Dyke-Davidoff-Masson Syndrome as a rare congenital hemiatrophy: a case report

Introduction: Dyke-Davidoff-Masson syndrome (DDMS) is a rare condition in childhood with very few cases reported in sub-Saharan Africa. Typically, the patient presents with facial asymmetry, seizures, and hemiparesis. Radiological findings include cerebral hemiatrophy, ipsilateral lateral ventricular dilatation, and hypertrophy of the calvarium and sinuses. Case presentation: We present the report of a 3-year-old male with weakness of right upper and lower limbs, facial asymmetry, and seizures. Physical examination showed a well-nourished child with a squint of the right eye. There were brisk tendon reflexes with right-sided hemiplegia which is spastic with left limb preference. Computer tomography of the brain showed atrophy of the left cerebral hemisphere, ipsilateral lateral ventricular dilatation, and thickening of the ipsilateral cranium. The diagnosis of Dyke-Davidoff-Masson syndrome was made. He was treated with sodium valproate and lamotrigine and presently physiotherapy and he had remained seizure-free. Conclusion: Dyke-Davidoff-Masson syndrome still affects children despite being a rare condition. There is a need for appropriate clinical and radiological assessment for the diagnosis of DDMS. Early identification and appropriate treatment will improve the general outcome of children with DDMS.

Cruveilhier’s Unrecognized Case (c1831) of Dyke-Davidoff-Masson Syndrome

In his serially published atlas of pathology, <i>Anatomie Pathologique du Corps Humain</i> (1829–1842), French anatomist and pathologist Jean Cruveilhier (1791–1874) provided an early clinical-pathologic description of Dyke-Davidoff-Masson syndrome. Cruveilhier’s case was initially published around 1830, more than a century before the clinical and radiologic report of Dyke and colleagues in 1933 based on a series of patients studied with pneumoencephalography. Although Dyke and colleagues were apparently unaware of Cruveilhier’s prior description, Cruveilhier’s case manifested all of the key osseous and neuropathological features of Dyke-Davidoff-Masson syndrome as later elaborated by Dyke and colleagues: (1) cerebral hemiatrophy with <i>ex vacuo</i> dilation of the lateral ventricle, (2) ipsilateral thickening of the diploe of the skull, and (3) ipsilateral hyper-pneumatization of the frontal sinuses. In addition, Cruveilhier noted crossed cerebral-cerebellar atrophy in his case and correctly inferred a “crossed effect” between the involved cerebral hemisphere and the contralateral cerebellum. Cruveilhier’s pathological case from 1830 clearly anticipated both the cases reported more than a century later by Dyke and colleagues based on pneumoencephalography and the more recent case reports recognized with computed tomography or magnetic resonance imaging.

Dyke-Davidoff-Masson Syndrome: A case Report in a patient presenting with drug-resistant epilepsy and pseudoseizures

Context: Dyke-Davidoff-Masson (DDM) syndrome is a rare neurological condition, first described in 1933. Characteristics include cerebral hemiatrophy, contralateral hemiparesis, seizures, and cognitive dysfunction, combined into different degrees and patterns. Brain magnetic resonance imaging (MRI) is used to perform diagnosis throughout its specific findings. Case Report: A eighteen-year-old female presented to our service with a history of cognitive dysfunction and seizures since early childhood, which persistence even with adequate use of antiepileptic drugs. During Investigation were found signs and symptoms compatible with DDM syndrome, and evidence of pseudoseizures captured in a video electroencephalography monitoring. Conclusion: DDM syndrome is a rare condition that must be part of differential diagnosis in patients with seizures and cerebral hemiatrophy. Management is based on adequate control of seizures and other comorbidities.

Dyke Davidoff syndrome: a rare cause of cerebral hemiatrophy in 11 months old

Dyke-Davidoff-Masson syndrome (DDMS) is an uncommon condition, characterized radiologically by cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses, of unknown frequency resulting from brain injury due to large no of causes; especially in early life. Mostly presents early in life with seizures, learning difficulty, contralateral hemiparesis and facial symmetry. Here we present a case of 11 months old female child with developmental delay, visual abnormality, microcephaly and spastic hemiplegia. CT-brain done which was suggestive of infantile type of cerebral hemiatrophy or DDMS.

Abnormalities of brain development as a cause of epileptic seizures

Brain abnormalities can cause resistant epileptic seizures and significant neurological deficits. In this article we present the clinical cases of cerebral hemiatrophy (DykeDavidoffMasson syndrome) and polymicrogyria with adescription of their classical clinical features and radiological and electrophysiological characteristics. The classical signs of DykeDavidoffMasson syndrome in neuroimaging are cerebral hemiatrophy, calvarial thickening, and hyperpneumatization of the frontal sinuses. Polymycrogyria characterized by a gross defect of the cortex with multiple small superficial gyri can manifest as hemi- or tetraparesis, focal epilepsy, and developmental delay. Further study of brain abnormalities, obtaining high-quality images with neuroimaging and their careful analysis can help improve the efficiency of diagnosis and treatment of patients with this pathology.