Dyke-Davidoff-Masson syndrome: A rare case of hemiatrophy with chronic seizure disorder
Dyke-Davidoff-Masson syndrome (DDMS) is a rare neurological disorder characterized by cerebral hemiatrophy, leading to seizures, hemiparesis, and cognitive deficits. We report the case of a 20-year-old female with a history of chronic seizure disorder and left-sided hemiparesis. The patient experien...
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Elsevier
2025-03-01
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| Series: | Radiology Case Reports |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S193004332401327X |
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| author | Utkarsh Pradeep Anjalee Chiwhane Sourya Acharya Pradeep Kumar Paschyanti Kasat |
| author_facet | Utkarsh Pradeep Anjalee Chiwhane Sourya Acharya Pradeep Kumar Paschyanti Kasat |
| author_sort | Utkarsh Pradeep |
| collection | DOAJ |
| description | Dyke-Davidoff-Masson syndrome (DDMS) is a rare neurological disorder characterized by cerebral hemiatrophy, leading to seizures, hemiparesis, and cognitive deficits. We report the case of a 20-year-old female with a history of chronic seizure disorder and left-sided hemiparesis. The patient experienced her first seizure at 6 months of age, followed by recurrent generalized tonic seizures throughout childhood. Despite treatment, she continued to have mild seizure episodes (brief changes in consciousness or less noticeable symptoms) twice a month. Additionally, left-sided motor weakness had been present since infancy, significantly affecting her daily activities. Magnetic Resonance Imaging (MRI) revealed right-sided cerebral atrophy with extensive cystic encephalomalacia, ex vacuo dilatation of the right lateral ventricle, and gliosis in the right fronto-parietal and fronto-temporal regions. Compensatory bony changes, including asymmetrical thickening of the calvarium and enlargement of the right frontal sinus, were also noted. Wallerian degeneration on the right side was observed. These imaging findings, along with the patient's clinical presentation, were consistent with the diagnosis of Dyke-Davidoff-Masson Syndrome. Management was focused on controlling seizures with antiepileptic medications and improving motor function through physical therapy. This case underscores the importance of early recognition of DDMS in patients with early-onset seizures and hemiparesis. Early diagnosis and appropriate intervention are essential for optimizing patient outcomes and improving quality of life. |
| format | Article |
| id | doaj-art-7042a45e330a4dc79f73c61fa24c7568 |
| institution | Kabale University |
| issn | 1930-0433 |
| language | English |
| publishDate | 2025-03-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Radiology Case Reports |
| spelling | doaj-art-7042a45e330a4dc79f73c61fa24c75682025-01-18T05:03:56ZengElsevierRadiology Case Reports1930-04332025-03-0120314701474Dyke-Davidoff-Masson syndrome: A rare case of hemiatrophy with chronic seizure disorderUtkarsh Pradeep0Anjalee Chiwhane1Sourya Acharya2Pradeep Kumar3Paschyanti Kasat4Department of Medicine, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education & Research, Sawangi, Meghe, Wardha, India; Corresponding author.Department of Medicine, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education & Research, Sawangi, Meghe, Wardha, IndiaDepartment of Medicine, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education & Research, Sawangi, Meghe, Wardha, IndiaDepartment of Medicine, Meerut Kidney Hospital, Meerut, IndiaDepartment of Radiology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education & Research, Sawangi, Meghe, Wardha, IndiaDyke-Davidoff-Masson syndrome (DDMS) is a rare neurological disorder characterized by cerebral hemiatrophy, leading to seizures, hemiparesis, and cognitive deficits. We report the case of a 20-year-old female with a history of chronic seizure disorder and left-sided hemiparesis. The patient experienced her first seizure at 6 months of age, followed by recurrent generalized tonic seizures throughout childhood. Despite treatment, she continued to have mild seizure episodes (brief changes in consciousness or less noticeable symptoms) twice a month. Additionally, left-sided motor weakness had been present since infancy, significantly affecting her daily activities. Magnetic Resonance Imaging (MRI) revealed right-sided cerebral atrophy with extensive cystic encephalomalacia, ex vacuo dilatation of the right lateral ventricle, and gliosis in the right fronto-parietal and fronto-temporal regions. Compensatory bony changes, including asymmetrical thickening of the calvarium and enlargement of the right frontal sinus, were also noted. Wallerian degeneration on the right side was observed. These imaging findings, along with the patient's clinical presentation, were consistent with the diagnosis of Dyke-Davidoff-Masson Syndrome. Management was focused on controlling seizures with antiepileptic medications and improving motor function through physical therapy. This case underscores the importance of early recognition of DDMS in patients with early-onset seizures and hemiparesis. Early diagnosis and appropriate intervention are essential for optimizing patient outcomes and improving quality of life.http://www.sciencedirect.com/science/article/pii/S193004332401327XDyke-Davidoff-Masson syndromeCerebral hemiatrophySeizuresHemiparesisEncephalomalaciaNeurological disorder |
| spellingShingle | Utkarsh Pradeep Anjalee Chiwhane Sourya Acharya Pradeep Kumar Paschyanti Kasat Dyke-Davidoff-Masson syndrome: A rare case of hemiatrophy with chronic seizure disorder Radiology Case Reports Dyke-Davidoff-Masson syndrome Cerebral hemiatrophy Seizures Hemiparesis Encephalomalacia Neurological disorder |
| title | Dyke-Davidoff-Masson syndrome: A rare case of hemiatrophy with chronic seizure disorder |
| title_full | Dyke-Davidoff-Masson syndrome: A rare case of hemiatrophy with chronic seizure disorder |
| title_fullStr | Dyke-Davidoff-Masson syndrome: A rare case of hemiatrophy with chronic seizure disorder |
| title_full_unstemmed | Dyke-Davidoff-Masson syndrome: A rare case of hemiatrophy with chronic seizure disorder |
| title_short | Dyke-Davidoff-Masson syndrome: A rare case of hemiatrophy with chronic seizure disorder |
| title_sort | dyke davidoff masson syndrome a rare case of hemiatrophy with chronic seizure disorder |
| topic | Dyke-Davidoff-Masson syndrome Cerebral hemiatrophy Seizures Hemiparesis Encephalomalacia Neurological disorder |
| url | http://www.sciencedirect.com/science/article/pii/S193004332401327X |
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