123
Views
0
CrossRef citations to date
0
Altmetric
REVIEW

Primary Angiitis of the Central Nervous System in Adults: A Comprehensive Review of 76 Biopsy-Proven Case Reports

ORCID Icon, ORCID Icon &
Pages 5083-5094 | Received 03 Sep 2023, Accepted 31 Oct 2023, Published online: 06 Nov 2023

References

  • Harbitz F. Unknown forms of arteritis, with special reference to their relation to syphilitic arteritis and periarteritis nodosa. Am J Med Sci. 1922;163(2):250–271. doi:10.1097/00000441-192202000-00010
  • Calabrese LH, Mallek JA. Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine. 1988;67(1):20–39. doi:10.1097/00005792-198801000-00002
  • Scolding NJ, Joseph F, Kirby PA, et al. Abeta-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy. Brain. 2005;128(Pt 3):500–515. doi:10.1093/brain/awh379
  • Gandham EJ, Patel B, Mathew V, Raju KP. Primary central nervous system angiitis mimicking a space-occupying lesion. Indian J Neurosurg. 2022. doi:10.1055/s-0042-1743263
  • Miller DV, Salvarani C, Hunder GG, et al. Biopsy findings in primary angiitis of the central nervous system. Am J Surg Pathol. 2009;33(1):35–43. doi:10.1097/PAS.0b013e318181e097
  • Salvarani C, Brown RD, Christianson TJH, Huston J, Giannini C, Hunder GG. Long-term remission, relapses and maintenance therapy in adult primary central nervous system vasculitis: a single-center 35-year experience. Autoimmun Rev. 2020;19(4):102497. doi:10.1016/j.autrev.2020.102497
  • de Boysson H, Parienti JJ, Mawet J, et al. Primary angiitis of the CNS and reversible cerebral vasoconstriction syndrome: a comparative study. Neurology. 2018;91(16):e1468–e1478. doi:10.1212/WNL.0000000000006367
  • Beuker C, Strunk D, Rawal R, et al. Primary angiitis of the CNS: a systematic review and meta-analysis. Neurol Neuroimmunol Neuroinflamm. 2021;8(6):e1093. doi:10.1212/NXI.0000000000001093
  • Campi A, Benndorf G, Martinelli V, Terreni MR, Scotti G. Spinal cord involvement in primary angiitis of the central nervous system: a report of two cases. Am J Neuroradiol. 2001;22(3):577–582.
  • Iwase T, Ojika K, Mitake S, et al. Involvement of CD45RO+T lymphocyte infiltration in a patient with primary angiitis of the central nervous system restricted to small vessels. Eur Neurol. 2001;45(3):184–185. doi:10.1159/000052120
  • Ay H, Sahin G, Saatci I, Soylemezoglu F, Saribas O. Primary angiitis of the central nervous system and silent cortical hemorrhages. Am J Neuroradiol. 2002;23(9):1561–1563.
  • Hassan AS, Trobe JD, McKeever PE, Gebarski SS. Linear magnetic resonance enhancement and optic neuropathy in primary angiitis of the central nervous system. J Neuro Ophthalmol. 2003;23(2):127–131. doi:10.1097/00041327-200306000-00004
  • Schwab P, Lidov HGW, Schwartz RB, Anderson RJ. Cerebral amyloid angiopathy associated with primary angiitis of the central nervous system: report of 2 cases and review of the literature. Arthritis Rheum. 2003;49(3):421–427. doi:10.1002/art.11049
  • Paisansinsup T, Manno EM, Moder KG. Cauda equina syndrome as a clinical presentation of primary angiitis of the central nervous system (PACNS). JCR. 2004;10(5):265–268. doi:10.1097/01.rhu.0000141508.17269.8e
  • Lukas C, Keyvani K, Bornke C. Primary angiitis of the central nervous system presenting with subacute and fatal course of disease: a case report. BMC Neurol. 2005;5(1):4. doi:10.1186/1471-2377-5-16
  • 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):622–626. doi:10.1016/j.surneu.2006.01.023
  • Arias M, Osorio XR, Dapena D, Arias-Rivas S, Vazquez F. Recurrent leukoencephalopathy with microhemorrhages: gradient-echo MRI study diagnostic value in CNS primary angiitis. Mult Scler J. 2008;14(8):1139–1141. doi:10.1177/1352458508094642
  • Duhon B, Renner D, Jensen R. Primary CNS angiitis presenting as short-term memory loss: a case report and literature review. Int J Rheum Dis. 2008;11(3):303–307. doi:10.1111/j.1756-185X.2008.00370.x
  • Nabika S, Kiya K, Satoh H, et al. Primary angiitis of the central nervous system mimicking dissemination from brainstem neoplasm: a case report. Surg Neurol. 2008;70(2):182–185. doi:10.1016/j.surneu.2007.05.008
  • Salvarani C, Brown RD, Calamia KT, et al. Efficacy of tumor necrosis factor a blockade in primary central nervous system vasculitis resistant to immunosuppressive treatment. Arthritis Rheum. 2008;59(2):291–296. doi:10.1002/art.23337
  • Chenevier F, Renoux C, Marignier R, et al. Primary angiitis of the central nervous system: response to mycophenolate mofetil. J Neurol Neurosurg Psychiatry. 2009;80(10):1159–1161. doi:10.1136/jnnp.2008.154567
  • Qu SB, Khan S, Liu H. Primary central nervous system vasculitis mimicking brain tumour: case report and literature review. Rheumatol Int. 2009;30(1):127–134. doi:10.1007/s00296-009-0914-7
  • Kinsella JA, O’Brien W, Mullins GM, Brewer J, Whyte S. Primary angiitis of the central nervous system with diffuse cerebral mass effect and giant cells. J Clin Neurosci. 2010;17(5):674–676. doi:10.1016/j.jocn.2009.09.031
  • Morishige M, Abe T, Kamida T, et al. Cerebral vasculitis associated with amyloid angiopathy-case report. Neurol Med Chir. 2010;50(4):336–338. doi:10.2176/nmc.50.336
  • Rigby H, Easton A, Bhan V. Amyloid beta-related angiitis of the central nervous system: report of 3 cases. Can J Neurol Sci. 2011;38(4):626–630. doi:10.1017/S0317167100012178
  • Tanei T, Nakahara N, Takebayashi S, Ito M, Hashizume Y, Wakabayashi T. Primary angiitis of the central nervous system mimicking tumor-like lesion -case report. Neurol Med Chir. 2011;51(1):56–59. doi:10.2176/nmc.51.56
  • Coronel-Restrepo N, Bonilla-Abadia F, Cortes OA, et al. Primary angiitis of the central nervous system: a report of three cases from a single Colombian center. Case Rep Neurol Med. 2013;2013:4. doi:10.1155/2013/940438
  • Hirano K, Fukae J, Hieda S, et al. Eosinophilic Meningitis Caused by Primary Angiitis of the Central Nervous System. Intern Med. 2013;52(12):1393–1396. doi:10.2169/internalmedicine.52.9578
  • Muccio CF, Di Blasi A, Esposito G, Brunese L, D’Arco F, Caranci F. Perfusion and spectroscopy magnetic resonance imaging in a case of lymphocytic vasculitis mimicking brain tumor. Polish J Radiol. 2013;78(3):66–69. doi:10.12659/PJR.884011
  • Rao NM, Prasad PS, Flippen CC, et al. Primary angiitis of the central nervous system presenting as unilateral optic neuritis. J Neuro Ophthalmol. 2014;34(4):380–385. doi:10.1097/WNO.0000000000000147
  • Shiner EA, Zagami AS. An illustrative case of primary angiitis of the central nervous system. SAGE Open Med Case Rep. 2014;2:4. doi:10.1177/2050313X14559638
  • Bajaj BK, Pandey S, Ramanujam B, Wadhwa A. Primary angiitis of central nervous system: the story of a great masquerader. J Neurosci Rural Pract. 2015;6(3):399–+. doi:10.4103/0976-3147.158781
  • Fang CW, Chen YC, Liao IC, Lin CCK. Primary granulomatous angiitis of the central nervous system with amyloid angiopathy a case report and literature review. Neurologist. 2015;19(3):73–78. doi:10.1097/NRL.0000000000000014
  • Killeen T, Jucker D, Went P, et al. Solitary tumour-like mass lesions of the central nervous system: primary angiitis of the CNS and inflammatory pseudotumour. Clin Neurol Neurosurg. 2015;135:34–37. doi:10.1016/j.clineuro.2015.05.008
  • Kim S, Kim DK. Psychosis in primary angiitis of the central nervous system involving bilateral thalami: a case report. Gen Hosp Psychiatry. 2015;37(3):3. doi:10.1016/j.genhosppsych.2015.03.006
  • Kim SI, Kim SH, Cho HJ, et al. Mass-forming primary angiitis of central nervous system with Rosai-Dorfmann disease-like massive histiocytosis with emperipolesis. Pathol Int. 2015;65(8):420–425. doi:10.1111/pin.12317
  • Benson CE, Knezevic A, Lynch SC. Primary central nervous system vasculitis with optic nerve involvement. J Neuro Ophthalmol. 2016;36(2):174–177. doi:10.1097/WNO.0000000000000328
  • Bonstrup M, Ott K, Glatzel M, Magnus T. Frontal lobe dementia syndrome as a first manifestation of primary angiitis of the central nervous system (PACNS). Clin Neurol Neurosurg. 2016;141:92–94. doi:10.1016/j.clineuro.2015.12.016
  • Sun L, Zhu LJ, Zhao T, et al. A rare case of tumor-mimicking primary angiitis of the central nervous system. Mol Clin Oncol. 2016;4(5):827–829. doi:10.3892/mco.2016.784
  • Al Share B, Zakaria A, Hiner E, Iskenderian Z, Warra N. Primary angiitis of the center nervous system: a clinical challenge diagnosed postmortem. Case Rep Neurol Med. 2017;2017:4. doi:10.1155/2017/3870753
  • Fisher A, Rahman H, Farrell M, Hennessy M. Progressive fatal myelopathy secondary to isolated spinal cord vasculitis. Front Neurol. 2017;8:4. doi:10.3389/fneur.2017.00705
  • Matar RK, Alshamsan B, Alsaleh S, et al. New onset refractory status epilepticus due to primary angiitis of the central nervous system. Epilepsy Behav Case Rep. 2017;8:100–104. doi:10.1016/j.ebcr.2017.07.005
  • Zhu DS, Yang XL, Lv HH, et al. Seizure syndrome as a first manifestation of solitary tumor-like mass lesion of PACNS Two case reports. Medicine. 2017;96(9):5.
  • Al-Sharydah AM, Al-Abdulwahhab AH, Al-Suhibani SS, Al-Issawi WM, Al-Safran FS. Primary central nervous system vasculitis disguised as tumor-like granulomatous angiitis and multifocal subdural hematomas: a case report and literature review. Interdiscip Neurosurg. 2018;13:119–123. doi:10.1016/j.inat.2018.05.002
  • He DA, Cai G, Li Y, et al. Primary angiitis of the central nervous system mimicking sporadic Creutzfeldt-Jakob disease: a case study. Alzheimer Dis Assoc Disord. 2018;32(3):258–261. doi:10.1097/WAD.0000000000000242
  • Caputi L, Erbetta A, Marucci G, et al. Biopsy-proven primary angiitis of the central nervous system mimicking leukodystrophy: a case report and review of the literature. J Clin Neurosci. 2019;64:42–44. doi:10.1016/j.jocn.2019.03.021
  • Jin H, Qu Y, Guo ZN, Cui GZ, Zhang FL, Yang Y. Primary Angiitis of the Central Nervous System Mimicking Glioblastoma: a Case Report and Literature Review. Front Neurol. 2019;10:8. doi:10.3389/fneur.2019.01208
  • Kon T, Funamizu Y, Suzuki C, et al. A long interval from a spinal cord lesion to a subsequent brain lesion in primary central nervous system vasculitis. Intern Med. 2019;58(10):1485–1489. doi:10.2169/internalmedicine.1667-18
  • Leonardi J, Saleh C, Jaszczuk P, et al. Primary angiitis of the central nervous system: from psychiatry to neurology. Case Rep Neurol Med. 2019;2019:4. doi:10.1155/2019/8074258
  • Spence S, Ng D, Casault C. Atypical presentation of fulminant primary central nervous system angiitis. J Neuroimmunol. 2019;330:1–4. doi:10.1016/j.jneuroim.2019.01.019
  • Arif S, Arif S, Liaqat J, Muhammad WW, Palwa AR. Need for swift diagnosis of primary angiitis of central nervous system: a case with focal motor seizures of hand progressing to aphasia. Cureus. 2020;12(10):13.
  • Bernstein JE, Podkovik S, Kashyap S, Ghanchi H, Ananda AK. Primary angiitis of the central nervous system presenting as a cerebral mass lesion: a case report and literature review. Cureus. 2020;12(6):8.
  • Borcheni M, Abdelazeem B, Malik B, Gurugubelli S, Kunadi A. Primary central nervous system vasculitis as an unusual cause of intracerebral hemorrhage: a case report. Cureus. 2021;13(3):8.
  • Su X, Han L, Li MX, et al. Novel method using DW-MRI and ADC images to guide stereotactic biopsy for the diagnosis small primary angiitis of the central nervous system: a case report. Eur J Med Res. 2021;26(1):10. doi:10.1186/s40001-021-00529-3
  • Zhang GD, Yang CX, Chang JJ, et al. Primary angiitis of the central nervous system mimicking a cerebellar tumor. Br J Neurosurg. 2021;35(3):367–369. doi:10.1080/02688697.2018.1464122
  • Sarhan FM, Al-Jasim A, Alaraj RS, Abedalkhader SF, Ghanim Z. Right arm weakness and mouth deviation as a presentation of Primary Angiitis of the Central Nervous System treated with rituximab: a case-report. Ann Med Surg. 2022;79:5. doi:10.1016/j.amsu.2022.104040
  • Tanner JA, Richie MB, Cadwell CR, et al. Amyloid-beta related angiitis presenting as eosinophilic meningitis: a case report. BMC Neurol. 2022;22(1):7. doi:10.1186/s12883-022-02638-w
  • Tamargo RJ, Connolly ES, McKhann GM, et al. Clinicopathological review: primary angiitis of the central nervous system in association with cerebral amyloid angiopathy. Neurosurgery. 2003;53(1):136–143. doi:10.1227/01.NEU.0000068864.20655.31
  • Kumar RS, Singh A, Rathore C, Kesavadas C. Primary angiitis of central nervous system: tumor-like lesion. Neurol India. 2010;58(1):147–149. doi:10.4103/0028-3886.60417
  • Ho MG, Chai WX, Vinters HV, et al. Unilateral hemispheric primary angiitis of the central nervous system. J Neurol. 2011;258(9):1714–1716. doi:10.1007/s00415-011-5993-1
  • De Boysson H, Arquizan C, Guillevin L, Pagnoux C. Rituximab for primary angiitis of the central nervous system: report of 2 patients from the French COVAC cohort and review of the literature. J Rheumatol. 2013;40(12):2102–2104. doi:10.3899/jrheum.130529
  • Lyra TG, Martin MDM, Carvalho RD, et al. Pseudotumoral presentation of primary central nervous system vasculitis. Arq Neuropsiquiatr. 2013;71(5):333–335. doi:10.1590/0004-282X20130032
  • Pizzanelli C, Tavoni A, Pelliccia V, et al. Multiple life-threatening relapses in a woman with primary angiitis of the central nervous system mimicking brain tumour: a case report. Clin Exp Rheumatol. 2014;32(2):S143–S144.
  • Safouris A, Stricker J, Michotte A, Voumvourakis K, Gazagnes MD, Tsivgoulis G. Biopsy-proven fulminant primary angiitis of the central nervous system with normal arteriography: a challenging diagnosis of recurrent ischemic strokes. Neurol Sci. 2014;35(1):135–137. doi:10.1007/s10072-013-1528-0
  • Salvarani C, Brown RD, Morris JM, Huston J, Hunder GG. Unilateral chronic relapsing primary central nervous system vasculitis. Clin Exp Rheumatol. 2014;32(2):S139–S140.
  • Moussaddy A, Levy A, Strbian D, Sundararajan S, Berthelet F, Lanthier S. Inflammatory cerebral amyloid angiopathy, amyloid-beta-related angiitis, and primary angiitis of the central nervous system similarities and differences. Stroke. 2015;46(9):E210–E213. doi:10.1161/STROKEAHA.115.010024
  • Burghaus L, Kabbasch C, Deckert M, et al. FET PET in primary central nervous system vasculitis. Clin Nucl Med. 2018;43(9):E322–E323. doi:10.1097/RLU.0000000000002197
  • Chu L, Eustace M, Pittman N. Primary angiitis of the central nervous system presenting with headache and ataxia. Can J Neurol Sci. 2018;45(5):583–584. doi:10.1017/cjn.2018.264
  • Van Rooij JLM, Rutgers DR, Spliet WGM, Frijns CJM. Vessel wall enhancement on MRI in the diagnosis of primary central nervous system vasculitis. Int J Stroke. 2018;13(9):NP24–NP27. doi:10.1177/1747493018789276
  • Wang CR, Peng SL, Huang HW. Isolated cervical cord involvement in primary central nervous system vasculitis. J Rheumatol. 2018;45(7):962–963. doi:10.3899/jrheum.171212
  • Moseley BD, Smith JH, Dhamija RR, Jones LK. Primary angiitis of the central nervous system presenting with microhemorrhages on gradient echo imaging. Neurol India. 2019;67(5):1374–1375. doi:10.4103/0028-3886.271267
  • Agarwal A, Reddy PS, Vishnu VY, Garg A. Primary central nervous system vasculitis presenting with isolated headache. J Neurosci Rural Pract. 2020;11(04):678–679. doi:10.1055/s-0040-1715999
  • Messmer B, Butts M. Relapsing primary central nervous system vasculitis treated with rituximab. J Clin Rheumatol. 2020;26(6):E206–E207. doi:10.1097/RHU.0000000000001075
  • Muccio CF, Tedeschi E, Elefante A, Caranci F, Cerase A. Primary central nervous system vasculitis mimicking a brain tumor on conventional magnetic resonance imaging: the usefulness of perfusion-weighted imaging. A case report. Acta Neurol Belg. 2020;120(2):475–477. doi:10.1007/s13760-018-0907-y
  • Paramasivan NK, Sundaram S, Sharma DP, Sreedharan SE, Sylaja PN. Rituximab for refractory primary angiitis of the central nervous system: experience in two patients. Mult Scler Relat Disord. 2021;51:3. doi:10.1016/j.msard.2021.102907
  • Santyr B, Pejhan S, Zhang Q, Budhram A. Unilateral primary angiitis of the central nervous system. Ann Neurol. 2021;90(6):999–1000. doi:10.1002/ana.26234
  • Han X, Pang Z, Wang Z, Xu S, Lin Y. A case of primary angiitis of the central nervous system presenting with diffuse cerebral microbleeds and recurrent intracranial hemorrhage. Neurol Sci. 2019;40(2):417–419. doi:10.1007/s10072-018-3595-8
  • Salvarani C, Hunder GG, Morris JM, Brown RD, Christianson T, Giannini C. Aβ-related angiitis: comparison with CAA without inflammation and primary CNS vasculitis. Neurology. 2013;81(18):1596–1603. doi:10.1212/WNL.0b013e3182a9f545
  • Das S, Goswami RP, Sinha D, Shobhana A, Purkayastha S, Datta A. Mycophenolate mofetil as induction and maintenance immunosuppressive therapy in adult primary central nervous system vasculitis: a prospective observational study. Clin Rheumatol. 2023;42(8):2155–2162. doi:10.1007/s10067-023-06602-y
  • de Boysson H, Boulouis G, Aouba A, et al. Adult primary angiitis of the central nervous system: isolated small-vessel vasculitis represents distinct disease pattern. Rheumatology. 2017;56(3):439–444. doi:10.1093/rheumatology/kew434
  • de Boysson H, Parienti JJ, Arquizan C, et al. Maintenance therapy is associated with better long-term outcomes in adult patients with primary angiitis of the central nervous system. Rheumatology. 2017;56(10):1684–1693. doi:10.1093/rheumatology/kex047
  • de Boysson H, Arquizan C, Touze E, et al. Treatment and long-term outcomes of primary central nervous system vasculitis: updated results from the French Registry. Stroke. 2018;49(8):1946–1952. doi:10.1161/STROKEAHA.118.021878