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LETTER TO EDITOR
Year : 2012  |  Volume : 60  |  Issue : 1  |  Page : 106-108

Massive vasculitic cerebellar infarction in patient with systemic lupus erythematosus


Department of Neurology, College of Medicine, Hanyang University, Seoul, Korea

Date of Submission07-Nov-2011
Date of Decision08-Dec-2011
Date of Acceptance02-Jan-2011
Date of Web Publication7-Mar-2012

Correspondence Address:
Seung-Hyun Kim
Department of Neurology, College of Medicine, Hanyang University, Seoul
Korea
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.93603

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How to cite this article:
Kim YS, Park BS, Baek W, Kim SH. Massive vasculitic cerebellar infarction in patient with systemic lupus erythematosus. Neurol India 2012;60:106-8

How to cite this URL:
Kim YS, Park BS, Baek W, Kim SH. Massive vasculitic cerebellar infarction in patient with systemic lupus erythematosus. Neurol India [serial online] 2012 [cited 2019 Oct 16];60:106-8. Available from: http://www.neurologyindia.com/text.asp?2012/60/1/106/93603


Sir,

Patients with systemic lupus erythematosus (SLE) can present with various neurological syndromes, referred as neurolupus (NL). Among NL syndromes, stroke is a well-documented complication. [1] However, stroke associated with central nervous system (CNS) vasculitis is extremely rare. [2],[3] To the best of our knowledge, vasculitis associated with a massive cerebellar biopsy-proven infarction has not previously been reported, and this prompted us to describe this patient.

A 17-year-old female, a diagnosed case of SLE on mizoribine 50 mg/day and deflazacort 30 mg/day, was admitted with a three-day history of fever, headache and nausea. On admission, her temperature was 38.7 and blood pressure was 103/56 mmHg. Neurological examinations were unremarkable except for mild neck stiffness. Initial brain computerized tomography (CT) scan and cerebrospinal fluid (CSF) examination were normal. Laboratory tests showed: low white blood cell count (1900/ mm 3 ), platelet count of 110,000/ mm 3 , positive anti-dsDNA (1:80), low serum complement C3 and C4 (27.4 mg/dl and 7.7 mg/dl, respectively) and CH50 (5.7 U/ml). These findings were consistent with highly active SLE. She was empirically treated with intravenous amoxacillin clavulanate 1.2 g twice a day. On the third hospital day, her mental status deteriorated to stupor. Fluid-attenuated inversion recovery (FLAIR) brain magnetic resonance imaging (MRI) revealed high signal intensity in the left cerebellum with mass effect and obstructive hydrocephalus [Figure 1]a and b. In addition, diffusion-weighted imaging (DWI) showed restricted diffusion [Figure 1]c and d. However, CT-cerebral angiography did not show any luminal narrowing of the vertebral artery [Figure 1]e. The patient underwent emergency decompressive craniectomy and left cerebellar posterior lobectomy. Pathology of cerebellar lesion revealed parenchymal change with intense microglial infiltration and multiple hemorrhages suggestive of cerebellar infarction [Figure 2]a and b. In addition, vascular change with perivascular and vascular neutrophilic infiltration and massive fibrin leakage was noted, favoring SLE-associated vasculitis [Figure 2]c and d. Patient improved gradually and became alert three weeks after surgery. Brain MRI revealed reduction of cerebellar edema and mass effect [Figure 1]f. At the three-month follow-up, her neurological deficit had improved and she was able to walk by herself.
Figure 1: FLAIR MRI (a,b) and diffusion MRI (c,d) on hospital Day 3 showing high signal intensities on the left cerebellar hemisphere, consistent with acute cerebellar infarction. However, computed tomography angiography did not show any stenosis of the vertebral artery (e). Follow-up FLAIR MRI after 61 days of decompressive surgery reveals diminished cerebellar edema and mass effect (f)

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Figure 2: Parenchymal change with microglial infiltration and multiple hemorrhages pointing to acute infarction [H and E stain (a, × 100), CD 68 stain (b, × 400)]. Perivascular and vascular neutrophilic infiltration and massive fibrin leakage is seen, consistent with vasculitis [H and E stain (c, × 400), fibrin stain (d, × 400)]

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Cerebral infarction is more prevalent in SLE patients than in the general population, in up to 15% of the SLE patients. [3] The common causes of cerebral infarction in patients with SLE include cardio-embolism, hypercoagulable states and atherosclerosis. [3],[4],[5] However, our patient presented massive cerebellar infarction without any previously supposed mechanisms. She had serological markers of highly active SLE, high titers of anti-dsDNA and low complement level. Pathology of the lesion also showed features of vasculitis. In view of these findings, we suspected that the cerebellar infarction was due to vasculitis caused by highly active SLE. CNS vasculitis not associated with CNS infection, drug or anti-phospholipid antibody is extremely rare in patients with SLE, and there are only a few autopsy-proven case reports. [6],[7] These reports describe bilateral multiple cerebral infarcts with necrotizing vasculitis [6] or extensive bilateral cerebral infarction with acute occlusive large-vessel disease. [7] However, our patient differed from those patients because she presented with life-threatening cerebellar infarction and vasculitic pathology in a single region. Another noteworthy finding was that her lesion was confined to the cerebellum. The reported involvement of the cerebellum in NL is only 2% and the common pathologic finding is reversible vasculopathy. [8] Our patient had restriction on DWI and pathological evidence of infarction and vasculitis suggesting the possibility of vasculitis of the posterior inferior cerebellar artery and occlusion resulting in massive cerebellar infarction.


 » Acknowledgment Top


This work was supported by the cluster research fund of Hanyang University (HY-2009-C)".

 
 » References Top

1.Unterman A, Nolte JE, Boaz M, Abady M, Shoenfeld Y, Zandman-Goddard G. Neuropsychiatric syndromes in systemic lupus erythematosus: A meta-analysis. Semin Arthritis Rheum 2011;41:1-11.  Back to cited text no. 1
[PUBMED]  [FULLTEXT]  
2.Mitsias P, Levine SR. Large cerebral vessel occlusive disease in systemic lupus erythematosus. Neurology 1994;44:385-93.  Back to cited text no. 2
[PUBMED]    
3.Futrell N, Millikan C. Frequency, etiology, and prevention of stroke in patients with systemic lupus erythematosus. Stroke 1989;20:583-91.  Back to cited text no. 3
[PUBMED]  [FULLTEXT]  
4.Bessant R, Duncan R, Ambler G, Swanton J, Isenberg DA, Gordon C, et al. Prevalence of conventional and lupus-specific risk factors for cardiovascular disease in patients with systemic lupus erythematosus: A case-control study. Arthritis Rheum 2006;55:892-9.  Back to cited text no. 4
[PUBMED]  [FULLTEXT]  
5.Roman MJ, Shanker BA, Davis A, Lockshin MD, Sammaritano L, Simantov R, et al. Prevalence and correlates of accelerated atherosclerosis in systemic lupus erythematosus. N Engl J Med 2003;349:2399-406.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  
6.Goel D, Reddy SR, Sundaram C, Prayaga AK, Rajasekhar L, Narsimulu G. Active necrotizing cerebral vasculitis in systemic lupus erythematosus. Neuropathology 2007;27:561-5.  Back to cited text no. 6
[PUBMED]  [FULLTEXT]  
7.Conen KL, Jeanneret C, Hecker B, Cathomas G, Biedermann BC. Acute occlusive large vessel disease leading to fatal stroke in a patient with systemic lupus erythematosus: Arteritis or atherosclerosis? Arthritis Rheum 2006;54:908-13.  Back to cited text no. 7
[PUBMED]  [FULLTEXT]  
8.Appenzeller S, Cendes F, Costallat LT. Cerebellar ataxia in systemic lupus erythematosus. Lupus 2008;17:1122-6.  Back to cited text no. 8
[PUBMED]  [FULLTEXT]  


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