Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery 2016; 35(03): 218-221
DOI: 10.1055/s-0036-1586238
Case Report | Relato de Caso
Thieme Publicações Ltda Rio de Janeiro, Brazil

Cerebral Aneurysm in Takayasu's Arteritis: A Case Report and Review of the Literature

Aneurisma cerebral na arterite de Takayasu: relato de caso e revisão da literatura
Breno Bezerra Arruda Câmara
1  Instituto de Ensino e Pesquisa, Santa Casa de Belo Horizonte, Belo Horizonte, Minas Gerais, Brazil
2  Neurosurgery, Santa Casa de Misericórdia de Belo Horizonte, Belo Horizonte, Minas Gerais, Brazil
,
Marcos Antônio Dellaretti Filho
2  Neurosurgery, Santa Casa de Misericórdia de Belo Horizonte, Belo Horizonte, Minas Gerais, Brazil
,
Carlos Eduardo Romeu de Almeida
2  Neurosurgery, Santa Casa de Misericórdia de Belo Horizonte, Belo Horizonte, Minas Gerais, Brazil
,
Daniel Espíndola Ronconi
2  Neurosurgery, Santa Casa de Misericórdia de Belo Horizonte, Belo Horizonte, Minas Gerais, Brazil
,
Marco Aurélio Vieira Couto
2  Neurosurgery, Santa Casa de Misericórdia de Belo Horizonte, Belo Horizonte, Minas Gerais, Brazil
,
Renata Ferreira de Souza
2  Neurosurgery, Santa Casa de Misericórdia de Belo Horizonte, Belo Horizonte, Minas Gerais, Brazil
› Author Affiliations
Further Information

Address for correspondence

Breno Bezerra Arruda Câmara, MD
Instituto de Ensino e Pesquisa da Santa Casa de Belo Horizonte
Belo Horizonte, Minas Gerais
Brazil   

Publication History

02 April 2016

08 June 2016

Publication Date:
03 August 2016 (online)

 

Abstract

Cerebral aneurysms are rarely observed in patients with Takaysu's arteritis. To our knowledge, this report presents the first case described in Brazil. Hemodynamic stress caused by obstructions of the cervical vessels developes cerebral aneurysms in these patients and they have higher rates of multiple and posterior circulation aneurysms than general population. In the majority of cases, microsurgical treatment is indicated mainly due to changes in the cervical vessels that preclude an endovascular approach.


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Resumo

Aneurismas cerebrais são raramente observados em pacientes com arterite de Takayasu. Este é o primeiro caso desta associação descrito no Brasil. O estresse hemodinâmico causado pelas obstruções vasculares cervicais desenvolve os aneurismas cerebrais nestes pacientes e eles apresentam maiores taxas de aneurismas múltiplos e de acometimento da circulação cerebral posterior. Na maioria dos casos, o tratamento microcirúrgico está indicado pois as alterações anatômicas dos vasos cervicais dificultam a abordagem endovascular.


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Introduction

Takayasu's arteritis is a chronic inflammatory disease that affects medium and large caliber arteries, particularly the aorta and its main branches.[1] Cerebral aneurysms and subarachnoid hemorrhage are rarely observed in these patients, and only 25 cases have been described in the literature. Hemodynamic stress seems to be of crucial importance in the anatomical distribution of cerebral aneurysms.[2] [3] [4] [5] Treatment follows the same criteria as those for the general population.[5] The purpose of this study was to report the first known case in Brazil and review the literature.


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Case Report

In 2003, 11 years before our evaluation, a female patient attended a routine medical evaluation in which the absence of left radial pulse was identified. She was referred to a cardiologist, who suspected Takayasu's arteritis and prescribed a carotid duplex scan that indicated 70–80% stenosis in the common carotid arteries. A duplex scan of the lower limbs showed no changes. Aortography showed: 80% stenosis of the renal arteries ([Fig. 1A]); occlusion of the middle third of the subclavian arteries and extensive collateral branches to the intercostal arteries; 80% stenosis of the common carotid arteries ([Fig. 1B]); occlusion of the right external carotid artery; and 95% stenosis of the right vertebral artery and dominant left vertebral artery. A cerebral angiography identified bilateral paraclinoid aneurysms close to the emergence of the ophthalmic arteries (measuring 5 mm and 1 mm).

The patient's treatment began with prednisone, methotrexate and folic acid in 2004, with follow-up every six months, and no reports of clinical worsening or complications. In December 2011, she became pregnant and was referred to the Santa Casa de Belo Horizonte due to high obstetric risk. The patient developed pregnancy-specific hypertensive disorder, so the birth was performed by caesarean section without complications, followed by tubal ligation, and the normalization of blood pressure at two months postpartum.

In January 2014, at 27 years old, she was submitted to a new cerebral angiography at Santa Casa de Belo Horizonte that confirmed the presence of bilateral paraclinoid aneurysms ([Fig. 1C-D]); the left aneurysm was larger (6.1 mm and 2.2 mm). She was then referred for treatment of the cerebral aneurysms. Upon admission, she was asymptomatic, and showed no evidence of any event or neurological symptoms, while the physical examination demonstrated impalpable brachial and radial pulses, a mild aortic diastolic murmur, and lower blood pressure in her arms. All laboratorial tests and inflammatory markers were normal.

The patient was submitted to treatment by microsurgery using the left pterional craniotomy approach and clipping of the left and right aneurysms ([Fig. 1E-F]).

Zoom Image
Fig. 1 (A) Aortography shows bilateral stenosis of the renal arteries. (B) Stenosis and dilations of the common carotid artery. (C) Aneurysm of the left ICA. (D) Baby aneurysm of the right ICA. (E and F) Control arteriography showing complete exclusion of the aneurysms.

In the immediate postoperative period, she developed left amaurosis, but presented no other complications during hospitalization. A control arteriography was performed, showing complete exclusion of the aneurysms. One month later, while attending the outpatient clinic, she continued to present the amaurotic symptoms, but showed good performance status in everyday activities, scoring 90 on the Karnofsky Performance Scale.


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Discussion

Takayasu's arteritis is a chronic inflammatory disease and a rare type of primary inflammatory vasculitis of unknown cause that affects medium and large caliber arteries, including the aorta and its main branches.[1]

Its pathophysiology includes transmural granulomatous inflammation with fibrinoid necrosis, which can result in vascular stenosis, occlusion, dilation and the formation of aneurysms.[6] [7] Takayasu's arteritis is a rare disease that primarily affects young women and presents higher incidence in Asian countries, but it is not limited to these regions.[8] [9]

The clinical pathology is characterized by a systemic inflammatory phase, associated with other nonspecific systemic symptoms, such as fever, weight loss, sweating, myalgia, arthralgia, anorexia and asthenia. A vascular inflammation phase follows, characterized by pain in the course of the affected vessel, and it culminates in the final stage, characterized by fibrosis of the vessels and ischemia, clinically represented by changes in pulse, arterial murmurs and tremors, paresthesia and claudication, mainly in the arms.[10] [11]

Diagnosis depends on the fulfillment of at least three of the American College of Rheumatology criteria, which are: patient age < 40 years old; decreased brachial pulses; claudication of the extremities; a difference in systolic blood pressure in the upper limbs greater than 10 mm Hg; murmurs in the subclavian arteries and aorta; and angiographic changes in the aorta and its main branches. A combination of these criteria shows 90.5% sensitivity and 97.8% specificity.[9] [12]

Involvement of the central nervous system occurs in 20% of patients, usually through ischemic stroke.[13] In contrast, cerebral aneurysms and subarachnoid hemorrhage are very rare presentations,[4] with only 25 cases currently described in the literature.

In our review of the literature, we found 22 papers reporting 18 aneurysms in the posterior circulation, and 24 in the anterior circulation, in which the internal carotid, anterior communicating and basilar arteries were the most affected ([Table 1]).[4] [5] [14] [15] [16] [17] [18] [19] [20] Thus, the proportion of involvement of the posterior circulation in these patients is much higher (42.8%) than in the general population,[5] [14] where ∼ 90% of aneurysms occur in the anterior circulation. The hemodynamic stress caused by the obstruction or occlusion of the carotid system is considered the main factor related to the genesis of cerebral aneurysms in Takayasu's arteritis, since the majority of studies observed no inflammation in the intracranial vessels affected.[2] [3] [4] [5] Studies using animal models, in which occlusion of the neck vessels was performed, led to the development of intracranial aneurysms that mimicked the pathophysiology proposed.[2] Only one patient showed no stenosis in the cervical vessels.[4] These patients also have a higher incidence of multiple aneurysms[14] (11 of the 25 cases reported).

Table 1

Summary of reported cases of cerebral aneurysms associated with Takayasu arteritis

Report (year)

Age/sex

Symptom

Location

Niitsu (1972)

26Y/F

sSAH

Lt. ICA-PComA

Izawa (1980)

55Y/F

sSAH

Lt. VA

Kumagai (1981)

41Y/F

sSAH

BA

Lt. PCA

Kumagai (1981)

55Y/F

sSAH

Bil. SCA

Arita (1981)

48Y/F

Incidental

AComA

Imaizumi (1982)

48Y/F

sSAH

AComA

Masuzawa (1982)

26Y/F

sSAH

Lt. PComA

Lt. MCA

Masuzawa (1982)

54Y/F

sSAH

BA top

Satoh (1983)

38Y/F

sSAH

Rt. ACA

Matsuzawa (1984)

55Y/M

sSAH

Rt. VA

Wakabayashi (1985)

56Y/F

sSAH

BA top

Mizuno (1985)

58Y/F

sSAH

BA

Masuzawa (1986)

64Y/F

sSAH

Lt. ICA-OphA

Lt. ICA- PComA

BA top

AICA

Kurimoto (1987)

48Y/F

sSAH

AComA

Sunami (1987)

58Y/F

sSAH

VA

Rt. PCA

Dimitrijevic' (1989)

53Y/F

sSAH

MCA

Nishimura (1994)

48Y/F

sSAH

AComA

Lt. ICA

Rt. MCA

Asaoka (1998)

54Y/F

Incidental

AComA

Lt. ICA

Kanda (2004)

48Y/F

sSAH

Rt. VA

Sonwalkar (2004)

50Y/F

sSAH

Lt. ICA-OphA

Lt. ICA- PcomA

Takayama (2008)

70Y/F

Incidental

BA top

Lt. PCA

Weiss (2008)

18M/F

sSAH

Rt. ACA

Lt. ICA

Maramattom (2010)

29Y/M

sSAH

AComA

He (2013)

30Y/F

sSAH

AComA

He (2013)

55Y/F

Incidental

BA top

Lt. PCA

AcomA

Present case

27Y/F

Incidental

Bil. ICA- OphA

Abbreviations: ACA, anterior cerebral artery; AComA, anterior communicating artery; AICA, anterior inferior cerebellar artery; BA, basilar artery; F, female; Bil., bilateral; ICA, internal carotid artery; Lt., left; M, male; MCA, middle cerebral artery; OphA, ophthalmic artery segment; PCA, posterior cerebral artery; PComA, posterior communicating artery; Rt., right; SCA, superior cerebellar artery; sSAH, spontaneous subarachnoid hemorrhage; VA, vertebral artery; Y, years. Adapted from Takayama et al[20] (2008), Kanda et al[4] (2004), and Masuzawa et al[15] (1984).


The treatment of cerebral aneurysms in Takayasu's arteritis follows the same recommendations as those for the general population.[5] He et al report the control of inflammatory markers before neurosurgical treatment in their two cases,[5] but there is no evidence for delaying treatment for this reason. Masuzawa et al highlight the importance of treating these aneurysms, since these patients usually have high blood pressure that is often difficult to control, increasing the risk of complications related to cerebral aneurysm. The permanence of hemodynamic stress caused by arterial blockages also favors the risk of rupture or the appearance of new aneurysms.[15] Microsurgical treatment is recommended in most cases because, as described by Maramattom, the presence of altered anatomical cervical vessels, whether due to stenosis, dilations or accentuated tortuosity, can make access to the aneurysm by the endovascular approach unfeasible.[16] In this case, the cervical vessels stenosis and the aneurysm neck size (7 mm) were relevant in the surgical decision.


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Conclusion

Despite the inflammatory involvement of vessels in Takayasu's arteritis, causing stenosis and dilation, these alterations are rarely found in the cerebral circulation.[4] The case reported here is a rare association of two infrequent pathologies, and there is no evidence of causality between them,[14] but this association has specific characteristics that directly influence its management. The anatomical aspects and the patient's condition shall define the best treatment.[4]


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Address for correspondence

Breno Bezerra Arruda Câmara, MD
Instituto de Ensino e Pesquisa da Santa Casa de Belo Horizonte
Belo Horizonte, Minas Gerais
Brazil   


Zoom Image
Fig. 1 (A) Aortography shows bilateral stenosis of the renal arteries. (B) Stenosis and dilations of the common carotid artery. (C) Aneurysm of the left ICA. (D) Baby aneurysm of the right ICA. (E and F) Control arteriography showing complete exclusion of the aneurysms.