Dissection Syndromes Clinical Presentation

  • Author: Chelsea S Kidwell, MD; Chief Editor: Helmi L Lutsep, MD   more...
 
Updated: Sep 19, 2011
 

History

  • The most frequent presenting complaints with cervicocephalic dissections are ischemic symptoms that include transient ischemic attack (TIA) or stroke (cerebrovascular accident).
  • Up to two thirds of patients complain of ipsilateral neck, scalp, or head pain, occurring in both carotid and vertebral artery dissections.
  • Up to one fourth of patients report pulsatile tinnitus or a subjective bruit, particularly with carotid artery dissections.
  • In a large, pooled, observational study of 982 patients with cervical artery dissections, significant differences were observed in patients with internal carotid artery dissections (ICAD) compared to vertebral artery dissections (VAD). Most notably, patients with ICAD were older, more often men, more frequently had a recent infection, and less frequently had cerebral ischemia. In contrast, patients with VAD more frequently reported a history of neck trauma and cervical pain and less frequently presented with headache. Of those patients with cerebral ischemia, ICAD patients had more severe baseline National Institutes of Health Stroke Scale scores.[1]
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Physical

  • Extracranial carotid artery
    • Cerebral ischemia occurs in at least 75% of reported cases (TIAs in 30%, infarcts in 45-50%).
    • Neurologic deficits reflect the ultimate site of ischemia in the ipsilateral anterior circulation.
    • In extracranial carotid dissections, local symptoms may occur as the intramural hematoma expands outward, compressing local structures.
    • Examination findings may include the following:
      • Ipsilateral partial Horner syndrome (32-82% of patients in various series)
      • Ipsilateral cranial nerve palsies, particularly cranial nerves IX, X, XI, and XII (5-12% of patients in various series)
      • Audible bruit (up to 20% of patients)
  • Intracranial carotid artery
    • Patients with intracranial carotid dissections usually present with headache followed by a major ischemic stroke.
    • Some patients initially may present with a seizure, syncope, or altered level of consciousness.
    • One fifth of patients develop subarachnoid hemorrhage.
  • Extracranial vertebral artery
    • This dissection is characterized by headache (often occipital) or neck pain and signs of ischemia in the posterior circulation.
    • Infarcts in the territory of the posterior inferior cerebral artery (commonly with a lateral medullary syndrome) are frequent.
  • Intracranial vertebrobasilar dissection: This dissection may present with symptoms of posterior circulation ischemia (particularly brainstem), subarachnoid hemorrhage (occurs in one half of patients), or both.
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Causes

  • Major blunt trauma to the head and neck can produce cervicocephalic dissection. In spontaneous dissections (dissection in absence of major trauma), a history of minor trauma is a precipitating factor in at least 25% of dissections. The remaining cases appear to be truly spontaneous.
  • Types of trauma associated with cervicocephalic dissections include chiropractic neck manipulations[2] , sporting activities, coughing, sneezing, sexual activity, and more intense forms of blunt trauma (eg, motor vehicle accidents, falls, strangulation, hanging).
  • Arteriopathies have been associated with cervicocephalic dissections.
    • Fibromuscular dysplasia (the most common underlying arteriopathy, found in as many as 15% of patients)
    • Extreme vessel tortuosity
    • Marfan syndrome
    • Ehlers-Danlos syndrome
    • Alpha-1-antitrypsin deficiency
    • Cystic medial necrosis
    • Type 1 collagen point mutation
    • Other connective tissue disorders
    • Moyamoya disease
    • Meningovascular syphilis
  • Associations also have been reported with systemic infections, hypertension, migraine, elevated homocysteine levels, alcohol use, and oral contraceptive use.
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Contributor Information and Disclosures
Author

Chelsea S Kidwell, MD  Professor, Department of Neurology, Georgetown University; Medical Director, Georgetown University Stroke Center

Chelsea S Kidwell, MD is a member of the following medical societies: American Academy of Neurology, American Heart Association, American Society of Neuroimaging, and National Stroke Association

Disclosure: Nothing to disclose.

Coauthor(s)

Richard E Burgess, MD, PhD  Assistant Professor, Department of Neurology, Georgetown University Hospital; Medical Director, Clinical Stroke Service

Richard E Burgess, MD, PhD is a member of the following medical societies: American Academy of Neurology and American Heart Association

Disclosure: Nothing to disclose.

Specialty Editor Board

William J Nowack, MD  Associate Professor, Epilepsy Center, Department of Neurology, University of Kansas Medical Center

William J Nowack, MD is a member of the following medical societies: American Academy of Neurology, American Clinical Neurophysiology Society, American Epilepsy Society, American Medical Electroencephalographic Association, American Medical Informatics Association, and Biomedical Engineering Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Howard S Kirshner, MD  Professor of Neurology, Psychiatry and Hearing and Speech Sciences, Vice Chairman, Department of Neurology, Vanderbilt University School of Medicine; Director, Vanderbilt Stroke Center; Program Director, Stroke Service, Vanderbilt Stallworth Rehabilitation Hospital; Consulting Staff, Department of Neurology, Nashville Veterans Affairs Medical Center

Howard S Kirshner, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Heart Association, American Medical Association, American Neurological Association, American Society of Neurorehabilitation, National Stroke Association, Phi Beta Kappa, and Tennessee Medical Association

Disclosure: Nothing to disclose.

Chief Editor

Helmi L Lutsep, MD  Professor, Department of Neurology, Oregon Health and Science University School of Medicine; Associate Director, Oregon Stroke Center

Helmi L Lutsep, MD is a member of the following medical societies: American Academy of Neurology and American Stroke Association

Disclosure: Co-Axia Consulting fee Review panel membership; AGA Medical Consulting fee Review panel membership; Concentric Medical Consulting fee Review panel membership

Additional Contributors

I would like to thank Jeffrey L. Saver, MD, for his thoughtful review of this article.

References

  1. Debette S, Grond-Ginsbach C, Bodenant M, Kloss M, Engelter S, Metso T, et al. Differential features of carotid and vertebral artery dissections: The CADISP Study. Neurology. Sep 7 2011;[Medline].

  2. Paciaroni M, Bogousslavsky J. Cerebrovascular complications of neck manipulation. Eur Neurol. 2009;61(2):112-8. [Medline].

  3. Kim YK, Schulman S. Cervical artery dissection: pathology, epidemiology and management. Thromb Res. Apr 2009;123(6):810-21. [Medline].

  4. Engelter ST, Rutgers MP, Hatz F, Georgiadis D, Fluri F, Sekoranja L. Intravenous thrombolysis in stroke attributable to cervical artery dissection. Stroke. Dec 2009;40(12):3772-6. [Medline].

  5. Arauz A, Márquez JM, Artigas C, Balderrama J, Orrego H. Recanalization of vertebral artery dissection. Stroke. Apr 2010;41(4):717-21. [Medline].

  6. Arnold M, Kurmann R, Galimanis A, Sarikaya H, Stapf C, Gralla J, et al. Differences in demographic characteristics and risk factors in patients with spontaneous vertebral artery dissections with and without ischemic events. Stroke. Apr 2010;41(4):802-4. [Medline].

  7. Debette S, Leys D. Cervical-artery dissections: predisposing factors, diagnosis, and outcome. Lancet Neurol. Jul 2009;8(7):668-78. [Medline].

  8. Debette S, Markus HS. The genetics of cervical artery dissection: a systematic review. Stroke. Jun 2009;40(6):e459-66. [Medline].

  9. Georgiadis D, Arnold M, von Buedingen HC, Valko P, Sarikaya H, Rousson V. Aspirin vs anticoagulation in carotid artery dissection: a study of 298 patients. Neurology. May 26 2009;72(21):1810-5. [Medline].

  10. Schwartz NE, Vertinsky AT, Hirsch KG, Albers GW. Clinical and radiographic natural history of cervical artery dissections. J Stroke Cerebrovasc Dis. Nov-Dec 2009;18(6):416-23. [Medline].

 
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Cerebral angiogram of a left internal carotid dissection showing gradual vessel tapering to occlusion.
Axial T1-weighted MRI demonstrating a crescent sign (arrow) in a patient with a left internal carotid artery dissection.
 
 
 
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