Primary CNS Vasculitis of Childhood Follow-up
- Author: Jefferson R Roberts, MD; Chief Editor: Lawrence K Jung, MD more...
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.
See the list below:
- 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.
See the list below:
- With early recognition and prompt treatment, prognosis can be excellent. 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.
Twilt M, Benseler SM. The spectrum of CNS vasculitis in children and adults. Nat Rev Rheumatol. 2011 Dec 20. [Medline].
Cellucci T, Benseler SM. Diagnosing central nervous system vasculitis in children. Curr Opin Pediatr. 2010 Dec. 22(6):731-8. [Medline].
Twilt M, Benseler SM. Central nervous system vasculitis in adults and children. Handb Clin Neurol. 2016. 133:283-300. [Medline].
Cravioto H, Feigin I. Noninfectious granulomatous angiitis with a predilection for the nervous system. Neurology. 1959 Sep. 9:599-609. [Medline].
Calabrese LH, Furlan AJ, Gragg LA, Ropos TJ. Primary angiitis of the central nervous system: diagnostic criteria and clinical approach. Cleve Clin J Med. 1992 May-Jun. 59(3):293-306. [Medline].
Herlin T, Nielsen S. [Primary childhood vasculitis--new classification criteria]. Ugeskr Laeger. 2008 Sep 1. 170(36):2784-7. [Medline].
Hajj-Ali RA, Calabrese LH. Diagnosis and classification of central nervous system vasculitis. J Autoimmun. 2014 Jan 31. [Medline].
Benseler SM, Silverman E, Aviv RI, Schneider R, Armstrong D, Tyrrell PN. Primary central nervous system vasculitis in children. Arthritis Rheum. 2006 Apr. 54(4):1291-7. [Medline].
Benseler SM, deVeber G, Hawkins C, et al. Angiography-negative primary central nervous system vasculitis in children: a newly recognized inflammatory central nervous system disease. Arthritis Rheum. 2005 Jul. 52(7):2159-67. [Medline].
Saini AG, Sankhyan N, Bhattad S, Vyas S, Saikia B, Singhi P. CNS vasculitis and stroke in neonatal lupus erythematosus: A case report and review of literature. Eur J Paediatr Neurol. 2014 Jan 25. [Medline].
Matsell DG, Keene DL, Jimenez C, Humphreys P. Isolated angiitis of the central nervous system in childhood. Can J Neurol Sci. 1990 May. 17(2):151-4. [Medline].
[Guideline] Riviello JJ Jr, Ashwal S, Hirtz D, et al. Practice parameter: diagnostic assessment of the child with status epilepticus (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2006 Nov 14. 67(9):1542-50. [Medline].
Hutchinson C, Elbers J, Halliday W, Branson H, Laughlin S, Armstrong D, et al. Treatment of small vessel primary CNS vasculitis in children: an open-label cohort study. Lancet Neurol. 2010 Nov. 9(11):1078-84. [Medline].
Cekinmez EK, Cengiz N, Erol I, Kizilkilic O, Uslu Y. Unusual cause of acute neurologic deficit in childhood: primary central nervous system vasculitis presenting with basilar arterial occlusion. Childs Nerv Syst. 2009 Jan. 25(1):133-6. [Medline].
Lanthier S, Lortie A, Michaud J, Laxer R, Jay V, deVeber G. Isolated angiitis of the CNS in children. Neurology. 2001 Apr 10. 56(7):837-42. [Medline].
Yaari R, Anselm IA, Szer IS, Malicki DM, Nespeca MP, Gleeson JG. Childhood primary angiitis of the central nervous system: two biopsy-proven cases. J Pediatr. 2004 Nov. 145(5):693-7. [Medline].
Aviv RI, Benseler SM, Silverman ED, et al. MR imaging and angiography of primary CNS vasculitis of childhood. AJNR Am J Neuroradiol. 2006 Jan. 27(1):192-9. [Medline].
Aviv RI, Benseler SM, DeVeber G, et al. Angiography of primary central nervous system angiitis of childhood: conventional angiography versus magnetic resonance angiography at presentation. AJNR Am J Neuroradiol. 2007 Jan. 28(1):9-15. [Medline].
Torres J, Loomis C, Cucchiara B, Smith M, Messé S. Diagnostic Yield and Safety of Brain Biopsy for Suspected Primary Central Nervous System Angiitis. Stroke. 2016 Jun 28. [Medline].
Bitter KJ, Epstein LG, Melin-Aldana H, Curran JG, Miller ML. Cyclophosphamide treatment of primary angiitis of the central nervous system in children: report of 2 cases. J Rheumatol. 2006 Oct. 33(10):2078-80. [Medline].
Elbers J, Benseler SM. Central nervous system vasculitis in children. Curr Opin Rheumatol. 2008 Jan. 20(1):47-54. [Medline].