Cervical arterial dissection: clinical characteristics in a neurology service in São Paulo, Brazil.

Cervical arterial dissection accounts for only a small proportion of ischemic stroke but arouses scientific interest due to its wide clinical variability. OBJECTIVE This study aimed to evaluate its risk factors, outline its clinical characteristics, compare treatment with antiaggregation or anticoagulation, and explore the prognosis of patients with cervical arterial dissection. METHODS An observational, retrospective study using data from medical records on patients with cervical arterial dissection between January 2010 and August 2015. RESULTS The total number of patients was 41. The patients' ages ranged from 19 to 75 years, with an average of 44.5 years. The most common risk factor was smoking. Antiaggregation was used in the majority of patients (65.5%); 43% of all patients recanalized in six months, more frequently in patients who had received anticoagulation (p = 0.04). CONCLUSION The presence of atherosclerotic disease is considered rare in patients with cervical arterial dissection; however, our study found a high frequency of hypertension, smoking and dyslipidemia. The choice of antithrombotic remains controversial and will depend on the judgment of the medical professional; the clinical results with anticoagulation or antiaggregation were similar but there was more recanalization in the group treated with anticoagulation; its course was favorable in both situations. The recurrence of cervical arterial dissection and stroke is considered a rare event and its course is favorable.

Cervical arterial dissection: clinical characteristics in a neurology service in São Paulo, Brazil / Dissecção arterial cervical: características clínicas em um serviço de neurologia de São Paulo, Brasil

ABSTRACT Cervical arterial dissection accounts for only a small proportion of ischemic stroke but arouses scientific interest due to its wide clinical variability. Objective: This study aimed to evaluate its risk factors, outline its clinical characteristics, compare treatment with antiaggregation or anticoagulation, and explore the prognosis of patients with cervical arterial dissection. Methods: An observational, retrospective study using data from medical records on patients with cervical arterial dissection between January 2010 and August 2015. Results: The total number of patients was 41. The patients' ages ranged from 19 to 75 years, with an average of 44.5 years. The most common risk factor was smoking. Antiaggregation was used in the majority of patients (65.5%); 43% of all patients recanalized in six months, more frequently in patients who had received anticoagulation (p = 0.04). Conclusion: The presence of atherosclerotic disease is considered rare in patients with cervical arterial dissection; however, our study found a high frequency of hypertension, smoking and dyslipidemia. The choice of antithrombotic remains controversial and will depend on the judgment of the medical professional; the clinical results with anticoagulation or antiaggregation were similar but there was more recanalization in the group treated with anticoagulation; its course was favorable in both situations. The recurrence of cervical arterial dissection and stroke is considered a rare event and its course is favorable.

RESUMO As dissecções arterais cervicais correspondem somente a uma pequena proporção dos casos de acidente vascular cerebral (AVC) isquêmico, mas despertam interesse científico devido à sua alta variabilidade clínica. Objetivos: Este estudo destina-se a avaliar os fatores de risco, desfechos clínicos, comparar o tratamento com anticoagulação e antiagregação, e avaliar o prognóstico desses pacientes. Métodos: Estudo observacional, retrospectivo utilizando dados de prontuários de pacientes com dissecção arterial cervical entre os períodos de janeiro de 2010 e agosto de 2015. Resultados: O número de pacientes foi 41. A idade foi de 19 a 75 anos, com idade média de 44,5 anos. O fator de risco mais comum encontrado foi o tabagismo. Antiagregação foi utilizada na maioria dos pacientes (65,5%); 43% dos pacientes apresentaram recanalização em seis meses, sendo esta mais frequentemente observada nos pacientes que receberam anticoagulação (p = 0,04). Conclusão: A presença de doença aterosclerótica é considerada rara em pacientes com dissecção arterial cervical. Entretanto, nosso estudo encontrou alta frequência de hipertensão arterial, tabagismo e dislipidemia. A escolha pela terapia antitrombótica permanece controversa e dependerá do julgamento clínico do médico; os resultados clínicos com anticoagulação ou antiagregação foram similares, mas houve maior taxa de recanalização no grupo tratado com anticoagulação. A recorrência de dissecação arterial cervical e AVC foi considerada rara e o curso, favorável.

A Unique Recurrent Stroke Case due to Bilateral Vertebral Artery Dissection with Familial Hirschsprung Disease.

Vertebral artery (VA) dissection is one major cause of brain infarction in young and middle-aged adults. Risk factors for VA dissection are hypertension, diabetes mellitus, hyperlipidemia, trauma, and genetic factors. A 32-year-old man with familial Hirschsprung disease at the age of 2 presented cerebellar ischemic stroke due to bilateral VA dissections. A stroke recurred within 17 days despite oral dual antiplatelet therapy. Bilateral VA dissections and recurrent dissections are related to genetic mutations associated with connective tissue diseases. A part of familial Hirschsprung disease has genetic factors in common with cerebrovascular disease. There may be a common genetic background between his VA dissection and Hirschsprung disease.

Congenital anomaly of the posterior arch of the atlas: a rare risk factor for posterior circulation stroke.

We report an extremely rare case involving a posterior arch anomaly of the atlas, causing a vertebral artery dissection (VAD) induced posterior circulation stroke. A 16-year-old girl was admitted to hospital because of new onset dizziness. VAD related multiple infarction in the posterior circulation was revealed. The congenital posterior arch anomaly of the atlas, along with instability of the atlantoaxial joint, were discovered accidentally during follow-up. This is the first case of ischemic stroke related to atlantal posterior arch aplasia and atlantoaxial instability. Although rare, it reminds us that investigation of the craniovertebral junction should be considered when stroke occurs in young patients.

[Dissection of the cervical arteries]. / Dissektion af halskar.

Dissection of the cervical arteries is an important cause of stroke in young adults. A haematoma in the wall of the cervical vessel leads to stenosis or occlusion and thereby risk of stroke. The most frequent local symptoms in carotid-artery dissection are head- and neck pain accompanied by Horner's syndrome, while typical symptoms in vertebral-artery dissection are pain in the back of the neck and head. The mural haematoma is best visualized by magnetic resonance imaging. Antithrombotic versus anticoagulation treatment to prevent stroke have recently shown to be equally effective.

Aspirin versus anticoagulation in intra- and extracranial vertebral artery dissection.

BACKGROUND AND PURPOSE: To evaluate the incidence and predictors of ischaemic recurrent stroke and the adverse events of antithrombotic therapy in patients with first intra- or extracranial vertebral artery dissection (VAD) who were treated with aspirin or oral anticoagulation (OA). METHODS: A 21-year database of consecutive patients with confirmed diagnoses of VAD (n = 110, 63% men; mean age 37.9 ± 8.5 years) without intracerebral hemorrhage and who were treated with aspirin or OA were analyzed retrospectively. In all cases, the admission diagnosis was ischaemic stroke. Three groups were defined according to the site of the dissection: (i) extracranial, (ii) intracranial, and (iii) intra-/extracranial. Clinical follow-up was obtained by neurologic examination. Outcome measures were (i) recurrent ischaemic events (ischaemic stroke or transient ischaemic attack) and (ii) intra- and extracranial major bleeding. RESULTS: No difference in age, smoking, or hypertension was found between patients treated with OA (n = 49) and those treated with aspirin (n = 50). Extracranial artery dissection (49%) had preponderance over intracranial (27%) or intra-/extracranial (23%) location. During the follow-up, recurrent ischaemic events were rare (one case). There were no bleeding complications. The treatment that was used did not influence the functional outcome or recanalization. A good functional outcome (modified Rankin score ≤ 2) was observed in 82 patients. CONCLUSIONS: Although this was a non-randomized study, our data suggest that the frequency of recurrent ischaemic stroke in patients with intra- or extracranial VAD is low and most likely independent of the type of antithrombotic treatment.

Triple and quadruple spontaneous cervical artery dissection: presenting characteristics and long-term outcome.

BACKGROUND: Spontaneous cervicocephalic artery dissection (sCAD) of more than two cervical arteries is rare. PATIENTS AND METHODS: Vascular and potential sCAD risk factors, triggering events, clinical and neuroimaging findings, and outcome of patients with multiple sCAD were studied. Patients were drawn from prospective hospital-based sCAD registries. RESULTS: Of 740 consecutive patients with sCAD, 11 (1.5%) had three, and one had four (0.1%) sCAD. Eight of these 12 patients were women. One patient had additional dissections of the celiac trunk and hepatic artery. Vascular risk factors included hypertension (n = 1), hypercholesterolaemia (n = 6), current smoking (n = 5) and migraine (n = 6). No patient had a family history of sCAD, fibromuscular dysplasia (FMD) or connective tissue disease. SCAD was preceded by a minor trauma in five and infection in four patients. Clinical manifestations included ischaemic stroke (n = 8), transient ischaemic attack (n = 3), headache (n = 9), neck pain (n = 4), Horner syndrome (n = 5), pulsatile tinnitus (n = 2) and dysgeusia (n = 1). Brain MRI revealed ischaemic infarcts that affected one vessel territory in seven and two territories in two patients. The 3-month outcome was favourable (modified Rankin scale score 0-1) in 10 patients (83%). No new recurrent stroke or sCAD occurred during a mean follow-up of 50 (SD 29) months. CONCLUSION: Multiple sCAD occurred preferentially in women and caused clinical symptoms and signs mainly in one vascular territory. In none of the patients was FMD or any other underlying arteriopathy apparent. The majority of multiple sCAD was preceded by a minor trauma or infection. Clinical outcome was favourable in most patients, and long-term prognosis benign. The data suggest that transient vasculopathy may be a major mechanism for multiple sCAD.

Conservative treatment of ruptured vertebrobasilar dissecting aneurysm.

Ruptured vertebrobasilar dissecting aneurysm is usually treated surgically because rebleeding negatively affects outcome. However, the risk of rebleeding decreases markedly once several hours have passed from the initial bleeding. Moreover, surgery-related complications are not rare. We describe seven patients with ruptured vertebrobasilar dissecting aneurysm. To prevent rebleeding during the acute stage, we treated all seven patients conservatively with fentanyl instead of emergency surgery. During the follow-up period (mean 20 months), no patient suffered rebleeding. Conservative treatment with fentanyl administration may be a good option for management of ruptured vertebrobasilar dissecting aneurysm during the acute stage.

[Spontaneous carotid and vertebral arteries dissection in a multiethnic population]. / Dissecção espontânea das artérias carótidas e vertebrais em uma população multiétnica.

Spontaneous dissection of the carotid and vertebral arteries (SDCVA) is considered a rare cause of stroke, particularly in countries with multiethnic population. The objective was to evaluate the clinical and neuroimaging features of patients with SDCVA from a multiethnic population. Sixty-six patients diagnosed with SDCVA were studied at two tertiary hospitals at São Paulo. An initial questionnaire was completed and patients were followed prospectively. Among the patients studied, 82% were caucasian, 53% were male and the average age was 41.7 years old. The most frequent cardiovascular risk factors found were systemic hypertension and tobacco use. Other aspects evaluated were history of previous migraine, initial treatment and prognosis. In conclusion, although the population studied was multiethnic, there was a marked predominance of caucasians. The analysis of clinical and neuroimaging data from patients with SDCVA allows a better understanding of the disease, leading to an earlier diagnosis and more appropriate treatment.

Dissecção espontânea das artérias carótidas e vertebrais em uma população multiétnica / Spontaneous carotid and vertebral arteries dissection in a multiethnic population

A dissecção espontânea das artérias carótidas e vertebrais (DEACV) é considerada uma causa rara de acidente vascular cerebral, particularmente em países com população multiétnica. O objetivo desse estudo foi avaliar características clínicas e de neuroimagem dos pacientes com DEACV em uma população multiétnica. Foram estudados 66 pacientes com diagnóstico de DEACV em dois hospitais terciários de São Paulo. Aplicou-se um questionário inicial e os pacientes foram seguidos prospectivamente. Dos pacientes estudados, 82 por cento eram brancos, 53 por cento eram homens e a média de idade foi 41,7 anos. Os fatores de risco cardiovasculares mais freqüentes foram hipertensão arterial e tabagismo. Outros aspectos avaliados foram história prévia de enxaqueca, tratamento inicial e prognóstico. Concluiu-se que apesar da população estudada ser multiétnica, houve um marcante predomínio de brancos. A análise das características clínicas e de neuroimagem dos pacientes com DEACV possibilita um melhor conhecimento da doença, levando a um diagnóstico precoce e tratamento mais adequado.

Spontaneous dissection of the carotid and vertebral arteries (SDCVA) is considered a rare cause of stroke, particularly in countries with multiethnic population. The objective was to evaluate the clinical and neuroimaging features of patients with SDCVA from a multiethnic population. Sixty-six patients diagnosed with SDCVA were studied at two tertiary hospitals at São Paulo. An initial questionnaire was completed and patients were followed prospectively. Among the patients studied, 82 percent were caucasian, 53 percent were male and the average age was 41.7 years old. The most frequent cardiovascular risk factors found were systemic hypertension and tobacco use. Other aspects evaluated were history of previous migraine, initial treatment and prognosis. In conclusion, although the population studied was multhiethnic, there was a marked predominance of caucasians. The analysis of clinical and neuroimaging data from patients with SDCVA allows a better understanding of the disease, leading to an earlier diagnosis and more appropriate treatment.

Fibromuscular dysplasia may herald symptomatic recurrence of cervical artery dissection.

BACKGROUND: The prevalence of fibromuscular dysplasia (FMD) in patients with cervical artery dissection (CAD) is unknown. Our objectives were to assess the risk of CAD recurring as a stroke or a transient ischemic attack and the association of these events with FMD. METHODS: We prospectively included and followed 103 consecutive patients who had been admitted for a CAD. The median follow-up was 4 years (range 4 months to 10 years). The main criteria for inclusion were a mural hematoma demonstrated by cervical magnetic resonance imaging and/or signs suggesting CAD on 2 other investigations. FMD was diagnosed on the so-called string of beads pattern by digital subtraction angiography. RESULTS: Five patients had CAD recurrence (60% occurred late). Four of these 5 patients had FMD. In 4 patients, CAD recurrence involved another cervical artery. CONCLUSION: The rate of symptomatic CAD recurrence was 1% per year and was often related to FMD.

Spontaneous dissection of the carotid and vertebral arteries: the 10-year UCSD experience.

The etiology of spontaneous dissection of the carotid and vertebral arteries without antecedent trauma remains unclear. The goal of this 10-year review was to examine factors regarding presentation, diagnosis, treatment, and outcome for all patients at our institution who were diagnosed with spontaneous carotid dissections (SCD) or spontaneous vertebral dissections (SVD) with no prior trauma history. A retrospective chart analysis was performed involving all discharges from UCSD Medical Center from 1995 to 2005. Patients were selected for inclusion based on the diagnosis of carotid or vertebral dissection with no associated traumatic or iatrogenic cause for their presentation. Characteristics of these patients' medical risk factors, presenting symptoms, diagnostic method and time, treatment, and outcomes were analyzed. A total of 20 patients (10 male, age 44.8 +/- 12.9 yrs; 10 female, age 39.6 +/- 14.9 yrs) were included for study. These patients represented 12 cases of SCD and nine SVD. On presentation, a majority of patients with both SVD and SCD reported headache as their primary complaint while a significantly higher rate of nausea (25% vs. 67%, p < 0.01) was reported in SVD. SVD was associated with a significantly longer diagnostic time (11 hr vs. 16 hr, p < 0.01). The most commonly performed diagnostic exam in both SCD and SVD was magnetic resonance angiography (MRA). Anticoagulation was the primary treatment in 11 of 12 SCD and all nine SVD. One patient with persistent, symptomatic bilateral carotid dissection after anticoagulation was treated with stent placement resulting in unilateral intracranial hemorrhage (ICH). Length of stay was significantly longer in SVD (5 d vs. 7 d, p < 0.02). A significantly higher incidence of persistent neurologic deficits on discharge was seen in SCD (71% vs. 33%, p < 0.02). Radiographic evidence of cerebral infarction on discharge had a stronger correlation with clinical deficits in SCD. Although there were only two cases, those treated with endovascular therapy in the setting of SCD suffered complications related to the intervention. On discharge, there did not seem to be a correlation between persistent neurologic deficits and radiographic evidence of infarction in SVD reflecting that recovery after these episodes may not be predictable based on the appearance of the infarction.

Spontaneous artery dissection in a patient with Human Immunodeficiency Virus (HIV) infection

INTRODUÇÃO: A relação entre AVC e infecção pelo vírus da imunodeficiência humana (HIV) pode ser atribuída em alguns casos a uma vasculopatia subjacente, assim como ocorre nas dissecções arteriais cervicais espontâneas. RELATO DO CASO: Relatamos o caso de um paciente com infecção pelo HIV que desenvolveu uma síndrome de Wallemberg devido a dissecção da artéria vertebral. Os exames laboratoriais revelaram aumento da homocisteina sérica e proteína C reativa. CONCLUSÃO: Este é o primeiro caso na literatura descrevendo a associação entre dissecção arterial e infecção pelo HIV. Sugerimos que o diagnóstico de dissecção arterial deve ser lembrado como um possível mecanismo de AVC isquêmico em pacientes com infecção pelo HIV.

[Strokes associated with cervical artery dissection, and systemic mastocytosis: an unfortuitous association? A report of two cases]. / Accident vasculaire cérébral sur dissection artérielle, au cours des mastocytoses systémiques: une association non fortuite? A propos de deux cas.

INTRODUCTION: Neuropsychiatric symptoms in systemic mastocytosis are usually cognitive and affective changes. EXEGESIS: We describe here two systemic mastocytosis patients without eosinophilia presenting strokes associated with cervical artery dissection. CONCLUSION: These observations are the first reported and they suggest that systemic mastocytosis could be add to the predisposing factors of spontaneous cervical artery dissections.

Aneurysmal forms of cervical artery dissection : associated factors and outcome.

BACKGROUND AND PURPOSE: The natural history of aneurysmal forms of cervical artery dissection (CAD) is ill defined. The aims of this study were to assess (1) clinical and anatomic outcome of aneurysmal forms of extracranial internal carotid artery (ICA) and vertebral artery (VA) dissections and (2) factors associated with aneurysmal forms of CAD. METHODS: Seventy-one consecutive patients with CAD were reviewed. Aneurysmal forms of CAD were identified from all available angiograms by 2 neuroradiologists. The frequency of arterial risk factors, of multiple vessel dissections, and of artery redundancies was compared in patients with and without aneurysm. Patients with aneurysm were invited by mail to undergo a final clinical and radiological evaluation. RESULTS: Of the 71 patients, 35 (49.3%) had a total of 42 aneurysms. Thirty aneurysms were located on a symptomatic artery (ICA, 23; VA, 7) and 12 on an asymptomatic artery (ICA, 10; VA, 2). Patients with aneurysm had multiple dissections of cervical vessels (18/35 versus 7/36; P:=0.005) and arterial redundancies (20/35 versus 11/36; P:=0.02) more frequently than patients without aneurysm. They were also more often migrainous (odds ratio=2.7 [95% CI, 0.8 to 8.5]) and tobacco users (odds ratio=2.2 [95% CI, 0.7 to 6.3]). Clinical and anatomic follow-up information was available for 35 (100%) and 33 patients (94%), respectively. During a mean follow-up of >3 years, no patient had signs of cerebral ischemia, local compression, or rupture. At follow-up, 46% of the aneurysms involving symptomatic ICA were unchanged, 36% had disappeared, and 18% had decreased in size. Resolution was more common for VA than for ICA aneurysms (83% versus 36%). None of the aneurysms located on an asymptomatic ICA had disappeared. CONCLUSIONS: Although aneurysms due to CAD frequently persist, patients carry a very low risk of clinical complications. This favorable clinical outcome should be kept in mind before potential harmful treatment is contemplated.

[Neurological complications after roller coaster rides: an emerging new risk?]. / Le mal des montagnes russes. Emergence d'un nouveau risque?

OBJECTIVE: To describe neurological complications occurring after roller-coaster rides. PATIENTS AND METHODS: We report 6 cases of complications occurring after roller-coaster rides and analyze published data. RESULTS: Complications seen our patients included 5 cervicoencephalic arterial dissections, one with brainstem dysfunction due to extending syringobulbia. Reported data include one cervicoencephalic arterial dissection, one case of carotid artery occlusion, 3 cases of subdural hematoma, one with subarachnoid hemorrhage, one with cerebrospinal fluid leak, and one with Brown-Séquard syndrome secondary to an enterogenous cyst of the spinal cord. In all patients, pain was the first symptom experienced. In 71.4% of cases, it occurred immediately after the trauma. Marfanís syndrome may be the only risk factor identifiable prior to exposure. The mechanisms of most complications are poorly understood but are likely to involve sudden head and neck flexion-extension movements. CONCLUSION: Neurological complications occurring after roller-coaster rides are highly uncommon, but may leave invalidating sequelae or be fatal. Clinicians should be aware of these complications so these patients can be given proper care early, particularly at the stage when pain is the only sign. Early management could help limit the consequences of these complications.