Azygos Vein Stenosis in Frontotemporal Dementia Sagging Brain Syndrome

• DOI: 10.3174/ajnr.a8532
• Publication Date: 2025-01-23T19:25:19Z
• Authors: Wouter I. Schievink; Marcel M. Maya; Rola Saouaf; H. Gabriel Lipshutz; Rachelle B. Taché; Daniel Scoffings; Jeremy D. Schmahmann
• Keywords: Azygos vein

<h3>BACKGROUND AND PURPOSE:</h3> Symptoms indistinguishable from behavioral-variant frontotemporal dementia (bvFTD) can develop in patients with spontaneous intracranial hypotension associated severe brain sagging. An underlying spinal CSF leak be identified only a minority of these and the success rate nondirected treatments, such as epidural blood patching dural reduction surgery, is low. The disability bvFTD sagging syndrome high and, because importance venous system pathophysiology leaks general, we have investigated systemic circulation those recalcitrant symptoms. <h3>MATERIALS METHODS:</h3> We reviewed medical records imaging studies 21 consecutive whom no could found who underwent (MR- or CT-venography). SIH Disability Assessment Score (SIHDAS) questionnaire was completed to assess severity <h3>RESULTS:</h3> mean age 3 women 18 men 50 years (range, 26–68 years). Seven were stenosis. Endovascular stent placement moderate high-grade azygos vein stenosis resulted prompt remarkable improvement symptoms 2 (SIHDAS: very mild disability) 1 patient disability). Treatment internal jugular inferior vena cava each did not result any (5 patients) surgical (2 interruption multiple pathways clinical improvement. <h3>CONCLUSIONS:</h3> main conduit between space this study demonstrates that isolated may cause loss syndrome. In study, yield finding clinically important treatable lesion among relatively low (15%). However, quality safe noninvasive available, eg, MR-venography, should considered for exhausted treatments devastating condition, focusing on system.