Intracranial aneurysm associated with chronic mucocutaneous candidiasis

• Published in: European journal of pediatrics Vol. 167; no. 4; pp. 461 - 463
• Main Authors: Marazzi, M. G., Bondi, E., Giannattasio, A., Strozzi, M., Savioli, C.
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer-Verlag 01.04.2008; Springer; Springer Nature B.V
• Subjects: Biological and medical sciences; Candidiasis, Chronic Mucocutaneous - complications; Candidiasis, Chronic Mucocutaneous - diagnosis; Cerebral Angiography; Diagnosis, Differential; Fatal Outcome; General aspects; Humans; Infant; Intracranial Aneurysm - diagnosis; Intracranial Aneurysm - etiology; Male; Medical sciences; Medicine; Medicine & Public Health; Neurology; Pediatrics; Short Report; Tomography, X-Ray Computed; Vascular diseases and vascular malformations of the nervous system; Fluconazole; Intracranial aneurysm; Candida albicans; Pediatrics; Nervous system diseases; Candidiasis; Mycosis; Cardiovascular disease; Cerebral disorder; Fungi; Infection; Vascular disease; Antifungal agent; Chronic; Association; intracranial aneurysm; Central nervous system disease; Triazole derivatives; Mucocutaneous; Fungi Imperfecti; Fluconazole· Intracranial aneurysm; Cerebrovascular disease
• ISSN: 0340-6199; 1432-1076
• DOI: 10.1007/s00431-007-0490-3

Chronic mucocutaneous candidiasis (CMC) is a syndrome characterised by immune deficiency, often presenting familial dominant inheritance and association with autoimmune endocrinopathies. We report on a patient with CMC who died at 5 years of age of a brain haemorrhage following the rupture of a basilar-artery aneurysm. Candida hyphae in the basilar artery were found at autopsy. A common immunologic abnormality in CMC is the failure of patient’s T-lymphocytes to produce cytokines, which are essential for expression of cell-mediated immunity to Candida . Therefore, long-term treatment is mandatory.