No abstract

Introducción: El síndrome de Marfán es un trastorno multisistémico del tejido conectivo de herencia autosómica dominante, de expresión variable. La ectasia dural es un compromiso frecuente, pero poco conocido, que puede asociarse a síndrome de hipotensión endocraneana (SHE).Objetivo: Presentar un caso de cefalea invalidante secundario a SHE, para advertir de esta rara complicación, que debe tenerse presente en niños portadores de conectivopatías, en especial síndrome de Marfán.Caso Clínico: Adolescente femenina de 13 años, portadora de sindrome de Marfán, de diagnóstico clínico según criterios de Ghent 2010, que consultó por cefalea ortostatica invalidante de 6 meses de evolución. La Resonancia Magnetica (RM) de cerebro mostró múltiples signos de hipotensión endocraneana, mientras que la RM de columna total mostró una ectasia dural que determinó la dilatación del saco tecal y remodelación posterior de los cuerpos vertebrales, especialmente a nivel del sacro. Se realizó tratamiento con parche sanguíneo autólogo epidural con buena respuesta clínica.Conclusiones: La ectasia dural, frecuente en el sindrome de Marfán, es una causa predisponente a fuga de líquido cefaloraquideo (LCR), que podría causar cefalea ortostática segundaria al SHE.

Background: Spontaneous cervical artery dissection (sCAD) is a leading cause of ischemic stroke in young patients. Studies using high-resolution magnetic resonance imaging and positron emission tomography have suggested vessel wall inflammation to be a pathogenic factor in sCAD. Computed tomography (CT) attenuation of perivascular adipose tissue (PVAT) is an established non-invasive imaging biomarker of inflammation in coronary arteries, with higher attenuation values reflecting a greater degree of vascular inflammation. Objectives: We evaluate the CT attenuation of PVAT surrounding the internal carotid artery (PVAT_carotid) with and without spontaneous dissection. Methods: Single-centre prospective observational study of 56 consecutive patients with CT-verified spontaneous dissection of the internal carotid artery (ICA) admitted between 2011 and 2018. Of these patients, 6 underwent follow-up CTA. 22 patients who underwent CTA for acute neurological symptoms but did not have dissection formed the control group. Using semiautomated research software, PVAT_carotid was measured as the mean Hounsfield Unit (HU) attenuation of adipose tissue within a defined volume of interest surrounding the ICA. Results: PVAT_carotid was significantly higher around dissected ICA compared with non-dissected contralateral ICA in the same patients (-58.7±10.2 vs. -68.9±8.1 HU, P<0.0001) and ICA of patients without dissection (-58.7±10.2 vs. -69.3±9.3 HU, P<0.0001). After a median follow-up of 89 days, there was a significant reduction in PVAT_carotid around dissected ICA (from -57.5±13.4 to -74.3±10.5 HU, P<0.05); while no change was observed around non-dissected contralateral ICA (from -71.0±4.4 to -74.1±4.1 HU, P=0.19). ICA dissection was an independent predictor of PVAT_carotid following multivariable adjustment for age and the presence of ICA occlusion. Conclusion: PVAT_carotid is elevated in the presence of sCAD and may decrease following the acute event.