Vertebrobasilar dilatative arteriopathy (dolichoectasia)
Min Lou
Department of Neurology, the Second Affiliated Hospital of Zhejiang University, School of Medicine, Hangzhou, China.
Search for more papers by this authorLouis R. Caplan
Department of Neurology, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Massachusetts, USA
Search for more papers by this authorMin Lou
Department of Neurology, the Second Affiliated Hospital of Zhejiang University, School of Medicine, Hangzhou, China.
Search for more papers by this authorLouis R. Caplan
Department of Neurology, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Massachusetts, USA
Search for more papers by this authorAbstract
Dolichoectasia (dilatative arteriopathy) describes marked elongation, widening, and tortuosity of arteries. The intracranial vertebral and basilar arteries are preferentially involved. Dolichoectatic arteries usually have an abnormally large external diameter and a thin arterial wall, with degeneration of the internal elastic lamina, multiple gaps in the internal elastica, thinning of the media secondary to reticular fiber deficiency, and smooth muscle atrophy. The most important clinical presentations of dilatative arteriopathy include acute brain ischemia; a progressive course related to compression of cranial nerves, the brain stem, or the third ventricle; and catastrophic outcome caused by vascular rupture. Flow in dilated arteries can become bidirectional, resulting in reduced antegrade flow and thrombus formation. Elongation and angulation of arteries can stretch and distort the orifices of arterial branches, leading to decreased blood flow, especially in penetrating branches.
References
- 1 Caplan, L.R. 2005. Dilatative arteriopathy (dolichoectasia): What is known and not known. Ann. Neurol. 57: 469–471.
- 2 Smoker, W.R., M.J. Price, W.D. Keyes, et al . 1986. High-resolution computed tomography of the basilar artery, 1: normal size and position. Am. J. Neuroradiol. 7: 55–60.
- 3 Pico, F., J. Labreuche, A. Cohen, et al . 2004. Intracranial arterial dolichoectasia is associated with enlarged descending thoracic aorta. Neurology 63: 2016–2021.
- 4 Dziewas, R., M. Freund, P. Ludemann, et al . 2003. Treatment options in vertebrobasilar dolichoectasia: case report and review of the literature. Eur. Neurol. 49: 245–247.
- 5 Yu, Y.L., I.F. Moseley, P. Pullicino & W.I. McDonald. 1982. The clinical picture of ectasia of the intracerebral arteries. J. Neurol. Neurosurg. Psychiatry 45: 29–36.
- 6 Pico, F., J. Labreuche, P.J. Touboul & P. Amarenco. 2003. Intracranial arterial dolichoectasia and its association with carotid atherosclerosis and stroke subtype. Neurology 61: 1736–1742.
- 7 Smoker, W.R., J.J. Corbett, L.R. Gentry, et al . 1986. High-resolution computed tomography of the basilar artery, 2: vertebrobasilar dolichoectasia: clinical-pathologic correlation and review. Am. J. Neuroradiol. 7: 61–72.
- 8 Ubogu, E.E. & O.O. Zaidat. 2004. Vertebrobasilar dolichoectasia diagnosed by magnetic resonance angiography and risk of stroke and death: a cohort study. J. Neurol. Neurosurg. Psychiatry 75: 22–26.
- 9 Ionita, C.C., A.R. Xavier, J. Farkas & P. Pullicino. 2004. Intracranial arterial dolichoectasia and its relation with atherosclerosis and stroke subtype. Neurology 63: 596; author reply 596.
- 10 Sacks, J.G. & R. Lindenburg. 1969. Dolicho-ectatic intracranial arteries: symptomatology and pathogenesis of arterial elongation and distention. Johns Hopkins Med. J. 125: 95–106.
- 11 Gautier, J.C., J.J. Hauw, A. Awada, et al . 1988. Dolichoectatic intracranial arteries. Association with aneurysms of the abdominal aorta. Rev. Neurol. (Paris) 144: 437–446.
- 12 Hegedüs, K. 1985. Ectasia of the basilar artery with special reference to possible pathogenesis. Surg. Neurol. 24: 463–469.
- 13 Hirsch, C.S. & U. Roessmann. 1975. Arterial dysplasia with ruptured basilar artery aneurysm: report of a case. Hum. Pathol. 6: 749–758.
- 14 Passero, S. & G. Filosomi. 1998. Posterior circulation infarcts in patients with vertebrobasilar dolichoectasia. Stroke 29: 653–659.
- 15 Pico, F., J. Labreuche, D. Seilhean, et al . 2007. Association of small-vessel disease with dilatative arteriopathy of the brain: neuropathologic evidence. Stroke 38: 1197–1202.
- 16 Pico, F., Y. Biron, M.G. Bousser & P. Amarenco. 2005. Concurrent dolichoectasia of basilar and coronary arteries. Neurology 65: 1503–1504.
- 17 Lamblin, N., C. Bauters, X. Hermant, et al . 2002. Polymorphisms in the promoter regions of MMP-2, MMP-3, MMP-9 and MMP-12 genes as determinants of aneurysmal coronary artery disease. J. Am. Coll. Cardiol. 40: 43–48.
- 18 Silverman, I.E., D.M. Berman, G.L. Dike, et al . 2000. Vertebrobasilar dolichoectasia associated with Marfan syndrome. J. Stroke Cerebrovasc. Dis. 9: 196–198.
- 19 Zambrino, C.A., A. Berardinelli, A. Martelli, et al . 1999. Dolichovertebrobasilar abnormality and migraine-like attacks. Eur. Neurol. 41: 10–14.
- 20 Mitsias, P. & S.R. Levine. 1996. Cerebrovascular complications of Fabry's disease. Ann. Neurol. 40: 8–17.
- 21 Grant Steen, R., J.W. Langston, R.J. Ogg, et al . 1998. Ectasia of the basilar artery in children with sickle cell disease: relationship to hematocrit and psychometric measures. J. Stroke Cerebrovasc. Dis. 7: 32–43.
- 22 Gavazzi, C., W. Borsini, L. Guerrini, et al . 2006. Subcortical damage and cortical functional changes in men and women with Fabry disease: a multifaceted MR study. Radiology 241: 492–500.
- 23 Garzuly, F., L. Maródi, M. Erdös, et al . 2005. Megadolichobasilar anomaly with thrombosis in a family with Fabry's disease and a novel mutation in the alpha-galactosidase A gene. Brain 128(Pt 9): 2078–2083.
- 24 Schievink, W.I., V.E. Torres, D.O. Wiebers & J. Huston, III. 1997. Intracranial arterial dolichoectasia in autosomal dominant polycystic kidney disease. J. Am. Soc. Nephrol. 8: 1298–1303.
- 25 Graf, S., A. Schischma, K.E. Eberhardt, et al . 2002. Intracranial aneurysms and dolichoectasia in autosomal dominant polycystic kidney disease. Nephrol. Dial. Transplant. 17: 819–823.
- 26 Rautenberg, W., A. Aulich, Rother, et al . 1992. Stroke and dolichoectatic intracranial arteries. Neurol. Res. 14(Suppl 2): 201–203.
- 27 Levine, R.L., P.A. Turski, T.M. Grist. 1995. Basilar artery dolichoectasia: Review of the literature and six patients studied with magnetic resonance angiography. J. Neuroimaging 5: 164–170.
- 28 Passero, S.G. & S. Rossi. 2008. Natural history of vertebrobasilar dolichoectasia. Neurology 70: 66–72.
- 29 Ince, B., G.W. Petty, R.D. Brown, Jr., et al . 1998. Dolichoectasia of the intracranial arteries in patients with first ischemic stroke: a population-based study. Neurology 50: 1694–1698.
- 30 Hennerici, M., W. Rautenberg & A. Schwartz. 1987. Transcranial Doppler ultrasound for the assessment of intracranial arterial flow velocity. II. Evaluation of intracranial arterial disease. Surg. Neurol. 27: 523–532.
- 31 Schwartz, A., W. Rautenberg & M. Hennerici. 1993. Dolichoectatic intracranial arteries: review of selected aspects. Cerebrovasc. Dis. 5: 273–279.
- 32 Kumral, E., A. Kisabay, C. Ataç, et al . 2005. The mechanism of ischemic stroke in patients with dolichoectatic basilar artery. Eur. J. Neurol. 12: 437–444.
- 33 Welsh, L.W., J.J. Welsh & B. Lewin. 2000. Basilar artery and vertigo. Ann. Otol. Rhinol. Laryngol. 109: 615–622.
- 34 Nakatomi, H., H. Segawa, A. Kurata, et al . 2000. Clinicopathological study of intracranial fusiform and dolichoectatic aneurysms: insight on the mechanism of growth. Stroke 31: 896–900.
- 35 Pico, F., J. Labreuche, P.-J. Touboul, et al . 2005. Intracranial arterial dolichoectasia and small vessel disease in stroke patients. Ann. Neurol. 57: 472–479.
- 36 Rabb, C.H. & S.L. Barnwell. 1998. Catastrophic subarachnoid hemorrhage resulting from ruptured vertebrobasilar dolichoectasia: case report. Neurosurgery 42: 379–382.
- 37 Flemming, K.D., K. Josephs & E.F. Wijdicks. 2000. Enlarging vertebrobasilar dolichoectasia with subarachnoid hemorrhage heralded by recurrent ischemia. Case illustration. J. Neurosurg. 92: 504.
- 38 Passero, S.G., B. Calchetti & S. Bartalini. 2005. Intracranial bleeding in patients with vertebrobasilar dolichoectasia. Stroke 36: 1421–1425.
- 39 Peterson, N.T., P.M. Duchesneau, E.L. Westbrook & M.A. Weinstein. 1977. Basilar artery ectasia demonstrated by computed tomography. Radiology 122: 713–715.
- 40 He, J., P.K. Whelton, B. Vu & M.J. Klag. 1998. Aspirin and risk of hemorrhagic stroke. A meta-analysis of randomized controlled trials. JAMA 280: 1930–1935.
- 41 Saloheimo, P., S. Juvela & M. Hillbom. 2001. Use of aspirin, epistaxis, and untreated hypertension as risk factors for primary intracerebral hemorrhage in middle-aged and elderly people. Stroke 32: 399–404.
- 42 De Georgia, M., J. Belden, L. Pao, et al . 1999. Thrombus in vertebrobasilar dolichoectatic artery treated with intravenous urokinase. Cerebrovasc. Dis. 9: 28–33.
- 43 Rahman, E.A., J.D. Trobe & S.S. Gebarski. 2002. Hemifacial spasm caused by vertebral artery dolichoectasia. Am. J. Ophthalmol. 133: 854–856.
- 44 Barker, F.G. II, P.J. Jannetta, D.J. Bissonette, et al . 1995. Microvascular decompression for hemifacial spasm. J. Neurosurg. 82: 201–210.
- 45 Love, S., D.A. Hilton & H.B. Coakham. 1998. Central demyelination of the Vth nerve root in trigeminal neuralgia associated with vascular compression. Brain Pathol. 8: 1–11.
- 46 Lye, R.H. 1986. Basilar artery ectasia: an unusual cause of trigeminal neuralgia. J. Neurol. Neurosurg. Psychiatry 49: 22–28.
- 47 Kasner, S.E., G.T. Liu & S.L. Galetta. 1997. Neuro-ophthalmologic aspects of aneurysms. Neuroimag. Clin. N. Am. 7: 679–692.
- 48 Goldenberg-Cohen, N. & N.R. Miller. 2004. Noninvasive neuroimaging of basilar artery dolichoectasia in a patient with an isolated abducens nerve paresis. Am. J. Ophthalmol. 137: 365–367.
- 49 Zhu, Y., K. Thulborn, K. Curnyn, et al . 2005. Sixth cranial nerve palsy caused by compression from a dolichoectatic vertebral artery. J. Neuroophthalmol. 25: 134–135.
- 50 Guirgis, M.F., B.L. Lam & S.F. Falcone. 2001. Optic tract compression from dolichoectatic basilar artery. Am. J. Ophthalmol. 132: 283–286.
- 51 Castelnovo, G., L. Jomir, A. Le Bayon, et al . 2003. Lingual atrophy and dolichoectatic artery. Neurology 61: 1121.
- 52 Staikov, I.N. & H.P. Mattle. 1994. Vertebrobasilar dolichoectasia and exertional headache. J. Neurol. Neurosurg. Psychiatry 57: 1544.
- 53 Savitz, S.I., M. Ronthal & L.R. Caplan. 2006. Vertebral artery compression of the medulla. Arch. Neurol. 63: 234–241.
- 54 Siddiqui, A., N.S. Chew & K. Miszkiel. 2008. Vertebrobasilar dolichoectasia: a rare cause of obstructive hydrocephalus: case report. Br. J. Radiol. 81: e123–e126.
- 55 Ekbom, K., T. Greitz & E. Kugelberg. 1969. Hydrocephalus due to ectasia of the basilar artery. J. Neurol. Sci. 8: 465–477.
- 56 Milandre, L., B. Bonnefoi, P. Pestre, et al . 1991. Vertebrobasilar arterial dolichoectasia. Complications and prognosis. Rev. Neurol. (Paris) 147: 714–722.
- 57 Laforêt, P., P. Petiot, M. Nicolino, et al . 2008. Dilative arteriopathy and basilar artery dolichoectasia complicating late-onset Pompe disease. Neurology 70: 2063–2066.
- 58 Breig, A., K. Ekbom, T. Greitz, et al . 1967. Hydrocephalus due to elongated basilar artery. A new clinicoradiological syndrome. Lancet 1: 874–875.