Malignant Isolated Cortical Vein Thrombosis as the Initial Manifestation of Primary Antiphospholipid Syndrome: Lessons on Diagnosis and Management From a Case Report

Abstract

Background: Antiphospholipid syndrome (APS) with isolated cortical vein thrombosis (ICoVT) is an extremely rare but potentially malignant entity. It is particularly challenging to diagnose APS-related ICoVT because of the non-specific clinical manifestations and the frequent absence of typical neuroimaging. Moreover, there is currently limited knowledge on the clinical features and management strategies for the condition. Delays in diagnosis and treatment may lead to life-threatening consequences.

Case presentation: We present a rare case of a 74-year-old Chinese woman who presented with sudden onset of headache and right arm weakness that mimicked acute ischemic stroke. Her initial computed tomography was unremarkable, and intravenous thrombolysis was performed. Serial neuroimages confirmed ICoVT 4 days after symptom onset, and low-molecular-weight heparin (LMWH) was started at a dose of 0.4 ml twice per day, according to the 2019 Chinese guidelines. The workup for the predisposing causes of ICoVT revealed triple positivity APS. LMWH dose was adjusted according to the anti-Xa chromogenic assay. However, the patient's condition deteriorated rapidly, and there was a progressive enlargement of the venous infarction despite treatment with anticoagulants. Transtentorial herniation developed on day 12, and decompressive craniectomy was immediately performed. The patient's symptoms did not improve significantly after surgery, and she remained aphasic and hemiplegic at the 3-month follow-up, with a modified Rankin Scale score of 5.

Conclusion: ICoVT is a rare yet potentially fatal manifestation of APS, and its diagnosis and treatment are extremely challenging. Timely diagnosis, prompt treatment, and close monitoring are essential to improve the clinical prognosis of patients with APS-related ICoVT.

Keywords: anticoagulation; antiphospholipid syndrome; case report; cortical vein thrombosis; decompressive craniectomy; magnetic resonance.

Figure 1

Neuroradiological data from onset to day 4. (A, B) CT images before and after intravenous thrombolysis showed no abnormalities except punctate hyperdensities within the left frontal sulci (red arrows). (C, D) CT angiography on day 4 showed filling defects of cortical veins (red arrows) and compensatory expansion of the adjacent veins (green arrows). (E, F) MRI on day 3 showed hyperintense T1WI and FLAIR lesions neighboring the falx cerebri and the left parietal cortex (red arrows), which were initially diagnosed as subarachnoid hemorrhages, indicating subacute thrombosis in the cortical veins. (G) MRI on day 4 showed venous infarction in the frontal lobe (green arrow) and the absence of the flowing-void effect in the cortical veins (red arrows). (H) Susceptibility-weighted imaging on day 4 showed curvilinear, serpentine, and exaggerated susceptibility within the sulcus, which was suggestive of thrombosed veins during the acute (red arrows) and subacute phases (greed arrow).

Figure 2

Neuroradiological data after day 4. (A, B) CT images on days 6 and 10 showed progressive enlargement of the venous infarction lesion in the frontoparietal cortex (red arrows). (C) MRI on day 12 showed venous infarction lesions with a large acute hematoma in the left frontoparietal lobe, which resulted in transtentorial herniation (red arrows). (D) CT scan after decompression craniectomy showed large areas of low-density lesions (red arrow). (E) MR black-blood-thrombus imaging (MRBTI) on day 7 showed vein thrombosis that extended proximally to the superior sagittal sinus (red arrow). The superior sagittal sinus was free of thrombosis (green arrow). (F) MR venography showed filling defects of the left frontal and partial cortical veins (red arrows). (G, H) DSA on day 8 showed normal venous flow of the right hemisphere (G, green arrow) compared with multiple thromboses in the left superior and internal cerebral veins of the left hemisphere (H, red arrows).

Figure 3

Timeline of the present case.