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. 2020 Jul 3;8:281. doi: 10.3389/fped.2020.00281

Table 2.

Differential diagnosis of cPACNS.

DIFFERENTIAL DIAGNOSIS OF PACNS
CNS-involvement in systemic inflammatory/rheumatic diseases
For example, Takayasu arteritis, polyarteritis nodosa, Kawasaki disease, Henoch-Schönlein purpura, Behçet disease, granulomatosis with polyangiitis, microscopic polyangiitis, systemic lupus erythematosus, juvenile dermatomyositis, inflammatory bowel disease, hemophagocytic lymphohistiocytosis, ADA 2-deficiency, TREX1-associated diseases (e.g., Aicardi-Goutieres syndrome)
Secondary vasculitis to other causes
For example, in malignancy, infections, drugs
Infectious encephalitis
For example, varicella zoster virus–post-varicella angiopathy
Thromboembolic disease
For example, in coagulation disorders, hemoglobinopathies, coronary artery disease, congenital heart disease
Reversible vasoconstriction syndrome
Moyamoya syndrome
Primary moyamoya MYMY1–MYMY6 (e.g., RNF213)
Secondary moyamoya syndrome, e.g., in Down syndrome, sickle cell anemia, neurofibromatosis type 1
Neurometabolic diseases
For example, Fabry disease (GLA), homocystinuria
Fibromuscular dysplasia
Migrainous infarction
(ICHD3: 1.4.3.)
Genetic vasculopathies
For example, NOTCH3, HTRA1
Genetic structural alterations of vessels
For example, COL4A1, ACTA2, MOPD2
Structural vasculopathies
(causing mostly hemorrhagic stroke): For example, arterial dissection, arteriovenous malformations, aneurysm, cavernous malformation