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Year : 2010  |  Volume : 58  |  Issue : 3  |  Page : 457-459

Cerebral miliary micro aneurysms in polyarteritis nodosa : Report of two cases

Department of Neuroradiology, AIIMS, New Delhi, India

Date of Acceptance01-Feb-2010
Date of Web Publication17-Jul-2010

Correspondence Address:
Sandeep Sharma
Department of Neuroradiology, AIIMS Ansari Nagar, New Delhi -110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.65840

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 » Abstract 

Cerebral involvement is rare in polyarteritis nodosa (PAN) and is mostly characterized by ischemic events and intracranial hemorrhages secondary to cerebral aneurysms is extremely rare. We report two patients of PAN with multiple intracranial aneurysms. One patient presented with intracerebral hemorrhage and in the other patient multiple intracranial aneurysms were incidental findings and were asymptomatic. Both our cases suggest that multiple intracranial aneurysms are not very uncommon in PAN and cerebral angiography should be considered while doing abdominal angiogram in these patients.

Keywords: Cerebral micro aneurysms, poly arteritis nodosa, SAH

How to cite this article:
Sharma S, Kumar S, Mishra N K, Gaikwad S B. Cerebral miliary micro aneurysms in polyarteritis nodosa : Report of two cases. Neurol India 2010;58:457-9

How to cite this URL:
Sharma S, Kumar S, Mishra N K, Gaikwad S B. Cerebral miliary micro aneurysms in polyarteritis nodosa : Report of two cases. Neurol India [serial online] 2010 [cited 2022 Sep 26];58:457-9. Available from: https://www.neurologyindia.com/text.asp?2010/58/3/457/65840

 » Introduction Top

Polyarteritis nodosa (PAN) is a rare systemic necrotizing vasculitis of small and medium sized arteries with predilection for certain organ systems. The reported prevalence is less than 31 per million population. More often occurs in males and in the age group of 40 to 60 years. [1],[2] Involvement of cerebral vascular system in PAN is very uncommon. [3] We report two patients with multiple tiny aneurysms scattered throughout the cerebral vasculature.

 » Case Reports Top


A 13-years boy presented to the emergency with one episode of seizure followed by unconsciousness. Computed tomography (CT) brain [Figure 1] revealed a large left frontal hematoma. Past history was significant: he used to have on and off fever and had weight loss since five months. He had undergone appendicectomy two months before this admission for recurrent abdominal pain. He also had transient right facial weakness for two days one month before. Angiography revealed multiple tiny aneurysms involving the visceral, muscular [Figure 2] and cerebral [Figure 3] arteries, highly suggestive of PAN. Clinically he fulfilled the Americal College of Rheumatology (ACR) criteria [4] for the diagnosis of PAN. Post angiogram CT [Figure 1] b-d revealed multiple nodular hyper dense lesions corresponding to the larger aneurysms seen on cerebral angiography. Serology was negative for c-ANCA and HBsAg while acute phase reactants and liver enzymes were elevated. Diagnosis of PAN was established based on the algorithm proposed by Watt`s et al. [5] Patient responded to corticosteroids and cycloposphomide during the short follow-up.

Case 2

A 32-years male was referred for cerebral angiography for an incidental right posterior cerebral artery (PCA) aneurysm detected during CT angiography done for spontaneous right perirenal hematoma [Figure 4]. He presented to the emergency department two months before for sudden onset left flank pain. CT abdomen done at that time showed a left perirenal hematoma and renal artery pseudo aneurysm which was treated endovascularly [Figure 4]. He had history of weight loss and grossly deranged renal parameters and the angiography was limited to renal arteries at that time. Digital subtraction angiography (DSA) [Figure 5] revealed scattered small aneurysms involving visceral arteries, small cerebral aneurysms and a relatively larger aneurysm arising from the hippocampal branch of right PCA. Contrast CT [Figure 4]c head revealed a nodular lesion in the body region of right hippocampus. Patient fulfilled the diagnostic criteria for PAN which included ACR, [4] Chapel Hill Consensus Conference (CHCC) criteria [6] and surrogate/serologic markers. Serology was negative for c-ANCA or HBsAg. He responded to steroid and cycloposphomide and showed weight gain and resolution of fever.

 » Discussion Top

Cerebral vessels may be involved in PAN and patients may present with diffuse encephalopathy, seizure or stroke. [3] Till 2000 only 19 cases of stroke, both ischemic and hemorrhagic strokes have been documented. [3] To the best of knowledge till date only four cases, including our first patient, of PAN with intracerebral hemorrhage have been reported. [3],[7]

Aneurysms in patients with PAN have commonly been demonstrated in the gastrointestinal system and kidney. [8,9] Intracranial aneurysms are exceptionally rare. [10],[11],[12],[13] Documentation of abdominal visceral artery aneurysms are considered characteristic of PAN, especially when the aneurysms are small and multiple. Studies reporting the angiographic spectrum in PAN and its diagnostic utility have mostly not performed cerebral angiography. [9] The few reports of cerebral involvement in PAN have focused mostly on ischemic events. [3],[7] Of the nine cases of PAN with intracranial aneurysms reviewed by Oomura et al,[13] only one case had innumerable intracranial aneurysms. The anigraphic findings in both of our patients suggest that multiple small intracranial aneurysms are not uncommon in PAN and cerebral angiography should be included in all the patients of PAN undergoing abdominal angiogram. The suggested treatment for cerebral aneurysms in PAN is conservative. [12] Kidney involvement is seen in more than 70% of patients with PAN [13] and changes include infarcts as seen in our first case or uncommonly aneurysm-related hemorrhage as seen in our second case.

The diagnosis of PAN is difficult using both ACR [4] and CHCC [6] criteria. Using ACR criteria, many patients qualify to be grouped into more than one disease category, while using CHCC criteria most of the patients do not qualify to be grouped in the PAN category. Inclusion of surrogate and serologic markers in the algorithm [5],[6],[14],[15] appears to be encouraging but angiography is essential and is relatively specific if numerous micro aneurysms are seen in visceral arteries and probably in the cerebral circulation.

 » References Top

1.Mohammad AJ, Jacobsson LT, Mahr AD, Sturfelt G, Segelmark M. Prevalence of Wegener's granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg-Strauss syndrome within a defined population in southern Sweden. Rheumatology (Oxford) 2007;46:1329-37  Back to cited text no. 1      
2.Bonsib SM. Polyarteritis nodosa. Semin Diagn Pathol 2001;18:14-23.  Back to cited text no. 2  [PUBMED]    
3.Reichart MD, Bogousslavsky J, Janzer RC. Early lacunar strokes complicating polyarteritis nodosa Thrombotic microangiopathy. Neurology 2000;54:883-9.   Back to cited text no. 3  [PUBMED]  [FULLTEXT]  
4.Lightfoot RW Jr, Michel BA, Bloch DA, Hunder GG, Zvaifler NJ, McShane DJ, et al. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum 1990;33:1088-93.  Back to cited text no. 4  [PUBMED]    
5.Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, et al. Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 2007;66:222-7.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]  
6.Segelmark M, Selga D. The challenge of managing patients with polyarteritis nodosa. Curr Opin Rheumatol 2007;19:33-8.   Back to cited text no. 6  [PUBMED]  [FULLTEXT]  
7.Provenzale JM, Allen NB. Neuroradiologic findings in polyarteritis nodosa. Am J Neuroradiol 1996;17:1119-26.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]  
8.Jee KN, Ha HK, Lee IJ, Kim JK, Sung KB, Cho KS, et al. Radiologic findings of abdominal polyarteritis nodosa. AJR Am J Roentgenol 2000;174:1675-79.   Back to cited text no. 8  [PUBMED]  [FULLTEXT]  
9.Stanson AW, Friese JL, Johnson CM, McKusick MA, Breen JF, Sabater EA, et al. Polyarteritis Nodosa: Spectrum of Angiographic Findings. Radiographics 2001;21:151-9.  Back to cited text no. 9  [PUBMED]  [FULLTEXT]  
10.Takahashi JC, Sakai N, Iihara K, Sakai H, Higashi T, Kogure S, et al. Subarachnoid hemorrhage from a ruptured anterior cerebral artery aneurysm caused by polyarteritis nodosa. Case report. J Neurosurg 2002;96:132-4  Back to cited text no. 10      
11.Thompson B, Burns A. Subarachnoid hemorrhages in vasculitis. Am J Kidney Dis 2003;42:582-5.  Back to cited text no. 11  [PUBMED]  [FULLTEXT]  
12.Oomura M, Yamawaki T, Naritomi H, Terai T, Shigeno K. Polyarteritis Nodosa in association with subarachnoid hemorrhage. Intern Med 2006;45:655-8.  Back to cited text no. 12  [PUBMED]  [FULLTEXT]  
13.Hekali P, Kivisaari L, Standerskjfld-Nordenstam CG, Pajari R, Turto H. Renal Complications of Polyarteritis Nodosa: CT Findings. J Comput Assist Tomogr 1985;9:333-8.  Back to cited text no. 13      
14.Kallenberg CG. The last classification of vasculitis. Clinic Rev Allerg Immunol 2008;35:5-10.  Back to cited text no. 14      
15.Watts RA, Scott DG. Recent developments in the classification and assessment of vasculitis. Best Pract Res Clin Rheumatol 2009;23:429-43.  Back to cited text no. 15  [PUBMED]  [FULLTEXT]  


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

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