CASE 13750 Published on 19.06.2016

Dyke Davidoff Mason syndrome



Case Type

Clinical Cases


Samar Hamid1, Sarwanand Khetpal

Department of Radiology, Jinnah Postgraduate Medical Center, Karachi, Pakistan

14 years, female

Area of Interest Neuroradiology brain ; Imaging Technique MR
Clinical History
14-year-old female patient with right-sided hemiparesis and convulsions.
Imaging Findings
Atrophy of the left cerebral hemisphere with exvacuuo dilatation of the left lateral ventricle associated with slight midline shift towards the left side. (Fig. 1) Relative thickening of the skull vault with relatively much more bone marrow / diploic space as compared to the right side. (Fig. 2) Hyperpneumatization of ipsilateral frontal and sphenoid sinuses. (Fig. 3) Elevation of the left petrous ridge along with larger ipsilateral mastoid air cells. (Fig. 4)
Right cerebral compensatory hemihypertrophy causing compression of right lateral ventricle. (Fig. 1)
Intracranial part of the internal carotid artery shows normal flow void and diameter on either side.
Corpus callosum and cerebellar vermis appear unremarkable.
No mass lesion is seen.
Sellar region appears unremarkable.
Posterior fossa appears unremarkable.
Dyke-Davidoff-Masson syndrome (DDMS) refers to atrophy of one cerebral hemisphere which is usually due to insult to the developing brain during perinatal period or early childhood. The underlying aetiology is cerebral insult. Prenatal causes include congenital anomalies, cerebral infarction, vascular malformations and infections. Perinatal causes are birth trauma, hypoxia and intracranial bleed. Postnatal hemiatrophy can develop secondary to cerebral trauma, tumours, infections and febrile seizures. The congenital type, in contrast to the acquired, shows enlargement of calvarium, diploic space and paranasal sinuses. These compensatory cranial changes occur to take up the relative vacuum created by the atrophied cerebral hemisphere. [1]

Clinical perspective
The clinical features are variable and depend on the extent of brain injury. Usually, patients present with seizures, facial asymmetry, contralateral hemiparesis, mental retardation, speech and language disorders. [2]

Imaging perspective
The typical radiological features are cerebral hemiatrophy with ipsilateral compensatory hypertrophy of the skull and sinuses. There are three well-recognized patterns on MR imaging.
Pattern I corresponds to diffuse cortical and subcortical atrophy. Pattern II corresponds to diffuse cortical atrophy coupled with porencephalic cysts. And pattern III corresponds to previous infarction with gliosis in the middle cerebral artery (MCA) territory. In our case, pattern I was present. The atrophied cerebral hemisphere will have prominent sulcal spaces if the vascular insult occurs after the end of sulcation. However, if ischaemia occurs during embryogenesis when the formation of gyri and sulci is deficient, prominent sulcal spaces will be absent.[3]

A proper history, thorough clinical examination, and radiological findings provide the correct diagnosis. In cases of intractable seizures, physicians should consider DDMS as one of the differential diagnoses. Treatment should focus on control of the seizures with suitable anticonvulsants. Along with drugs, physiotherapy and speech therapy play a significant role in long-term management of the child. Hemispherectomy is the treatment of choice for children with intractable disabling seizures. [4] Our patient was put on anticonvulsants and physiotherapy was commenced.

Take Home Message
Dyke-Davidoff-Masson syndrome is a rare epilepsy syndrome which needs to be differentiated from Sturge Weber syndrome, basal ganglia germinoma, Silver- Russel syndrome, Linear nevus syndrome, Fishman syndrome, and Rasmussen encephalitis. [5]
MRI is the modality of choice and aids in evaluating cerebral hemiatrophy and bony structural changes and thus differentiating between congenital and acquired types of DDMS.
It is very important for a neurologist / radiologist to diagnose the condition at its earliest and the treatment should focus on optimum control of seizures along with domiciliary physiotherapy.
Differential Diagnosis List
Dyke Davidoff Masson syndrome
Chronic Rasmussen encephalitis
Sturge-Weber syndrome
Final Diagnosis
Dyke Davidoff Masson syndrome
Case information
DOI: 10.1594/EURORAD/CASE.13750
ISSN: 1563-4086