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Table 2 Differential diagnoses of PACNS [6, 9]

From: Primary angiitis of the CNS (PACNS) and Behçet disease

Other inflammatory diseases
 − Autoimmune encephalitides
 − Susac-Syndrome
Non-inflammatory diseases
 − Atherosclerosis
 − Neurofibromatosis
 − Fibromuscular Dysplasia, Ehlers-Danlos IV, Marfan-Syndrome
 − Genetic microangiopathies (COL4A1, CTC1, TREX)
 − CADASIL, CARASIL
 − Leukodystrophies
 − MELAS
 − Moyamoya angiopathy
 − Fabry disease
 − Sneddon syndrome
 − Hypercoagubility
Demyelinating diseases
 − Multiple Sclerosis
 − ADEM
 − NMOSD
CNS-Involvement in Systemic Vasculitis
 − Large vessel vasculitides: Giant cell arteriitis, Takayasu Arteriitis
 − Medium size vessel vasculitides: Polyarteriitis nodosa, Kawasaki disease
 − Small vessel vasculitides: ANCA-associated vasculitides (Granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis) Immunocomplex-associated diseases (IgA-Vasculitis – Schönlein-Henoch Purpura, cyoglobulinemic vasculitis)
Involvement in Infections
 − Embolisms of subacute bacterial endocarditis
 − VZV-Vasopathy
 − HSV, HIV, Hepatitis B, C, Parvoviruses Borreliae Lues, Tuberculosis, Rickettsias, fungi, protozoans
 − parainfectious syndromes
Involvement in Systemic Diseases
 − Systemic Lupus erythematodes
 − Sjögren-Syndrome
 − Sclerodermia
 − Neurosarcoidosis
 − Neurobehçet
Reversible Cerebral Vasoconstriction Medication Syndromes or Drug-induced Malignant Syndromes Diseases
 − Primary CNS-Lymphoma
 − Intravascular Lymphoma
 − Lymphomatoid Granulomatosis