Positron emission tomography in Dyke-Davidoff-Masson syndrome

Dyke-Davidoff-Masson syndrome is clinically characterized by hemiparesis, hemiplegia, seizures, mental retardation, and facial asymmetry secondary to congenital or early childhood vascular insult. A 21-year-old man with Dyke-Davidoff-Masson syndrome presented with uncontrolled seizures. The authors present the magnetic resonance (MR) and positron emission tomography (PET) findings of this syndrome.     

Dyke-Davidoff-Masson syndrome: A case report with a literature review

Dyke-Davidoff-Masson syndrome (DDMS) is an uncommon neurological disease defined as cerebral hemiatrophy with a contralateral motor deficit, facial asymmetry, and seizures. Classic imaging findings are cerebral hypoplasia, ventriculomegaly, paranasal sinus hyper-pneumatization, and compensatory osseous enlargement. The diagnosis of DDMS is based on the correlation between clinical and neuroimaging features. The management of DDMS is based on anticonvulsant medication with physiotherapy. We describe an unusual case of DDMS presented with frequent and persistent seizures.     

Dyke-Davidoff-Masson syndrome: A rare case of hemiatrophy of brain—Case report from Nepal

Dyke-Davidoff-Masson syndrome (DDMS) is a rare neurological disorder that results from brain injury during intrauterine or early years of life. Prominent cortical sulci, dilated lateral ventricles, cerebral hemiatrophy, hyperpneumatization of the sinus, and compensatory hypertrophy of the skull are the characteristic findings. We describe a female patient who presented with a history of seizure, right-sided body weakness, and neuroimaging features of left cerebral hemiatrophy, dilatation of left lateral ventricle, left frontal sinus hyperpneumatization, asymmetric calvarial thickening, and elevation of the petrous ridge.     

Dyke-Davidoff-Masson综合征MRI特征(附5例报告并文献复习)

目的分析Dyke-Davidoff-Masson综合征(Dyke-Davidoff-Masson syndrome,DDMS)的MRI特征。方法回顾性分析5例DDMS患者的临床及MRI表现并进行文献复习。结果 5例均为右侧大脑萎缩并同侧侧脑室扩张,其中中线结构向患侧轻度移位1例,同侧丘脑、豆状核萎缩1例,大脑脚萎缩4例;颅骨增厚5例,额窦及乳突小房增大3例,岩骨嵴抬升2例。结论 DDMS的MRI表现具有特征性,结合相应临床征象,能定性诊断DDMS。     

Left hemisphere and male sex dominance of cerebral hemiatrophy (Dyke-Davidoff-Masson Syndrome)

Although radiological findings of cerebral hemiatrophy (Dyke-Davidoff-Masson Syndrome) are well known, there is no systematic study about the gender and the affected side in this syndrome. Brain images in 26 patients (mean aged 11) with cerebral hemiatrophy were retrospectively reviewed. Nineteen patients (73.5%) were male and seven patients (26.5%) were female. Left hemisphere involvement was seen in 18 patients (69.2%) and right hemisphere involvement was seen in eight patients (30.8%). We conclude that male gender and left side involvement are frequent in cerebral hemiatrophy disease.     

戴克-大卫杜夫-梅森综合征CT、MRI诊断及鉴别诊断(附3例报告并文献复习)

目的 探讨戴克-大卫杜夫-梅森综合征(Dyke-Davidoff-Masson syndrome,DDMS)的临床及影像学表现.方法 回顾性分析3例DDMS患者的临床及影像学资料并作文献复习.结果 3例均出现单侧大脑萎缩,同侧侧脑室扩大,中线结构向患侧偏移,1例出现脑软化及脑穿通畸形,1例出现丘脑及豆状核萎缩.3例出现患侧颅骨增厚,2例出现鼻窦过度发育,2例颅窝缩小,1例乳突窦增大及岩骨嵴抬升.结论 DDMS具有比较典型的影像学表现,结合临床表现影像学可以正确诊断.     

Midaortic syndrome associated with fetal alcohol syndrome

Midaortic syndrome (MAS) is an uncommon condition characterized by progressive narrowing of the abdominal aorta and its branches and impressive formation of collateral circulation. It affects children and young adults and presents predominantly as untreatable hypertension. Fetal alcohol syndrome (FAS) refers to a constellation of physical, behavioral, and cognitive abnormalities secondary to alcohol exposure in utero. The authors present an unusual association between a hypoplastic abdominal aorta and fetal alcohol syndrome. The patient discussed in this article presented with severe hypertension that was successfully treated with renal angioplasty.     

Rothmund-Thomson syndrome,Klippel-Feil syndrome,and osteosarcoma

We report on a 33 year old woman with Rothmund-Thompson syndrome, Klippel-Feil syndrome and osteosarcoma. We briefly discuss the relationship of these diseases and suggest that the cause for mental retardation is cerebral atrophy as shown on imaging.     

Budd-Chiari syndrome

Modern imaging techniques are presented in a case of Budd-Chiari syndrome. The etiological, clinical and therapeutic aspects of this rare type of hepatic vein occlusion are discussed.     

Boerhaave's syndrome

Spontaneous esophageal perforation (Boerhaave syndrome) is a life-threatening emergency. It usually has misleading clinical manifestations and causes severe complications. In the presence of the clinical manifestations: vomiting, pain, emphysema, this diagnosis must be considered and confirmed by radiological evaluation of the chest and the esophagus. We report two cases and we describe the severity of this syndrome, its cause, its pathogenesis, and its characteristic clinical features and radiological signs.     

Pippow's syndrome

For the first time since the initial report of aplasia of vertebral joints and brachydactyly occurring in a sibship, a similar family history of this rare dystostosis is presented. The following triad of symptoms is significant: a) hypoplasia of the laminae and of the articular and transverse processes of the vertebrae in the thoracolumbar region; b) cranial shift of the vertebral junctions; and c) brachydactyly. "Pippow's syndrome" and "Pippow's dysostosis" are suggested as possible names for this condition.