Table 2.
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 |