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Intracranial hypotension in a girl with Marfan syndrome: case report and review of the literature
Authors:E. Cheuret  T. Edouard  M. Mejdoubi  P. Acar  C. Pienkowski  C. Cances  S. Lebon  M. Tauber  Y. Chaix
Affiliation:1.Unité de Neurologie Pédiatrique,H?pital Purpan,Toulouse Cedex,France;2.Unité d’Endocrinologie Pédiatrique,H?pital Purpan,Toulouse,France;3.Unité de Cardiologie Pédiatrique,H?pital Purpan,Toulouse,France;4.Unité de Neuroradiologie,H?pital Purpan,Toulouse,France
Abstract:
Introduction A 14-year-old girl, followed in our department for Marfan syndrome, presented with postural headache for a month. Neurological examination was normal. The diagnosis of intracranial hypotension syndrome was suspected. Discussion Bilateral subdural hematomas were found on brain magnetic resonance imaging (MRI), and spinal MRI showed large lumbosacral arachnoid diverticula; no cerebrospinal fluid leaks could be found. Despite bed rest and hydration for 2 weeks, postural headache remained. Epidural blood patching was also performed. Subsequently, the patient became asymptomatic and could stand up after 1 day. Brain MRI did not find recurrent subdural hematoma after 1 month. Dural ectasia is one of the major criteria of Marfan syndrome, and it is often poorly symptomatic. Intracranial hypotension is a rare complication especially in children, and management is not standardized. In this case report, blood patching was sufficient. Further research into the diagnosis and management of spontaneous intracranial hypotension is required.
Keywords:Marfan syndrome  Intracranial hypotension  Dural ectasia  Epidural blood patch
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