Abstract
BackgroundDDMS is a rare disease diagnosed by clinical and radiological characteristics. But the complexity of radiological and clinical manifestations of DDMS has become a challenge diagnostically. To date, the reported cases with DDMS had highly varied clinical manifestations including seizures, contralateral hemiplegia/hemiparesis, facial asymmetry, mental retardation, etc. In addition to typical clinical findings, some new characteristics have been recently added to the spectrum of DDMS. However, few cases have been reported to be associated with neuropsychiatric symptoms according to the literature. This study aimed to investigate the neuropsychiatric manifestations associated with Dyke-Davidoff-Masson syndrome (DDMS) and related imaging findings.MethodsThis study included 7 patients diagnosed with DDMS between 2014 and 2020. The clinical characteristics, neuropsychiatric manifestations, and radiological results were retrospectively evaluated.ResultsSeven patients (five males and two females) with a mean age of 28.0 ± 9.73 (range 15.0–41.0) years were included. Five patients were admitted to the psychiatric unit due to psychological and behavioral disorders. Two patients were referred to the neurology unit mainly due to epilepsy. Six patients had epileptic seizures, 4 had hemiplegia, 3 had mental retardation, 2 patients had external ear deformities, and 2 had facial asymmetry. Neuropsychiatric symptoms were presented in 6 (85.7 %) cases. Cases 2–6 developed affective disorders. Deficits in verbal communication, impairment of social interaction, lack of insight, adulia and hypobulia appeared in cases 1–4. Schizophrenia with apathy, and epileptic schizoid psychosis were observed in cases 4 and 5 respectively. Case 6 had behavioral disorders, hyperactivity, tic disorder, mental retardation, anxiety, catatonic symptoms and suicidal tendency. Case 7 had seizures and mental retardation, and no psychiatric symptoms were presented. Radiological examinations showed unilateral cerebral atrophy, enlarged lateral ventricles, and various compensatory hypertrophy of the skull in all cases. The midline structure has shifted to the affected side in 5(71.4 %) cases. Atrophy of the basal ganglia or brain stem was observed in 4(57.1 %) cases.ConclusionsThe hallmark imaging manifestations of DDMS facilitated the diagnosis in most cases. This study illustrated that a variety of psychoneurotic disorders and ear abnormalities were correlated with DDMS.
Highlights
Davidoff-Masson syndrome (DDMS) is a rare disease diagnosed by clinical and radiological characteristics
The underlying causes of DDMS still remains to be controversial, but it is usually considered to be a disorder secondary to ischemia, infarction, trauma, infection, and brain hemorrhage in fetal or early childhood period [2]. It is a rare condition characterized by cerebral hemiatrophy, ipsilateral compensatory skull hypertrophy, and hyperpneumatization of paranasal sinuses based on radiographic features
Case 5 was a 20-year-old male patient who was referred to our clinic due to paroxysmal headache, dizziness and irritability, and epileptic schizoid psychosis. He was diagnosed with DDMS through electroencephalogram and Computed Tomography (CT) scan
Summary
DDMS is a rare disease diagnosed by clinical and radiological characteristics. But the complexity of radiological and clinical manifestations of DDMS has become a challenge diagnostically. The reported cases with DDMS had highly varied clinical manifestations including seizures, contralateral hemiplegia/hemiparesis, facial asymmetry, mental retardation, etc. This study aimed to investigate the neuropsychiatric manifestations associated with Dyke-Davidoff-Masson syndrome (DDMS) and related imaging findings. The underlying causes of DDMS still remains to be controversial, but it is usually considered to be a disorder secondary to ischemia, infarction, trauma, infection, and brain hemorrhage in fetal or early childhood period [2] It is a rare condition characterized by cerebral hemiatrophy, ipsilateral compensatory skull hypertrophy, and hyperpneumatization of paranasal sinuses based on radiographic features. In addition to the typical radiological and clinical findings described above, some new characteristics have recently been added to the spectrum of DDMS. Previous studies were reviewed and aimed to investigate the neuropsychiatric manifestations associated with DDMS and explored the related imaging findings
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