Introduction
Dyke-Davidoff-Masson syndrome (DDMS) is a rare neurological disease characterized by cerebral hypoplasia or hemiatrophy, contralateral weakness, facial palsy, and seizures. It was first defined by Dyke, Davidoff, and Masson in 1933 and usually affects the pediatric population.
Diagnosis
The diagnosis of DDMS relies on comparing clinical symptoms with neuroimaging results. Key imaging indicators include cerebral hypoplasia, ventriculomegaly, paranasal sinus hyper-pneumatization, and compensatory osseous enlargement.
Development of the Brain
During the first year of life, the human brain reaches half of its adult size, with three-fourths attained by the age of three. Improper brain growth can cause changes in head size and shape, leading to conditions like DDMS.
Causes and Manifestations
DDMS occurs due to damage to the developing brain, either during pregnancy or in early childhood. It can manifest at any age, and common causes include trauma, infections, strokes, lack of oxygen during birth, inflammation, or congenital abnormalities.
Radiological Imaging
In 1933, Dyke, Davidoff, and Masson outlined the syndrome through observations of changes seen in plain radiographs and pneumoencephalograms. The condition is divided into two types: Infantile (congenital) and Acquired.
Clinical Presentation
Clinical features of DDMS include recurrent seizures, facial asymmetry, contralateral hemiparesis, and intellectual disability.
Differential Diagnosis
Differential diagnoses include Hemimegalencephaly, Sturge-Weber syndrome, Rasmussen encephalitis, Fishman syndrome, and Silver-Russell syndrome.
Treatment
The treatment of DDMS primarily focuses on symptomatic treatment, often utilizing anticonvulsive medication to control seizures. Hemispherectomy is a potential intervention for severe cases.
Clinical Finding
- Patient Data: 15-year-old male
- Presentation: Seizure and left-side hemiparesis
- CT Brain Finding: Right cerebral atrophy with fronto-parietal encephalomalacia and ex vacuo dilatation of the right lateral ventricle. Ipsilateral compensatory skull vault thickening is also noted.