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Primary CNS Vasculitis of Childhood Clinical Presentation

  • Author: Jefferson R Roberts, MD; Chief Editor: Lawrence K Jung, MD  more...
Updated: Jul 06, 2016


A broad spectrum of clinical presentation in patients with primary CNS vasculitis of childhood is observed, ranging from an insidious onset of headache,[14] cognitive decline, behavior changes or psychosis, an acute stroke, seizures, or status epilepticus. Optic neuritis (in particular bilateral) and cranial nerve palsies have also been seen. The presentation may be affected by the type of vascular lesion (large-medium vs small vessel), as well as the location of discrete lesions seen on MRI and angiography.

  • Large-medium vessel disease frequently presents with focal deficits, including acute hemiparesis, hemisensory deficit, or fine motor deficit. Diffuse deficits are also seen in this condition and may include headache, concentration and cognitive deficits, behavior and personality changes, and seizures. Neurocognitive dysfunction and headaches are reportedly more frequent in progressive large-medium vessel disease, whereas hemiparesis is a more common presentation in nonprogressive large-medium vessel disease.[8]
  • Small vessel disease also has a wide variety of presentations, including seizures (either acute onset or a chronic seizure disorder), headache, neurocognitive deficits, and psychiatric symptoms, including psychosis. Focal deficits that involve gross motor skills, fine motor skills, and sensory function are also seen.[9, 15]
  • Constitutional symptoms are uncommon in patients with large-medium vessel disease but can be present in a minority of patients with small vessel disease. These may include fever, fatigue, and flulike symptoms. In general, such systemic symptoms should prompt investigation for infection or other secondary cause of CNS vasculitis.
  • Eliciting features of systemic inflammatory disease that exclude the diagnosis of primary CNS vasculitis is important. These may include rashes, arthritis, respiratory symptoms, urinary abnormalities, and GI symptoms among others.


The physical examination findings generally reflect the neurological symptoms that are present, as well as any constitutional features that are part of the patient's disease. Most importantly, as with the history, signs of systemic inflammatory disease that may indicate secondary rather than primary CNS vasculitis should be sought.

  • A full neurological examination that includes cranial nerve, fine and gross motor, sensory, cerebellar, and reflex testing is essential.
  • Assessment of neurocognitive deficits is important and may include objective tools such as the Pediatric Stroke Outcome Measure.
  • Some patients may be able to compensate to a certain degree for long-standing cognitive impairment; as such, deficits may not be readily apparent on informal testing. A formal assessment completed by a neuropsychologist or other professional with expertise in this field is often helpful in fully delineating the degree of dysfunction.
  • The systemic examination should be thorough because findings such as oral ulcers, alopecia, lymphadenopathy, hepatosplenomegaly, rash, arthritis, or respiratory abnormalities may indicate an underlying systemic disease responsible for the neurological symptoms.


See the list below:

  • The cause of primary CNS vasculitis of childhood is unknown. Given the commonalities noted between CNS vasculitis and postvaricella angiopathy, an infectious trigger may be responsible; however, none has been identified to date.
  • By definition, any case of CNS vasculitis caused by an underlying systemic disease is not primary CNS vasculitis.
Contributor Information and Disclosures

Jefferson R Roberts, MD Chief of Rheumatology Service, Tripler Army Medical Center; Assistant Clinical Professor of Medicine, Uniformed Services University of the Health Sciences

Jefferson R Roberts, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology, Society for Simulation in Healthcare

Disclosure: Nothing to disclose.


Phalgoon A Shah, MD Resident Physician, Department of Medicine, Tripler Army Medical Center

Phalgoon A Shah, MD is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.


Disclosure: Nothing to disclose.

James J Kim, MD Resident Physician, Department of Internal Medicine, Tripler Army Medical Center

James J Kim, MD is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Chief Editor

Lawrence K Jung, MD Chief, Division of Pediatric Rheumatology, Children's National Medical Center

Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Jayant Deodhar, MD Associate Professor in Pediatrics, BJ Medical College, India; Honorary Consultant, Departments of Pediatrics and Neonatology, King Edward Memorial Hospital, India

Disclosure: Nothing to disclose.

Clare M Hutchinson, MD Lecturer, Part-Time Faculty, Department of Pediatrics, University of Toronto Faculty of Medicine; Pediatrician, Pediatric Education Co-Lead, Department of Child and Teen Health, North York General Hospital, Canada

Disclosure: Nothing to disclose.

Susanne Maria Benseler, MD Pediatric Rheumatologist, Section Chief, Alberta Children's Hospital; Associate Professor, Department of Pediatrics, University of Calgary Faculty of Medicine, Canada

Disclosure: Nothing to disclose.


The authors thank Jorina Elbers, William Halliday, Suzanne Laughlin, Helen Branson, Harvey Lim, Robyn Westmacott, and Derek Armstrong for their significant contributions.

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