Abstract
This article aims to present and interpret two instances of Dyke-Davidoff-Masson syndrome
(DDMS), with the main features of cerebral hemiatrophy and compensatory skull changes,
focusing on the clinical spectrum, imaging features, and variations in timing of presentation.
Two male patients with left cerebral hemisphere involvement were evaluated. The first
case was an 8-year-old child presenting with intellectual disability, delayed milestones,
right hemiparesis, abnormal jerky movements, and generalized seizures. The second
case was a 22-year-old adult with hemiparesis and generalized seizures since birth.
Both underwent detailed neurological examination and magnetic resonance imaging (MRI)
on a 3T scanner, including time-of-flight (TOF) angiography to assess cerebral vasculature.
MRI of the first patient demonstrated left cerebral hemiatrophy with dilatation of
the ipsilateral lateral ventricle and sulcal prominence, mild falcine deviation toward
the affected side, and compensatory hypertrophy of the left calvarial bones (temporal,
parietal, frontal, occipital) with enlargement of the frontal sinus and mastoid air
cells and elevation of the left petrous ridge. Mild attenuation of the left middle
cerebral artery (MCA) segments was seen on TOF imaging. The second patient's MRI revealed
left cerebral hemisphere atrophy with gliosis in the MCA territory, left ventricular
dilatation, ipsilateral sulcal prominence, and a neuroglial cyst in the left insular
cortex with similar bony changes as those of the previous patient. TOF angiography
showed attenuation of the left MCA segments without filling defects. DDMS is associated
with a certain specific set of clinical and radiological features. Early recognition
through MRI and computed tomography is essential for appropriate management and improved
outcomes. These cases illustrate the variability in presentation and emphasize the
importance of considering DDMS in the differential diagnosis of unilateral cerebral
atrophy with neurological deficits.
Keywords
Dyke-Davidoff-Masson syndrome - cerebral hemisphere atrophy - calvarial hypertrophy
- middle cerebral artery
