Isolated Superficial Sylvian Vein Thrombosis With Long Cord Sign: Case Report and Review of the Literature

Abstract

Isolated cortical vein thrombosis (ICVT) is extremely rare. Only single case or small series of ICVT have been reported; clinical details are still uncertain. We report a case of isolated superficial sylvian vein thrombosis with exceedingly long cord sign. A 14-year-old female with severe sudden onset headache visited our hospital. Fluid attenuated inversion recovery and echo-planar T 2 * susceptibility-weighted imaging (T 2 *SW) showed a long cord sign on the surface of the sylvian fissure. The patency of dural sinuses and deep cerebral veins were confirmed by magnetic resonance venography (MRV), and diagnosis of ICVT was made. She recovered completely without anticoagulant agents. To clarify the clinical characteristics of ICVT, we reviewed 51 ICVT cases in the literature. In many cases, T 2 *SW was the most useful examination to diagnose ICVT. In contrast with general cerebral venous thrombosis, MRV and conventional angiography were either supporting or useless. Anastomotic cortical veins were involved frequently; symptoms of gyri around the veins were common. It also suggested that ICVTs of the silent area might have been overlooked because of nonspecific symptoms, and more patients with ICVT may exist. In cases involving patients with nonspecific symptoms, the possibility of ICVT should be considered.

Isolated cortical vein thrombosis: case series

“…The youngest ICVT cases reported previously occurred in two 14-yearold patients. 20,43 There was a female predominance in this case series, which is also consistent with previously published data. 22,23 Patients with ICVT may present with a variety of symptoms, including focal or generalized seizures, headaches, hemiparesis, sensory disturbances, and other focal deficits, often without signs of elevated intracranial pressure.…”

“…A recent review also concluded that MRV results are neither objective nor definitive, and their role in ICVT diagnosis is either supportive or useless. 20 In this case series, MRV was most useful in confirming the patency of the venous sinuses and deep cerebral veins and determining recanalization of the ICVT at follow-up.…”

“…9,14,15,18,22,29,30,35,47 Headaches and seizures were the most common clinical symptoms among the 7 patients in this case series, which is consistent with historical data. 20,22,29,35,47 Common risk factors for ICVT include: 1) a hypercoagulable state due to, for example, oral contraceptive use, postpartum status, or hematological malignancies; 2) head trauma; 3) intracranial hypotension; and 4) infections of the ear, nose, and throat. 20,22,29,35,40,47 Five patients had a least 1 risk factor for ICVT; of these 5 patient, 3 had two risk factors for ICVT.…”

A case of isolated cortical venous thrombosis presenting radiographically as a subacute multifocal leukoencephalopathy, and review of literature

“…ICVT is a rare and poorly understood condition that has only been documented in case reports or case series (116 total patients studied in a comprehensive 2014 review of the literature)1 and represents less than 0.063% of all cerebral strokes 2. It frequently involves anastomotic surface vessels such as the vein of Trolard, the vein of Labbé, the superficial Sylvian vein and the precentral, postcentral and central veins.…”

“…In patients presenting with ICVT as a solitary diagnosis, the mortality rate is 0%; with good outcome 1. While most patients who are diagnosed with ICVT receive anticoagulation and it is considered the standard of care, outcomes may not be different between patients who receive anticoagulation and those who do not 2…”