Rameshbabu Sushmitha, Nallathambi Naveenkumar, Shaaruk D, Mohammed Imaduddin, Syed Neha
Institute of Internal Medicine, Madras Medical College, Chennai, India.
Junior Resident, Royal care Hospital, Coimbatore, India.
Radiol Case Rep. 2025 Jul 8;20(9):4844-4848. doi: 10.1016/j.radcr.2025.06.017. eCollection 2025 Sep.
Dyke-Davidoff-Masson syndrome (DDMS) is a rare neurological condition resulting from prenatal or early childhood brain injury, characterized by seizures, hemiparesis, facial asymmetry, and intellectual disability, with imaging findings including cerebral hemiatrophy, calvarial thickening, and mastoid cell hyperpneumatization. We present the case of a 13-year-old male who exhibited recurrent convulsive seizures predominantly affecting his left side. MRI revealed right-sided cerebral hemiatrophy with ipsilateral calvarial thickening and mastoid hyperpneumatization, consistent with DDMS. Notably, this case was further complicated by coexisting metabolic syndrome, a rare comorbidity in DDMS, which increased the complexity of diagnosis and management. This case highlights the importance of considering DDMS in pediatric patients presenting with focal seizures and hemiparesis, and emphasizes the need to recognize atypical clinical associations such as metabolic syndrome. Early diagnosis and a multidisciplinary approach are essential to improve outcomes and guide future research into broader manifestations of DDMS.
戴克-戴维多夫-马森综合征（DDMS）是一种罕见的神经系统疾病，由产前或儿童早期脑损伤引起，其特征为癫痫发作、偏瘫、面部不对称和智力残疾，影像学表现包括大脑半球萎缩、颅骨增厚和乳突气房过度气化。我们报告了一例13岁男性病例，该患者主要表现为左侧反复发作的惊厥性癫痫发作。磁共振成像（MRI）显示右侧大脑半球萎缩，同侧颅骨增厚和乳突气化过度，符合DDMS表现。值得注意的是，该病例并发代谢综合征，这在DDMS中是一种罕见的合并症，增加了诊断和管理的复杂性。该病例强调了在出现局灶性癫痫发作和偏瘫的儿科患者中考虑DDMS的重要性，并强调需要认识到诸如代谢综合征等非典型临床关联。早期诊断和多学科方法对于改善预后以及指导未来对DDMS更广泛表现的研究至关重要。
