Advances in Primary Angiitis of the Central Nervous System

Primary angiitis of the central nervous system (PACNS) is a rare idiopathic inflammatory syndrome targeting the vessels of the brain and spinal cord. Clinical presentation is variable, insidious, and non-specific; headache and encephalopathy are the most common symptoms. Multiple strokes affecting numerous vascular territories may be seen, and both focal and diffuse neurologic dysfunction may be present. Cerebrospinal fluid (CSF) analysis is crucial; a normal CSF along with normal brain parenchymal imaging carries a high negative predictive value in excluding PACNS. The role of imaging continues to evolve, and most patients have abnormal vascular imaging; however, the specificity of imaging for PACNS has historically been poor. Cerebral and meningeal biopsy is a valuable tool in confirming the diagnosis and excluding mimics. PACNS generally responds to immunosuppressive therapy. Failure to respond should prompt evaluation for an alternative diagnosis. Given the rarity of this disorder, exclusion of mimics such as the reversible cerebral vasoconstriction syndromes (RCVS) and infectious processes is essential.

[1]  A. Singhal,et al.  Tumour-like mass lesion: an under-recognised presentation of primary angiitis of the central nervous system , 2008, Annals of the rheumatic diseases.

[2]  C. Arquizan,et al.  Rituximab for Primary Angiitis of the Central Nervous System: Report of 2 Patients from the French COVAC Cohort and Review of the Literature , 2013, The Journal of Rheumatology.

[3]  A. Ducros Reversible cerebral vasoconstriction syndrome , 2012, The Lancet Neurology.

[4]  G. Hunder,et al.  Adult primary central nervous system vasculitis , 2012, The Lancet.

[5]  L. Calabrese,et al.  Primary Angiitis of the Central Nervous System: REPORT OF 8 NEW CASES, REVIEW OF THE LITERATURE, AND PROPOSAL FOR DIAGNOSTIC CRITERIA , 1988, Medicine.

[6]  I. Pesaresi,et al.  3-T Magnetic Resonance Angiography in Primary Angiitis of the Central Nervous System , 2013, Journal of computer assisted tomography.

[7]  R. Hajj-Ali,et al.  Primary Angiitis of the Central Nervous System and Reversible Cerebral Vasoconstriction Syndrome , 2013, Current Atherosclerosis Reports.

[8]  S. Jones,et al.  High-Resolution MRI Vessel Wall Imaging: Spatial and Temporal Patterns of Reversible Cerebral Vasoconstriction Syndrome and Central Nervous System Vasculitis , 2014, American Journal of Neuroradiology.

[9]  A. Singhal,et al.  Reversible cerebral vasoconstriction syndromes: analysis of 139 cases. , 2011, Archives of neurology.

[10]  Catherine Oppenheim,et al.  Primary Angiitis of the Central Nervous System: Description of the First Fifty‐Two Adults Enrolled in the French Cohort of Patients With Primary Vasculitis of the Central Nervous System , 2014, Arthritis & rheumatology.

[11]  M. Waters,et al.  Vessel wall enhancement in herpes simplex virus central nervous system vasculitis , 2013, Journal of Clinical Neuroscience.

[12]  J. Safdieh,et al.  The varicella zoster virus vasculopathies , 2008, Neurology.

[13]  Catherine Oppenheim,et al.  Revised primary angiitis of the central nervous system: Description of the first 52 adults enrolled in the french COVAC' cohort. , 2014 .

[14]  J. Mullikin,et al.  Early-onset stroke and vasculopathy associated with mutations in ADA2. , 2014, The New England journal of medicine.

[15]  J. Wiltfang,et al.  Brain-reactive β-amyloid antibodies in primary cns angiitis with cerebral amyloid angiopathy , 2011, Neurology.

[16]  A. Singhal,et al.  Primary angiitis of the CNS , 2011, The Lancet Neurology.

[17]  Jonathan M. Morris,et al.  Aβ-related angiitis , 2013, Neurology.

[18]  J. Parisi,et al.  Biopsy Findings in Primary Angiitis of the Central Nervous System , 2009, The American journal of surgical pathology.

[19]  A. Ducros,et al.  Reversible cerebral vasoconstriction syndrome and cervical artery dissection in 20 patients , 2013, Neurology.

[20]  S. Weigand,et al.  Primary central nervous system vasculitis: analysis of 101 patients , 2007, Annals of neurology.

[21]  D. Mikulis,et al.  Vessel Wall MRI to Differentiate Between Reversible Cerebral Vasoconstriction Syndrome and Central Nervous System Vasculitis: Preliminary Results , 2012, Stroke.

[22]  S. Benseler,et al.  The spectrum of CNS vasculitis in children and adults , 2012, Nature Reviews Rheumatology.