Dyke-Davidoff-Masson syndrome: Classical imaging findings

Singh, Paramdeep; Saggar, Kavita; Ahluwalia, Archana

doi:10.4103/1817-1745.76108

Cited by 45 publications

(38 citation statements)

References 2 publications

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“…Pattern I corresponds to diffuse cortical and subcortical atrophy. Pattern II corresponds to diffuse cortical atrophy coupled with a porencephalic cyst, while pattern III corresponds to previous infarction with gliosis in the middle cerebral artery (MCA) territory [10]. The clinical profile and imaging features in our patient are in keeping with acquired DDMS pattern III.…”

Section: Discussionsupporting

confidence: 65%

Dyke–Davidoff–Masson syndrome in a Nigerian

Adebayo

Bakare

Bello

et al. 2017

Epilepsy & Behavior Case Reports

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Dyke–Davidoff–Masson syndrome (DDMS) is a rare, but important cause of drug-resistant seizures. Dyke–Davidoff–Masson syndrome is a constellation of clinical features that consists of hemiparesis, seizure, facial asymmetry, and intellectual disability with distinct neuroimaging features. A 27-year-old lady presented to us with drug-resistant epilepsy, hemiparesis, and intellectual disability that necessitated her withdrawal from school. Her brain magnetic resonance imaging (MRI) showed cerebral hemiatrophy, calvarial thickening, and hyperpneumatization of the frontal sinuses consistent with DDMS. We discuss the diagnostic and therapeutic implications of DDMS and advocate early referral and evaluation of people with epilepsy in sub-Saharan African settings.

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Section: Discussionsupporting

confidence: 65%

Dyke–Davidoff–Masson syndrome in a Nigerian

Adebayo

Bakare

Bello

et al. 2017

Epilepsy & Behavior Case Reports

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“…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].…”

Section: Introductionmentioning

confidence: 99%

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

2017

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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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“…The compensatory cranial changes occur to take up the relative vacuum created by the atrophied or hypoplastic cerebral hemisphere. [7] Hageman et al . proposed the terms cerebral hemihypoplasia or unilateral cerebral hypoplasia for the primary (congenital) cerebral atrophy owing to the fact that there is a lack of cerebral development rather than atrophy.…”

Section: Discussionmentioning

confidence: 99%

“…[6] MRI has the ability to bring light changes in the cerebral hemispheres as well as highlighting bony structures, thus differentiating between congenital and acquired types of DDMS. [7]…”

Section: Discussionmentioning

confidence: 99%

Dyke–Davidoff–Masson syndrome: A rare cause of cerebral hemiatrophy in children

Thakkar

Dave

2016

J Pediatr Neurosci

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Dyke–Davidoff–Masson syndrome (DDMS) is an uncommon condition, in which the diagnosis is mainly done by various clinical presentations along with positive radiological findings. Patients have facial asymmetry, seizures, learning difficulties, and contralateral hemiparesis. The radiological discoveries of the same incorporate cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses. Here, we report a case of a 10-year-old female child who presented with a single episode of convulsion, mental retardation, and contralateral hemiparesis. Magnetic resonance imaging of the brain showed unilateral atrophy of the left cerebral hemisphere with dilatation of ipsilateral lateral ventricle and ipsilateral sulcal prominence. These findings were suggestive of the diagnosis of DDMS.

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Dyke-Davidoff-Masson syndrome: Classical imaging findings

Abstract: A 15-year-old female presented with seizures, right-sided hemiparesis, hemiatrophy of the right side of the body and mental retardation. MRI brain revealed characteristic features diagnostic of congenital type of cerebral hemiatrophy or Dyke–Davidoff–Masson syndrome.

Cited by 45 publications

References 2 publications

Dyke–Davidoff–Masson syndrome in a Nigerian

Dyke–Davidoff–Masson syndrome in a Nigerian

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

Dyke–Davidoff–Masson syndrome: A rare cause of cerebral hemiatrophy in children

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