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Case Report
Dyke-Davidoff Masson Syndrome: A rare cause of cerebral hemi-atrophy and aggression
1 BA, Medical Student Year 4, Rutgers Robert Wood Johnson Medical School, Piscataway, New Jersey, USA
2 MD, Resident Physician Year 3, Delaware Psychiatric Center, Delaware, USA
3 MD, Attending Physician, Department of Psychiatry, Christiana Care Health System, Delaware, USA
Address correspondence to:
Smit P. Shah
112 Montgomery Street Apt 3G, Highland Park, NJ 08904,
USA
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Article ID: 100969Z01SS2018
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Shah SP, Shah PP, Vardi C. Dyke-Davidoff Masson Syndrome: A rare cause of cerebral hemiatrophy and aggression. Int J Case Rep Images 2018;9:100969Z01SS2018.ABSTRACT
Introduction: Dyke-Davidoff Masson Syndrome (DDMS) is a rare neurological disorder characterized by cerebral asymmetry secondary to unilateral brain atrophy, ipsilateral ventricular dilatation, convulsions, mental retardation, learning disabilities and behavioural changes. These patients can potentially have complex medical management that requires multidisciplinary team based approach.
Case Report: We describe a case of a 16-year-old female with developmental delay who presented with new onset seizures along with aggressive head-banging behaviour and mild motor weakness in right upper and lower extremeties. Radiological imaging of brain demonstrated left sided cerebral atrophy which was more prominent in both frontal and temporal lobes along with ipsilateral ventricular enlargement, enlarged left sphenoid sinus and prominent left mastoid air cells.
Conclusion: Dyke-Davidoff Masson Syndrome (DDMS) is a rare neurological disorder characterized by cerebral hemiatrophy, ventricular dilatation, seizures, behavioural changes and learning disabilities. Patients can have compensatory skull thickening on the affected side along with unilateral frontal and ethmoidal sinus anomalies. Causes of DDMS include cerebral trauma, infections, hemorrhage and ischemia.
Keywords: Cerebral, Hemi-atrophy, Headbanging, Pediatric, Unilateral
SUPPORTING INFORMATION
Author Contributions
Smit P. Shah - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Pooja P. Shah - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Christina Vardi - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this case report.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2018 Smit P. Shah et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
