Dyke-Davidoff-Masson Syndrome

Clinical History: A 27-year-old man had a history of hemiplegia and seizures since childhood. The seizures were partially controlled with medication. The present hospital visit was due to breakthrough seizure activity.
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Figure 1: Axial T2 and T1 images at level of lateral ventricles demonstrates atrophy of the right cerebral hemisphere with enlarged sulcal spaces and a midline shift to left. The right cerebral cortex is thinned, the basal ganglia are small and there is mild increased T2 signal in the deep white matter.

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Figure 2: T1W image at the same level as in Figure 1 shows calvarial thickening along with the cerebral atrophy. Note the thickened fat density falx. 

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Figure 3: Axial T2 image at level of midbrain reveals atrophy of the cerebral peduncle. Hypertrophy of the frontal sinuses is also evident.

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Figure 4: Coronal T2 image showing atrophy of the left cerebellar hemisphere along with right cerebral atrophy.

Diagnosis: Dyke-Davidoff-Masson Syndrome (Cerebral Hemiatrophy)

Discussion: MRI of the brain showed atrophy of the right cerebral hemisphere, midline shift to right and associated sulcal widening and prominence of the right lateral ventricle. The grey matter is thinned and there is reduced volume and T2 hyperintensity of the deep white matter. Atrophy of the right thalamus, basal ganglia and cerebral peduncle was present.  The contra lateral cerebellar hemisphere was atrophied – this was best demonstrated on the coronal images. Associated compensatory calvarial hypertrophy and enlarged frontal sinuses were also present.

Cerebral hemiatrophy may be congenital or acquired. The basic underlying etiology is a vascular insult with resultant hypoperfusion and impaired growth or destruction of neuronal tissue. The congenital variety is usually an intrauterine vascular occlusion. Acquired causes are more numerous and may be due to ischemia, vasculitis, trauma, tumors, prolonged infantile seizures, hemorrhagic conditions or infections.

Magnetic resonance imaging is the imaging of choice for evaluation of the extent of parenchymal loss and also for assessment of possible etiology. Calvarial overgrowth and enlargement of paranasal sinuses occurs in congenital and infantile types of hemiatrophy as a compensatory mechanism and can be differentiated from the adult acquired type where bony changes are not a feature. Enlarged sulcal spaces are present when the vascular insult occurs after birth or after sulcation. This is in contrast with congenital type when the insult is in the early intrauterine period before sulcation has completed and therefore sulcal widening is not a feature.

Dr Arti Chaturvedi MD
Senior Consultant, Department of Radiodiagnosis
Fortis International Hospital, Noida, India

References:

1. Singh P, Saggar K, Ahluwalia A. Dyke-Davidoff-Masson syndrome: Classical imaging findings. J Pediatr Neurosci 2010;5:124-5.

2. Shetty DS, Lakhkar BN, John JR. Dyke-Davidoff-Masson syndrome . Neurol India 2003;51:136