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Figures
- Figure 1
Pathogenesis of PACNS From the point of pathology, the natural processes of PACNS were suggested to follow 3 steps: 1) endothelial cell swelling and hyperplasia, and lymphocytic infiltration; 2) subintimal fibrinoid necrosis of small vessels and histiocytic infiltration; and 3) marked granulomatous inflammation. From the point of pathophysiology, ischemia and infarction or hemorrhage were considered to be results of the inflammation process, which leads to the obstruction of the vessel lumen, hypercoagulable states, vasomotor tone dysfunction, and destruction of the vascular wall.
Tables
Adult PACNS* Headache Cognitive dysfunction Hemiparesis Consistent neurologic deficit or stroke Visual symptoms Transient ischemic attack Aphasia Seizures Ataxia Intracranial hemorrhage Large-medium vessel cPACNS† Focal neurologic deficits 1. Acute hemiparesis 2. Hemisensory loss 3. Fine motor skill loss 4. Hemifacial weakness Headaches Seizures Diffuse neurologic deficit Small-vessel cPACNS Any neurologic or psychiatric symptoms Secondary central nervous system vasculitis Systemic vasculitides: Takayasu arteritis, giant cell arteritis, polyarteritis nodosa, Kawasaki disease, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, Behçet disease, etc. Vasculitis associated with systemic diseases: lupus vasculitis, rheumatoid vasculitis, Gougerot Sjögren’s syndrome, etc. Infections: viral (e.g., herpes zoster, HIV1), bacterial (e.g., tuberculosis, syphilis), fungal (e.g., aspergillosis, cryptococcus), mycoplasmal, etc Cancer-associated: Hodgkin and non-Hodgkin lymphoma, leukemia, etc. Nonvasculitic autoimmune and inflammatory brain diseases Neuromyelitis optica, N-Methyl-D-aspartate receptor–mediated encephalitis, Susac syndrome, optic neuritis, multiple sclerosis, acute demyelinating encephalomyelitis, Rasmussen encephalitis. Non-inflammatory vasculopathies RCVS:2 Call-Fleming syndrome, postpartum angiopathy, migrainous vasospasm, drug-induced arteritis, BACNS3. Others such as fibromuscular dysplasia, Moyamoya disease, intracranial dissection, radiation vasculopathy, etc. Miscellaneous thromboembolic disease, bacterial endocarditis, anti-phospholipid syndrome and other hypercoagulable states, cardiac myxoma embolism, cholesterol atheroembolism, hemoglobin disorders, etc. HIV - human immunodeficiency virus, RCVS - reversible cerebral vasoconstriction syndrome, BACNS - benign angiitis of the central nervous system
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