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Table of Contents    
Year : 2019  |  Volume : 67  |  Issue : 1  |  Page : 113-114

Primary angiitis of the central nervous system – An Indian experience

Department of Neurology, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication7-Mar-2019

Correspondence Address:
Dr. Achal Kumar Srivastava
Department of Neurology, Room No. 60, GF, Neurosciences Center, All India Institute of Medical Sciences, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.253599

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How to cite this article:
Srivastava AK, Reddy Sura PK. Primary angiitis of the central nervous system – An Indian experience. Neurol India 2019;67:113-4

How to cite this URL:
Srivastava AK, Reddy Sura PK. Primary angiitis of the central nervous system – An Indian experience. Neurol India [serial online] 2019 [cited 2022 Nov 30];67:113-4. Available from: https://www.neurologyindia.com/text.asp?2019/67/1/113/253599

Primary angiitis of central nervous system (PACNS) or primary CNS vasculitis is an uncommon and poorly understood disease worldwide, affecting the brain and spinal cord, with the earliest case reported by Harbitz in 1922.[1] In 1988, the diagnostic criteria for PACNS, as suggested by Calabrese and Mallek, included the development of a neurologic deficit unexplained by any other processes, plus the presence of either an angiogram with characteristic features of vasculitis or a CNS biopsy showing vasculitis.[2] Relying on the angiographic findings remains uncertain as many of the nonvasculitic disorders have similar findings.[3] With advances in imaging and awareness of the patients, there is more literature coming up on PACNS, but still there are uncertainties in the clinical features, the response to therapy, and long-term outcomes.[4] There is no literature available that defines the clinical spectrum and long-term outcomes in any Indian cohort.

“Primary angiitis of the CNS clinical profile and outcomes in 45 patients” is the first study of Indian cohort with CNS vasculitis. The largest two cohorts with PACNS were from Mayo Clinic and a French cohort.[5],[6] In the Mayo Clinic series, the incidence of primary CNS vasculitis in the United States was estimated to be two to four cases per 10,00,000 person-years. Most of the studies did not show much gender difference.[5] The median age at diagnosis was 50 years, and 50% of the patients were between 37 and 59 years of age. The nature of inflammatory infiltrate in primary CNS vasculitis is T-cell mediated. Immunohistochemical staining of a biopsy sample shows predominant infiltration by cluster of differentiation (CD) 45R0+ T cells in and around small cerebral vessels.[7] Three types of histological patterns had been reported in PACNS –the granulomatous, lymphocytic, and necrotizing pattern. In the Mayo Clinic cohort, the most common type of histological pattern seen was the granulomatous one, and in the French cohort, the most common pattern was the lymphocytic pattern.[5],[6] In the present cohort from India, most of the patients showed a lymphocytic pattern.

The clinical manifestations at the diagnosis are variable with most of the patients presenting with headaches, focal neurological deficits, and cognitive deficits, as seen in the Mayo group.[5] Intracerebral hemorrhage as presentation was seen in 11%–12% of cases in the Mayo Clinic cohort,[5] and the present cohort had shown it in around 8% of patients. Spinal cord involvement was reported in 5% of the Mayo cohort,[5] whereas in the present cohort, only one person showed any evidence of myelopathy. Cerebrospinal fluid analysis was abnormal in 80%–90% in the Mayo cohort [5] and more than 50% in the French cohort.[6] The changes consist of a mildly increased leucocyte count and total protein concentration. The cerebral angiography supports the diagnosis of PACNS, but the limitation is its low specificity.[2] The suggestive angiographic findings are alternating segments of stenosis with normal or dilated intervening segments and arterial occlusions. Other abnormalities are the presence of delayed arterial emptying and anastomotic channels.[8]

A recent systematic review on the diagnosis of PACNS suggested that when both angiography and biopsy were performed, the disagreement between them was more than five times likely than the agreement. Involvement of the vessels with different calibres in different PACNS subtypes could be an explanation for the poor agreement between the tests. It is still unclear as to how accurately these entities can be defined based on the current criteria.[9] The treatment strategies used in PACNS are derived from the other vasculitides groups. There is no randomized clinical trial on the medical management of PACNS. Rarity of the disease is an obvious reason. Studies, including those by Cupps et al., reported the effectiveness of cyclophosphamide (CYP) in combination with corticosteroids.[10] The recent French cohort group had shown that maintenance therapy with an immunosuppressant combined with glucocorticoids leads to the best long-term clinical and functional outcomes in patients with primary CNS vasculitis after having achieved the remission with either the glucocorticoids alone or in combination with another immunosuppressant.[11] In the Mayo cohort, relapses were more frequent in patients treated with glucocorticoids alone initially, when compared with the combination therapy of glucocorticoids and CYP. In the present cohort, all patients received steroids and 24.4% of patients received CYP along with steroids.

The present cohort is the first large clinical study of PACNS from India. It has addressed the factors which could influence the clinical outcome and long-term relapse rate. The major limitation of the study is its retrospective nature, which is bound to cause certain known problems. Histopathology was not available for all patients as many patients did not agree for a biopsy.[12]

The latest data suggest a more promising role of immunosuppressants in PACNS, but only a few patients in the present cohort received additional immunosuppressant therapy. A clarification on which patients need CYP at the disease onset needs to be established. The role of other immunosuppressants should be studied as substitutes for CYP.

The novel imaging technique of high-resolution magnetic resonance (MR) vessel wall imaging is a very important development which is currently being used to differentiate vasculitis from intracranial atherosclerotic disease. It helps in assessing the vasculitic activity and in selecting the intracranial target for biopsy.

Despite the limitations, this study certainly gives a glimpse of the clinical features, prevailing management patterns, and the outcome of PACNS among Indian patients. Newer vascular MR imaging techniques should be used for securing an improved diagnosis. The role of immunosuppressants other than CYP in the management of PACNS needs to be explored.

  References Top

Harbitz F. Unknown forms of arteritis, with special reference to theirrelation to syphilitic arteritis and periarteritis nodosa. Am J Med Sci 1922;163:250-72.  Back to cited text no. 1
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 (Baltimore) 1988;67:20-39.  Back to cited text no. 2
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;233:878-82.  Back to cited text no. 3
Oon S, Roberts C, Gorelik A, Wicks I, Brand C. Primary angiitis of the central nervous system: Experience of a Victorian tertiary-referral hospital. Intern Med J 2013;43:685-92.  Back to cited text no. 4
Salvarani C, Brown RD Jr, Christianson T, Miller DV, Giannini C, Huston J 3rd, et al. An update of the Mayo Clinic cohort of patients with adult primary central nervous system vasculitis: Description of 163 patients. Medicine (Baltimore) 2015;94:e738.  Back to cited text no. 5
de Boysson H, Zuber M, Naggara O, Neau JP, Gray F, Bousser MG, et al. French Vasculitis Study Group and the French NeuroVascular Society. 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 Rheumatol 2014;66:1315-26.  Back to cited text no. 6
Iwase T, Ojika K, Mitake S, Katada E, Katano H, Mase M, et al. Involvement of CD45RO+ T lymphocyte infiltration in a patient with primary angiitis of thecentral nervous system restricted to small vessels. Eur Neurol 2001;45:184-85.  Back to cited text no. 7
Ferris EJ, Levine HL. Cerebral arteritis: Classification. Radiology 1983;109:327-41.  Back to cited text no. 8
McVerry F, McCluskey G, McCarron P, Muir KW, McCarron MO. Diagnostic test results in primary CNS vasculitis: A systematic review of published cases. Neurol Clin Pract 2017;7:256-65.  Back to cited text no. 9
Cupps TR, Moore PM, Fauci AS. Isolated angiitis of the centralnervous system. Prospective diagnostic and therapeutic experience. Am J Med 1983;74:97-105.  Back to cited text no. 10
de Boysson H, Arquizan C, Touzé E, Zuber M, Boulouis G, Naggara O, et al. Treatment and long-term outcomes of primary central nervous system vasculitis. Stroke 2018;49:1946-52.  Back to cited text no. 11
Sundaram S, Menon D, Khatri P, Sreedharan SE, Jayadevan ER, Sarma P, et al. Primary angiitis of the central nervous system: Clinical profiles and outcomes of 45 patients. Neurol India 2019;67:105-12.  Back to cited text no. 12
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