Cerebral arterial dolichoectasia with seizure Case report JOSEPH R. THOMPSON, M.D., PHILIP R. WEINSTEIN, M.D., AND CHARLES R. SIMMONS, M.D. Loma Linda University Medical Center, Loma Linda, California v' Cerebral angiography, performed after a seizure in a patient with a life-long history of typical hemiplegic migraine, disclosed markedly dolichoectatic anterior and middle cerebral arteries. No abnormality of the adjacent capillary or venous structures was present. A positive brain scan was attributed to ischemia induced by vasospasm rather than to the corresponding large tortuous anterior and middle cerebral arteries. There were no permanent sequelae and the patient has been free of seizures on Dilantin and phenobarbital over a 3-year follow-up period. Angiographic demonstration or description of a similar ectatic set of anterior and middle cerebral arteries could not be found in the literature. The concurrence of seizures and hemiplegic migraine adds to the peculiarity of this case. KEY WORDS migraine 9 c e r e b r a l arteries 9 dolichoectasia 9 ectasia 9 seizures Case Report RTERIAL dolichoectasia (elongationdistension) is an uncommon condition that may involve intracranial This 39-year-old right-handed man was adarteries, but rarely causes gigantic enlarge- mitted for neurodiagnostic evaluation 1 ment. When severe enlargement does occur, it month after his first grand mal seizure. He involves the arteries at the base of the brain. had not slept for 24 hours when he developed This entity usually presents symptoms like his typical prodromata of migraine and took those of an intracranial t u m o r ? Our search of ergotamine tartrate (Cafergot) which brought the literature has not revealed a previous case relief. About 30 minutes later he experienced involving cerebral arteries with the exclusion nausea and dizziness which progressed to loss of the carotid or basilar arteries. The oc- of consciousness and generalized seizure. currence of migraine headache complicated The patient had a history of two or three by hemiplegia and the presentation with a typical episodes of hemiplegic migraine per seizure adds interest to this unusual case. year since the age of 11 years. The attacks A J. Neurosurg. / Volume 44 / April, 1976 509 J. R. Thompson, P. R. Weinstein and C. R. Simmons F~. 1. Lateral left common carotid arteriogram. Upper Left." Early arterial phase shows markedly enlarged anterior cerebral artery (black arrow) from its carotid origin. Note large middle cerebral artery branches and distortion of normal Sylvian configuration. The temporal branches are mysteriously spared (open arrows). Upper Right: Later arterial phase shows striking prominence of the pericallosal artery (arrow). Lower Left." At end of arterial phase no early draining veins or capillary aggregates are seen. Venous phase (not shown) was completely normal. began with right arm weakness and progressed to the face and palate causing speech difficulty. Within 10 minutes, retroorbital tension and burning pain occurred followed by a severe bilateral, pounding headache that was disabling for several hours. He had not been evaluated for his migraine previously and had been placed on medications only recently. He had taken chlorpheniramine maleate (Chlortrimeton) and Cafergot at the onset of migraine prodromata and had twice aborted an attack. Similar attacks of hemiplegic migraine were experienced by his mother throughout her life but no other incidence of migraine or seizure was present in the family. Other medical history, and physical and neurological examinations were negative. There were no cranial bruits. Skull x-ray films and electroencephalogram were normal. Lumbar puncture did not suggest subarachnoid hemorrhage and cerebospinal fluid examina510 tion was normal. Brain scan showed diffuse, but definitely increased activity in the left frontal lobe with most prominence in the anterior cerebral distribution. Bilateral common carotid and left vertebral angiograms were performed (Fig. 1) because of the recent seizure and positive brain scan. Right carotid and vertebrobasilar systems were completely normal including both posterior cerebral arteries. A diffuse luminal enlargement of profound degree began at the left internal carotid artery bifurcation and involved selectively the entire anterior cerebral artery and most branches of the middle cerebral artery (Fig. 2). No unusual capillary staining was present and the cortical and deep venous configuration of the left cerebral hemisphere was completely normal. No mass effect was present. The patient has remained free of seizures on diphenylhydantoin (Dilantin) and phenobarbital during the three years of follow-up. J. Neurosurg. / Volume 44 / April, 1976 Cerebral arterial dolichoeetasia FI~. 2. Left: Left common carotid arteriogram, anteroposterior view, shows normal-sized supraclinoid internal carotid and proximal middle cerebral arteries. Note enlargement in anterior cerebral artery more marked above anterior communicating level. Note proximal rightward positioning. Right: Right carotid injection shows completely normal right-sided arterial pattern except for cross-filling in left anterior cerebral artery (arrow). Vertebrobasilar system (not shown) was also normal. He has remained on active duty in the Air Force. Discussion Although the angiographic findings in this case suggest a vascular malformation, a purely arterial malformation has never been verified angiographically. I,~,5 7 If, as Bergstrand, et al., 1 claimed, pure arterial angiomas do not exist, the dilated cerebral arteries in our patient would have to have resulted from some acquired process. The appearance most closely resembles that of arterial dolichoectasia, although the unilaterality, luminal uniformity, and cerebral hemispheric location are atypical. Patients with dolichoectasia have not been reported to present with seizures but characteristically have shown cranial nerve deficits, 8,9 and occasionally hydrocephalus, a findings which suggest the presence of an underlying intracranial tumor. The cause of dolichoectasia has not been established. Histological changes occur in the elastic tissue and the muscle fibers of the media. Arteriosclerosis when present is believed to be incidental. Examples of the entity are reported in children 8 and are unJ. Neurosurg. / Volume 44 / April, 1976 associated with arteriosclerosis. Because other diseases may cause ectatic arterial change, syphilis, arteriosclerosis, Ehlers-Danlos syndrome, pseudoxanthoma elasticum, and nonsyphilitic vasculitis should be considered in the gamut of possibilities. Acknowledgments The authors are grateful to Brenda Holden for the manuscript preparation and to James Simmons for the photography. References 1. Bergstrand H, Olivecrona H, TSnnis W: Gefiissmissbildungen und Ger~issgeschwiiistedes Gehirns. Leipzig: Georg Thieme, 1936 2. Brasch F, quoted by Wyburn-Mason R: The Vascular Abnormalities and Tumors of the Spinal Cord and Its Membranes. London: Henry Kimpton, 1943 3. Breig A, Ekbom K, Greitz T, et al: Hydrocephalus due to elongated basilar artery. A new clinicoradiological syndrome. Lancet 1:874-875, 1967 4. Krayenbiihl HA, Ya~argil MG: Cerebral Angiography. Philadelphia: JB Lippincott, 1968 5. Krayenbiihl HA, Yaw MG: Das Hirnaneurysma. Basel: JR Geigy, 1958 511 J. R. Thompson, P. R. Weinstein and C. R. Simmons 6. Russell DS, Rubenstein LJ: Pathology of Tumors of the Nervous System. London: Edward Arnold, 1959 7. Sacks JG, Lindenburg R: Dolicho-ectatic intracranial arteries: symptomatology and pathogenesis of arterial elongation and distension. Johns Hopkins Med J 125:95-106, 1969 8. Wallace S, Jaffe ME: Cerebral arterial ectasia with saccular aneurysms. Radiology 88:90-93, 1967 512 9. Wyburn-Mason R: The Vascular Abnormalities and Tumors of the Spinal Cord and its Membranes. London: Henry Kimpton, 1943 Address reprint requests to: Joseph R. Thompson, M.D., Department of Radiology, Loma Linda University Medical Center, Loma Linda, California 92354. J. Neurosurg. / Volume 44 / April, 1976