Yaşar BÜKTE, ASLIHAN SEMİZ OYSU, Faysal EKİCİ, SALİH HATTAPOĞLU, Zakir SAKCI, Elif FİDAN

Dyke-Davidoff-Masson sendromu (serebral hemiatrofi): Radyolojik bulgular

Amaç: Dyke-Davidoff-Masson Sendromu (DDMS) olan olguların kesitsel kranial görüntüleme bulgularının sunulması amaçlanmıştır. Yöntemler: Bilgisayarlı tomografi (BT) ve/veya manyetik rezonans görüntüleme (MRG) ile unilateral serebral hemisferik atrofisi saptanmış olan 16 olgunun bulguları retrospektif olarak değerlendirildi. Olguların 8i kadın ve 8i erkek olup, yaşları 5 ile 53 (ortalama: 24) arasında de- ğişiyordu. Olguların 6sı çocuk ve 10u erişkindi. Olguların 5inde BT ve 13ünde MRG tetkiki yapılmıştı. Olguların görüntüleri retrospektif olarak incelenerek serebral parankimal bulgular ve kompansatuar kranial bulgular not edildi. Bulgular: Tüm olgularda unilateral serebral hemisfer atrofisi, ipsilateral kortikal sulkuslarda ve lateral ventrikülde genişleme mevcuttu. Hemisferik atrofi ile birlikte 6 olguda (%37,5) korpus kallozumun ipsilateral atrofisi, 13 olguda (%81) ipsilateral talamik atrofi, 8 olguda (%50) ipsilateral parahipokampal atrofi, 7 olguda (%44) ipsilateral serebral pedinkülde atrofi ve 3 olguda (%19) ipsilateral pons atrofisi saptandı. Olguların 13 tanesinde (%81) gliotik sinyal değişiklikleri gözlendi. Kompansatuar bulgulardan unilateral kalvariyal kalınlaşma 4 olguda (%25) fokal, 12 olguda (%75) diffüz olarak izlendi. Frontal sinüs ipsilateral yarımında genişleme 15 olguda (%94) ve ipsilateral temporal kemik havalanmasında artış ise 5 olguda (%31) mevcuttu. Sonuçlar: DDMS, serebral hemiatrofi ve kalvarial hipertrofi bulguları ile kendini gösteren bir sendromdur. Kesitsel radyolojik görüntüleme bulguları olgular arasında çeşitlilik gösterebilir.

Dyke-Davidoff-Masson syndrome (cerebral hemiatrophy): Radiological findings

Objective: The aim of this study was to present crosssectional cranial imaging findings of cases with DykeDavidoff-Masson syndrome (DDMS). Methods: The findings of 16 cases in whom unilateral cerebral hemispheric atrophy was detected at computed tomography and/or magnetic resonance imaging were retrospectively evaluated. The cases consisted of 8 females and 8 males, the ages ranged between 5 and 53 (mean:24). Six of the cases were children and 10 were males. Five of the patients had CT and 13 had MRI scan. The images were retrospectively evaluated and cerebral parenchymal findings and compensatory cranial findings were noted. Results: All cases had unilateral cerebral hemispheric atrophy, ipsilateral cortical sulcal and lateral ventricular dilatation. Together with hemispheric atrophy ipsilateral atrophy of corpus callosum in 6 cases (37.5%), ipsilateral thalamic atrophy in 13 cases (81%), ipsilateral parahippocampal atrophy in 8 cases (50%), ipsilateral cerebral pedincular atrophy in 7 cases (44%) and ipsilateral pontine atrophy in 3 cases (19%) were detected. Gliotic signal changes were observed in 13 cases (81%). Of compensatory findings, unilateral calvarial thickening was focal in 4 cases (25%), and diffuse in 12 cases (75%). There was expansion in ipsilateral half of frontal sinus in 15 cases (94%) and expansion in temporal bone aeration in 5 cases (31%). Conclusion: DDMS is a syndrome presenting with findings of cerebral hemiatrophy and calvarial hypertrophy. Cross-sectional radiological imaging findings may be variable among cases.

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