Postural headache in marfan syndrome associated with spinal cysts and liquor hypotension

Neuropediatrics
N C VoermansE J van Lindert

Abstract

We here report a 13-year-old Marfan patient who suffered from severe, medication-resistant, intermittent headache, which was provoked when getting into an upright position and immediately relieved by lying down or after intravenous rehydration. The postural benefit and the sudden relief after intravenous hydration suggested (intermittent) intracranial hypotension, although a normal opening pressure on lumbar punction was observed and no cerebrospinal fluid (CSF) leakage was identified. Imaging studies revealed severe dural ectasia at lumbosacral level, and two intradural cysts and two extradural presacral cysts were detected. Most likely, altered hydrodynamics in intra- and extracranial spinal meningeal cysts caused intermittent CSF hypotension above these cysts, resulting in intermittent intracranial hypotension. Surgical marsupialisation of the intradural cysts proved to be effective. This resulted in a significant reduction of the headache during the clinical follow-up of eight years.

Citations

Feb 7, 2015·American Journal of Medical Genetics. Part C, Seminars in Medical Genetics·Marco CastoriPaola Grammatico
Aug 15, 2013·The Journal of Spinal Cord Medicine·Paul M Arnold, Jan Teuber
Mar 5, 2013·The Journal of Pediatrics·Wouter I SchievinkLiliana Sloninsky
Sep 14, 2011·Cephalalgia : an International Journal of Headache·Marco A ArrudaMarcelo E Bigal
Dec 12, 2019·American Journal of Medical Genetics. Part a·Thy Thy VanemSvend Rand-Hendriksen
Jul 8, 2021·Neuropediatrics·Lisa BallmannMiriam Michel

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