Image series - 1

Image series – 2 Bone window.

Legend: The above images show right sided cerebral atrophy with frontal, sphenoidal and mastoid air sinus hypertrophy and calvarial thickening.

Introduction: DDMS is a rare condition characterized clinically by variable degrees of facial asymmetry, seizures, contralateral hemiparesis, mental retardation and learning disabilities. Both sexes and any of the hemispheres may be affected but male gender and left hemisphere involvement are more frequent. Age of presentation depends on time of neurologic insult and characteristic changes may be seen only in adolescence.

Etiology: This syndrome is considered to be due to cerebral insult in fetal or early childhood period. Early insult leads to complete unilateral cerebral involvement whereas late insult causes more localized changes. Decreased unilateral blood flow in co-arctation of aorta can sometimes cause cerebral hemiatrophy. The cerebral atrophy is actually a lack of cerebral development rather than atrophy.

Clinical features: Depends on the extent of brain injury. Varying degrees of atrophy of one half of body, sensory loss, speech and language disorder, mental retardation or learning disability and psychiatric manifestations like schizophrenia may also be present. A proper history, thorough clinical examination and radiologic findings provide the correct diagnosis.

Radiological features: The radiological features are classical (reflecting unilateral cerebral atrophy with compensatory overgrowth of adjacent structures) with asymmetry of cerebral hemispheric growth with atrophy on one side, ipsilateral osseous hypertrophy and hyperpneumatization of sinuses and mastoid cellular system. There is widening of ipsilater calvarial diploic space, elevation of greater wing of sphenoid and tilting of the planum sphenoidale.

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