body sensation. He returned to part-time employment. Repeat angiography 3 months later showed persistence of the left vertebral artery dissection with partial healing of both his vertebral artery pseudoaneurysms.

Discussion The two cases described represent vertebrobasilar dissections occurring in chronic headache patients, one with classic migraine and the other with cluster migraines. Both had a long history of headaches but developed new symptoms clearly different and more severe than their usual headaches. In the first case, mild trauma in the form of exercise may have contributed to the dissection. In the second case, risk factors included hypertension and neck manipulation. Chiropractic manipulation began after the onset of the atypical headache, but it may have actually worsened a pre-existing dissection. In addition, there was angiographic evidence of prior dissection in the opposite vertebral artery. The association between migraine and arterial dissection has, for the most part, been anecdotal. There are 40 cases of arterial dissection associated with migraine in the English-speaking literature (4-13). It is unclear whether migraines cause vessel wall damage or whether there is an intrinsic vessel wall abnormality in the migraine patient. One etiology that has been postulated is that chronic vasodilation and constriction of the cervicocranial arteries weakens the vessel wall predisposing to intimal tears (11). Autopsies of patients with an antemortem diagnosis of "migraine stroke" reveal intimal hyperplasia, adventitial thickening, and narrowing of some vessels (10,14). These findings support the concept that chronic migraineurs suffer damage to their cerebral vessels. In 1953, Sinclair described a young woman with a long history of migraines who developed a fatal middle cerebral artery stroke (11). Autopsy revealed internal carotid and middle cerebral artery dissection. Ante- and postmortem studies failed to reveal an alternative etiology. Affected vessel walls were noted to have edema and fibrosis. Spudis et al. (12) reported a second case of fatal dissection of the internal carotid and middle cerebral arteries in a chronic migraineur. At autopsy, however, no abnormal cerebral vasculature was noted. Alexander et al. (4) described a dissection of the basilar artery in a migraineur, which also failed to demonstrate cerebral vascular abnormalities at autopsy. Youl et al. (13) reported a migraineur with

internal carotid and vertebral artery dissections who was subequently diagnosed with Marfan's syndrome. A review of 13 cases of vertebral artery dissection by Mas etal. (8) revealed that Sof l S (46%) hada prior history of migraine. Mokri reported a history of migriane in 11% of 36 patients with internal carotid artery dissections and in 8% of those with vertebral artery dissections (9,15). A recent case control study by D'Anglejan-Chatillon et al. (6) showed a significant correlation between dissection and migraine (p < 0.05). Twenty of 50 patients (40%) with angiographically documented dissection were chronic migraineurs, whereas 24 of 100 (24%) controls suffered from migraines. Their data was also published in the French literature as a larger series of 62 carotid artery dissections (16). Of those 62 patients, 21 (34%) suffered migraines. Characteristics of 40 patients with dissection and migraine reported in the literature are summarized in Table 1. The age range was 27-45 years with a mean of 38 years and a slight preponderance of women. Of those patients whose headache character was described, half reported an increase in severity or new neurologic deficit, whereas half did not report any change. Most patients previously reported suffered common migraines (72%) rather than classic or complicated migraine (27%). Associated risk factors included concurrent oral contraceptive use (16%), neck manipulation (12.5%), smoking (8%), Marfan's syndrome (one case), fibromuscular dysplasia (one case), and hypertension (one case). Anticoagulation is frequently recommended for the treatment of cervicocranial dissections. With dissection and subsequent thrombus formation, there is concern of subsequent vessel occlusion and embolic events. This is supported by the fact that 15% of angiograms that show dissection also show distal artery occlusion suggesting embolism (17,18). A 7-day course of intravenous heparin therapy followed by 3 months of coumadin therapy is common practice. Aspirin instead of coumadin has been suggested in cases in which coumadin is contraindicated (17,18). The data in the literature relating migraine and arterial dissection are unclear, but there appears to be an association. More controlled studies are needed to determine the association, since it may impact on the way one treats migraineurs. It may be possible to identify a population of migraine sufferers who would benefit from pharmacologic prophylaxis or who should avoid high-risk activities. In the meantime, we would stress that a change in the quality or severity of headaches in migraine patients may indicate dissection. In those patients with

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M A. YENARI ET AI.

Table 1.

Summary of characteristics ill migraineurs with dissection reported ill the literature of 40 cases

Study

No. of cases

Sinclair, 1953 (11)

1

27/F

None

Spudis et al., 1974 (12) Bladin, 1974 (5)

1

30/F

None

2

421F 45/M

N/A N/A

Alexander et aI., 1979 (4) Mokri et aI., 1986 (9) Mas et aI., 1987 (8)

1

30/M

4

Dehaene and Meeus, 1989 (7) D'Anglejan et aI., 1989 (6) Youl et aI., 1990 (13) Schuaib,1991 (10)

Age/sex

Risk factors

Migraine type/ pattern change? Common/neurodeficit Classic/no

Dissection location

Follow-up

1 eICA/iMCA

Death

1 elCA 1 eICA 1 eVA

Death

N/A

Common/NzA Common/more severe Classic/no

BA

Death Recovery with deficit Death

N/A

N/A

N/A/no

N/A

N/A

6

27-40/ SF, 1M

N/A

SeVA 1 e, iVA

S good outcome/l deficit

1

40/F

4 OCP, 2 chiropractic, 1 FMD, 1 smoking, 1 HTN N/A

N/A

1 eVA

N/A

20

x = 42/ 12F, 8M 31/M

N/A

4 classic, 16 common/N/A

N/A

N/A

Marfan's, smoking

Common/worse

Recovery

1 mild trauma

2 common, 1 classici all worse

leVA 1 eICA 1 BA 2eICA

1 3

29/M 28/F 33/M

Recovery

Abbreviations: OCP, oral contraceptives; FMD, fibromuscular dysplasia; HTN, hypertension; N/A, data not available; x, mean; ICA, internal carotid artery; VA,vertebral artery; MCA, middle cerebral artery; BA, basilar artery; i, intracranial; e, extracranial.

high clinical suspicion, routine MRI offers high yield in detecting dissections as was demonstrated in our first case. MRI characteristics of dissection include absence of flow void or hemorrhage into a vessel wall. Angiography remains the gold standard; however, MR angiography appears to be a promising new technology (19). We suggest migraine patients who develop a change in the quality or severity of their headaches or experience new neurologic symptoms should be more thoroughly investigated with MRI and, if indicated, angiography.

3. 4. 5. 6. 7.

Acknowledgment: We thank Dr. Gregory W. Albers for graciously reviewing this manuscript and offering helpful suggestions.

8. 9.

References

10.

1. Sacquegna T, Andreoli A, Baldrati A, et al, Ischemic stroke in young adults: the relevance of migrainous infarction. Cephalalgia 1989;9:255-8. 2. Solomon GD, Spaccavento LJ. Lateral medullary syn40

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MIGRAINE AND VERTEBROBASlLAR DISSECTION

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Atlll

Neurol1982;12:75.

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17. Hart RG, Easton JD. Dissection of cervical and cerebral arteries. Neurol Clin 1983;1:155-81. 18. Anson J, Crowell RM. Review article: cervicocranial arterial dissection. Stroke 1991;29:89-96. 19. Bui LN, Brant-Zawadzki M, Verghese P, Gillan G. Magnetic resonance angiography of cervicocranial dissection. Stroke 1993;24:126-31.·

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