antiphospholipid antibodies are circulating immunoglobulins that are associated with both venous and arterial thrombotic events .
the antiphospholipid antibody syndrome is defined by 2 major criteria : the occurrence of at least 1 clinical thrombotic event or pregnancy morbidity ( either 1 otherwise unexplained fetal death after 10 weeks gestation , 1 premature birth before 34 weeks gestation because of preeclampsia , eclampsia or placental insufficiency , or 3 unexplained pregnancy losses before 10 weeks gestation ) , and the presence of antiphospholipid antibodies measured on 2 or more occasions at least 12 weeks apart .
venous thrombosis is the most common systemic manifestation of the antiphospholipid antibody syndrome , but within the central nervous system , arterial thrombosis is more common than venous thrombosis .
arterial stroke is the presenting symptom in 13% of patients with the antiphospholipid antibody syndrome [ 2 , 3 ] .
imaging studies of patients with the antiphospholipid antibody syndrome have emphasized the parenchymal changes noted on ct and mri .
these studies have shown that subcortical infarcts and hyperintense white matter foci are common in patients presenting both with and without clinical strokes .
fewer studies have reported on the angiographic findings in stroke patients with the antiphospholipid antibody syndrome .
these studies have shown predominantly intracranial stem or branch occlusions and irregularities of the cerebral vessel walls consistent with vasculopathy [ 4 , 5 ] .
these angiographic findings suggest a link between the antiphospholipid antibody syndrome and cerebral arteriopathies and raise the possibility that arteriopathies and a hypercoagulable state are both implicated in the increased risk of arterial stroke .
whereas most arteriopathies in stroke patients with the antiphospholipid antibody syndrome involve the intracranial circulation , occasionally the extracranial internal carotid artery is affected .
little is known about this manifestation and about the natural progression of stenosis of the extracranial internal carotid artery in patients with the antiphospholipid antibody syndrome .
we describe a young patient who experienced progressive steno - occlusive disease of her bilateral extracranial internal carotid arteries in the setting of antiphospholipid antibodies .
a 39-year - old woman was transferred to our hospital after being found unconscious at home .
she was confused , but was able to give a history of left - sided weakness with decreased use of her left hand and difficulty bearing weight on her left leg that had begun abruptly 3 days prior to presentation .
she had had one prior outside hospital admission 1 year earlier for a left middle cerebral artery stroke that presented with expressive aphasia and right arm and leg weakness .
limited mri at that time revealed 2 areas of restricted diffusion in the left parietal lobe with increased flair signal and associated cortical / subcortical enhancement .
axial t1-weighted images showed the absence of a flow void in the left internal carotid artery , while the right internal carotid artery appeared patent based on the presence of a normal flow void .
the patient underwent stereotactic biopsy of the left parietal lesion to rule out a neoplasm .
she was discharged to inpatient rehabilitation , and over the subsequent months her speech and motor function completely recovered .
three years prior to her current presentation , the patient had had a miscarriage at 12 weeks gestation .
her history was also significant for intravenous methamphetamine use , ending 13 years prior to presentation , and an 18 pack - year smoking history .
she did not use birth control pills and did not have a history of deep venous thrombosis or pulmonary embolism .
her family history was remarkable for systemic lupus erythematosus in her father and a brother who was diagnosed with thrombotic thrombocytopenia purpura . on examination , her blood pressure was 126/80 mm hg , and her heart rate was 91 beats / min and regular .
she was alert and oriented to person , but not place , time , or situation .
her speech was fluent , with intact repetition and naming and without aphasia or dysarthria .
cranial nerve examination was significant for a left homonymous hemianopsia and left lower facial weakness .
her motor exam revealed left hemiparesis , left hyperreflexia , and a left babinski sign .
she had intact sensation of light touch bilaterally , but extinction on the left with double simultaneous stimuli .
abnormal coagulation studies included a prolonged activated partial thromboplastin time at 42.6 s ( normal range 25.137.6 s ) which did not correct with a 1:1 mix with normal plasma , a thromboplastin inhibition test that was strongly positive for lupus anticoagulant , and a dilute russell 's viper venom test that was prolonged at 64.3 s ( normal 2946 s ) which corrected with the addition of phospholipids .
other coagulation studies , which included anticardiolipin and anti-2 glycoprotein antibodies , were within normal limits .
erythrocyte sedimentation rate ( 55 mm / h ) and c - reactive protein ( 5.7
mg / l ) were moderately elevated , and the antinuclear antibody assay was elevated at 1:160 .
other studies were normal or negative , including complete blood count , platelet count , renal , liver , and thyroid functions , hemoglobin a1c , and ldl cholesterol .
a ct scan showed areas of evolving infarction in the right anterior and middle cerebral artery distributions .
ct angiography showed bilateral internal carotid artery occlusions extending from the level of the bifurcation to the supraclinoid segments .
mri showed areas of acute infarction within the right middle cerebral artery - anterior cerebral artery watershed territory and chronic infarcts in the left middle cerebral artery - anterior cerebral artery watershed territory ( fig .
1 ) . conventional angiography confirmed bilateral internal carotid artery occlusions in the proximal cervical segments , with reconstitution of the carotid arteries at the supraclinoid segments via the ethmoidal branches of the distal internal maxillary arteries and the ophthalmic arteries ( fig .
the posterior circulation was normal , with extensive collaterals to the anterior circulation via the posterior communicating arteries .
no abnormalities concerning for vasculopathy or dissection were noted in the aortic arch or in the carotid , vertebral , subclavian , renal , or femoral arteries .
our patient met clinical and laboratory criteria for a diagnosis of the antiphospholipid antibody syndrome .
she was young , had a characteristic history of prior spontaneous abortion at more than 10 weeks gestation , and had a strongly positive thromboplastin inhibitor test consistent with lupus anticoagulant antibodies .
her presentation with strokes secondary to bilateral carotid artery occlusions suggests an association between the antiphospholipid antibody syndrome and a vasculopathy involving the extracranial segments of the internal carotid arteries . the causal relationship between the antiphospholipid antibody syndrome and bilateral carotid occlusions
is further supported by the absence of reasonable alternative causes of carotid occlusions in this young patient .
specifically , there was no history of head or neck trauma and no evidence of dissection or atherosclerosis on imaging .
she was a smoker , but she had no history of hypertension , hyperlipidemia , or other vascular risk factors .
her medical history indicates that vasculopathy of the internal carotid arteries in the setting of the antiphospholipid antibody syndrome can be rapidly progressive .
mri at that time was notable for an absence of a flow void in the left internal carotid artery , consistent with a left carotid occlusion , and the presence of a flow void in the right internal carotid artery , indicating patency of that vessel . within 1 year , however , she presented with a right middle cerebral artery stroke , and workup revealed progression to bilateral carotid artery occlusions . in summary ,
the findings suggest that the antiphospholipid antibody syndrome may be implicated in pathological changes that can result in occlusions of the proximal segments of the internal carotid arteries .
consequently , young stroke patients who present with unexplained internal carotid artery occlusions may benefit from testing for the presence of antiphospholipid antibodies .
 a 39-year - old woman presented with a right - hemispheric stroke 1 year after she had suffered a left - hemispheric stroke . 
 her diagnostic workup was notable for bilateral occlusions of the internal carotid arteries at their origins and a positive lupus anticoagulant antibody test . 
 there was no evidence of carotid dissection or another identifiable cause for her carotid occlusions . 
 these findings suggest that the antiphospholipid antibody syndrome may be implicated in the pathological changes that resulted in occlusions of the extracranial internal carotid arteries . 
 young stroke patients who present with unexplained internal carotid artery occlusions may benefit from testing for the presence of antiphospholipid antibodies . 
Introduction
Case Report
Discussion