cerebral venous sinus thrombosis as an i”>American Journal of Emergency Medicine 31 (2013) 888.e1-888.e3

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American Journal of Emergency Medicine

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Case Report

Cerebral venous sinus thrombosis as an initial manifestation of primary Antiphospholipid syndrome?

Abstract

Cerebral venous sinus thrombosis is a rare neurologic manifesta- tion of antiphospholipid syndrome. We report a case of a 49-year-old woman who presented to the emergency department with Recurrent episodes of transient clumsiness of the left upper extremity. The results of unenhanced brain computed tomography and electroen- cephalography were unremarkable. Serial neuroimages revealed superior sagittal sinus thrombosis. Thrombophilia screen disclosed positive lupus anticoagulant. In this case report, we aim to emphasize the significance of recognizing an Unusual presentation of antipho- spholipid syndrome.

A 49-year-old, unmarried, Han Chinese woman with no significant medical history presented with sudden-onset left clumsy thumb followed by floppy left arm lasting approximately 10 minutes. On arrival at the emergency department, her symptoms subsided spontaneously. There were no previous histories of hypertension, diabetes mellitus, or autoimmune disorders. On clinical examination, she was found to be fully conscious and had normal blood pressure, but was mildly febrile (38.0?C). Result of the Neurological examination was unremarkable. There were no significant findings in routine laboratory tests (complete blood count, electrolytes, kidney and liver function tests), inflammatory markers, analysis for rheumatic diseases (antinuclear antibody, Rheumatoid factor, anti-double-stranded DNA antibody), cerebro- spinal fluid study, electroencephalography, and unenhanced com- puted tomographic scan of the brain except mild thrombocytopenia (128,000 platelets per microliter). In spite of management with Antiplatelet agents (acetylsalicylic acid), several recurrent episodes of transient left upper limb paresis with a duration between 20 minutes and 2 hours were noticed at the emergency department and during the initial hospitalization.

In considering Transient ischemic attacks , magnetic resonance imaging (MRI) of the brain was performed. The MRI of the brain showed vasogenic edema over the right precentral gyrus; T2-star and susceptibility-weighted MRI in the area corresponded with thrombus and microhemorrhages (Fig. 1A-D). Brain MR venography disclosed focal poor signal intensity at the middle portion of the superior sagittal sinus (Fig. 2A). Results of thrombo- philia screen that included homocysteine levels, protein C, Protein S, antithrombin III activity, and anticardiolipin immunoglobulins G

? Authorship: All authors had access to the data and played a role in writing this manuscript.

and M were unremarkable; however, lupus anticoagulant was positive at presentation and 1 year later. She was started on anticoagulant therapy, initially with low-molecular-weight heparin (Fraxiparine, Sanofi Winthrop Industrie, Notre Dame de Bonceville, France) and later with oral warfarin. Her symptoms of transient, episodic left upper extremity paresis markedly diminished. There were no further Neurological deficits after she was discharged from the hospital. Recanalization of the thrombosis was demonstrated on the MR venography 3 months later (Fig. 2B).

Antiphospholipid syndrome (APS) is an autoimmune disease characterized by recurrent vascular thrombosis and/or pregnancy morbidity, mainly associated with antiphospholipid antibodies (aPL), including anticardiolipin antibody, anti-?₂ glycoprotein I, and lupus anticoagulant [1]. The most common neurological manifestation of APS is arterial stroke, taking charge of high morbidity and mortality [2]. The deep venous thrombosis of lower extremities occurred commonly in patients with APS, but rarely cerebral venous sinus thrombosis [3], that accurate diagnosis of CVST in APS patients is usually delayed.

The traditional definition of TIAs was sudden-onset neurological dysfunction with symptoms lasting less than 24 hours due to focal cerebral ischemia with or without infarcts. Recently, tissue-based TIA has been defined as “an episode of transient Neurologic dysfunction resulting from focal brain, spinal cord, or retinal ischemia, without acute infarction” [4]. Our patient presented with recurrent episodes of neurological deficits lasting less than 24 hours, with a normal result in the cranial computed tomographic study, and had TIAs as the Initial diagnosis. However, serial neuroimages showed superior sagittal sinus thrombosis; and APS was confirmed finally.

Cerebral venous thrombosis is a rare form of cerebrovascular disease, which accounts for 0.5% to 1.0% of all strokes [5]. The most prevalent clinical presentations of CVST are isolated Intracranial hypertension, headache, focal neurological abnormalities, seizures, and encephalopathy [6]. Our patient with the final diagnosis of primary APS presented rarely with cerebral venous thrombosis as an initial manifestation. In this case, there were neither risk factors for atherosclerosis nor other predisposed factors for secondary throm- bosis. The precise mechanism of nerve system involvement in APS remains unclear. Previous studies demonstrated that aPL promote a prothrombotic state by activating endothelium cells, platelets, and monocytes [7]. There is also evidence that the anti-?₂ glycoprotein I antibodies and complement activation play a critical role in the pathophysiology of aPL-induced thrombosis [8].

The long-term vital and functional prognosis of cerebral venous thrombosis is much better than that of arterial stroke [9]. Antic- oagulation is regarded as safe and efficacious therapy for cerebral

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888.e2 Case Report / American Journal of Emergency Medicine 31 (2013) 888.e1–888.e3

Fig. 1. Brain MR images including T2-weighted image (A), diffusion-weighted imaging (B), and apparent diffusion coefficient map (C) demonstrate high signal intensity in the right precentral region (arrow). Susceptibility-weighted MRI reveals the thrombus and microhemorrhages (D).

venous thrombosis with or without the appearance of intracranial hemorrhage [10]. Therefore, we initiated low-molecular-weight heparin (Fraxiparine) for the treatment and shifted to oral warfarin subsequently. The Therapeutic effect was good.

In summary, CVST is an uncommon neurologic manifestation in APS; delayed diagnosis is not unusual. The occurrence of recurrent episodes of transient focal neurological deficits should raise the clinical suspicion of CVST, and APS should be taken into account in patients without definite risk factors for thrombosis.

Chia-Lin Tsai MD Department of Neurology, Tri-Service General Hospital National Defense Medical Center, Taipei, Taiwan

E-mail address: [email protected]

Dueng-Yuan Hueng MD, PhD Department of Neurological Surgery, Tri-Service General Hospital National Defense Medical Center, Taipei, Taiwan

E-mail address: [email protected]

Fig. 2. Magnetic resonance venography of the brain illustrates thrombosis of the superior sagittal sinus (A, arrow) and recanalization of the thrombosis 3 months later (B, arrow).

Case Report / American Journal of Emergency Medicine 31 (2013) 888.e1–888.e3 888.e3

Wen-Long Tsao MD

Department of Neurology, Buddhist Dalin Tzu Chi General Hospital

Chiayi, Taiwan E-mail address: [email protected]

Jiann-Chyun Lin MD, PhD Department of Neurology, Tri-Service General Hospital National Defense Medical Center, Taipei, Taiwan

E-mail addresses: [email protected], [email protected] http://dx.doi.org/10.1016/j.ajem.2012.12.020

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