A rare syndrome, Dyke-Davidoff-Masson Syndrome (DDMS), with a diagnostic conundrum, and the way it was solved is presented. A 13-yearold boy presented with recurrent seizures for the past 10 years. He had been treated with anticonvulsant medication which was satisfactory at first but later the seizures recurred. Recently, the frequency of the seizures increased with preictal dizziness and postictal drowsiness. Physical examination revealed mild left hemiparesis and left deviated gait irregularity. He was mentally alert but had not achieved all the developmental milestones as compared to normal child of his age. CT and MRI scan of the head showed hemiatrophic cerebral parenchyma with prominent sulci and encephalomalacia. 24-hour intensive video EEG monitoring revealed suppression of alpha rhythm and local slow wave activity on the side of the atrophic hemisphere. PET-CT showed highly functional left cerebral hemisphere and less functional right cerebral hemisphere. The patient underwent functional hemispherectomy under neurophysiological monitoring and the nonfunctional brain tissues were resected while selectively preserving the functional areas detected by fMRI and PET-CT scan. During follow up, the patient was seizure free as well as without difficulties in performing his daily activities and communications. Functional hemispherectomy for DDMS patient has a good prognosis.KeywOrds: Dyke-Davidoff-Masson syndome, Cerebral hemiatrophy, Seizure, Functional hemispherectomy ÖZ Nadir bir sendrom olan ve tanı koyması güç olan Dyke-Davidoff-Masson Sendromu (DDMS) ve tanı konma şekli sunulmaktadır. 13 yaşında bir erkek son 10 yıldır tekrarlayan havalelerle başvurdu. Antikonvülsan ilaçlarla tedavi başlangıçta tatminkar olmuş ama daha sonra havaleler tekrarlamıştı. Son zamanlarda havale sıklığı artmış ve preiktal başdönmesi ve postiktal uyku hali başlamıştı. Fizik muayene hafif sol hemiparezi ve sola deviasyonlu yürüme bozukluğu gösterdi. Mental olarak uyanıktı ama kendi yaşındaki normal bir çocuğun gelişimsel referans noktalarının tümüne ulaşmamıştı. Baş BT ve MRG taraması belirgin sulkuslar ve ensefalomalasi ile hemiatrofik serebral parankim gösterdi. Yapılan 24 saatlik yoğun video EEG izleme, atrofik hemisfer tarafında alfa ritim baskılanması ve yerel yavaş dalga aktivitesi ortaya çıkardı. PET-BT çok işlevsel bir sol serebral hemisfer ve daha az işlevsel bir sağ serebral hemisfer gösterdi. Hastaya nörofizyolojik izleme altında işlevsel hemisferektomi yapılıp işlevsel olmayan beyin dokuları rezeksiyonu gerçekleştirilirken fMRG ve PET-BT taraması ile saptanan işlevsel bölgeler selektif olarak korundu. Takip sırasında hasta havale geçirmedi ve ayrıca günlük faaliyetleri ve iletişimini devam ettirmekte zorluk çekmedi. DDMS hastası için işlevsel hemisferektominin prognozu iyidir.
Intracranial clear-cell meningioma (CCM) is rarely reported in the literature since it has to be distinguished from other subtypes of meningioma. Most of the CCMs are intraspinal, according to the related literature. We report a case of occipital parietal CCM in a 6-year-old child, review all the 35 intracranial CCMs that have been reported since 1995 to present and discuss their clinical, radiological and histopathologic characteristics.
The Enterprise stent is very useful for endovascular embolization of wide-necked intracranial aneurysms, because it is easy to navigate and place precisely. The overall morbidity and mortality rates are low.
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