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

Abstract

BackgroundAntiphospholipid 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 PresentationWe 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.ConclusionICoVT 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.

Lupus, antiphospholipid syndrome, and stroke: An attempt to crossmatch

“…For example, aCL IgA has been found positive at moderate titers in a case of isolated cortical vein thrombosis. 73 However, the titers were not repeated for confirmation. Similarly, rare data suggest the involvement of aβ2GPI IgA in CVT pathophysiology although no follow-up confirmatory test was performed.…”

“…Similarly, rare data suggest the involvement of aβ2GPI IgA in CVT pathophysiology although no follow-up confirmatory test was performed. 73 No direct evidence exists to support a relationship between aPS/PT IgG or aPS/PT IgM and CVT. Given that aPS/PT IgG might be related to LAC suggests a potential role for this isotype in CVT, although more studies are needed to study such an association.…”

Cerebral venous thrombosis in patients with autoimmune disease, hematonosis or coronavirus disease 2019: Many familiar faces and some strangers

“…48 The predominant forms of clinical presentation are acute and subacute, with headaches being the most prevalent symptom reported in 74% of published cases. 15,49 In a single-center study of the management of venous stroke patients with APS by Shen et al, 50 local endovascular thrombolysis, endovascular mechanical thrombectomy, and internal jugular vein stenting were utilized in patients who suffered from anticoagulant inefficacy, with satisfying outcomes. Glucocorticoids and intravenous immunoglobulin and/or plasma exchange therapy are applied in catastrophic APS-associated venous stroke, which is characterized by microvascular thrombosis resulting in multiorgan failure.…”

“…APS with venous stroke as the earliest presentation was initially described in 2008 48 . The predominant forms of clinical presentation are acute and subacute, with headaches being the most prevalent symptom reported in 74% of published cases 15,49 …”

“… 48 The predominant forms of clinical presentation are acute and subacute, with headaches being the most prevalent symptom reported in 74% of published cases. 15 , 49 …”