From pathophysiology to symptoms of chronic cerebrospinal venous insufficiency

Chronic Cerebrospinal Venous Insufficiency (CCSVI), and its variant limited to the internal jugular veins, is a multifactorial condition characterized by reduced extracranial venous outflow due to valvular abnormalities, intraluminal lesions, structural anomalies, and extrinsic compressions. This results in dysfunction of the glymphatic system with cerebrospinal fluid stasis, increased intracranial pressure, and cerebral hypoperfusion. The objective of this study was to collect and integrate all symptoms potentially caused by CCSVI and to explain the underlying pathophysiological mechanism. A systematic analysis of the recent literature concerning CCSVI was performed, focusing on the anatomical causes, diagnostic evaluation (Color Doppler Ultrasound, CDUS; Computed Tomography, CT angiography; Magnetic Resonance, MR venography; catheter venography; Single-Photon Emission Computed Tomography, SPECT/CT), and the clinical implications of reduced extracranial venous outflow. The alteration of venous outflow through the internal jugular veins leads to intracranial hypertension, cerebral hypoperfusion, and dysfunction of the glymphatic system with stagnation of cerebrospinal fluid. These mechanisms determine a heterogeneous symptomatologic picture that includes dysesthesia, ipsilateral facial pain, morning soft tissue swelling, vertigo, hypoacusis, diplopia, headache, chronic fatigue, and bladder control disorders, as well as other related symptoms. Scientific evidence also shows symptomatic improvement following angioplasty or autologous venous stenting. CCSVI is a multifactorial syndrome that requires both a multimodal diagnostic approach and a multidisciplinary therapeutic strategy.