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 |