Elsevier

The Lancet Neurology

Volume 14, Issue 6, June 2015, Pages 640-654
The Lancet Neurology

Review
Epidemiology, pathophysiology, diagnosis, and management of intracranial artery dissection

https://doi.org/10.1016/S1474-4422(15)00009-5Get rights and content

Summary

Spontaneous intracranial artery dissection is an uncommon and probably underdiagnosed cause of stroke that is defined by the occurrence of a haematoma in the wall of an intracranial artery. Patients can present with headache, ischaemic stroke, subarachnoid haemorrhage, or symptoms associated with mass effect, mostly on the brainstem. Although intracranial artery dissection is less common than cervical artery dissection in adults of European ethnic origin, intracranial artery dissection is reportedly more common in children and in Asian populations. Risk factors and mechanisms are poorly understood, and diagnosis is challenging because characteristic imaging features can be difficult to detect in view of the small size of intracranial arteries. Therefore, multimodal follow-up imaging is often needed to confirm the diagnosis. Treatment of intracranial artery dissections is empirical in the absence of data from randomised controlled trials. Most patients with subarachnoid haemorrhage undergo surgical or endovascular treatment to prevent rebleeding, whereas patients with intracranial artery dissection and cerebral ischaemia are treated with antithrombotics. Prognosis seems worse in patients with subarachnoid haemorrhage than in those without.

Introduction

Cervicocephalic artery dissection, which corresponds with a haematoma in the wall of a cervical or an intracranial artery, is an important cause of stroke in children and young and middle-aged adults.1, 2, 3 Although dissection of the extracranial cervical arteries has been extensively studied and described,4, 5, 6, 7, 8, 9, 10, 11, 12 less information is available about pure intracranial artery dissection (ie, not including the cervical portion of the artery).4 Early reports were exclusively based on autopsy series, hence biased towards the most severe cases of intracranial artery dissection.13, 14 Several possible reasons are available for the absence of information about intracranial artery dissections. First, intracranial artery dissection happens less frequently than cervical artery dissection in non-Asian countries, where the largest series of patients who had cervical artery dissection have been reported so far.9, 10, 11, 12 Second, patients who have cervical artery dissection and mainly present with headache, cervical pain, and ischaemic stroke are mostly seen by neurologists, whereas patients with intracranial artery dissection can also develop a subarachnoid haemorrhage and are therefore managed not only by neurologists, but also by neurosurgeons and interventional neuroradiologists, all of whom might have an incomplete picture of the disease. As a result, no consensus is agreed on for the diagnostic criteria and optimum treatment of patients with intracranial artery dissections.

In this Review we provide a comprehensive overview of reported studies into the epidemiology, pathophysiology, diagnosis, management, and outcome of spontaneous intracranial artery dissections, in addition to proposing a consensus statement by a group of international experts from various specialties and countries about the diagnosis and management of intracranial artery dissections.

Section snippets

Epidemiology

The incidence of intracranial artery dissections is unknown, but is probably lower than that of symptomatic cervical artery dissection (2·6–3·0 per 100 000 people per year15, 16) in populations of European ethnic origin. The proportion of intracranial artery dissections in all cervicocephalic dissections substantially varies between ethnic origin and age groups, and also depends on study recruitment strategies and ascertainment methods used. Recruitment of patients for studies through neurology

Anatomy of the intracranial carotid and vertebral arteries

The intradural portion of the internal carotid artery starts at the clinoid segment of the artery (C6), from which the ophthalmic artery originates in most patients. The intradural portion of the vertebral artery is called the V4 segment, from which the anterior spinal artery and posterior inferior cerebellar artery originate (figure 1).

By contrast with cervical arteries, intradural arteries are characterised by a well developed internal elastic lamina, a paucity of elastic fibres in the media,

Risk factors and predisposing conditions

Risk factors for intracranial artery dissections are unknown. No comparisons exist between putative risk factors in patients with intracranial artery dissection and healthy controls. In the few studies that included both patients with cervical artery dissection and those with intracranial artery dissection, distribution of vascular risk factors did not differ between the two groups,31 except for one study37 showing a higher prevalence of hypertension in patients with intracranial artery

Clinical presentation and radiological features

Clinical presentation of intracranial artery dissections is not specific. The two main manifestations are subarachnoid haemorrhage and cerebral ischaemia.31 In most reported series (table 1), intracranial artery dissections with subarachnoid haemorrhage represent 50–60% of all intracranial artery dissections. Subarachnoid haemorrhage occurs if the arterial wall of an intracranial artery dissection in the intradural portion ruptures. Between 30% and 78% of patients with intracranial artery

Treatment options

Optimum treatment for patients with intracranial artery dissections is unknown. No randomised trials exist and only observational studies with small sample sizes are available, thus providing a very low level of evidence.

Patients with intracranial artery dissection with subarachnoid haemorrhage are usually treated with surgical or endovascular procedures because up to 40% of patients have rebleeding within the first days after the event.30, 87 If patients are in very poor clinical health or the

Outcome

Because of treatment and publication biases, little is known about the natural history of intracranial artery dissection. Overall, intracranial artery dissection has a more severe course than cervical artery dissection, with a more ominous outcome in patients with subarachnoid haemorrhage than in those without subarachnoid haemorrhage.30, 101 Table 3 and the appendix summarise outcomes reported in individual studies.

Conclusions and future directions

Intracranial artery dissection is an uncommon and presumably underdiagnosed cause of both ischaemic stroke and subarachnoid haemorrhage. Diagnosis of intracranial artery dissection is often difficult because of non-specific clinical presentation; low sensitivity of radiological methods for pathognomonical signs, such as a mural haematoma, intimal flap, or double lumen, in view of the small size of the arteries; and the dynamic nature of the disease. We propose terminology and grading of imaging

Search strategy and selection criteria

References for this Review were identified through searches of PubMed with the terms “intracranial”, “intradural”, “intracranial aneurysm”, or “intracranial artery diseases” in combination with “dissection”, “vertebral artery dissection”, “carotid artery, internal, dissection”, or “aneurysm, dissecting” between PubMed inception and Dec 1, 2014. We also identified scientific papers by reviewing reference lists of relevant articles and through searches of the authors' personal files. We

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