Clinical Neuroscience

[Severe intracranial hypotension in an adolescent with Marfan syndrome. Case report]

ROSDY Beáta1, KOLLÁR Katalin1, MÓSER Judit1, VÁRALLYAY György2, KORDÁS Mariann3

JUNE 02, 2009

Clinical Neuroscience - 2009;62(05-06)

[Spontaneous intracranial hypotension is a rare complication of connective tissue disorders. One of them is Marfan syndrome. It predisposes the patient to meningeal diverticula. Possibly after minor unrecognised head trauma or secondary to Valsalva manoeuvre cerebrospinal fluid leak from meningeal diverticula can happen. It causes postural headache. We describe a 15 year old adolescent female newly diagnosed with Marfan syndrome, who presented with intractable postural headache. Our patient's brain MRI showed bilateral frontal subdural effusion, narrow ventricles, downward displacement of the brainstem, enlarged cervical venous plexi, dural ectasias and wide nerve root sleeves. Radionuclide cisternography demonstrated CSF leaks at multiple sites. She could not be treated conservatively, but was successfully treated by epidural saline injections. Control brain and cervical MRI confirmed her healing, too. At the two and half year follow up visit, she was completely well. So we recommend this easiest technic to use before epidural autolog blood patches.]

AFFILIATIONS

  1. Fôvárosi Önkormányzat Heim Pál Gyermekkórháza, Neurológia Osztály, Budapest
  2. Szentágothai János Tudásközpont, Semmelweis Egyetem, Budapest
  3. Országos Idegsebészeti Tudományos Intézet, Budapest

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