Primary Angiitis of the Central Nervous System and Reversible Cerebral Vasoconstriction Syndromes
Vasculitis that exclusively involves the central nervous system (CNS) is among the most complex and poorly understood forms of vascular inflammatory disease. The variable terminology for CNS vasculitis continues to pose significant challenges. CNS vasculitis that is not associated with a known underlying cause or some form of systemic vasculitis is termed primary angiitis of the CNS (PACNS). Some cases of PACNS confirmed by histopathology have granulomatous features of inflammation. The term “granulomatous inflammation of the nervous system” has been used for such cases. For practical purposes, now GANS and PACNS are considered to be largely synonymous terms. PACNS presents in a subacute manner, with an elapse of many months between the time of first symptom onset and diagnostic confirmation. The clinical hallmarks of PACNS are headache, slowly-evolving encephalopathy, and multi-focal strokes. Fever, other constitutional symptoms, and extra-CNS manifestations are absent. Acute phase reactants tend to be normal in PACNS. Lumbar puncture typically reveals a lymphocytic pleocytosis.
KeywordsPosterior Reversible Encephalopathy Syndrome Brain Biopsy Reversible Posterior Leukoencephalopathy Syndrome Pituitary Apoplexy Reversible Cerebral Vasoconstriction Syndrome
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