"When should primary angiitis of the central nervous system (PACNS) be suspected?": literature review and proposal of a preliminary screening algorithm.
CNS vasculitis
Cerebral angiography
Magnetic resonance imaging
PACNS
Primary angiitis of the CNS
Journal
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
ISSN: 1590-3478
Titre abrégé: Neurol Sci
Pays: Italy
ID NLM: 100959175
Informations de publication
Date de publication:
Nov 2020
Nov 2020
Historique:
received:
09
01
2020
accepted:
05
07
2020
pubmed:
11
8
2020
medline:
15
5
2021
entrez:
11
8
2020
Statut:
ppublish
Résumé
Primary angiitis of the CNS (PACNS) is a process causing variously combined neurological disturbances. Its rarity and kaleidoscopic presentation make it difficult to diagnose and even to suspect. (1) To provide an up-to-date review on PACNS and (2) to create a preliminary screening algorithm based on clinical and radiological first-level data, useful to suspect PACNS and guide further investigations. Review of PUBMED case series on PACNS, published from 2002 to 2017, collection of frequencies of clinical and neuroimaging features and calculation of median values. Classification of features as "major" or "minor" if frequency was higher or lower than median value. Combination of features in sets of criteria represented by all possible combinations of major and minor clinical and neuroradiological features. Application of criteria to published PACNS case reports and selection of the ones best identifying patients with definite PACNS. We reviewed 24 case series. "Major" clinical features were headache, stroke, cognitive impairment, focal neurological deficits; "minor" were seizures, altered consciousness, psychiatric disorders. "Major" neuroradiological features were multiple parenchymal lesions, parenchymal/meningeal contrast enhancement, magnetic resonance angiography vessel abnormalities, vessel wall enhancement; "minor" were parenchymal/subarachnoid hemorrhage, single parenchymal lesion. The selected sets of criteria able to identify all PACNS patients were (1) one clinical (major/minor) + one major neuroradiological feature; and (2) Two clinical (≥ 1 major) + one minor neuroradiological feature. Our review provides a detailed clinical/neuroradiological picture of PACNS. The proposed algorithm should be regarded as a preliminary screening tool to move the first steps towards PACNS diagnosis that needs validation.
Sections du résumé
BACKGROUND
BACKGROUND
Primary angiitis of the CNS (PACNS) is a process causing variously combined neurological disturbances. Its rarity and kaleidoscopic presentation make it difficult to diagnose and even to suspect.
OBJECTIVE
OBJECTIVE
(1) To provide an up-to-date review on PACNS and (2) to create a preliminary screening algorithm based on clinical and radiological first-level data, useful to suspect PACNS and guide further investigations.
METHODS
METHODS
Review of PUBMED case series on PACNS, published from 2002 to 2017, collection of frequencies of clinical and neuroimaging features and calculation of median values. Classification of features as "major" or "minor" if frequency was higher or lower than median value. Combination of features in sets of criteria represented by all possible combinations of major and minor clinical and neuroradiological features. Application of criteria to published PACNS case reports and selection of the ones best identifying patients with definite PACNS.
RESULTS
RESULTS
We reviewed 24 case series. "Major" clinical features were headache, stroke, cognitive impairment, focal neurological deficits; "minor" were seizures, altered consciousness, psychiatric disorders. "Major" neuroradiological features were multiple parenchymal lesions, parenchymal/meningeal contrast enhancement, magnetic resonance angiography vessel abnormalities, vessel wall enhancement; "minor" were parenchymal/subarachnoid hemorrhage, single parenchymal lesion. The selected sets of criteria able to identify all PACNS patients were (1) one clinical (major/minor) + one major neuroradiological feature; and (2) Two clinical (≥ 1 major) + one minor neuroradiological feature.
CONCLUSION
CONCLUSIONS
Our review provides a detailed clinical/neuroradiological picture of PACNS. The proposed algorithm should be regarded as a preliminary screening tool to move the first steps towards PACNS diagnosis that needs validation.
Identifiants
pubmed: 32776287
doi: 10.1007/s10072-020-04583-3
pii: 10.1007/s10072-020-04583-3
pmc: PMC7567723
doi:
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
3135-3148Commentaires et corrections
Type : ErratumIn
Références
Hajj-Ali R, Furlan A, Abou-Chebel A, Calabrese L (2002) Benign angiopathy of the central nervous system: cohort of 16 patients with clinical course and long-term followup. Arthritis Rheum 47:662–669
pubmed: 12522842
Calabrese LH, Mallek JA (1988) Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine (Baltimore) 67:20–39
Salvarani C, Brown RD, Calamia KT, Christianson TJH, Weigand SD, Miller DV, Giannini C, Meschia JF, Huston J, Hunder GG (2007) Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol 62:442–451
pubmed: 17924545
Twilt M, Benseler SM (2012) The spectrum of CNS vasculits in children and adults. Nat Rev Rheumatol 8:97–107
Volcy M, Toro ME, Uribe CS, Toro G (2004) Primary angiitis of the central nervous system: report of five biopsy-confirmed cases from Colombia. J Neurol Sci 227:85–89
pubmed: 15546596
MacLaren K, Gillespie J, Shrestha S et al (2005) Primary angiitis of the central nervous system: emerging variants. Q J Med 98:643–654
Kuker W, Gaertner S, Naegle T et al (2008) Vessel wall contrast enhancement: a diagnostic sign of cerebral vasculitis. Cerebrovasc Dis 26:23–29
pubmed: 18511868
pmcid: 2813800
Molloy ES, Singhal AB, Calabrrese LH (2008) Tumor-like mass-lesion:an under-recognised presentation of primary angiitis of the central nervous system. Ann Rheum Dis 67:1732–1735
pubmed: 18625623
Lee Y, Kim J, Kim E, Park SH, Yim YJ, Sohn CH, Chang KH (2009) Tumor-mimicking primary angiitis of the central nervous system: initial and follow-up MR features. Neuroradiology 51:651–659
pubmed: 19529928
Kraemer M, Berlit P (2011) Primary central nervous system vasculitis: clinical experiences with 21 new European cases. RheumatolInt 31:463–472
Pizzanelli C, Catarsi E, Pelliccia V, Cosottini M, Pesaresi I, Puglioli M, Moretti P, Tavoni A (2011) Primary angiitis of the central nervous system: report of eight cases from a single Italian center. J Neurol Sci 307:69–73
pubmed: 21636100
Nèel A, Auffray-Calvier E, Guillon B et al (2012) Challenging the diagnosis of primary angiitis of the central nervous system: a single-center retrospective study. J Rheumatol 39:1026–1034
pubmed: 22467936
Pfefferkorn T, Linn J, Habs M et al (2012) Black blood MRI in suspected large artery primary angiitis of the central nervous system. J Neuroimaging XX:1–5
Pourmahmoodian H, Ali GOH, Harrirchian MH et al (2012) Primary angiitis of the central nervous system. Acta MedicaIranica 50:216–221
Coronel-Restrepo N, Bonilla-Abadía F, Cortes OA et al (2013) Primary angiitis of the central nervous system: a report of three cases from a single colombian center. Case Rep Neurol Med 2013:940438
pubmed: 23738167
pmcid: 3664475
Cosottini M, Canovetti S, Pesaresi I, Desideri I, Pizzanelli C, Catarsi E, Puglioli M, Tavoni A, Bonuccelli U, Bartolozzi C (2013) 3-T magnetic resonance angiography in primary angiitis of the central nervous system. J Comput Assist Tomogr 37:493–498
pubmed: 23863522
Geri G, Saadoun D, Guillevin R, Crozier S, Lubetzki C, Mokhtari K, Amoura Z, Wechsler B, le Boutin D, Costedoat-Chalumeau N, Samson Y, Cacoub P (2014) Central nervous system angiitis: a series of 31 patients. Clin Rheumatol 33:105–110
pubmed: 24096639
Oon S, Roberts C, Gorelik A, Wicks I, Brand C (2013) Primary angiitis of the central nervous system: experience of a Victorian tertiary-referral hospital. Intern Med J 43:685–692
pubmed: 23176349
Vera-Lastra O, Sepúlveda-Delgado J, Cruz-DomínguezMdel P et al (2015) Primary and secondary central nervous system vasculitis: clinical manifestations, laboratory findings, neuroimaging, and treatment analysis. Clin Rheumatol 34:729–738
pubmed: 25425493
Salvarani C, Brown RD Jr, Christianson TJ et al (2015) Adult primary central nervous system vasculitis treatment and course: analysis of one hundred sixty-three patients. Arthritis Rheum 67:1637–1645
Singhal AB, Topcuoglu MA, Fok JW, Kursun O, Nogueira RG, Frosch MP, Caviness VS Jr (2016) Reversible cerebral vasoconstriction syndromes and primary angiitis of the central nervous system: clinical, imaging, and angiographic comparison. Ann Neurol 79:882–894
pubmed: 27043703
Becker J, Horn PA, Keyvani K, Metz I, Wegner C, Brück W, Heinemann FM, Schwitalla JC, Berlit P, Kraemer M (2017) Primary central nervous system vasculitis and its mimicking diseases - clinical features, outcome, comorbidities and diagnostic results - a case control study. Clin Neurol Neurosurg 156:48–54
pubmed: 28324788
De Boysson H, Boulouis G, Aouba A et al (2017) Adult primary angiitis of the central nervous system: isolated small-vessel vasculitis represents distinct disease pattern. Rheumatology (Oxford) 56:439–444
De Boysson H, Zuber M, Naggara O et al (2014) 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. Arthritis Rheum 66:1315–1326
Niu L, Wang L, Yin X, Li XF, Wang F (2017) Role of magnetic resonance imaging in the diagnosis of primary central nervous system angiitis. Exp Ther Med 14:555–560
pubmed: 28672966
pmcid: 5488425
Schuster S, Bachmann H, Thom V, Kaufmann-Buehler AK, Matschke J, Siemonsen S, Glatzel M, Fiehler J, Gerloff C, Magnus T, Thomalla G (2017) Subtypes of primary angiitis of the CNS identified by MRI patterns reflect the size of affected vessels. J Neurol Neurosurg Psychiatry 88:749–755
pubmed: 28705900
Peng LJ, Qian HR, Mao LL, Xia DY, Qi XK (2017) A clinical analysis of 5 patients with infratentorial primary angiitis of central nervous system. ZhonghuaNeiKe Za Zhi 56:284–289
Harsha KJ, Jagtap SA, Kapilamoorthy TR, Kesavadas C, Thomas B, Radhakrishnan N (2017) CNS small vessel vasculitis: distinct MRI features and histopathological correlation. Neurol India 65:1291–1294
pubmed: 29133703
Al Share B, Zakaria A, Hiner E, Iskenderian Z, Warra N (2017) Primary angiitis of the center nervous system: a clinical challenge diagnosed postmortem. Case Rep Neurol Med 2017:3870753
pubmed: 28758039
pmcid: 5516732
Kumar PP, Rajesh A, Kandadai RM, Purohit AK, Sundaram C (2017) Primary CNS vasculitis masquerading as glioblastoma: a case report and review. Asian J Neurosurg 12:69–71
pubmed: 28413538
pmcid: 5379810
Takahashi K, Sato H, Hattori H, Takao M, Takahashi S, Suzuki N (2017) Case report of a 28-year-old male with the rapid progression of steroid-resistant central nervous system vasculitis diagnosed by a brain biopsy. RinshoShinkeigaku 57:509–514
Lee JS, Jung TY, Lee KH, Kim SK (2017) Primary central nervous system vasculitis mimicking a cortical brain tumor: a case report. Brain Tumor Res Treat 5:30–33
pubmed: 28516076
pmcid: 5433948
Arif S, Liaqat J, Nawaz KH, Hashmat A (2017) Focal hemispheric central nervous system vasculitis: an unusual form of primary angiitis. J Ayub Med Coll Abbottabad 29:367
pubmed: 28718271
Mizuno Y, Shigeto H, Yamada T, Maeda N, Suzuki SO, Kira J (2016) A case of primary central nervous system vasculitis diagnosed by second brain biopsy and treated successfully. RinshoShinkeigaku. 56:186–190
Sun LI, Zhu L, Zhao T, Wang D, Ma D, Zhang R, Fang S (2016) A rare case of tumor-mimicking primary angiitis of the central nervous system. Mol Clin Oncol 4:827–829
pubmed: 27123289
pmcid: 4840726
Benson CE, Knezevic A, Lynch SC (2016) Primary central nervous system vasculitis with optic nerve involvement. J Neuroophthalmol 36:174–177
pubmed: 26693942
Pillai SH, Sreedharan SE, Menon G, Kannoth S, Pn S (2016) Primary CNS vasculitis presenting as intraventricular bleeding. Ann Indian Acad Neurol 19:406–408
pubmed: 27570401
pmcid: 4980972
Fang CW, Chen YC, Liao IC, Lin CC (2015) Primary granulomatous angiitis of the central nervous system with amyloid angiopathy: a case report and literature review. Neurologist. 19:73–78
pubmed: 25692513
Kim S, Kim DK (2015) Psychosis in primary angiitis of the central nervous system involving bilateral thalami: a case report. Gen Hosp Psychiatry 37:275.e1–275.e3
Bajaj BK, Pandey S, Ramanujam B, Wadhwa A (2015) Primary angiitis of central nervous system: the story of a great masquerader. J Neurosci Rural Pract 6:399–401
pubmed: 26167025
pmcid: 4481796
Kim SI, Kim SH, Cho HJ, Kim H, Chung CK, Choi SH, Park SH (2015) Mass-forming primary angiitis of central nervous system with Rosai-Dorfmann disease-like massive histiocytosis with emperipolesis. Pathol Int 65:420–425
pubmed: 26044211
Killeen T, Jucker D, Went P, Muthurajah V, Woon K, Cesnulis E, Czaplinski A (2015) Solitary tumour-like mass lesions of the central nervous system: primary angiitis of the CNS and inflammatory pseudotumour. Clin Neurol Neurosurg 135:34–37
pubmed: 26010394
Gan C, Maingard J, Giles L, Phal PM, Tan KM (2015) Primary angiitis of the central nervous system presenting as a mass lesion. J Clin Neurosci 22:1528–1531
pubmed: 26119979
Huang M, Steele WJ, Baskin DS (2015) Primary central nervous system vasculitis preceded by granulomatous hypophysitis: case report with a review of the literature. Surg Neurol Int 6(Suppl 16):S407–S413
pubmed: 26539311
pmcid: 4597298
Okunomiya T, Kageyama T, Tanaka K, Kambe D, Shinde A, Suenaga T (2014) Lymphocytic primary angiitis of the central nervous system with fan-shaped linear enhancement converging to the lateral ventricles: a case report. RinshoShinkeigaku. 54:709–714
Safouris A, Stricker J, Michotte A, Voumvourakis K, Gazagnes MD, Tsivgoulis G (2014) Biopsy-proven fulminant primary angiitis of the central nervous system with normal arteriography: a challenging diagnosis of recurrent ischemic strokes. Neurol Sci 35:135–137
pubmed: 23995894
Michiels V, Bissay V, Michotte A (2014) Neuropsychological functioning in a patient with primary angiitis of the CNS. J Neuropsychiatr Clin Neurosci 26:E23
Gaillard N, Bertrand JL, Dumitrana A, Sablot D (2014) Microaneurysms in primary angiitis of the central nervous system revealed by MRI. Cerebrovasc Dis 38:308–310
pubmed: 25412995
Rao NM, Prasad PS, Flippen CC 2nd et al (2014) Primary angiitis of the central nervous system presenting as unilateral optic neuritis. J Neuroophthalmol 34:380–385
pubmed: 25409482
Noh HJ, Choi JW, Kim JP, Moon GJ, Bang OY (2014) Role of high-resolution magnetic resonance imaging in the diagnosis of primary angiitis of the central nervous system. J Clin Neurol 10:267–271
pubmed: 25045382
pmcid: 4101106
Orr SL, Dos Santos MP, Jurencak R, Michaud J, Miller E, Doja A (2014) Central nervous system venulitis presenting as migraine. Headache. 54:541–544
pubmed: 23879203
Lyra TG, Martin Mda G, Carvalho Rdo C et al (2013) Pseudotumoral presentation of primary central nervous system vasculitis. ArqNeuropsiquiatr. 71:333–335
Rosenberg J, Mahta A, Koppula K, Borys E, Kesari S (2013) Cyclophosphamide responsive primary angiitis of the CNS in a 61-year-old female. Clin Neuropathol 32(1):66–68
pubmed: 23006473
Okeda R, Ito K, Tsumura K, Eishi Y (2013) Primary granulomatous angiitis of the CNS preferentially involving small veins with a granulomatous leukoencephalitis-like lesion in the cerebrum. Neuropathology. 33:547–552
pubmed: 23279573
Yu XL, Liu AF, Ma L, Yan CZ, Zhao YY, Shan PY (2011) Primary angiitis of the central nervous system: a case report. Chin Med J 124:2782–2785
pubmed: 22040443
Ghavanini AA, Munoz DG (2011) Primary cerebral angiitis associated with amyloid angiopathy. Arch Neurol 68:1202–1203
pubmed: 21911703
Ho MG, Chai W, Vinters HV, Hathout G, Mishra S, Yim C, Valdes-Sueiras M, Nishimura R (2011) Unilateral hemispheric primary angiitis of the central nervous system. J Neurol 258:1714–1716
pubmed: 21416209
pmcid: 3165118
Tanei T, Nakahara N, Takebayashi S, Ito M, Hashizume Y, Wakabayashi T (2011) Primary angiitis of the central nervous system mimicking tumor-like lesion--case report. Neurol Med Chir (Tokyo) 51:56–59
Pires C, Foreid H, Barroso C, Ferro JM (2011) Rapidly progressive dementia due to leukocytoclastic vasculitis of the central nervous system. BMJ Case Rep
John S, Hajj-Ali RA (2014) CNS vasculitis. Semin Neurol 34:405–412
pubmed: 25369436
Carandang CG, Grant AL (2008) Delirium and isolated angiitis of the central nervous system: a case report and review. CNS Spectrums 13:209–213
pubmed: 18323754
Diamanti S, Longoni M, Agostoni EC (2019) Leading symptoms in cerebrovascular diseases: what about headache? Neurol Sci 40(Suppl 1):147–152
pubmed: 30891639
Mandal J, Chung SA (2017) Primary angiitis of the central nervous system. Rheum Dis Clin N Am 43:503–518
Beuker C, Schmidt A, Strunk D, Sporns PB, Wiendl H, Meuth SG, Minnerup J (2018) Primary angiitis of the central nervous system: diagnosis and treatment. Ther Adv Neurol Disord 11:175628641878507