Dyke-Davidoff-Masson Sendromlu Olguların Değerlendirilmesi

Dyke-Davidoff-Masson Sendromu(DDMS) nadir görülen, temel olarak serebral hemiatrofi, karşı tarafta hemipleji/hemiparezi, epilepsi ve mental retardasyon ile karakterize bir hastalıktır. Etiyolojisine göre akut DDMS ve konjenital DDMS olarak ayrılır. Retrospektif olarak yapılan bu çalışmada çocuk nörolojisi kliniğinde DDMS tanısı ile takip edilen hastaların demografik, klinik ve radyolojik bulgularının sunulması amaçlanmıştır. Çalışmaya 7 erkek 3 kız olmak üzere 10 hasta dahil edildi. Hastaların yaş ortalaması 8.3±1.2 yıldı. Çalışmamızda 8 hasta konjenital DDMS, perinatal serebral iskemisi olan bir hasta ve merkezi sinir sistemi enfeksiyonu geçiren bir hasta olmak üzere toplam 2 hasta ise akut DDMS olarak kabul edildi. Yedi hastada sol, 3 hastada ise sağ serebral hemiatrofi vardı. Yedi hastada hemiatrofi tarafında ensefalomalazik değişiklikler ve 7 hastada hemiatrofi tarafında lateral ventrikül dilatasyonu saptandı. Tüm hastalarda epilepsi vardı ve 5’i dirençli epilepsi hastasıydı. Ortalama DDMS tanısı konulduğu yaş 7±1.08 yıl, ilk beyin görüntülemesi yapılan yaş ise 3.4±1.2 yıldı. Hastaların ilk beyin görüntüleme yaşları ile DDMS olarak tanı konuldukları yaş arasında yaklaşık 2 kat fark var olması, DDMS’ nin bilinirliğinin yeterli olmadığını düşündürmektedir. Hemipleji/hemiparezi, epilepsi ve mental retardasyonu olan, beyin görüntülemesinde hemiatrofi saptanan hastalarda DDMS mutlaka ön tanılar arasında yer almalıdır.

Anahtar Kelimeler:

Dyke-Davidoff-Masson Sendromu, serebral hemiatrofi, hemipleji, epilepsi

Evaluation of Patients with Dyke-Davidoff-Masson Syndrome

Dyke-Davidoff-Masson syndrome (DDMS) is characterized by cerebral hemiatrophy, contralateral hemiplegia/hemiparesis, epilepsy and mental retardation. The purpose of this retrospective study was to emphasize the demographic, clinical and imaging findings of 10 child (7 boys, 3 girls) with DDMS. The mean age of the patients was 8.3 ± 1.2 years. Eight of our patients were considered as congenital DDMS. The remaining 2 patients were considered as congenital DDMS. In these patients, symptoms appeared following postnatal hypoxic ischaemia and central nervous system infection. Seven patients had left and 3 patients had right cerebral hemiatrophy. Ipsilateral lateral ventricular dilatation and encephalomalacia were observed in 7 patients. All patients had unilateral hemiparesis / hemiplegia and mental/motor retardation. Five patients had resistant epilepsy. The mean age for the diagnosis of DDMS was 7 ± 1.08 years, and the initial brain imaging age was 3.4 ± 1.2 years. There were approximately two times the difference between the first brain imaging ages of patients and the age at which they were diagnosed as DDMS. This suggests that the awareness of DDMS is not sufficient. DDMS should be among the preliminary diagnoses in patients with hemiplegia / hemiparesis, epilepsy, and mental retardation and with hemiatrophy in brain imaging.

Keywords:

Dyke-Davidoff-Masson Syndrome, cerebral hemiatrophy, hemiplegia, epilepsy,

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