Clinical spectrum of Dyke-Davidoff-Masson syndrome in the adult: An atypical presentation and review of literature

Jose Danilo Bengzon Diestro, Maria Kristina Casanova Dorotan, Alvin Camacho, Katerina Tanya Perez-Gosiengfiao, Leonor Isip Cabral-Lim

Research output: Contribution to journalArticle

Abstract

Dyke-Davidoff-Masson Syndrome (DDMS) is a rare condition usually diagnosed in paediatric patients with clinical features of hemiparesis, seizures, mental retardation and contralateral cerebral hemiatrophy on neuroimaging. This report follows the case of a 22-year-old man presenting with seizures and hemiatrophy and hemiparesis. On review of cases the most common neuroimaging findings were cerebral hemiatrophy (100%) followed by hemicalvarial thickening (71.4%) and hyperpneumatisation of sinuses (71.4%). Apart from our patient, all nine cases with data on epilepsy control had drug-resistant epilepsy. The onset of seizures in adulthood, block vertebra, short stature, absence of mental retardation and well-controlled epilepsy on monotherapy makes our case exceptional - even bringing to mind the possibility of a DDMS variant. This report exhaustively reviews the wide range of clinical and radiological manifestations of DDMS in the adult, thereby adding to the literature on an unusual syndrome that causes significant neurological morbidity.

Original languageEnglish (US)
Article number224170
JournalBMJ Case Reports
Volume2018
DOIs
StatePublished - Jan 1 2018
Externally publishedYes

    Fingerprint

Keywords

  • epilepsy and seizures
  • neurology

ASJC Scopus subject areas

  • Medicine(all)

Cite this