Primary Angiitis of the Central Nervous System

  • Hossein Pourmahmoodian Mail Department of Neurology, Iranian Center of Neurological Research, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran.
  • Hossein Ali Ghelichnia Omrani Department of Neurology, Iranian Center of Neurological Research, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran.
  • Mohammad Hossein Harrirchian Department of Neurology, Iranian Center of Neurological Research, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran.
  • Mojdeh Ghabaee Department of Neurology, Iranian Center of Neurological Research, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran.
  • Babak Zamani Department of Neurology, Iranian Center of Neurological Research, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran.
  • Majid Ghaffarpour Department of Neurology, Iranian Center of Neurological Research, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Keywords:
Vasculitis, Central nervous system, Angiography

Abstract

Primary angiitis of the central nervous system (PACNS) is an idiopathic disorder (vasculitis) restricted to the central nervous system (CNS). It often presents with focal neurological deficits suggesting stroke or a combination of confusion and headache. We herein report three cases with various combinations of fever, partial seizure, encephalopathy, paresis, headache and ataxia. One of them was initially treated as herpes simplex meningoencephalitis, but further investigations revealed primary angiitis. Primary angiitis of the CNS has protean manifestations and should always be considered in patients suspicious to have CNS infection or stroke, particularly who does not respond to the routine treatments. Clinical data, exclusion of differential diagnoses and typical angiography seem to be enough to justify the diagnosis in the majority of cases.

References

Moore PM, Richardson B. Neurology of the vasculitides and connective tissue diseases. J Neurol Neurosurg Psychiatry 1998;65(1):10-22.

Lie. JT. Primary (granulomatous) angiitis of the central nervous system: a clinicopathologic analysis of 15 new cases and a review of the literature. Hum Pathol 1992;23(2):164-71.

Siva A. Vasculitis of the nervous system. J Neurol 2001;248(6):451-68.

Ilhan A, Budak F. Primary Angiitis of the Central Nervous System: Unusual Clinical Presentation. Int J Angiol 2000;9(1):23-6.

Abu-Shakra M, Khraishi M, Grosman H, Lewtas J, Cividino A, Keystone EC. Primary angiitis of the CNS diagnosed by angiography. Q J Med 1994;87(6):351-8.

Moore PM. Central nervous system vasculitis. Curr Opin Neurol 1998;11(3):241-6.

Hankey GJ. Isolated angiitis/angiopathy of the central nervous system. Cerebrovasc Dis 1991;1:2-15.

Vollmer TL, Guarnaccia J, Harrington W, Pacia SV, Petroff OA. Idiopathic granulomatous angiitis of the central nervous system. Diagnostic challenges. Arch Neurol 1993;50(9):925-30.

Bhibhatbhan A, Katz NR, Hudon M, Clark AW, Hurlbert RJ, Zochodne DW. Primary angiitis of the spinal cord presenting as a conus mass: long-term remission. Surg Neurol 2006;66(6):622-5; discussion 625-6.

Derry C, Dale RC, Thom M, Miller DH, Giovannoni G. Unihemispheric cerebral vasculitis mimicking Rasmussen's encephalitis. Neurology 2002;58(2):327-8.

Rehman HU. Primary angiitis of the central nervous system. J R Soc Med 2000;93(11):586-8.

Kumar R, Wijdicks EF, Brown RD Jr, Parisi JE, Hammond CA. Isolated angiitis of the CNS presenting as subarachnoid haemorrhage. J Neurol Neurosurg Psychiatry 1997;62(6):649-51.

Wijdicks EF, Manno EM, Fulgham JR, Giannini C. Cerebral angiitis mimicking posterior leukoencephalopathy. J Neurol 2003;250(4):444-8.

Aviv RI, Benseler SM, Silverman ED, Tyrrell PN, Deveber G, Tsang LM, Armstrong D. MR imaging and angiography of primary CNS vasculitis of childhood. AJNR Am J Neuroradiol 2006;27(1):192-9.

Collie DA, Summers DM, Sellar RJ, Ironside JW, Cooper S, Zeidler M, Knight R, Will RG. Diagnosing variant Creutzfeldt-Jakob disease with the pulvinar sign: MR imaging findings in 86 neuropathologically confirmed cases. AJNR Am J Neuroradiol 2003;24(8):1560-9.

Schmidley JW. Isolated CNS angiitis: pathology. In: Schmidley JW, editor. Central Nervous System Angiitis. Woburn: Butterworth-Heine- mann; 2000. p. 1-28.

Fazekas F, Kleinert R, Roob G, Kleinert G, Kapeller P, Schmidt R, Hartung HP. Histopathologic analysis of foci of signal loss on gradient-echo T2*-weighted MR images in patients with spontaneous intracerebral hemorrhage: evidence of microangiopathy-related microbleeds. AJNR Am J Neuroradiol 1999;20(4):637-42.

Vollmer TL, Guarnaccia J, Harrington W, Pacia SV, Petroff OA. Idiopathic granulomatous angiitis of the central nervous system. Diagnostic challenges. Arch Neurol 1993;50(9):925-30.

Kadkhodayan Y, Alreshaid A, Moran CJ, Cross DT 3rd, Powers WJ, Derdeyn CP. Primary angiitis of the central nervous system at conventional angiography. Radiology 2004;233(3):878-82.

Calabrese LH, Mallek JA. Primary angiitis of the centralnervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine (Baltimore) 1988;67(1):20-39.

Scolding NJ, Joseph F, Kirby PA, Mazanti I, Gray F, Mikol J, Ellison D, Hilton DA, Williams TL, MacKenzie JM, Xuereb JH, Love S. Abeta-related angiitis: primaryangiitis of the central nervous system associated with cerebral amyloid angiopathy. Brain 2005;128(Pt 3):500-15.

Schmidley JW, Beadle BA, Trigg L. Co-occurrence of CADASIL and isolated CNS angiitis. Cerebrovasc Dis 2005;19(5):352-4.

Ducros A, Boukobza M, Porcher R, Sarov M, Valade D, Bousser MG. The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. Brain 2007;130(Pt 12):3091-101.

How to Cite
1.
Pourmahmoodian H, Ghelichnia Omrani HA, Harrirchian MH, Ghabaee M, Zamani B, Ghaffarpour M. Primary Angiitis of the Central Nervous System. Acta Med Iran. 50(3):216-221.
QRcode
Section
Articles