Jiali Lau
National University Hospital, Singapore
Title: Treatment Considerations for Psychological and Physiological Problems in Patients with Dyke-Davidoff-Mason Syn-drome in a Paediatric Population: A Review
Biography
Biography: Jiali Lau
Abstract
Statement of the problem: Dyke-Davidoff -Masson syndrome is a rare clinical condition with characteristic clinical and radiological findings. First described in 1933 by Dyke, Davidoff and Masson, literature on this condition has been scant with the prevalence of this syndrome still unknown with a number of these reports being the fi rst published cases in their respective countries. The classical clinical presentation includes intellectual disability with challenging behavior, recurrent seizures, contralateral hemiplegia or hemiparesis and facial asymmetry. We aim to review the treatment options for the various psychological and physiological issues that patients of this rare condition face.
Orientation: A literature search was conducted via several databases such as PubMed.
Findings: Based on our review of the existing literature, management strategies diff er for each patient with priorities depending upon the key clinical features present. Antipsychotic medications such as Risperidone may be eff ective for reducing challenging behavior in the short-term, however in the longer term there is a risk of signifi cant side effects. Th e management of the seizures, if present should be a priority which oft en necessitates the use of multiple antiepileptic medications. Some cases have demonstrated the eff ectiveness of hemispherectomy for adults with intractable unihemispheric epilepsy resulting in excellent long term seizure control. Management of the hemiparesis or hemiplegia depends on the severity of the disability with physiotherapy sessions and the use of orthotics useful in these instances. The involvement of allied health professionals such as physiotherapists and psychologists is desirable for the long term management of patients with Dyke-Davidoff -Mason syndrome. The prognosis for Dyke-Davidoff -Mason syndrome is better if the onset of hemiparesis is aft er two years of age and in absence of prolonged or recurrent seizure.
Conclusion: A multidisciplinary approach with the involvement of various specialties is advocated given the multifaceted issues faced by these patients.