Dyke-Davidoff-Masson syndrome that is not accompanied by epilepsy: A case report

Yıl 2013, Cilt: 3 Sayı: 2, 91 - 94, 01.06.2013

Yeşim Güzey Aras Davut Ceylan Dilcan Kotan Can Yaldız Ayhan Bölük

https://doi.org/10.5505/sakaryamj.2013.05706

Öz

Dyke-Davidoff-Masson Syndrome (DMMS), is a clinical entity with features of cerebral hemiatrophy, contralateral hemiparesia and epilepsy. The other findings of the disease are facial asymmetry, mental retardation, sensorineural hearing loss and psychiatric disorders. In radiological evaluation, the disease is marked by an atrophy in unilateral cerebral hemisphere, an expansion in sulcus and ventriculles in the same side, a thickening of the skull, an excessive extension in paranasal sinuses and an increase in airing. The patient, who had a febrile convulsion when seven months old and has never undergone a seizure since, applied to our clinic only with a complaint of dizziness. It is established in the electroencephalogram (EEG) that the female patient, 17 age, has a diffuse slow down in her left hemisphere. The patient, who is diagnosed to have DDMS in the light of clinical and radilogical findings, is presented here as it is a rare case and to emphasize that epilepsy may not necessarily accompany DDMS.

Anahtar Kelimeler

Dyke-Davidoff-Masson Syndrome, Serebral hemiatrphy, Seizure

Epilepsinin eşlik etmediği Dyke-Davidoff-Masson Sendromu: Bir olgu sunumu

Öz

Dyke-Davidoff-Masson Sendromu (DDMS) serebral hemiatrofi, kontralateral hemiparezi ve epilepsi ile karakterize klinik bir antitedir. Hastalığın diğer bulguları fasial asimetri, mental retardasyon, sensorinöral işitme kaybı, psikiyatrik bozukluklardır. Radyolojik incelemelerde ise ünilateral serebral hemisferlerde atrofi, aynı tarafta sulkus ve ventriküllerde genişleme, kafatası kalınlaşması, paranazal sinüslerde aşırı genişleme ve havalanma artışının olması ile karakterize bir tablodur. 7 aylıkken febril konvülsiyon geçiren ve daha sonra hiç nöbeti olmayan hasta polikliniğimize sadece baş dönmesi şikayeti ile müracaat etti. 17 yaşındaki kız olgunun elektroensefalogram (EEG)'ında sağ hemisferde diffüz yavaşlama tespit edildi. Klinik ve görüntüleme bulgularının eşliğinde DDMS tanısı konulan hasta ender görülen bir tablo olması ve epilepsinin eşlik etmeyebileceği vurgulanmak amacıyla sunulmuştur.

Anahtar Kelimeler

Dyke-Davidoff-Masson Sendromu, Serebral hemiatrofi, Nöbet

Kaynakça

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