Journal of Neurosciences in Rural Practice

  Table of Contents 
Year : 2015  |  Volume : 6  |  Issue : 3  |  Page : 401-402  


Department of Neurology, Stroke Unit, Brugmann University Hospital, Brussels, Belgium

Date of Web Publication16-Jun-2015

Correspondence Address:
Apostolos Safouris
Place Van Gehuchten 4, 1020 Bruxelles
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0976-3147.154565

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How to cite this article:
Safouris A. Commentary. J Neurosci Rural Pract 2015;6:401-2

How to cite this URL:
Safouris A. Commentary. J Neurosci Rural Pract [serial online] 2015 [cited 2019 May 21];6:401-2. Available from:

Angiitis of the central nervous system (ACNS) is a heterogeneous group of rare diseases that affect any age group and usually carry a grave prognosis. The first difficulty in diagnosing ACNS is its diverse clinical manifestations. There are neither pathognomonic clinical signs nor symptoms; the three most common clinical presentations are chronic or subacute headache, ischemic or hemorrhagic strokes and progressive encephalopathy (progressive cognitive impairment, psychiatric symptoms, seizures etc). In some cases of patients suffering from a known predisposing condition, inflammatory or other, the diagnosis can be easily attained but in most cases a complete work-up is indispensable in order to differentiate secondary from primary CNS angiitis (PACNS). In an excellent review on PACNS differential diagnosis, Birnbaum et al. [1] proposed the following general rules as a guide to the clinician:

  • For patients less than 40 years, think of reversible vasoconstriction syndrome
  • For patients older than 75, think of cerebral amyloid angiopathy
  • When basal meningeal inflammation is detected on imaging, think of tuberculosis or mycosis
  • Immunosuppressed patients (AIDS patients or under immunosuppressive treatment); exclude opportunistic infections and subacute bacterial endocarditis
  • When inflammatory markers are elevated, think about cancer, generalized autoimmune disease or infection.
After a complete diagnostic panel that excludes all secondary causes of angiitis (for a complete list of possible differential diagnosis, a complete table can be found in Néel et al.; [2] article in French but the table is accessible to English-speaking readers), PACNS emerges as a diagnosis of exclusion that can only be affirmed with certitude with a brain biopsy. Angiography has its role in diagnosis but there are reports not only of false-negative but also of false-positive results. It is a well-known fact that angiography tends to be less sensitive for small diameter arteries (less than 0.5 mm) and arteries with diameter smaller than 0.2 mm cannot be visualized; small-vessel vasculitis is therefore beyond its reach. Once considered highly specific, the finding of segmental stenosis and dilation of arteries has been also described in severe atherosclerosis, post irradiation angiopathy, infection and vasospasm and therefore should not considered pathognomonic. [3,4]

A brain biopsy remains the gold standard for diagnosis as it has been shown that in one in three patients suspected to suffer from PACNS, a biopsy proved a different diagnosis. It also has its limitations as one out of three biopsies did not provide a diagnosis. [5,6]

PACNS is a most challenging diagnosis. The present case by Bajaj et al. [7] highlights the main problems with diagnosing this detrimental but possibly treatable condition. Outside city context, where patients cannot be always easily followed, management is even more complicated. The authors should be praised for their persistence in obtaining brain biopsy results before reaching a diagnosis. A brain biopsy is certainly an invasive but usually necessary procedure in order to introduce an immunosuppressive treatment that is neither simple nor without side effects. [8] Since there are no randomized controlled trials for this rarely encountered disease, treatment recommendations come from expert opinion. Corticosteroids are first-line treatment with or without cyclophosphamide. Prognosis is not only dependent on disease progression but also on irreversible neurological sequelae before treatment initiation.

   References Top

Birnbaum J, Hellmann DB. Primary angiitis of the central nervous system. Arch Neurol 2009;66:704-9.  Back to cited text no. 1
Néel A, Pagnoux C, Guillevin L, Hamidou M. Central nervous system vasculitides: An update. Rev Med Interne 2012;33:381-9.  Back to cited text no. 2
Alhalabi M, Moore PM. Serial angiography in isolated angiitis of the central nervous system. Neurology 1994;44:1221-6.  Back to cited text no. 3
Vollmer TL, Guarnaccia J, Harrington W, Pacia SV, Petroff OA. Idiopathic granulomatous angiitis of the central nervous system. Diagnostic challenges. Arch Neurol 1993;50:925-30.  Back to cited text no. 4
Alrawi A, Trobe JD, Blaivas M, Musch DC. Brain biopsy in primary angiitis of the central nervous system. Neurology 1999;53:858-60.  Back to cited text no. 5
Chu CT, Gray L, Goldstein LB, Hulette CM. Diagnosis of intracranial vasculitis: A multi-disciplinary approach. J Neuropathol Exp Neurol 1998;57:30-8.  Back to cited text no. 6
Bajaj BK, Pandey S, Ramanujam B, Wadhwa A. Primary angiitis of CNS: The story of a great masquerader. J Neurosci Rural Pract 2015:399-401.  Back to cited text no. 7
  Medknow Journal  
P, Kraemer M. Cerebral vasculitis in adults: What are the steps in order to establish the diagnosis? Red flags and pitfalls. Clin Exp Immunol 2014;175:419-24.  Back to cited text no. 8


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