Chunchu Venkata Anirudh, Kommalapati Nishitha, Pemma Sai Sarath Kumar, Mane Manohar Manish Prajwal, Nalamalapu Rahul Reddy
Medicine, Avalon University School of Medicine, Willemstad, CUW.
Medicine, John F. Kennedy University, Willemstad, CUW.
Cureus. 2023 Feb 11;15(2):e34868. doi: 10.7759/cureus.34868. eCollection 2023 Feb.
Dyke-Davidoff-Masson syndrome (DDMS) is a rare neurological entity that is predominantly seen in childhood. Here, we present the case of a 13-year-old girl who was brought to the pediatric ward for general examination with a previous history of seizures, speech difficulty, facial deviation, and progressive left-sided hemiparesis that started at the age of two, followed by delayed developmental milestones. Computed tomography (CT) and magnetic resonance imaging (MRI) of the brain showed right cerebral hemiatrophy, ventriculomegaly, hyperpneumatization of the sinus, the decreased caliber of cortical veins, and skull thickening on the right were all characteristic findings of DDMS. Based on the history, clinical presentation, and imaging findings from CT and MRI, DDMS was confirmed. Identifying DDMS in a clinical setting can be challenging because of low awareness of the condition and varied clinical presentations. Although CT and MRI imaging are the gold standards in diagnosing DDMS, the early manifestations of the disease cannot be well-appreciated on a CT and would likely require an MRI. Since there is no standardized protocol for managing DDMS, the treatment is primarily symptomatic. Early identification and diagnosis of the syndrome are essential to aid the child's mental and physical development through a multidisciplinary approach. There is also a need to improve awareness of DDMS so that the condition can be considered a potential differential diagnosis amongst other similar conditions and does not get misdiagnosed. The lack of a proper protocol for the management of DDMS prompts more research for a better understanding and early identification of the condition.
戴克-戴维多夫-马森综合征(DDMS)是一种罕见的神经系统疾病,主要见于儿童期。在此,我们报告一例13岁女孩的病例,她因癫痫发作、言语困难、面部偏斜和进行性左侧偏瘫病史于2岁起发病,随后发育里程碑延迟,被送至儿科病房进行全面检查。脑部计算机断层扫描(CT)和磁共振成像(MRI)显示右侧大脑半球萎缩、脑室扩大、鼻窦气化过度、皮质静脉管径变细以及右侧颅骨增厚,这些都是DDMS的典型表现。根据病史、临床表现以及CT和MRI的影像学表现,确诊为DDMS。在临床环境中识别DDMS可能具有挑战性,因为对该疾病的认识不足且临床表现多样。尽管CT和MRI成像在诊断DDMS方面是金标准,但疾病的早期表现无法在CT上很好地显示,可能需要MRI检查。由于目前尚无管理DDMS的标准化方案,治疗主要是对症治疗。早期识别和诊断该综合征对于通过多学科方法促进儿童的身心发育至关重要。还需要提高对DDMS的认识,以便在其他类似疾病中将其视为潜在的鉴别诊断,避免误诊。缺乏适当的DDMS管理方案促使开展更多研究,以更好地了解和早期识别该疾病。