A case of spontaneous intracranial hypotension in a patient of Ehler Danlos syndrome

Spontaneous intracranial hypotension oriinally described in 1938, is a syndrome charcterized by postural headache and low cerebrospinal fluid (CSF) pressure. Headache may be associated with nausea, vomitting and less commonly diplopi or cranial neuropathies. We report 32 years old woman with Ehler Danlos Syndrome who spontaneously developed postural headache, nausea and vomiting. Neurologic examination was normal. LP and cranial MR1 supported the diagnosis. It is possibe that underlying connective tissue disorder Ehler Danlos Syndrome is the reason of spontaneous intracranial hypotension.

Ehler Danlos Sendromlu Hastada Geliþen Spontan Ýntrakraniyal Hipotansiyon

Spontan intrakraniyal hipotansiyon ilk kez 1938’de tanýmlanan postural baþ aðrýsý ve düþük beyin omurilik sývýsý (BOS) basýncý ile karakterize bir tablodur. Aðrýya eþlik eden ense sertliði, bulantý, kusma, diplopi, kraniyal nöropatiler daha az sýklýkta tanýmlanmaktadýr. Çalýþmada 4 haftalýk postural baþ aðrýsý öyküsü olan 32 yaþýnda kadýn hasta sunulmaktadýr. Ehler Danlos sendromu tanýsý almýþ olan hasta ani geliþen ve yatmakla düzelen baþ aðrýsý, bulantý, kusma tanýmlamaktadýr. Nörolojik muayenesinde özellik saptanmayan hastanýn lomber ponksiyon (LP) ve Kraniyal manyetik resonans görüntüleme (MRG) bulgularý tanýyý destekleyicidir. Travma ve LP öyküsü olmayan hastada tablonun Ehler Danlos Sendromu ile iliþkili olarak geliþmiþ olabileceði akla gelmektedir.