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Case 92

Dyke-Davidoff-Masson syndrome (Cerebral hemiatrophy)

A boy with learning difficulties and left-sided hemiparesis

Ozgur Kilickesmez

This 17 year old boy with left-sided hemiparesis, and seizures since infancy, presented for MRI of the cranium.

Findings

There is obvious atrophy of the right hemisphere, certainly in the temporal and frontal lobes with enlargement of the adjacent subarachnoid spaces, frontal, temporal and occipital horns of the right lateral ventricle. Also the right frontal sinus is larger than the left. Diploic spaces on the right seem to be widened. The other findings are ipsilateral diploic calvarial expansion, elevation of the petrous bone and orbital roof, and hypoplasia/atrophy of the cerebral peduncle.

Diagnosis

Dyke-Davidoff-Masson syndrome (Cerebral hemiatrophy)

Differential

Hemimegalencephaly (the ventricle on the affected side is smaller)
Focal cortical atrophy

Discussion

Cerebral hemiatrophy or Dyke-Davidoff-Masson syndrome is a condition characterized by seizures, facial asymmetry, contralateral hemiplegia or hemiparesis, and learning difficulties. These findings are due to cerebral injury that may occur early in life or in utero. Insult to immature brain results in neuronal loss and impaired brain growth. The causes in the prenatal period are congenital malformation, infection and vascular insult; in the perinatal period birth trauma, anoxia, hypoxia and intracranial haemorrhage. Postnatal causes are trauma, tumor, infection and prolonged febrile seizures.

The radiological features are unilateral loss of cerebral volume and associated compensatory bone alterations in the calvarium, such as thickening, hyperpneumatization of the paranasal sinuses and mastoid cells and elevation of the petrous ridge. Although computed tomography (CT) and MR are complimentary, it is felt that MR represents the imaging procedure of choice with respect to the assessment of the aetiology and extent of cerebral parenchymal involvement in patients presenting with a clinical combination of congenital or early onset of seizures, hemiparesis/plegia, and/or craniofacial asymmetry.

References

1. Romeo Araujo D, Pego Reigosa R, Branas Fernandez F, Martinez Vazquez F, Cortes Laino JA. [Contralateral cerebellar atrophy in the Dyke-Davidoff-Masson syndrome] Neurologia. 1999 Jun-Jul;14(6):320-1. PMID: 10439628
2. Aguiar PH, Liu CW, Leitao H, Issa F, Lepski G, Figueiredo EG, Gomes-Pinto F,Marino Junior R. MR and CT imaging in the Dyke-Davidoff-Masson syndrome. Report of three cases and contribution to pathogenesis and differential diagnosis. Arq Neuropsiquiatr. 1998 Dec;56(4):803-7. PMID: 10029885
3. Sener RN, Jinkins JR. MR of craniocerebral hemiatrophy. Clin Imaging. 1992 Apr-Jun;16(2):93-7. PMID: 1547482

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