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Axial CT images showing hypertrophied frontal sinus and mastoid air cells on right side.
Source publication
Dyke Davidoff Masson Syndrome (DDMS) is a rare condition with varied presentation. Characteristic features are cerebral hemiatrophy, enlargement
of ipsilateral ventricle, enlargement of ipsilateral air sinuses and clinical finding of contralateral hemiparesis or hemiplegia and seizures. DDMS may
have comorbid intellectual disability or speech disor...
Contexts in source publication
Context 1
... Resonance Imaging (MRI) revealed gliosis and atrophy of the right cerebral hemisphere with dilatation of the right lateral ventricle. There was hypertrophy of the frontal sinus and thickening of frontal, mastoid and occipital bone on the right side (Fig 1-4). The diagnosis of DDMS with Mild Intellectual Disability with Alcohol and Cannabis use Disorder was made. ...
Context 2
... Resonance Imaging (MRI) revealed gliosis and atrophy of the right cerebral hemisphere with dilatation of the right lateral ventricle. There was hypertrophy of the frontal sinus and thickening of frontal, mastoid and occipital bone on the right side (Fig 1-4). The diagnosis of DDMS with Mild Intellectual Disability with Alcohol and Cannabis use Disorder was made. ...
Citations
... In contrast, our patient had no mental retardation on examination. Some unique cases of DDMS have presented with psychiatric comorbidities like behavioral disturbance and substance use disorder, as described by Bhandarı et al. [20], and schizophrenia, as described by Puri et al. [21]. On the other hand, some cases have been diagnosed incidentally with no clinical manifestations of DDMS [7]. ...
Dyke-Davidoff-Masson syndrome (DDMS) is a rare congenital or acquired neurological disorder that most commonly affects the pediatric population but is also rarely reported in adults. DDMS results from brain injury in the intrauterine or early years of life. It is characterized by prominent cortical sulci, hyperpneumatization of the frontal sinus, unilateral cerebral hemiatrophy with ventricular dilation, and associated bony thickness of the cranial vault. Seizures and asymmetric hemiparesis are the most consistent findings in DDMS with facial asymmetry and mental retardation widely reported. Herein, we report a case of a 32-year-old female patient with DDMS presenting with a history of seizure and right-sided hemiparesis. Neuroimaging findings showed asymmetric cerebral encephalomalacia and gliosis with ex vacuo ventricular dilatation and calvarial diploic space widening. Our case report is unique in the sense that our patient presented with DDMS in adulthood with no signs of mental retardation or history of seizures during childhood and well-controlled seizures on monotherapy. Given the adult presentation of DDMS is unusual and rarely reported in the medical literature, our case report will help physicians to keep DDMS high on differential diagnoses in such cases. Awareness of the clinical features of DDMS on imaging can facilitate a timely and accurate diagnosis, thereby enabling appropriate and prompt management.
Introduction:
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.
Objective:
To provide information on the main clinical and radiological characteristics of this disease.
Methods:
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.
Results:
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.
Conclusion:
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.
