A Systematic Review Of The Clinical And Imaging Features Of Dyke-Davidoff-Masson Syndrome

ObjectivesDyke-Davidoff-Masson syndrome(DDMS)is a rare cerebral parenchyma dysplasia syndrome.The main clinical manifestations are refractory seizures,contralateral hemiparesis or hemiplegia,mental retardation and facial asymmetry.This disease is not common in clinic and it is easy to be ignored by clinicians.This study summarizes the clinical and imaging features of patients with DDMS to improve clinicians' understanding of rare diseases.Reduce misdiagnosis and missed diagnosis,and improve the prognosis of patients and improve the quality of life as much as possible.MethodsThe clinical data of 75 patients with DDMS were collected through PubMed,EMBASE,Medline,Cochrane Library and Chinese literature databases including CNKI,CQVIP and WANFANG.The sex and age distribution,clinical symptoms,neuroimaging manifestations,EEG features and etiology of DDMS patients were collected and analyzed.ResultsA total of 67 articles were retrieved,including 75 DDMS patients and including 40 males(53.3%)and 35 females(46.7%).72 cases(96.0%)had seizures.62 cases(82.7%)had hemiparesis or hemiplegia,of which 17.3% had hemiatrophy.Six patients less than 4 years old had growth and development retardation,such as turning over,sitting up,standing or walking delay.In the age > 4 years old group,52 cases(75.4%)had mental retardation.The decrease of intelligence in patients with DDMS was significantly differences in different genders,and seemed to be more obvious in males.It was not significantly related to the location of the focus,the complication of hemiplegia,the etiological type and the age of the first attack of epilepsy(P > 0.05).Hemispheric atrophy was found in 74 cases(98.7%),32 cases(42.7%)on the right side and 43 cases(57.3%)on the left side.There were significant enlargement of ventricle,sulcus and cistern in 60 cases(80.0%),midline shift in 13 cases(17.3%),midbrain atrophy in 15 cases(20.0%),contralateral cerebellar atrophy in 14 cases(18.7%),skull thickening in 50 cases(66.7%)and ipsilateral paranasal sinus enlargement in 49 cases(65.3%).The change of bone structure was not related to sex,etiology,age of onset and focus(P > 0.05).ConclusionsThe patients with DDMS often show seizures,mental retardation,hemiparesis or hemiplegia.In this study,there were more male patients than female patients,and the left cerebral hemisphere was more involved than the right hemisphere.Mental retardation in patients with DDMS,as an important factor seriously affecting the quality of life of patients,is not significantly related to the involved hemisphere,etiology,age of first onset of epilepsy and hemiplegia,but there are gender differences in this symptom,and male patients are more likely to develop mental retardation.Brain imaging is the most important means of DDMS diagnosis,and obvious imaging findings can be found on CT and MRI.Skull thickening and paranasal sinus enlargement are the characteristics of DDMS,which are not significantly related to sex,etiology,involved hemisphere and age of etiology.