EC Clinical and Medical Case Reports

Dyke-Davidoff-Masson syndrome (DDMS) is a rare neurological disorder that results from brain injury in intrauterine or the first years of life. Clinical manifestation are usually hemiplegia or hemiparesis, seizure, facial asymmetry, learning difficulties or mental retardation. Rarely, sensory symptoms or psychiatric disorders may be seen. Brain imaging finding include prominent cortical sulci, dilated lateral ventricle, cerebral hemiatrophy, hyper pneumatization of the frontal sinus, and compensatory hypertrophy of the skull. We describe a female patient with no previous childhood history of brain damage, who presented with generalized tonic-clonic seizure, right-sided body weakness, neuroimaging findings of cerebral hemiatrophy, left lateral ventricle dilatation and homolateral frontal sinus hyperpneumatization.

Keywords: Dyke Davidoff Masson Syndrome; Epilepsy; Cerebral Hemiatrophy; Magnetic Resonance Imaging

1. Anna Misyail., et al. “Dyke-Davidoff-Masson syndrome: a case report”. BMC Neurology 18 (2018): 76.
2. N Hamza., et al. “Dyke Davidoff Masson syndrome a rare cause of schizoaffective disorder: A case report and review of the literature”. L’encéphale3 (2019): 274-275.
3. Adebayo Philip B., et al. “Dyke-Davidoff-Masson Syndrome in a Nigerian”. Epilepsy and Behavior Case Reports (2016).
4. Zeynep Özözen Ayas., et al. “The clinico-radiological spectrum of Dyke-Davidoff-Masson syndrome in adults”. Neurological Sciences (2020).
5. Douglas J Lanska. “Cruveilhier’s Unrecognized Case (c1831) of Dyke-Davidoff-Masson Syndrome”. European Neurology 84 (2021): 300-306.
6. Atalar MH., et al. “Cerebral hemiatrophy (Dyke-Davidoff-Masson syndrome) in childhood: clinicoradiological analysis of 19 cases”. Pediatrics International 49 (2007): 70-75.
7. Gökçe E., et al. “Radiological imaging findings of Dyke-Davidoff-Masson syndrome”. Acta Neurologica Belgica (2017).
8. Chiron C., et al. “The right brain hemisphere is dominant in human infants”. Brain 20 (1997): 1057-1065.
9. Arora R and Rani JY. “Dyke–Davidoff–Masson syndrome: imaging features with illustration of two cases”. Quantitative Imaging in Medicine and Surgery3 (2015): 469-471.
10. Roy U., et al. “Adult presentation of Dyke–Davidoff–Masson syndrome: a case report”. Case Reports in Neurology1 (2016): 20-26.
11. Thomas-Sohl KA., et al. “Sturge–Weber syndrome: a review”. Pediatric Neurology 30 (2004): 303-310.
12. Zamora CA and Kontzialis M. “Teaching neuroimages: Dyke– Davidoff–Masson in Sturge–Weber syndrome”. Neurology16 (2015): e128.
13. Bekci T., et al. “A rare combination: Sturge–Weber syndrome and accompanying Dyke– Davidoff–Masson syndrome”. Acta Neurologica Belgica1 (2016): 79-81.
14. Amar DJ., et al. “Encephalocraniocutaneous lipomatosis (Fishman syndrome): a rare neurocutaneous syndrome”. Journal of Paediatrics and Child Health 36 (2000): 603-605.