Alpha-1-Antitrypsin Deficiency-Associated Cervical Artery Dissection: Report of Three Cases

Pezzini, Alessandro; Magoni, Mauro; Corda, Luciano; Pini, Lara; Medicina, Daniela; Crispino, M.; Pavia, Marco; Padovani, Alessandro; Grassi, Vittorio

doi:10.1159/000057899

Cited by 27 publications

(14 citation statements)

References 19 publications

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“…Major and minor cervical trauma [13][14][15][16][17] Arterial hypertension [18][19][20] Young age 12 Current use of oral contraceptives 21 Migraine [22][23][24] Fibromuscular dysplasia 11,[25][26][27][28][29][30][31][32] Ultrastructural connective tissue abnormalities 33 Vascular subtype of Ehlers-Danlos syndrome 34 Marfan syndrome 18,35 Turner syndrome 19,36 Williams syndrome 37 Familial cases 18,38 Hereditary hemochromatosis 39 Osteogenesis imperfecta type I 40 α1-Antitrypsin deficiency 41,42 677T genotype MTHFR [43][44][45] Hyperhomocysteinemia 46 Cystic medial necrosis of intracranial vessels 47 Styloid process length 48 ICAM-1 E4690 K gene polymorphism 49 Autosomal-dominant polycystic kidney disease 50 Infections [51][52][53][54] Moyamoya disease …”

Section: Table 1 Factors Associated With CDmentioning

confidence: 99%

Cervical Arterial Dissections and Association With Cervical Manipulative Therapy

Biller¹,

Sacco²,

Albuquerque³

et al. 2014

Stroke

175

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3155Purpose-Cervical artery dissections (CDs) are among the most common causes of stroke in young and middle-aged adults.The aim of this scientific statement is to review the current state of evidence on the diagnosis and management of CDs and their statistical association with cervical manipulative therapy (CMT). In some forms of CMT, a high or low amplitude thrust is applied to the cervical spine by a healthcare professional. Methods-Members of the writing group were appointed by the American Heart Association Stroke Council's Scientific Statements Oversight Committee and the American Heart Association's Manuscript Oversight Committee. Members were assigned topics relevant to their areas of expertise and reviewed appropriate literature, references to published clinical and epidemiology studies, morbidity and mortality reports, clinical and public health guidelines, authoritative statements, personal files, and expert opinion to summarize existing evidence and to indicate gaps in current knowledge. Results-Patients with CD may present with unilateral headaches, posterior cervical pain, or cerebral or retinal ischemia (transient ischemic or strokes) attributable mainly to artery-artery embolism, CD cranial nerve palsies, oculosympathetic palsy, or pulsatile tinnitus. Diagnosis of CD depends on a thorough history, physical examination, and targeted ancillary investigations. Although the role of trivial trauma is debatable, mechanical forces can lead to intimal injuries of the vertebral arteries and internal carotid arteries and result in CD. Disability levels vary among CD patients with many having good outcomes, but serious neurological sequelae can occur. No evidence-based guidelines are currently available to endorse best management strategies for CDs. Antiplatelet and anticoagulant treatments are both used for prevention of local thrombus and secondary embolism. Case-control and other articles have suggested an epidemiologic association between CD, particularly vertebral artery dissection, and CMT. It is unclear whether this is due to lack of recognition of preexisting CD in these patients or due to trauma caused by CMT. Ultrasonography, computed tomographic angiography, and magnetic resonance imaging with magnetic resonance angiography are useful in the diagnosis of CD. Follow-up neuroimaging is preferentially done with noninvasive modalities, but we suggest that no single test should be seen as the gold standard.

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Section: Table 1 Factors Associated With CDmentioning

confidence: 99%

Cervical Arterial Dissections and Association With Cervical Manipulative Therapy

Biller¹,

Sacco²,

Albuquerque³

et al. 2014

Stroke

175

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“…However, our results are in the same direction as previous case reports in which an association between deficient protease inhibitor phenotypes (PIZ and PIS) of A1-AT and the presence of sCAD was observed. 4,5,7,8 An association between age and A1-AT levels was found in all subjects, suggesting that levels of A1-AT, like other acute-phase proteins, increase with age. Because sCAD cases were younger than controls, it is possible to speculate that low levels of A1-AT in sCAD reflect their younger age.…”

Section: Discussionmentioning

confidence: 85%

Levels of α ₁ -Antitrypsin in Plasma and Risk of Spontaneous Cervical Artery Dissections

Vila

Millán

Ferrer

et al. 2003

Stroke

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Background and Purpose-Abnormalities of dermal connective tissue have been detected in patients with spontaneous cervical artery dissections (sCAD), suggesting an underlying structural defect of the arterial wall. ␣ 1 -Antitrypsin (A1-AT) is a circulating serine proteinase inhibitor of proteolytic enzymes that helps to maintain the integrity of elastic and collagen fibers. Methods-To test the hypothesis that moderate deficiency of A1-AT may be a risk factor for sCAD, 22 cases with sCAD and 113 controls were included in the study. Key Words: alpha 1-antitrypsin Ⅲ aneurysm, dissecting Ⅲ risk factors G enetic and environmental factors have been proposed in the pathogenesis of spontaneous cervical artery dissections (sCAD). 1 Abnormalities of dermal connective tissue have been detected in most patients with sCAD, suggesting an underlying structural defect of the arterial wall. 2 In such patients, damaged collagen and elastic fibers might predispose to arterial wall rupture in points of weakness after mechanical stress. 1,2 The presence and intensity of vascular risk factors (smoking, homocysteine, hypertension, oral contraceptives, infections, etc) might explain the sCAD cluster at about the middle of life despite the congenital structural defect of the vessel. Genetically determined alterations of the extracellular matrix may be an important field to investigate the genesis of sCAD. 2,3 ␣ 1 -Antitrypsin (A1-AT) is a circulating serine proteinase inhibitor of proteolytic enzymes that contributes to the maintenance of the integrity of connective tissues. Deficiency of A1-AT may result in degradation of the arterial wall through inadequate protection against the proteolytic effect of elastase and collagenase. 4,5 A1-AT deficiency is a genetic and systemic disorder characterized not only by lung and liver disease but also by vascular manifestations, including sCAD, fibromuscular dysplasia, or aneurysms. 4,5 However, A1-AT deficiency ranges from severe forms with very low circulating levels (10% of normal) in homozygous patients to other forms with moderately low concentrations (60% to 70% of normal) in heterozygous patients. 4,5 To evaluate whether moderate deficiency of A1-AT may be a risk factor for sCAD, we performed a prospective case-control study comparing plasma levels of A1-AT in consecutive patients with sCAD and controls. Results-Patients MethodsWe examined 22 consecutive patients with stroke and sCAD confirmed by conventional angiography or MR angiography and cervical MRI. Controls (nϭ113) were stroke patients without sCAD admitted during the same period of time who had complete neurovascular studies (carotid ultrasounds, MR angiography, or conventional arteriography).We recorded demographic data and presence of vascular risk factors. A blood sample was taken at least 2 weeks after stroke onset in cases and controls to avoid the effect of the acute-phase response on plasma protein levels. A1-AT determinations were performed at the hospital laboratory following a standard immune nephelometric technique. Estab...

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“…Occasionally, however, the cardiovascular system is involved. Abdominal aortic aneurysm,1 spontaneous dissection of the thoracic aorta,2 coronary artery dissection,3 intracerebral aneurysms4 and cervical artery dissection5 have all been reported as concomitant vascular adverse events. Despite the pathogenesis of the combination of aneurysm and vascular dissection in the presence of AATD being not well established, the reported proportion of mortality accounted for cerebrovascular causes in AATD patients is about 5% 6…”

Section: Introductionmentioning

confidence: 99%

Elastic properties of the ascending aorta in patients with α1-antitrypsin deficiency (Z homozygotes)

Vizzardi

Corda

Pezzali

et al. 2012

Heart

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Alpha-1-Antitrypsin Deficiency-Associated Cervical Artery Dissection: Report of Three Cases

Cited by 27 publications

References 19 publications

Cervical Arterial Dissections and Association With Cervical Manipulative Therapy

Cervical Arterial Dissections and Association With Cervical Manipulative Therapy

Levels of α ₁ -Antitrypsin in Plasma and Risk of Spontaneous Cervical Artery Dissections

Elastic properties of the ascending aorta in patients with α1-antitrypsin deficiency (Z homozygotes)

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Alpha-1-Antitrypsin Deficiency-Associated Cervical Artery Dissection: Report of Three Cases

Cited by 27 publications

References 19 publications

Cervical Arterial Dissections and Association With Cervical Manipulative Therapy

Cervical Arterial Dissections and Association With Cervical Manipulative Therapy

Levels of α 1 -Antitrypsin in Plasma and Risk of Spontaneous Cervical Artery Dissections

Elastic properties of the ascending aorta in patients with α1-antitrypsin deficiency (Z homozygotes)

Contact Info

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Levels of α ₁ -Antitrypsin in Plasma and Risk of Spontaneous Cervical Artery Dissections