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Table of Contents
CASE REPORT
Year : 2021  |  Volume : 9  |  Issue : 2  |  Page : 113-115

A rare case report of primary central nervous system vasculitis


Department of Medicine, Bangalore Medical College and Research Institute, Bengaluru, Karnataka, India

Date of Submission20-Dec-2019
Date of Decision07-Mar-2020
Date of Acceptance31-Mar-2020
Date of Web Publication21-Apr-2021

Correspondence Address:
Dr. Madhumati
Professor, Department of Medicine, Bangalore Medical College and Research Institute, K R Market, Bengaluru - 560 002, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/AJIM.AJIM_90_19

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  Abstract 


Primary central nervous system (CNS) vasculitis is defined as an inflammation of cerebral vasculature without vasculitis of other organs. It represents a rare and poorly understood form of vascular inflammatory disease restricted to the brain and spinal cord. An average annual incidence rate of 2.4 cases per 1,000,000 persons per year has been reported all over the world. It affects medium and small-sized arteries of the brain parenchyma, spinal cord, and leptomeninges resulting in CNS dysfunction. Here, we report the case of a 19-year-old female brought with a history of headache, fever and vomiting, and altered sensorium, and on further clinical, biochemical and radiological evaluation, a final diagnosis of primary CNS vasculitis was made.

Keywords: Central nervous system, leptomeninges, primary central nervous system vasculitis


How to cite this article:
Madhumati, Chetan H. A rare case report of primary central nervous system vasculitis. APIK J Int Med 2021;9:113-5

How to cite this URL:
Madhumati, Chetan H. A rare case report of primary central nervous system vasculitis. APIK J Int Med [serial online] 2021 [cited 2021 Jun 12];9:113-5. Available from: https://www.ajim.in/text.asp?2021/9/2/113/314206




  Introduction Top


Primary central nervous system (CNS) vasculitis is defined as an inflammation of cerebral vasculature without vasculitis of other organs. It represents a rare and poorly understood form of vascular inflammatory disease restricted to the brain and spinal cord. An average annual incidence rate of 2.4 cases per 1,000,000 persons per year has been reported. It affects medium and small-sized arteries of the brain parenchyma, spinal cord, and leptomeninges resulting in CNS dysfunction. The diagnosis remains a challenge, since there are no universally accepted diagnostic criteria, and imaging findings may not be specific. Different types of clinical presentation have been described, which makes it even more difficult to identify. Unlike systemic vasculitis, this entity lacks positive autoantibodies. Here, we describe the clinical and radiological findings of our patient with primary CNS vasculitis treated in Bowring and Lady Curzon Hospital.


  Case Report Top


A 19-year-old female patient brought to casualty with a history of headache for 3 months, fever and vomiting for 4 days, and altered sensorium for 1 day. There was no history of convulsions or head trauma. Earlier, the patient was taken to a private hospital where she was intubated in view of low Glasgow Coma Scale. On clinical examination, she was suspected to have chronic meningitis. Computed tomography of the brain and cerebrospinal fluid (CSF) analysis were done which was suggestive of tuberculoma. The patient was started on antitubercular treatment (ATT) along with steroids but showed no improvement.

Later, the patient developed seizures of generalized tonic-clonic seizures semiology and anisocoria; neurologist opinion was taken for the same and advised for magnetic resonance imaging(MRI) and magnetic resonance angiogram(MRA) of brain and CSF analysis.

CSF analysis showed mildly elevated proteins and normal cells, antinuclear antibodies negative, and HIV negative; MRI [Figure 1] and MR Angiography [Figure 2] of the brain showed showed multiple infarcts, constriction of posterior communicating artery and irregular beading appearance. A diagnosis of primary CNS vasculitis was done based on MRI findings and started on injection methyl prednisolone 750 mg once a day for 5 days, and the patient showed improvement in sensorium. ATT was stopped after 1 month and started with oral steroids and then tablet azathioprine.
Figure 1: Infarct in the right pons

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Figure 2: Beading in the left posterior cerebral artery

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  Discussion Top


Introduction Primary CNS vasculitis is a rare inflammatory disorder involving the small and medium-sized blood vessels of the brain and spinal cord without systemic vasculitis. It has been described ever since 1959 with a variable and nonspecific symptoms and signs. In most of the cases, headache is the presenting symptom; it can also have symptoms such as hemiparesis, altered sensorium, aphasia, sensory disturbances, blurred vision, and giddiness.[1]

Till date, there is no exact etiology, but few postulated triggers are:

  • Bacterial triggers such as Mycoplasma, Rickettsia, and Treponema
  • Viral infections such as HIV, hepatitis C virus
  • Systemic lupus erythematosus, Churg–Strauss syndrome
  • Amyloid angiopathy.[2]


Criteria for diagnosis

The diagnosis of CNS vasculitis can be done using Calabrese and Mallek criteria. The diagnosis needs the presence of all three features:

  1. Presence of an unexplained acquired neurological deficit after extensive investigation for other causes
  2. Either classic angiographic evidence of vasculitis or histopathologic evidence of vasculitis within the CNS
  3. No evidence of systemic vasculitis or any other condition to which the angiographic or pathologic evidence can be attributed.[3]


Epidemiology

It is affecting around 2.4 per million person-years with a median age at onset of 50 years with equal sex distribution.[2]

Pathophysiology

Mycoplasma and Rickettsia activate the T-cells and lead to the inflammation of cerebral blood vessels. Infiltration of T-cell around small-sized arteries in CNS.[4]

Histopathology

Primary CNS vasculitis is a type of vasculitis affecting medium and small-sized arterial vessels. The three histological patterns observed are granulomatous, lymphocytic, and necrotizing vasculitis. In granulomatous vasculitis, vasculogenic mononuclear inflammation and well-formed granulomas with multinucleated cells are seen. The histological patterns are not related to symptoms or outcomes.[4]

Clinical features

It is usually insidious onset and slowly progressive disease, with few acute presentations. The symptoms are nonspecific; common presentations are headache, cognitive dysfunction, and stroke, whereas the less common are aphasia, visual field deficits, double vision, seizures, ataxia, and amnestic syndromes.[5]

It is a difficult task to diagnose primary CNS vasculitis because of its rarity and nonspecific clinical presentation. The presentation varies depending upon the cerebral arteries involved such as headache, confusion, seizures, and focal deficits.

Imaging

On fluid attenuated inversion recovery, it shows multiple infarct, hemorrhages cerebral angiography shows multifocal beading with alternating stenosis and dilatations of small-sized vessels, the sensitivity and specificity being 60% and 30%. Most of the times, CSF examination reveals mild pleocytosis and/or elevated proteins.[5]

Differential diagnosis

The differential diagnosis for CNS vasculitis includes systemic vasculitis, reversible cerebral vasoconstriction syndrome, premature intracranial atherosclerosis, fibromuscular dysplasia, moyamoya disease, brain neoplasm, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), posterior reversible encephalopathy syndrome, microvascular cerebral ischemia, demyelinating diseases, and multifocal cerebral thromboembolism.[6]

Treatment

  1. Induction therapy: At present, there are no evidence-based strategies on the treatment of CNS vasculitis. The available groups of drugs are corticosteroids, immunosuppresants, and biologicals. Induction therapy is aimed at providing remission and preventing worse outcomes. In a cohort study, it is revealed that the combination of cyclophosphamide and corticosteroids has fewer relapses, but the efficacy is equal to corticosteroid monotherapy. The current regimen used is intravenous (IV) methylprednisolone pulse (1 g daily for 3–5 days) or oral prednisone 1 mg/kg/day. Cyclophosphamide oral (2 mg/kg/day) for 3–6 months or IV (750 mg/m2/month)
  2. The maintenance therapy is aimed at reducing the number of relapses and long-term disabilities. The regimens used are oral azathioprine (1–2 mg/kg daily), mycophenolate mofetil (1–2 g daily), and methotrexate (10–25 mg/week).[7]



  Conclusion Top


We conclude, MRI and MR Angiogram of brain are needed in young patients presenting with nonspecific symptoms to rule out CNS vasculitis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Younger DS, Coyle PK. Central Nervous System Vasculitis due to Infection. Neurol Clin 2019;37:441-63.  Back to cited text no. 1
    
2.
De Boysson H, Zuber M, Naggara O, Neau JP, Gray F, Bousser MG, et al. 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. 2
    
3.
Calabrese LH, Duna GF, Lie JT. Vasculitis in the central nervous system. Arthritis Rheum 1997;40:1189-201.  Back to cited text no. 3
    
4.
Salvarani C, Brown RD Jr, Calamia KT, Christianson TJ, Huston J 3rd, Meschia JF, et al. Angiography-negative primary central nervous system vasculitis: A syndrome involving small cerebral vessels. Medicine (Baltimore) 2008;87:264-71.  Back to cited text no. 4
    
5.
Salvarani C, Brown RD Jr, Calamia KT, Christianson TJ, Weigand SD, Miller DV, et al. Primary central nervous system vasculitis: Analysis of 101 patients. Ann Neurol 2007;62:442-51.  Back to cited text no. 5
    
6.
Cañas CA, Cedeño SV, Carrascal E, Vélez JD. Diátesis trombótica en paciente con infección aguda por citomegalovirus. Acta Médica Colombiana 2009;341:36-139.  Back to cited text no. 6
    
7.
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. 7
    


    Figures

  [Figure 1], [Figure 2]



 

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