Dyke–Davidoff–Masson syndrome

doi:10.1016/S0899-7071(01)00318-7

Clinical Imaging

Volume 26, Issue 1, January–February 2002, Pages 13-17

https://doi.org/10.1016/S0899-7071(01)00318-7 Get rights and content

Abstract

We reported five cases of Dyke–Davidoff–Masson syndrome (DDMS) with different clinical and radiological findings. The evaluated parameters were the location of the lesions, midline structural shift effect, pathological and morphological changes on the ipsilateral calvarium, paranasal sinuses and mesencephalon, presence of compensatory contralateral hypertrophy. With the help of both magnetic resonance (MR) and computerized tomography (CT) images, changing degrees of all the evaluated parameters were observed in all five of our patients. In conclusion, no relationship was found between parenchymal and calvarial changes and between the time after onset of the disease and amount of the morphologic and pathological changes.

Introduction

The causes of cerebral hemiatrophy are multiple, and include both congenital and acquired lesions [1], [2], [3]. In 1933, Dyke, Davidoff and Masson described a series of nine patients with hemiplegia who had cranial asymmetry noted on plain film of the skull [4]. The asymmetry of cerebral hemispheric growth with atrophy on one side, ipsilateral compensatory osseous hypertrophy and hyperpneumatization of the paranasal sinuses and mastoid cells, and contralateral paresis are the underlying pathological processes of Dyke–Davidoff–Masson syndrome (DDMS). Several degrees of hemiparesis, hemiplegia, seizures, mental retardation and facial asymmetry can accompany this syndrome [1], [2], [5]. We reported five cases of DDMS with different clinical and radiological findings.

Section snippets

Materials and methods

Five patients (three male, two female) with symptoms like unilateral hemiparesis, mental retardation, seizures and speech difficulties were evaluated with computerized tomography (CT) and magnetic resonance (MR) imaging. The patients were 4–38 years old.

The MR images were obtained in three planes with T1-weighted, T2-weighted and proton density spin-echo techniques. CT images were obtained in axial plane.

The evaluated parameters were the location of the lesions, midline structural shift effect,

Results

In three of five patients, symptoms appeared after hemorrhagic disease of newborn, cerebral ischemia and encephalitis. The etiologies of these three DDMS cases were considered as acquired Fig. 1, Fig. 2, Fig. 3. In the remaining two patients, there were no known history of important disease and these cases were considered congenital Fig. 4, Fig. 5.

In all the patients, changing degrees of findings were observed such as: ipsilateral calvarial thickening, hyperpneumatization of the ipsilateral

Discussion

The unique features of DDMS are calvarial thickening at the site of cerebral atrophy and over development of the paranasal sinuses and petromastoid air cells as additional modes of filling space. The causes of DDMS are multiple and include both congenital and acquired lesions [3]. Symptoms and findings appear perinatally or a few months later and intrauterine cause is usually unknown [1]. In these patients, unilateral cerebral blood circulation anomalies were adduced [6]. It was reported that

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Cited by (52)

Dyke-Davidoff-Masson Syndrome: Main clinical and radiological findings- systematic literature review
2023, Seizure
Dyke-Davidoff-Masson syndrome (DDMS), or cerebral hemiatrophy, was first described in 1933. It is characterised by cerebral injury that causes hypoplasia in one of the cerebral hemispheres. The disease has different clinical degrees and two aetiologies: congenital and acquired. Radiological findings depend on the degree of injury and the patient's age at the time.
To provide information on the main clinical and radiological characteristics of this disease.
A systematic review of the PubMed, MEDLINE, and LILACS databases was conducted using only one keyword. Dyke-Davidoff-Masson syndrome. A total of 223 studies were identified, and the results are presented in tables and graphics.
The mean age of the patients was 19.44 (0–83 years), and the majority were male (55.32%). The most common types of epileptic seizures were generalised tonic-clonic seizures (31 cases), focal impaired awareness seizures (20 cases), focal motor seizures (13 cases), focal to bilateral tonic-clonic seizures (nine cases), and focal myoclonic seizures (one case). The main features of the disease were rapid deep tendon reflexes and extensor cutaneous-plantar tendon reflexes (30 cases - 16%), contralateral hemiparesis or hemiplegia (132 cases - 70%), gait alterations (16 cases - 9%), facial paralysis (nine cases - 5%), facial asymmetry (58 cases - 31%), limb asymmetry (20 cases - 11%), delayed developmental milestones (39 cases - 21%), intellectual disability (87 cases - 46%), and language/speech disorders (29 cases - 15%). Left hemisphere atrophy was the most prevalent.
DDMS is a rare syndrome, and several questions regarding this disease remain unanswered. This systematic review aims to elucidate the most common clinical and radiological aspects of the disease and emphasises the need for further investigation.
Dyke-Davidoff-Masson syndrome: Imaging diagnosis in an asymptomatic adult
2022, Radiology Case Reports
Dyke-Davidoff-Masson syndrome (DDMS) was first described in 1933 as a cerebral condition of hemispheric atrophy characterized clinically by contralateral hemiparesis, facial-asymmetry, seizures, and mental retardation. Neuroimaging findings include asymmetric thickening of the calvarium and enlargement of frontal and ethmoid sinuses. There have been 21 reported cases described in the literature with the syndrome undiagnosed until adult age, likely due to less severe or absent clinical findings or symptoms as described in the case presented in this report. This article describes a case where the Dyke-Davidoff-Masson imaging features were identified as an incidental finding on a CT scan of the brain performed for non-seizure related symptoms. A 54-year-old woman presented with weakness and gait difficulty and only upon further evaluation was she found to have cranial deformities. CT and MRI demonstrate encephalomalacia in the right frontal lobe anteriorly with gliosis and moderate unilateral cerebral atrophy, and extensive hypertrophy of the right frontal calvarium, right ethmoid cells and frontal sinuses.
Radiological examination of cranial, ear-nose-throat, and maxillofacial system findings of Dyke-Davidoff-Masson syndrome in 28 patients
2021, Neurology Perspectives
Citation Excerpt :
Also, 35.7% HP was observed in the mastoid bone in this study. Gökçe et al.1 75%, Taşdemir et al.12 reported 100% mastoid HP. Reported rates of mastoid HP in the referenced studies is clearly different, so, mastoid HP may be caused by other causes such as eustachian canal dysfunction.
It was aimed to detail the intracranial and ear–nose–throat-maxillofacial (ENT-MF) system findings of Dyke–Davidoff–Masson Syndrome (DDMS), and to examine the association in a larger sample according to current literature.
The study was designed retrospectively. Cranial magnetic resonance (MRI) and computed tomography (CT) Imaging records of patients were re-examined. Outcome parameters were identified as patients' demographics, the emergence of the central nervous system (CNS) pathologies, the asymmetric pneumatization of the skull bones, and ENT-MF system presentation. The correlation of the cranial and ENT-MF system findings was evaluated with Spearmen correlation analysis.
Radiological images of 28 patients were examined. The number of men was 21 (75.0%) and the number of females was 7 (25.0%). The mean age of the patients was 23.6 ± 10.6 (min. 1, max. 44). DDMS involvement detected on the right side in 15 (53.6%) of the patients. Cerebral atrophy (n = 28, 100%), lateral ventricular dilatation (n = 26, 96.3%) and corpus callosum damage (n = 19, 67.9%) were most frequent findings respectively. Cerebellar atrophy (n = 4, 14.3) and venous sinus dominance (n = 4, 14.3) were the rarest pathologies. Facial asymmetry (n = 15, 53.6%) and frontal sinus hyperpneumatization (n = 15, 53.6%) were the most frequent findings in the ENT-MF system. Increased calvarial thickness showed a high positive correlation with facial asymmetry and frontal sinus hyperpneumatization (p = 0.002, r = 0.571 and 0.024, r = 0.424, respectively).
DDMS may occur with different radiological findings depending on the level of cerebral damage. Facial asymmetry often accompanies the disease and it should be considered in clinical practice of facial plastic and maxillofacial surgery.
Se tuvo como objetivo detallar los hallazgos del sistema intracraneal y del sistema oído-nariz-garganta-maxilofacial (ENT-MF) del síndrome de Dyke-Davidoff-Masson (DDMS) y examinar la asociación en una muestra más amplia de acuerdo con la literatura actual.
El estudio se diseñó de forma retrospectiva. Se volvieron a examinar los registros de imágenes de los pacientes por resonancia magnética craneal (IRM) y tomografía computarizada (TC). Los parámetros de resultado se identificaron como la demografía de los pacientes, la aparición de patologías del sistema nervioso central (SNC), la neumatización asimétrica de los huesos del cráneo y la presentación del sistema ENT-MF. La correlación de los hallazgos del sistema craneal y ENT-MF se evaluó con el análisis de correlación de Spearmen.
Se examinaron imágenes radiológicas de 28 pacientes. El número de hombres fue 21 (75,0%) y el número de mujeres fue 7 (25,0%). La edad media de los pacientes fue de 23,6 ± 10,6 (mín. 1, máx. 44). La afectación del DDMS se detectó en el lado derecho en 15 (53,6%) de los pacientes. La atrofia cerebral (n = 28, 100%), la dilatación ventricular lateral (n = 26, 96,3%) y el daño del cuerpo calloso (n = 19, 67,9%) fueron los hallazgos más frecuentes, respectivamente. La atrofia cerebelosa (n = 4, 14,3) y la dominancia del seno venoso (n = 4, 14,3) fueron las patologías más raras. La asimetría facial (n = 15, 53,6%) y la hiperneumatización del seno frontal (n = 15, 53,6%) fueron los hallazgos más frecuentes en el sistema ORL-MF.
la EMDD puede presentarse con diferentes hallazgos radiológicos según el nivel de daño cerebral. La asimetría facial suele acompañar a la enfermedad y debe considerarse en la práctica clínica de la cirugía plástica facial y maxilofacial.
Severely infected pneumoceles of the frontal sinus in patients with mental retardation and brain atrophy treated by endoscopic sinus surgery
2018, Auris Nasus Larynx
Citation Excerpt :
These reports mentioned that abnormal enlargement of the frontal sinus may be caused by the space-occupying effect in the atrophic brain. Besides, the ipsilateral enlargement of paranasal sinuses and mastoid cells and calvarial thickening have been reported in patients with cerebral hemiatrophy such as Dyke–Davidoff–Masson syndrome and Sturge–Weber syndrome [8–10]. In such syndrome, hyperpneumatization of paranasal sinuses and mastoid cells and calvarial thickening are caused by the compensatory adaptation to unilateral atrophy of brain.
We herein present three cases of abnormally expanded frontal sinuses (pneumoceles) with severe infection in patients with mental retardation and brain atrophy. Two patients previously underwent laryngotracheal separation surgery, and bacteriological examinations of purulent nasal discharge revealed infections caused by drug-resistant bacteria such as Pseudomonas aeruginosa and Acinetobacter baumannii. As conservative medical treatments were ineffective, all three patients were treated by computed tomography-guided endoscopic sinus surgery. This navigation system is useful for safer surgery in the area of anatomic deformity. The clinical findings, possible etiologies and surgical treatment of these cases are discussed.
Temporo-spatial analyses define epileptogenic and functional zones in a case of Dyke-Davidoff-Masson syndrome
2011, Seizure
Citation Excerpt :
Dyke–Davidoff–Masson syndrome (DDMS) is a rare epilepsy syndrome that is characterized by a cluster of imaging features (cerebral hemiatrophy, homolateral skull hyperplasia, and hyperpneumatization of the paranasal sinus) and clinical features (seizures with or without mental retardation and contralateral hemiparesis).1,2
Dyke–Davidoff–Masson syndrome (DDMS) is a rare epilepsy syndrome that is characterized by cerebral hemiatrophy, homolateral skull hyperplasia, hyperpneumatization of the paranasal sinuses, seizures with or without mental retardation, and contralateral hemiparesis. We describe a case of DDMS in a 40-year-old female who had complex partial seizures with occasional secondary generalization since the age of 4 years. Her seizure frequency was 10–20 seizures/month even though she took four antiepileptic drugs. We applied magnetic resonance imaging (MRI), positron emission tomography (PET), functional MRI, and invasive electroencephalography (EEG) to define her epileptogenic and functional zones. Brain MRI showed prominent atrophy in the left frontal dorsal and lateral regions and mild atrophy of the left superior temporal gyrus and left parietal gyri. Interictal PET revealed decreased glucose metabolism in the atrophic regions. Functional MRI demonstrated that the inferior frontal and inferior parieto-occipital regions of the right hemisphere were activated by language testing. Invasive EEG revealed that the left lateral temporal lobe was the sole source of her seizures. Our results imply that the “metabolic border zone” rather than the atrophic region plays an important role in seizure activity, and that reorganization of functional zones occur after cerebral damage early in life.
Facial capillary malformation and dyke-davidoff-masson syndrome
2010, Pediatric Neurology
Citation Excerpt :
Male sex predominates, and the left hemisphere is most commonly affected in this syndrome [3]. The causes are multiple, and include both congenital and acquired lesions [4]. In the congenital type, the lesion commonly has a vascular origin, whereas several different cerebral injuries (e.g., trauma, infection, vascular abnormalities of the cerebral circulation, and ischemic and hemorrhagic states) can lead to the acquired type [5].
Described here is the case of a girl with a reticulated capillary malformation on the right side of her face, along with Dyke-Davidoff-Masson syndrome, as evidenced by microphthalmia and severe associated anomalies in the right eye, and right cerebral hemispheric atrophy and cerebral arteries malformations. Capillary malformations are a novel finding for children with Dyke-Davidoff-Masson syndrome.

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Dyke–Davidoff–Masson syndrome

Abstract

Introduction

Section snippets

Materials and methods

Results

Discussion

Clin Imaging

Ann Thorac Surg

MR imaging of cerebral hemiatrophy

J Comput Assist Tomogr

Cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses

Surg Gynecol Obstet