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The clinico-radiological spectrum of Dyke-Davidoff-Masson syndrome in adults

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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 ± 21 years. 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.

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Authors and Affiliations

  1. Department of Neurology, Sakarya University Training and Research Hospital, Adnan Menderes Road, Saglık St. No: 195, 54100, Adapazarı, Sakarya, Turkey

    Zeynep Özözen Ayas

  2. Department of Radiology, Sakarya University Training and Research Hospital, Adapazarı, Sakarya, Turkey

    Kıyasettin Asil

  3. Department of Neurology, Baskent University Faculty of Medicine, Ankara, Turkey

    Ruhsen Öcal

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  1. Zeynep Özözen Ayas
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  2. Kıyasettin Asil
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  3. Ruhsen Öcal
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Correspondence to Zeynep Özözen Ayas.

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The authors assert that all procedures contributing to this work comply with the ethical standards of the relevant national and institutional committees on human experimentation and with the Helsinki Declaration of 1975, as revised in 2008.

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Ayas, Z.Ö., Asil, K. & Öcal, R. The clinico-radiological spectrum of Dyke-Davidoff-Masson syndrome in adults. Neurol Sci 38, 1823–1828 (2017). https://doi.org/10.1007/s10072-017-3074-7

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  • DOI: https://doi.org/10.1007/s10072-017-3074-7

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