Intracranial hypotension as a contributor to isolated cortical vein thrombosis.

Abstract

Isolated cortical vein thrombosis (ICVT), a rare type of cerebral venous thrombosis (CVT), is diagnostically challenging in some cases, and intracranial hypotension (IH) is known to cause CVT. In this study, we reviewed the clinical and imaging characteristics of ICVT in patients with IH caused by spinal cerebrospinal fluid leakage, based on a literature review and investigation of cases from our hospital. Between January 1, 2007, and November 1, 2019, 735 patients were diagnosed with IH at our hospital; three patients developed ICVT (incidence ~ 0.4%, 3/735), and the literature review yielded an additional 23 cases. Therefore, 26 patients (mean age 35.9±11.4 years old) were included in this study. The most common symptoms were headache (100.0%, 26/26), focal neurological deficits (53.8%, 14/26), and seizure (34.6%, 9/26). The initial headache was orthostatic in 96.2% (25/26) of patients, and 38.5% (10/26) of patients reported a change in the headache pattern following diagnosis of ICVT. Neuroimaging findings associated with ICVT included the cord sign (61.5%, 16/26) and parenchymal brain lesions (46.2%, 12/26), such as intracerebral hemorrhage (30.8%, 8/26), hemorrhagic infarcts (11.5%, 3/26), and localized edema (11.5%, 3/26). The percentage of patients who received anticoagulation and epidural blood patch therapy was similar (69.2% [18/26] vs. 65.4% [17/26]), and most patients recovered completely (92.3%, 24/26). IH should be considered in the differential diagnosis in patients with ICVT. Knowledge of the relevant clinical and neuroimaging features is important to facilitate early diagnosis for favorable prognosis.