Case Report

Efficacy of Extracranial–Intracranial Bypass for Progressive Middle Cerebral Artery Occlusion Associated with Active €gren’s Syndrome: Case Report Sjo Hiroyuki Sakata, MD, PhD, Miki Fujimura, MD, PhD, Kenichi Sato, MD, PhD, Hiroaki Shimizu, MD, PhD, and Teiji Tominaga, MD, PhD

Sj€ ogren syndrome affecting the major cerebral arteries is rare, and an optimal therapeutic strategy to counteract such a lesion has not yet been established. We herein report a case of a 39-year-old woman with a history of primary Sj€ ogren syndrome, which had previously been treated with immunosuppressive therapy, manifesting with a crescendo transient ischemic attack because of left middle cerebral artery stenosis. Despite the administration of high doses of prednisolone and azathioprine for active Sj€ ogren syndrome, the frequency of crescendo transient ischemic attacks increased with the progression of stenosis and magnetic resonance imaging showed the development of subacute cerebral infarction. Single-photon emission computed tomography with N-isopropyl[123I]-p-iodoamphetamine revealed apparent hemodynamic compromise in the affected cerebral hemisphere. In light of the increased risk of further progression of cerebral infarction, we decided to perform surgical revascularization in spite of her active inflammatory condition. The patient underwent extracranial–intracranial bypass without complications and was treated with intensive immunosuppressive therapy during the perioperative period. Based on our findings, we recommend surgical revascularization for occlusive cerebrovascular disease with hemodynamic compromise in combination with intensive immunosuppressive therapy, even in the active inflammatory state of autoimmune diseases, if ischemic symptoms are medically uncontrollable. Key Words: Sj€ ogren’s syndrome—cerebral artery occlusion—cerebral infarction—immunosuppressive therapy. Ó 2014 by National Stroke Association

From the Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Japan. Received December 25, 2013; revision received February 20, 2014; accepted February 26, 2014. Address correspondence to Miki Fujimura, MD, PhD, Department of Neurosurgery, Tohoku University Graduate School of Medicine, 11 Seiryo-machi, Aoba-ku, Sendai City, Miyagi 980-8574, Japan. E-mail: [email protected] 1052-3057/$ - see front matter Ó 2014 by National Stroke Association http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2014.02.022

Primary Sj€ ogren syndrome is a chronic autoimmune disorder of the exocrine glands with associated lymphocytic infiltrates in the affected glands. Neurologic manifestations, including the involvement of both the peripheral and the central nervous systems, have been reported in approximately 20% of patients with Sj€ ogren syndrome.1 Although a recent study demonstrated the association between Sj€ ogren syndrome and the high risk of ischemic and hemorrhagic stroke because of small vessel

Journal of Stroke and Cerebrovascular Diseases, Vol. 23, No. 8 (September), 2014: pp e399-e402

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vasculitis, only a few cases in which the major cerebral arteries were affected have been reported.3-5 Therefore, an optimal therapeutic strategy to treat this rare condition has not yet been established, especially during the active stage of Sj€ ogren syndrome. We herein described a patient with Sj€ ogren syndrome who manifested with a crescendo transient ischemic attack (TIA) caused by the progressive occlusion of the middle cerebral artery (MCA), and was successfully managed by revascularization surgery under the intensive immunosuppressive therapy during the active stage of Sj€ ogren syndrome.

Case Report A 39-year-old woman had a 10-year history of primary Sj€ ogren syndrome, which was diagnosed with minor salivary gland biopsy (grade 4), Schirmer test, and positive

Sj€ ogren syndrome antigen A and antinuclear antibody. She had previously been treated with prednisolone and azathioprine to control the activity of this syndrome by the department of rheumatology. Eight months before being admitted to our department, magnetic resonance angiography showed the manifestation of asymptomatic bilateral M1 stenosis (Fig 1, A). She developed TIA with right hemiparesis and speech disturbance 2 months later. Because the frequency of TIA increased in spite of intensive immunosuppressive therapy (40 mg prednisolone and 100 mg azathioprine) over 6 months, she was referred to our department for surgical consideration. A neurologic examination on admission revealed mild motor aphasia. A serologic examination was positive for Sj€ ogren syndrome antigen A and antinuclear antibody and showed higher levels of C-reactive protein (5.1 mg/dL), whereas antiphospholipid antibody was negative. These findings suggested that Sj€ ogren syndrome was in the

Figure 1. (A) Temporal profile of magnetic resonance angiography showing bilateral middle cerebral artery (MCA) stenosis 8 months before surgery, progression of left MCA stenosis and improvements in right MCA stenosis 4 months before surgery, occlusion of the left MCA 1 month before surgery, and patency of superficial temporal artery–MCA bypass 1 day and 3 months after surgery (arrows). (B) Diffusion-weighted image 1 month before surgery revealing multiple subacute infarction in the left cerebral hemisphere (arrows). (C) Digital subtraction angiography, performed 1 month before surgery, demonstrating occlusion of the left MCA (arrow). Single-photon emission computed tomography with N-isopropyl[123I]-p-iodoamphetamine, performed 1 month before surgery (D) and 7 days after surgery (E), showing that the decrease in cerebral blood flow in the left hemisphere significantly improved after revascularization surgery.

€ CEREBRAL ARTERY OCCLUSION WITH SJOGREN’S SYNDROME

active stage. Magnetic resonance imaging revealed occlusion of the left M1 (Fig 1, A) and subacute infarction in the left MCA territory (Fig 1, B). Digital subtraction angiography confirmed left M1 occlusion just distal to the anterior temporal artery (Fig 1, C). Single-photon emission computed tomography with N-isopropyl[123I]-p-iodoamphetamine revealed a hypoperfusion area in the left MCA territory (Fig 1, D). Because TIA was medically uncontrollable, superficial temporal artery (STA)–MCA anastomosis was performed under treatment with 40 mg of prednisolone and 100 mg of azathioprine during the perioperative period. The stump of the parietal branch of the STA was anastomosed to the MCA (M4 segment), which supplied the frontal lobe. The temporary occlusion time of the MCA was 23 minutes. Atherosclerosis was not evident in the peripheral MCA during surgery. Magnetic resonance angiography performed 1 day after surgery, showed patent STA-MCA bypass without any signs of vasospasm at the site of anastomosis (Fig 1, A). Neither ischemic nor hemorrhagic complications were observed perioperatively. Postoperative single-photon emission computed tomography with Nisopropyl[123I]-p-iodoamphetamine during the acute stage after revascularization surgery revealed a significant improvement in cerebral blood flow in the left hemisphere (Fig 1, E). Her postoperative course was uneventful, and the patient was discharged 2 weeks after surgery without new neurologic deficits. Immunosuppressive therapy was continued and the recurrence of TIA was not observed during the follow-up period of 5 months.

Discussion Dynamic changes in the asymmetric steno-occlusive findings in the major cerebral arteries under the active state of Sj€ ogren syndrome appear to be unique to the present case. The exact mechanism underlying this rare condition has yet to be determined, although 2 immunologicallymediated mechanisms have been proposed for the etiopathogenesis of arterial stenosis in Sj€ ogren syndrome.6-8 First, vasculitis may participate in this pathology because of its association with the increased serum level of autoantibodies characterized by Sj€ ogren syndrome.6,7 Sj€ ogren syndrome has alternatively been reported to accelerate early atherosclerotic changes because of chronic inflammation induced by the autoimmune system.8 In the present case, left MCA stenosis progressed to occlusion, whereas reversible transient narrowing of the contralateral MCA was observed, which confirmed dynamic changes in the major cerebral arteries in a relatively short time under the active inflammatory state of Sj€ ogren syndrome. Furthermore, our patient did not have any general risk factors for atherosclerosis. Based on these findings, it is conceivable that vasculitis induced by active Sj€ ogren syndrome could have primarily contributed to

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the development of progressive cerebral artery stenosis in our case. In fact, intraoperative findings suggested the absence of atherosclerosis in the cerebral arteries. Another important differential diagnosis for this rare condition is reversible cerebral vasoconstriction syndrome (RCVS), which is characterized by severe headaches and diffuse segmental constriction of cerebral arteries that resolves spontaneously within 12 weeks. Because calcium channel blockers are widely used for the treatment and as the diagnostic tool for RCVS, preoperative administration of calcium channel blockers might have been useful to rule out the association with RCVS. Nevertheless, the lack of headache in her clinical course and prolonged vasoconstriction of the left MCA over 12 weeks suggested relatively lower possibility of the involvement of this entity,9 and we sought to avoid deterioration of her ischemic condition by unexpected hypotension. The optimal management of occlusive cerebrovascular disorders induced by Sj€ ogren’s syndrome has not yet been established because of the rarity of this association. Treatment with high doses of immunosuppressive agents potentially resolves stenosis of the major cerebral arteries associated with autoimmune diseases.3,10 Revascularization surgery is also a treatment of choice to restore cerebral perfusion in patients with the silent state of Sj€ ogren syndrome5; however, surgery is generally avoided in the active stage of the autoimmune disease.11 Intensive medical treatment failed to improve ischemic symptoms or the progression of MCA occlusion in the present case. Apparent hemodynamic compromise with subacute infarction was also found in the affected cerebral hemisphere. In light of the increased risk of further progression of cerebral infarction, we decided to perform surgical revascularization in spite of the active inflammatory condition. Sufficient revascularization was obtained without complications in our case by continuing the administration of high doses of prednisolone and azathioprine during the perioperative period. These findings suggest that extracranial–intracranial bypass in combination with intensive immunosuppressive therapy could be effective even in the active inflammatory state of autoimmune diseases.

References 1. Fox RI. Sjogren’s syndrome. Lancet 2005;366:321-331. 2. Zoller B, Li X, Sundquist J, et al. Risk of subsequent ischemic and hemorrhagic stroke in patients hospitalized for immune-mediated diseases: a nationwide follow-up study from Sweden. BMC Neurol 2012;12:41. 3. Ii Y, Shindo A, Sasaki R, et al. Reversible stenosis of large cerebral arteries in a patient with combined Sjogren’s syndrome and neuromyelitis optica spectrum disorder. Rheumatol Int 2008;28:1277-1280. 4. Matsuki Y, Kawakami M, Ishizuka T, et al. SLE and Sjogren’s syndrome associated with unilateral moyamoya

e402 vessels in cerebral arteries. Scand J Rheumatol 1997; 26:392-394. 5. Nagahiro S, Mantani A, Yamada K, et al. Multiple cerebral arterial occlusions in a young patient with Sjogren’s syndrome: case report. Neurosurgery 1996; 38:592-595. 6. Alexander EL, Craft C, Dorsch C, et al. Necrotizing arteritis and spinal subarachnoid hemorrhage in Sjogren syndrome. Ann Neurol 1982;11:632-635. 7. Alexander GE, Provost TT, Stevens MB, et al. Sjogren syndrome: central nervous system manifestations. Neurology 1981;31:1391-1396.

H. SAKATA ET AL. 8. Vaudo G, Bocci EB, Shoenfeld Y, et al. Precocious intimamedia thickening in patients with primary Sjogren’s syndrome. Arthritis Rheum 2005;52:3890-3897. 9. Ducros A. Reversible cerebral vasoconstriction syndrome. Lancet Neurol 2012;11:906-917. 10. Wang R, Xu Y, Lv R, et al. Systemic lupus erythematosus associated with moyamoya syndrome: a case report and literature review. Lupus 2013;22:629-633. 11. Saadoun D, Lambert M, Mirault T, et al. Retrospective analysis of surgery versus endovascular intervention in Takayasu arteritis: A multicenter experience. Circulation 2012;125:813-819.

Efficacy of extracranial-intracranial bypass for progressive middle cerebral artery occlusion associated with active Sjögren's syndrome: case report.

Sjögren syndrome affecting the major cerebral arteries is rare, and an optimal therapeutic strategy to counteract such a lesion has not yet been estab...
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