Abstract
Dyke-Davidoff-Masson syndrome (DDMS) is characterized by cerebral hemiatrophy, seizure, contralateral hemiplegia/hemiparesis, and mental retardation. In this study, clinical and radiological investigations of seven patients who were diagnosed with DDMS as adult age were evaluated and discussed. Seven patients (four male, three female) were included. The mean age±SD of the patients was 46±21years. Clinical presentation of six patients was epileptic seizure. One patient was presented with head trauma due to a fall. Two patients had complex partial seizures, three patients had generalized tonic-clonic seizures (GTC), and one had GTC and myoclonic seizure. Mental retardation was in five patients. A congenital cause was detected in one patient in the etiologic investigation and acquired causes in two patients. In four patients, the etiology was not identified. We observed left-hemisphere involvement in four patients and right-hemisphere involvement in three patients. Brain imaging was performed by CT only in four patients and by MRI only in three patients. All patients were diagnosed with DDMS at adulthood. Atrophy in basal ganglia was detected in five patients, and atrophy in brain stem in four patients. Calvarial thickening was observed in four patients. Three patients had hyperpneumatization in mastoid cells. Sinus hyperpneumatization, including the paranasal and frontal sinuses, was seen in six patients. DDMS can also be diagnosed in adulthood symptomatically (mild-severe) or asymptomatically in adulthood. As a result, DDMS is a syndrome with wide clinical and radiological spectra that can be variably symptomatic at different stages of life.
Highlights
Dyke-Davidoff-Masson syndrome (DDMS) is characterized by cerebral hemiatrophy, convulsions, facial asymmetry, controlateral hemiparesis/hemiplegia, and mental retardation
Diagnosis, which is important in terms of the approach to the disease and the treatment, is based on clinical findings, computerized tomography (CT), and magnetic resonance imaging (MRI)
Severe mental retardation was detected in 2 of the patients presenting with seizures, while mild mental retardation was detected in 3 patients
Summary
Dyke-Davidoff-Masson syndrome (DDMS) is characterized by cerebral hemiatrophy, convulsions, facial asymmetry, controlateral hemiparesis/hemiplegia, and mental retardation. Radiological characteristics include the loss of unilateral cerebral parenchyma, compensatory changes in the calvarial bones on the same side (such as thickening), hyperpneumatization of the paranasal sinuses, elevation of the temporal bone, brainstem atrophy, and thalamic atrophy [1,2]. Diagnosis, which is important in terms of the approach to the disease and the treatment, is based on clinical findings, computerized tomography (CT), and magnetic resonance imaging (MRI). Investigations of seven patients who were diagnosed with DDMS as adults are evaluated and discussed. Dyke-Davidoff-Masson syndrome (DDMS) is characterized by cerebral hemiatrophy, epileptic seizures, contralateral hemiplegia/hemiparesis, and mental retardation
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