Dyke⁃Davidoff⁃Masson syndrome: one case report

Dyke -Davidoff- Masson (DDM) Syndrome is rare neurological disorder commonly affecting the children but is rarely reported in adults as well. It mostly presents with seizure, hemiparesis followed by mental retardation and facial asymmetry. We here in report a case of 28 years old female (G3P2A0) with 34+ weeks of pregnancy who presented with generalized tonic clonic seizures and was incidentally diagnosed as DDM. She was managed with an External ventricular drain and antiepileptic medication. This case report aims to draw the attention of health care professionals to keep DDM as a differential in a patient with seizure.

BackgroundDyke–Davidoff–Masson syndrome is an uncommon constellation of radiological and clinical findings. Few reports describe co-occurring psychiatric manifestations. Systemic lupus erythematosus is a systemic disease with vascular, neurologic, and psychiatric involvement. To the best of our knowledge, no case reports have been made associating these entities.Case presentationWe present the case of a 21-year-old Mexican mestizo woman with a history of systemic lupus erythematosus diagnosed at 4 years of age, who developed focal impaired awareness seizures when she was 8-years old, which became treatment-resistant at age 15. Two years prior to our evaluation, she developed deep vein thrombosis; clinical and laboratory criteria were met to diagnose secondary antiphospholipid syndrome. After being treated with anticonvulsants, glucocorticoids, and immunosuppressants with only a partial response, she developed a severe major depressive episode 1 year prior to our assessment, including two suicide attempts. She was referred to the out-patient clinic of our department for evaluation; intellectual disability, depressive symptoms, and behavioral symptoms were documented. Imaging studies revealed structural abnormalities in the left cerebral hemisphere: cortical atrophy, enlargement of sulci and cisternal spaces, and hyperpneumatization of the frontal sinus. Treatment with an antidepressant was initiated and maintained for 1 year, added to anticonvulsants and immunosuppressants. Depressive and behavioral symptoms diminished and no suicidal ideation has been noted at follow-up.ConclusionsDyke–Davidoff–Masson syndrome was diagnosed, accompanied by clinical symptoms previously reported as epilepsy and intellectual disability. This case report illustrates the complexity of syndrome presentation in an adult female, constituting a diagnostic and therapeutic challenge. This constellation of symptoms and structural brain abnormalities should be kept in mind in patients with neuropsychiatric manifestations and systemic diseases with central nervous system involvement, especially when diagnosed at a young age.

Dyke-Davidoff-Masson syndrome (DDMS) is a rare disease affecting the brain with almost 100 cases previously reported, with only 21 cases among adults. Due to the intricacy of clinical manifestations and radiological findings, it is difficult to reach the diagnosis. It usually includes atrophy of the cerebral hemisphere, dilation of the lateral ventricle, hypertrophy of skull bones, and hyperpneumatization of air sinuses. Herein, we present a case of a 55-year-old female patient who presented with a new-onset seizure. This case emphasizes the importance of considering DDMS in the differential diagnosis of adult-onset seizures, especially in patients with a previous history of brain insult, and demonstrates the possibility of developing this condition despite the lack of childhood symptoms. To our knowledge, this is the first case reported in Jordan.

Postmortal diagnosis of a Dyke–Davidoff–Masson syndrome in a 75-year-old woman – A case report

Context: Dyke–Davidoff–Masson syndrome (DDM) is a rare condition characterized by cerebral hemiatrophy, epileptic seizures, contralateral hemiparesis, and delayed neuropsychomotor development. It can be congenital or acquired, with the latter commonly associated with neonatal hypoxia. Early diagnosis is essential for proper management. Case report: A 31-year-old female patient with a history of perinatal hypoxia and delayed neuropsychomotor development. Since the neonatal period, she has had difficult-to-control epileptic seizures with varied semiology, including focal tonic–clonic seizures, atonic seizures, and behavioral arrest with orofacial automatisms. She presents with moderate intellectual disability, psychomotor agitation, sleep–wake cycle inversion, right spastic hemiparesis, facial asymmetry, and an anomaly in the distal phalanx of the right thumb. EEG revealed rare epileptiform paroxysms in the left hemisphere, predominantly in frontotemporal regions. MRI showed extensive corticosubcortical encephalomalacia in the left cerebral hemisphere, in addition to thinning of the corpus callosum, Wallerian degeneration of the left corticospinal tract, and signs of mesial temporal sclerosis. There was also thickening of the left cranial vault, compatible with DDM. Discussion: DDM is characterized by epileptic seizures, hemiparesis, and cognitive impairment. The acquired form is related to perinatal insults. Radiological findings include cerebral hemiatrophy and cranial thickening. Treatment is symptomatic, focusing on seizure control and neuropsychomotor support. The patient showed clinical improvement with phenobarbital, lamotrigine, and valproic acid, along with folic acid supplementation. Conclusion: DDM should be considered in cases of perinatal insult with neurological manifestations. It has a good prognosis when diagnosed early and treated appropriately.

Dyke–Davidoff–Masson syndrome manifested by seizure in late childhood: a case report

Dyke-Davidoff-Masson syndrome (DDMS) is an important cause of intractable and drug-resistant seizures. It has varied clinical presentation and distinct neuroimaging features. Here, we describe a male patient presented with recurrent generalized tonic-clonic seizures, and cognitive deficit and characteristic neuroimaging features of marked sulcal all ventricular prominence seen on left side suggestive of hemiatrophy associated with mild calvarial thickening with pneumatization of sinuses seen on left side with dilatation of left petrous edge which is suggestive of DDMS. Early institution of neuroimaging in patients with intractable epilepsy will make early diagnosis and better outcome.

Dyke–Davidoff–Masson syndrome (DDMS), also known as cerebral hemiatrophy, is characterized by brain damage resulting in hypoplasia of one cerebral hemisphere. It is described as a rare disorder, primarily characterized by epileptic seizures and convulsions, as well as hemiparesis and cognitive impairments. We present the evolution of a child with DDMS and the neurodevelopmental milestones achieved from the onset of symptoms at 10 days of age to 3 years. Clinical features included persistent seizures and hemiparesis. Neuroimaging revealed right cerebral hemiatrophy with associated structural changes. The aim of this study is to present a case report of a pediatric patient with DDMS, detailing the clinical evaluation, therapeutic approaches, and disease progression, while describing the healthcare process and challenges associated with managing this rare condition in the Dominican Republic. Through this, we aim to improve the therapeutic strategies implemented in the region for the management of this infrequent condition and enhance the understanding of DDMS in the Caribbean.

Dyke–Davidoff–Masson syndrome: A rare case report