Primary CNS Vasculitis of Childhood Follow-up

Updated: Dec 14, 2018
  • Author: Jefferson R Roberts, MD; Chief Editor: Lawrence K Jung, MD  more...
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Follow-up

Further Outpatient Care

Ongoing close follow-up with a multidisciplinary team is important in primary CNS vasculitis of childhood. The particular needs of each patient should be identified and addressed as they arise during treatment. These may include educational support for reintegration in school, adequate seizure control, and emotional support of the family, among others.

  • In both small vessel disease and large-medium vessel disease, MRI should be repeated at 3 months and 6 months following disease onset to study the changes in parenchymal brain lesions. In large-medium vessel disease, little improvement in the vessel anatomy may be observed until 6 months after diagnosis, at which time conventional angiography should be performed. Of course, should clinical symptoms change or disease progression be suspected, early imaging is appropriate.

  • Once a diagnosis of small vessel disease is confirmed by biopsy findings, further biopsies do not need to be performed.

  • The involvement of psychiatrists to assist with behavioral symptoms secondary to inflammatory brain disease is often necessary; psychiatric medication may be needed.

  • Serial cognitive assessments with the Pediatric Stroke Outcome Measure are a useful way to quantify deficits.

  • Annual neuropsychological assessments for evaluation of cognitive deficits and identification of assistance needed in school should be performed.

  • Structured quality-of-life assessments using standardized questionnaires can provide insight into the impact of disease on a child's daily life.

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Complications

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  • Complications are mostly related to the immunosuppression and anticoagulation that constitute therapy for the different varieties of primary CNS vasculitis. Side effects of prednisone, such as weight gain, susceptibility to infection, hypertension, and osteopenia, can be seen with the prolonged course of corticosteroids that are a mainstay of treatment.

  • Complications related to disease, such as ongoing seizure disorder, may be noted. Flare of the disease is possible even while receiving therapy. The neurological signs that accompany this may be subtle, and repeating neuroimaging to ascertain the presence of new lesions on MRI or conventional angiography is often necessary. This may require increasing current immunosuppression or the institution of a different immunosuppressive medication to induce remission.

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Prognosis

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  • With early recognition and prompt treatment, prognosis can be excellent. [24] Neurological recovery may take place over months, during which time physical therapy, occupational therapy, speech language therapy, appropriate schooling, and seizure control should be continued. Families of patients affected with primary CNS vasculitis should be prepared for a prolonged rehabilitation period.

  • Although many patients exhibit complete neurological recovery, deficits may remain after completion of treatment. This may include focal and diffuse neurological deficits and behavioral and cognitive symptoms.

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