Page 110 - The Vasculitides Volumes 2

86 Aleksandra Mineyko and Adam Kirton

Table 2. Diagnostic Algorithm of Childhood Stroke Due to CNS Vasculitis*

History Neurological review of systems (focal, headaches, seizures, other)

Past Medical History

Family History

Infectious exposures/Risk factors for stroke

History of skin, ophthalmologic, joint involvement, recurrent fevers

Physical Examination Vital signs, peripheral pulses

General and neurological examinations

Rheumatologic assessment

Neuropsychological assessment

Laboratory Investigation Markers of Inflammation: CBC, CRP, ESR, IgG, C3, vWF

Prothrombotic Markers: Protein S, C, ATIII, fibrinogen, plasminogen,

homocysteine; factor V Ledien, PT gene mutations, lupus anticoagulant.

Autoantibodies: ANA, ENA, dsDNA, ANCA, aPL, ACL

(depending on the differential diagnosis: NMDAR, VGKC, GAD,

GABA)

Infectious serology: B. burgdorferi, VDRL, HIV, VZV

Lumbar CSF Analysis OP, cell count, protein, glucose, IgG, OCB

Cryptococcal antigen, VDRL, viral encephalitis register

paired serum and CSF B. burgdorferi and VZV serology

bacterial, fungal, TB cultures

specific autoantibodies depending on the differential diagnosis

Neuroimaging CT/CTA

MRI/MRA with gadolinium and vessel wall imaging

Cerebral angiography

Brain SPECT

PET/CT

Doppler ultrasound

Tissue Biopsies Brain and overlying meninges

Skin, nerve, muscle as indicated

*Adapted from [115 and 128]. Abbreviations: CBC, complete blood count; ATIII, CRP: C-reactive

protein, ESR, erythrocyte sedimentation rate, C3, C3 complement; ATIII, anti-thrombin III; VWF,

Von Williebrand factor; ELISA, enzyme linked immunosorbent assay; CSF, cerebrospinal fluid;

PT, prothrombin; LAC, lupus anticoagulant; ANA, antinuclear antibody; ENA, extractable nuclear

antibody; dsDNA, double-stranded DNA antibody; aPL and ACL, anti-phospholipid and anti-

cardiolipin antibodies; NMDAR, N-methyl-D-asparatate receptor; VGKC, voltage-gated calcium

channel, GABA, gamma-aminobutyric acid; VZV, varicella-zoster virus; CT/CTA, computed

tomography and angiography; MRI and MRA, magnetic resonance imaging and angiography;

SPECT, single photon emission computed tomography; PET/CT, positron emission tomography

and computed tomography; IgG, immunoglobulin G; VDRL, Venereal disease research laboratory;

TB, tuberculosis; OCB, oligoclonal bands; GAD, glutamate decarboxylase; C3, complement

component 3.

Differential Diagnosis

Numerous non-inflammatory conditions must be considered in the differential diagnosis
of CNS vasculitis. One mimic of large vessel CNS disease described above is arterial
dissection, an important cause of stroke which may extend intracranially more often in

Complimentary Contributor Copy