IS Case 245: Cerebral hemiatrophy - Dyke-Davidoff-Masson syndrome
Imaging Sciences URMC 2008
Publication Date: 2009-05-26
Patient is a 23-year-old male with seizures.
Axial CT images of the head demonstrate unilateral atrophy of the left cerebral hemisphere, with associated ipsilateral thickening of the calvarium. There is hyperpneumatization of the mastoid air cells and paranasal sinuses. There is Wallerian degeneration seen of the left cerebral peduncle and prominence of the right cerebellar sulci. The middle cranial fossa is reduced in size.
There are multiple causes of cerebral hemiatrophy, including both congenital and acquired lesions that may affect the immature brain, resulting in neuronal loss and impaired brain growth. Congenital malformations, infection, vascular insults, birth trauma, anoxia, intracranial hemorrhage, tumors, and prolonged febrile seizures have all been implicated. Rasmussen's encephalitis is a rare, chronic inflammatory disease usually seen in children under the age of 10 that can lead to the finding of unilateral cerebral atrophy and neurologic deficits.
With regard to the above patient, Dyke, Davidoff, and Masson described a series of nine patients with cranial asymmetry on skull plain films in 1933. The unique features of Dyke-Davidoff-Masson syndrome include calvarial thickening at the site of cerebral hemiatrophy and hyperpneumatization of the paranasal sinuses and mastoid air cells. There may be elevation of the petrous ridge and reduced size of the middle cranial fossa as well. 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. Clinically, patients demonstrate varying degrees of of hemiplegia, hemiparesis, seizures, mental retardation, and facial asymmetry. All of these features with the exception of facial asymmetry are documented clinical findings in the above patient. There is minimal documentation on the suspected etiology of the patient’s findings, as he is from a refugee camp in Asia and is not English-speaking.
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