title: Baroreflex failure, sympathetic storm, and cerebral vasospasm in fibulin-4 cutis laxa.
abstract: Sudden, severe, and life-threatening, the crises associated with baroreflex failure are diagnostically challenging, particularly in children, a population in which it has rarely been described. The baroreflex failure syndrome results from impaired afferent baroreceptive input and manifests with autonomic stimulation-induced surges in blood pressure and heart rate accompanied by distinct signs, including thunderclap headache, diaphoresis, and emotional instability. Although the adult literature includes cases of severe headache in baroreflex failure,(1) (,) (2) we present the first case of a child with recurrent thunderclap headache and cerebral vasospasm with baroreflex failure secondary to vascular complications of a rare genetic connective tissue disorder.
authors.affiliation: Section of Neurology, and Departments of Pediatrics, thilinie.rajapakse@albertahealthservices.ca.
authors.forename: Thilinie
authors.initials: T
authors.lastname: Rajapakse
authors.affiliation: Section of Neurology, and Departments of Pediatrics.
authors.forename: Aleksandra
authors.initials: A
authors.lastname: Mineyko
authors.affiliation: Departments of Pediatrics.
authors.forename: Caroline
authors.initials: C
authors.lastname: Chee
authors.affiliation: Departments of Clinical Neurosciences and Surgery and.
authors.forename: Suresh
authors.initials: S
authors.lastname: Subramaniam
authors.affiliation: Pediatrics and Cardiac Sciences, University of Calgary, Alberta, Canada Cardiology, and.
authors.forename: Frank
authors.initials: F
authors.lastname: Dicke
authors.affiliation: Medical Genetics, Alberta Children's Hospital, Alberta, Canada; and.
authors.forename: Francois P
authors.initials: FP
authors.lastname: Bernier
authors.affiliation: Section of Neurology, and Departments of Pediatrics.
authors.forename: Adam
authors.initials: A
authors.lastname: Kirton
mesh_terms: D001342:Autonomic Nervous System Diseases / Q000175:diagnosis / Q000235:genetics*; D017704:Baroreflex / Q000235:genetics*; D002343:Carotid Artery, Internal / Q000002:abnormalities / Q000473:pathology; D002533:Cerebral Angiography; D002648:Child; D003483:Cutis Laxa / Q000175:diagnosis / Q000235:genetics*; D004252:DNA Mutational Analysis; D016326:Extracellular Matrix Proteins / Q000235:genetics*; D051270:Headache Disorders, Primary / Q000175:diagnosis / Q000235:genetics; D006801:Humans; D018810:Magnetic Resonance Angiography; D008297:Male; D012021:Reflex, Abnormal / Q000235:genetics*; D013577:Syndrome; D014057:Tomography, X-Ray Computed; D020301:Vasospasm, Intracranial / Q000235:genetics*
chemical_list: C403672:EFEMP2 protein, human; D016326:Extracellular Matrix Proteins
keywords: baroreflex; child; cutis laxa; intracranial vasospasm; thunderclap headache