Primary angiitis of the central nervous system (PACNS): clinical features

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Primary angiitis of the central nervous system (PACNS): clinical features. Granulomatous angiitis: this affects small-sized vessels, Lymphocytic PACNS.

Classification

  • Granulomatous angiitis: this affects small-sized vessels
  • Lymphocytic PACNS
  • Angiographically defined PACNS: this affects medium-sized vessels
  • Mass/tumour-like lesions
  • Amyloid beta related cerebral angiitis
  • Haemorrhagic PACNS: this is associated with sympathomimetic drug use
  • Isolated spinal cord PACNS
  • Unilateral hemispheric PACNS
  • Unilateral relapsing PACNS
  • Isolated small vessel PACNS (svPACNS)

Risk factors

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Demographic features

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Headache

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Stroke and transient ischaemic attacks (TIAs)

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Other clinical features

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Outcomes

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Predictors of good prognosis

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References

  1. Hajj-Ali RA, Singhal AB, Benseler S, Molloy E, Calabrese LH. Primary angiitis of the CNS. Lancet Neurol 2011; 10:561-572.
  2. Rehman HU. Primary angiitis of the central nervous system. J R Soc Med 2000; 93:586-588.
  3. Binbaum J, Hellmann DB. Primary angiitis of the central nervous system. Arch Neurol 2009; 66:704-709.
  4. de Boysson H, Boulouis G, Dequatre N, et al; French Vasculitis Study Group. Tumor-like presentation of primary angiitis of the central nervous system. Stroke 2016; 47:2401-2404. 
  5. Topcuoglu MA, Jha RM, George J, Frosch MP, Singhal AB. Hemorrhagic primary CNS angiitis and vasoconstrictive drug exposure. Neurol Clin Pract 2017; 7:26-34.
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