Cerebral hemiatrophy unveiled: An in-depth radiological perspective on the diagnosis and clinical implications of Dyke-Davidoff-Masson syndrome
Dyke-Davidoff-Masson Syndrome (DDMS) is a rare neurological condition characterized by unilateral cerebral atrophy and compensatory cranial changes. It typically presents with a constellation of clinical features, including seizures, developmental delays, and hemiparesis, resulting from early brain...
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Elsevier
2025-04-01
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| Series: | Radiology Case Reports |
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| author | Rakshanda Agrawal, MBBS Vaishali Dhawan, MBBS Pratapsingh Parihar, MBBS Anjali Kumari, MBBS Komal Mishra, MBBS |
| author_facet | Rakshanda Agrawal, MBBS Vaishali Dhawan, MBBS Pratapsingh Parihar, MBBS Anjali Kumari, MBBS Komal Mishra, MBBS |
| author_sort | Rakshanda Agrawal, MBBS |
| collection | DOAJ |
| description | Dyke-Davidoff-Masson Syndrome (DDMS) is a rare neurological condition characterized by unilateral cerebral atrophy and compensatory cranial changes. It typically presents with a constellation of clinical features, including seizures, developmental delays, and hemiparesis, resulting from early brain injury or developmental anomalies. Radiological imaging, particularly Magnetic Resonance Imaging (MRI), plays a pivotal role in diagnosing DDMS by providing detailed visualization of the structural abnormalities and associated compensatory changes. This case report details the clinical and radiological findings of an 8-year-old female presenting with recurrent seizures, developmental delay, and left-sided hemiparesis. A neurological examination revealed cognitive impairment and significant left-sided weakness. Routine laboratory investigations were unremarkable, and an electroencephalogram (EEG) demonstrated focal epileptic activity. MRI findings were characteristic of DDMS, showing marked atrophy of the right cerebral hemisphere, ex vacuo dilatation of the lateral ventricle, ipsilateral calvarial thickening, and hyper pneumatization of the right sphenoid bone and bilateral mastoid air cells. Areas of cystic encephalomalacia with surrounding gliosis were noted in the right fronto-parieto-temporal regions, suggestive of prior ischemic or hypoxic injury. This case underscores the essential role of radiological imaging in diagnosing and managing DDMS. MRI confirms the diagnosis by highlighting hallmark features and helps differentiate it from other conditions with overlapping clinical and imaging findings, such as Sturge-Weber syndrome or Rasmussen encephalitis. Early recognition and diagnosis allow for optimized therapeutic strategies, including seizure management with antiepileptic medications and physiotherapy for motor deficits. Long-term follow-up is critical to monitor disease progression and adapt treatment plans. |
| format | Article |
| id | doaj-art-56b32626619a4958bbe74fb4f9a27e69 |
| institution | Kabale University |
| issn | 1930-0433 |
| language | English |
| publishDate | 2025-04-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Radiology Case Reports |
| spelling | doaj-art-56b32626619a4958bbe74fb4f9a27e692025-01-31T05:11:12ZengElsevierRadiology Case Reports1930-04332025-04-0120421012104Cerebral hemiatrophy unveiled: An in-depth radiological perspective on the diagnosis and clinical implications of Dyke-Davidoff-Masson syndromeRakshanda Agrawal, MBBS0Vaishali Dhawan, MBBS1Pratapsingh Parihar, MBBS2Anjali Kumari, MBBS3Komal Mishra, MBBS4Corresponding author.; Department of Radiology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education & Research, Wardha, Maharashtra 442001, IndiaDepartment of Radiology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education & Research, Wardha, Maharashtra 442001, IndiaDepartment of Radiology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education & Research, Wardha, Maharashtra 442001, IndiaDepartment of Radiology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education & Research, Wardha, Maharashtra 442001, IndiaDepartment of Radiology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education & Research, Wardha, Maharashtra 442001, IndiaDyke-Davidoff-Masson Syndrome (DDMS) is a rare neurological condition characterized by unilateral cerebral atrophy and compensatory cranial changes. It typically presents with a constellation of clinical features, including seizures, developmental delays, and hemiparesis, resulting from early brain injury or developmental anomalies. Radiological imaging, particularly Magnetic Resonance Imaging (MRI), plays a pivotal role in diagnosing DDMS by providing detailed visualization of the structural abnormalities and associated compensatory changes. This case report details the clinical and radiological findings of an 8-year-old female presenting with recurrent seizures, developmental delay, and left-sided hemiparesis. A neurological examination revealed cognitive impairment and significant left-sided weakness. Routine laboratory investigations were unremarkable, and an electroencephalogram (EEG) demonstrated focal epileptic activity. MRI findings were characteristic of DDMS, showing marked atrophy of the right cerebral hemisphere, ex vacuo dilatation of the lateral ventricle, ipsilateral calvarial thickening, and hyper pneumatization of the right sphenoid bone and bilateral mastoid air cells. Areas of cystic encephalomalacia with surrounding gliosis were noted in the right fronto-parieto-temporal regions, suggestive of prior ischemic or hypoxic injury. This case underscores the essential role of radiological imaging in diagnosing and managing DDMS. MRI confirms the diagnosis by highlighting hallmark features and helps differentiate it from other conditions with overlapping clinical and imaging findings, such as Sturge-Weber syndrome or Rasmussen encephalitis. Early recognition and diagnosis allow for optimized therapeutic strategies, including seizure management with antiepileptic medications and physiotherapy for motor deficits. Long-term follow-up is critical to monitor disease progression and adapt treatment plans.http://www.sciencedirect.com/science/article/pii/S1930043325000202Cerebral hemiatrophyDyke-Davidoff-Masson syndromeMagnetic resonance imagingNeurological deficitsPediatric seizuresCalvarial thickening |
| spellingShingle | Rakshanda Agrawal, MBBS Vaishali Dhawan, MBBS Pratapsingh Parihar, MBBS Anjali Kumari, MBBS Komal Mishra, MBBS Cerebral hemiatrophy unveiled: An in-depth radiological perspective on the diagnosis and clinical implications of Dyke-Davidoff-Masson syndrome Radiology Case Reports Cerebral hemiatrophy Dyke-Davidoff-Masson syndrome Magnetic resonance imaging Neurological deficits Pediatric seizures Calvarial thickening |
| title | Cerebral hemiatrophy unveiled: An in-depth radiological perspective on the diagnosis and clinical implications of Dyke-Davidoff-Masson syndrome |
| title_full | Cerebral hemiatrophy unveiled: An in-depth radiological perspective on the diagnosis and clinical implications of Dyke-Davidoff-Masson syndrome |
| title_fullStr | Cerebral hemiatrophy unveiled: An in-depth radiological perspective on the diagnosis and clinical implications of Dyke-Davidoff-Masson syndrome |
| title_full_unstemmed | Cerebral hemiatrophy unveiled: An in-depth radiological perspective on the diagnosis and clinical implications of Dyke-Davidoff-Masson syndrome |
| title_short | Cerebral hemiatrophy unveiled: An in-depth radiological perspective on the diagnosis and clinical implications of Dyke-Davidoff-Masson syndrome |
| title_sort | cerebral hemiatrophy unveiled an in depth radiological perspective on the diagnosis and clinical implications of dyke davidoff masson syndrome |
| topic | Cerebral hemiatrophy Dyke-Davidoff-Masson syndrome Magnetic resonance imaging Neurological deficits Pediatric seizures Calvarial thickening |
| url | http://www.sciencedirect.com/science/article/pii/S1930043325000202 |
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