Overview
Dyke-Davidoff-Masson syndrome (DDMS) is a rare congenital disorder characterized by hemiparesis, seizures, facial asymmetry, and intellectual disability, primarily diagnosed through imaging techniques 1.Diagnosis
Key Diagnostic Criteria: Unilateral hemiatrophy, absence of cerebral arteries (e.g., anterior and middle cerebral arteries), and facial asymmetry 1.
Recommended Tests: Antenatal ultrasonography and postnatal MRI are crucial for early detection and detailed assessment 1.
Novel Diagnostic Indicator: "Shifted falx sign" has been proposed as a new imaging marker for DDMS 1.Management
First-Line Treatments: Management focuses on symptomatic treatment, including anticonvulsants for seizures and physical therapy for motor deficits 1.
Adjunctive Treatments: Cognitive and developmental support therapies are essential for addressing intellectual disability and developmental delays 1.Special Populations
Pregnancy: Antenatal diagnosis via ultrasound can be achieved, highlighting the importance of routine prenatal imaging 1.
Pediatrics: Early postnatal MRI is critical for comprehensive evaluation and management planning 1.Key Recommendations
Antenatal ultrasound should be considered routine to detect early signs of DDMS, particularly unilateral hemiatrophy, facilitating timely postnatal imaging 1 (Evidence: Moderate).
Postnatal MRI is essential for definitive diagnosis and detailed assessment of cerebral anomalies in DDMS 1 (Evidence: Moderate).
Implement a multidisciplinary approach including neurology, physical therapy, and developmental support for comprehensive management 1 (Evidence: Expert opinion).References
1 Aggarwal A, Aggarwal AK, Kapoor A, Kapoor R, Bansal A. Hemiatrophy of brain: antenatal ultrasonography and MRI/postnatal MRI diagnosis with the introduction of "shifted falx sign". Journal of medical ultrasonics (2001) 2017. link