Primary CNS Vasculitis of Childhood Clinical Presentation
- Author: Jefferson R Roberts, MD; Chief Editor: Lawrence K Jung, MD more...
A broad spectrum of clinical presentation in patients with primary CNS vasculitis of childhood is observed, ranging from an insidious onset of headache, 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.
- 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.
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- 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.
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].