Page 105 - The Vasculitides Volumes 2

Childhood Vasculitic Stroke 81

inflammation on tissue biopsy [23]. Neurologically, it is most often associated with
contiguous extension of granulomas leading to pachymeningitis while reports of cerebral
vasculitis or AIS are rare. Similarly, children with EGPA rarely present with AIS [37].
IgAV/HSP is a common disorder of early childhood characterized by nonthrombocytopenic
purpura, arthralgia, nephritis, and abdominal pain [29, 30]. Stroke types associated with
IgAV/HSP include hemorrhagic and arterial ischemic strokes as well as sinus venous
thrombosis [29, 30]. However, pediatric stroke is rare in IgAV/HSP, described in only 1-8%
of patients [29, 38, 39].

Variable Vessel Vasculitis

Behçet syndrome is a rare primary systemic vasculitis of childhood characterized by
mucocutaneous ulcers and uveitis. CNS involvement includes idiopathic intracranial
hypertension and meningoencephalitis which occur in up to 50% of patients [29].
Cerebrovascular disease has been reported in 30% of adults. Occurrence in children is
recognized in isolated case reports but the true incidence is unknown and likely low. Venous
sinus thrombosis may be most common but arterial infarcts and aneurysms are also described
[40].

CNS Vasculitis

Isolated central nervous system vasculitis in children has been referred to in the literature
as cPACNS. Specific subtypes are distinguished based on vessel size, angiographic and
pathological findings, and the presence or absence of long-term progression [24, 25]. Large-
medium vessel vasculitis is divided into progressive and non-progressive types [24, 25, 41],
the latter syndrome being highly comparable to other childhood cerebral arteriopathies such
as TCA and FCA. Current classifications remain incomplete and controversial, as discussed
here and elsewhere [24, 25, 42] but provides a practical, clinically relevant framework. It
should be noted that the following discussion is based on evidence supporting a vasculitic
mechanism for large-medium cPACNS but that definitive diagnostic testing does not exist
and the acceptance of this mechanism varies widely within the pediatric stroke community.

Large-Medium Vessel Type
Most large/medium vessel primary CNS vasculitis in children is non-progressive and

unilateral. As such, it bears marked similarity to other cerebral arteriopathies causing stroke
in otherwise healthy children, namely TCA and FCA. It is possible they represent the same
disease or closely related diseases within a spectrum [41, 43]. The following features are
shared across these syndromes and are used in support of the clinical diagnosis [6, 21,
25, 42]:

Complimentary Contributor Copy