Dyke Davidoff Masson: A Case Report.

Dyke Davidoff-Masson syndrome is a rare neurological condition that results from brain injury during early childhood stages. The precise incidence of this condition is unknown, with a slight male predominance, and adult forms have been documented. Imaging findings reveal hemisphere atrophy along with ipsilateral compensatory skull changes and hyper-pneumatization of mastoid cells. The treatment approach involves anti-epileptic medications and hemispherectomy is reserved for cases with intractable seizures. This case report delineates the clinical manifestation and therapeutic approach employed in an 8-year-old male patient exhibiting pharmaco-resistant left hemi-body convulsive seizures. The magnetic resonance imaging (MRI) findings revealed right cerebral hemiatrophy, mesencephalon atrophy, ipsilateral calvarial hypertrophy and hyperpneumatization of mastoid cells. The objective of this study is to contribute to the existing literature by presenting this rare case report. We propose that in cases involving pediatric pharmaco-resistant epilepsy, it is essential to conduct further investigations to establish a comprehensive management strategy.

Dyke-Davidoff-Masson syndrome: A case report of an 11-year-old child managed for Erb's Palsy.

Dyke-Davidoff-Masson syndrome (DDMS) is a rare neurological anomaly encompassing clinical features of seizures, contralateral hemiparesis, facial asymmetry, and intellectual dysfunction. Radiographic findings include cerebral hemiatrophy and ipsilateral calvarial thickening. We encountered an 11-year-old male who presented with new-onset seizures and a 4-year history of weakness in the abduction of his right arm, previously being managed as Erb's palsy. Brain MRI revealed atrophy of the left cerebral hemisphere with ipsilateral dilated ventricle and osseous thickening, consistent with the congenital form of DDMS. We present this case of an atypical presentation of DDMS.

A Rare Pediatric Neurological Case: Dyke-Davidoff-Masson Syndrome Unprecedented at 10 Months.

This case report presents a rare occurrence of Dyke-Davidoff-Masson Syndrome (DDMS) in a 10-month-old male child, highlighting the atypical presentation of this neurological disorder in early infancy. The child initially presented with irritability, loss of appetite, and right-sided weakness following episodes of fever. A comprehensive medical history revealed the sudden onset of generalized tonic-clonic seizures, prompting further investigation. Diagnostic imaging, including CT and MRI, confirmed features consistent with DDMS, including cerebral hemiatrophy, ventricular enlargement, and calvarial thickening. Notably, the child's seizures were successfully managed with antiepileptic medication, leading to stabilized vital signs. This case emphasizes the importance of considering rare neurological disorders in pediatric patients with unusual presentations and underscores the challenges in diagnosing and managing DDMS in infancy. Further research is warranted to elucidate the underlying mechanisms, contributing factors, and optimal management strategies for DDMS in this age group.

Evaluation of the contralateral hemisphere with DWI in pediatric patients with Dyke-Davidoff-Masson syndrome.

INTRODUCTION: Dyke-Davidoff-Masson Syndrome (DDMS) is a clinical syndrome that causes different clinical symptoms and is defined by volume decrement in one cerebral hemisphere. In this study, we aimed to evaluate the involvement of the normal-appearing contralateral hemisphere in 16 pediatric patients with DDMS using diffusion-weighted imaging (DWI). MATERIALS AND METHODS: Brain MRIs were retrospectively reviewed between January 2014 and January 2023. Sixteen pediatric patients radiologically compatible with DDMS were included in the study. Sixteen children who had undergone brain MRI, most commonly for headaches and whose MRI findings had been completely normal, were included as the control group. Apparent diffusion coefficient (ADC) values of the deep gray and white matter of the normal-appearing hemisphere in the patient group were calculated and compared with that of the control group. RESULTS: The ADC values of the gray and white matters of the patient and control groups were not statistically different. However, in the patient group, the ADC values of the gray and white matters in males were remarkably lower than in females (p = 0.038, p = 0.037, respectively). CONCLUSION: The difference in the ADC values of the contralateral hemisphere between females and males in the patient group suggests that the normal-appearing hemisphere may have been affected by DDMS. Although, the exact mechanism of this effect is not known. Therefore, in patients with DDMS, contralateral hemisphere involvement in cerebral hemiatrophy and hemispherectomy should be evaluated clinically and radiologically.

Cerebral hemiatrophy and hemiparesis following hemiclonic status epilepticus in Dravet syndrome.

Dravet syndrome is currently considered as an developmental and epileptic encephalopathy and, recently, mandatory, alert, and exclusionary criteria have been proposed. Here, we describe three patients with Dravet syndrome with the typical early presentation including febrile and afebrile alternating hemiclonic seizures due to loss-of-function SCN1A variants. Subsequently, they developed episodes of febrile focal status epilepticus (SE) associated with hemiparesis and cerebral hemiatrophy with posterior focal seizures, as a consequence of Dravet syndrome. This sequence of events has been previously published in patients with Dravet syndrome and does not contradict the recent classification by the International League Against Epilepsy (ILAE). The ILAE guidance identifies "Focal neurological findings" as alert criteria and "MRI showing a causal focal lesion" as exclusionary criteria for making an initial diagnosis of Dravet syndrome at presentation. Our three patients would correspond to a severe phenotype, similar to the well-known presentation of generalized atrophy following prolonged status epilepticus. Common genetic findings in cases of diffuse and unilateral brain involvement may help explain these clinical presentations. Further genotype-phenotype studies may provide additional insights into this electroclinical behavior.

A Case Report of Rasmussen Encephalitis and its Stages: The Important Role of Imaging.

Relatively rare, Rasmussen encephalitis is a chronic inflammatory disease conditioning a single cerebral hemisphere. It is a progressive pathology with different stages clinically and on MRI. Therefore, imaging has an important role in evoking the diagnosis and also excluding other possible etiologies. We report a case of Rasmussen encephalitis with 2 different stages on MRI and also provide a review of the role of imaging in diagnostic criteria, differential diagnoses, and stages of this pathology.

Adult hemiconvulsive hemiatrophy syndrome: a novel clinicoradiologic disorder in adults.

The association of focal motor seizures with cerebral hemiatrophy is a recognised rare paediatric syndrome known as 'hemiconvulsion, hemiatrophy and epilepsy' (HHE). To date, HHE has not been reported in adults. We present four adult patients with striking similarities to HHE, following alcohol withdrawal in chronic alcoholics. We document the imaging findings in the acute and subacute phases, discuss the underlying mechanisms and present a hypothesis regarding the pathophysiology.

A Case Report on Dyke-Davidoff-Masson Syndrome: A Rare Cause of Hemiparesis.

Infantile hemiparesis resulting from Dyke-Davidoff-Masson syndrome (DDMS) is an uncommon condition, especially in patients with no positive natal history. The age of presentation is dependent on when the neurologic insult occurred, and distinctive alterations may not appear until puberty. The left hemisphere and the male gender are more frequently involved. Common findings that can be seen are seizures, hemiparesis, mental retardation, and facial changes. Characteristic MRI findings are dilation of the lateral ventricles, hemiatrophy of the cerebrum, frontal sinus hyperpneumatization, and compensatory hypertrophy of the skull. Here, we report a 17-year-old female patient who reported physiotherapy treatment after the attack of epilepsy, with the complaint of inability to use the right hand for functional activities and gait deviations. Patient examination revealed typical chronic hemiparesis of the right side with mild cognitive affection. Brain investigation confirms the diagnosis of DDMS.

Dyke-Davidoff-Masson Syndrome: A Case Report.

Dyke-Davidoff-Masson syndrome (DDMS) is a rare neurological entity that is predominantly seen in childhood. Here, we present the case of a 13-year-old girl who was brought to the pediatric ward for general examination with a previous history of seizures, speech difficulty, facial deviation, and progressive left-sided hemiparesis that started at the age of two, followed by delayed developmental milestones. Computed tomography (CT) and magnetic resonance imaging (MRI) of the brain showed right cerebral hemiatrophy, ventriculomegaly, hyperpneumatization of the sinus, the decreased caliber of cortical veins, and skull thickening on the right were all characteristic findings of DDMS. Based on the history, clinical presentation, and imaging findings from CT and MRI, DDMS was confirmed. Identifying DDMS in a clinical setting can be challenging because of low awareness of the condition and varied clinical presentations. Although CT and MRI imaging are the gold standards in diagnosing DDMS, the early manifestations of the disease cannot be well-appreciated on a CT and would likely require an MRI. Since there is no standardized protocol for managing DDMS, the treatment is primarily symptomatic. Early identification and diagnosis of the syndrome are essential to aid the child's mental and physical development through a multidisciplinary approach. There is also a need to improve awareness of DDMS so that the condition can be considered a potential differential diagnosis amongst other similar conditions and does not get misdiagnosed. The lack of a proper protocol for the management of DDMS prompts more research for a better understanding and early identification of the condition.

Dyke-Davidoff-Masson syndrome: A case report with a literature review.

Dyke-Davidoff-Masson syndrome (DDMS) is an uncommon neurological disease defined as cerebral hemiatrophy with a contralateral motor deficit, facial asymmetry, and seizures. Classic imaging findings are cerebral hypoplasia, ventriculomegaly, paranasal sinus hyper-pneumatization, and compensatory osseous enlargement. The diagnosis of DDMS is based on the correlation between clinical and neuroimaging features. The management of DDMS is based on anticonvulsant medication with physiotherapy. We describe an unusual case of DDMS presented with frequent and persistent seizures.

An Adult With Dyke-Davidoff-Masson Syndrome: A Case Report.

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.

Preserved visuospatial abilities in absence of the right hemisphere: A case of cerebral hemiatrophy with minimal cognitive impairment.

Cerebral hemiatrophy is a rare neurological condition, usually resulting in severe and diffuse cognitive impairment. In this paper we present a 69-year old woman with notable congenital hemiatrophy with strikingly preserved cognitive functions. Cognitive assessment indicated that although her executive functions were found impaired, the remaining cognitive domains were relatively unaffected. We argue that this unexpected cognitive profile may be explained by anomalous hemispheric lateralization, driven by neuroplasticity along the developmental course.

Dyke-Davidoff-Masson Syndrome: A Rare Cause of Acquired Cerebral Hemiatrophy.

Dyke-Davidoff-Masson syndrome is a rare disease of childhood which is clinically characterized by hemiparesis, refractory seizures, facial asymmetry, and mental retardation. The classical radiological findings are cerebral hemiatrophy, calvarial thickening, and hyperpneumatization of the frontal sinuses. Seizure refractory to medical management warrants surgical intervention with excellent outcome. Here, we are reporting two such cases who presented late and diagnosis was made on the basis of magnetic resonance imaging brain features. Both of our children responded to oral anticonvulsant and are on regular follow-up.

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

In his serially published atlas of pathology, Anatomie Pathologique du Corps Humain (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 ex vacuo 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 presenting as recurrent chronic headache in the late adult life.

Dyke-Davidoff-Masson syndrome (DDMS) (also referred to as cerebral hemiatrophy) is a rare condition characterized by seizures, facial asymmetry, contralateral spastic hemiplegia, or hemiparesis, with or without learning difficulties. It usually presents in the early childhood or late adolescence. The diagnosis is mainly associated with the presence of radiologic findings which include contralateral cerebral hemiatrophy with ipsilateral dilatation of the lateral ventricle and hypertrophy of the sinuses. Here, we report a case of a 49-year-old female patient who presented with chronic headache episodes for 10 years, and radiological findings revealed the DDMS.

DYKE-DAVIDOFF-MASSON SYNDROME-A Rare Cause of Cerebral Hemiatrophy in a 17-Years-Old Ethiopian Patient: A Case Report.

BACKGROUND: Dyke-Davidoff-Masson syndrome (DDMS) is a rare disease with unknown frequency, mainly presented with seizures, facial asymmetry, contralateral hemiparesis and mental retardation. Often, resulting from brain injury due to a multitude of causes, especially in early life, associated with birth asphyxia. Radiological findings include cerebral hemiatrophy/hypoplasia, calvarial thickening, and hyperpneumatization of the frontal sinuses. CASE PRESENTATION: We report the case of a 17-year-old male patient who presented to Neurology Clinic with complaints of left side body weakness, walking difficulty and poorly controlled seizure for the past 6 years. Brain MRI revealed atrophy of the right cerebral hemisphere. CONCLUSION: Dyke-Davidoff-Masson syndrome should be suspected in any patients who present with classical features and brain imaging showing hemiatrophy. Early identification and treatment is important in such patients, as it can improve patients prognosis and quality of life.

Clinico-radiological approach to cerebral hemiatrophy.

INTRODUCTION: Cerebral hemiatrophy is an uncommon neuroimaging finding of diverse etiologies, conventionally classified into two broad categories: congenital and acquired. The authors propose an alternative pragmatic clinical approach to cerebral hemiatrophy, classifying its diverse etiologies into a single event insult such as an in utero stroke, or a progressive disorder from an inflammatory or neoplastic process, the latter of which needs urgent intervention and will be the focus of our review paper. Illustrative cases will also be presented to facilitate the understanding of the discussed disorders. CONCLUSION: A systematic approach, linking both clinical and neuroimaging features, is important to facilitate the diagnostic workup of cerebral hemiatrophy. This may potentially help avoid large-scale investigations. Determining the underlying aetiology of cerebral hemiatrophy may impact treatment and prognostication as some conditions such as Rasmussen encephalitis and Parry-Romberg syndrome may benefit from timely implementation of immunomodulatory therapy.

Successful hemispherotomy in two refractory epilepsy patients with cerebral hemiatrophy and contralateral EEG abnormalities.

We describe two cases of refractory epilepsy with cerebral hemiatrophy and contralateral electroencephalographic (EEG) abnormalities, in which hemispherotomy of the atrophic hemisphere effectively controlled seizures. Case 1 was a 5-year-1-month-old girl with refractory bilateral asymmetrical tonic posturing seizures predominantly in the right arm. Magnetic resonance imaging showed left porencephaly corresponding to a left middle cerebral artery infarction. Case 2 was a 3-year-8-month-old boy with refractory bilateral asymmetrical tonic posturing seizures predominantly in the right arm due to atrophy of the left cerebral hemisphere after septic meningitis. Both patients had right hemiparesis and was incapable of pinching by the right hand. Contralateral interictal and ictal EEG abnormalities were observed. Interictal 99mTc-ethyl cysteinate dimer (99mTc-ECD) single photon emission computed tomography (SPECT) showed hypoperfusion and ictal 99mTc-ECD-SPECT showed hyperperfusion within the left cerebral hemisphere. Left hemispherotomy was performed. Cases 1 and 2 remained seizure-free at the last follow-up 18 months and 15 months, respectively, after surgery, and contralateral interictal EEG abnormalities disappeared. In patients with cerebral hemiatrophy and contralateral EEG abnormalities, epilepsy surgery may be considered when the laterality of seizure semiology, functional imaging findings and motor deficits were concordant with the atrophic side. Ictal SPECT is effective to confirm the epileptogenic hemisphere.

Radiological imaging findings of Dyke-Davidoff-Masson syndrome.

Radiological findings of Dyke-Davidoff-Masson syndrome (DDMS) in patients with different etiologies are presented in our study. The study included 12 patients (seven females, five males) for whom radiological examinations were requested due to reasons such as epilepsy, mental retardation, and/or hemiplegia. CT was performed in 12, MRI in 6, MRA in 1, and DSA in 1 patient. Following imaging findings were evaluated: cerebral and cerebellar involvement (laterality, encephalomalacia), affected territories, ventricular enlargement, sulcal enlargement, calvarial thickening, and paranasal sinus enlargement hyperaeration. Age range of the patients was 5-62 (mean 34.1 ± 21.7). Left hemicrania was affected in eight patients, right hemicrania in four. Ipsilateral calvarial thickening and lateral ventricular dilatation were observed in all patients. 11 patients had ipsilateral frontal sinus hyperaeration, sulcal enlargement and encephalomalacia. Wallerian degeneration of the mesencephalon and middle fossa hypoplasia was seen in ten patients, mastoid hyperaeration, third ventricular enlargement and thalamic involvement in nine, and corpus callosum, basal ganglion injury, and sphenoid sinus hyperaeration in eight. MCA, ACA, and PCA territories were involved in six patients. Only MCA territory involvement was seen in four patients. Cerebellar atrophy was contralateral in two patients. Symmetric bilateral atrophy was observed in one patient. DDMS can be encountered with different radiological findings based on cerebral damage formation process and the extent of damage. Patients may have different levels of cerebral hemiatrophy, ipsilateral carvarial thickening, and lateral ventricular dilatation.