Noonan Syndrome, Metabolic Syndrome and Stroke-in- the-Young: Coincidence, Causal or Contribution?
Jakris Eu-ahsunthornwattana MD, MSc*, Objoon Trachoo MD*, Donniphat Dejsuphong MD*, Atchara Tunteeratum MD*, Kanoknan Srichan RN*, Thanyachai Sura MD, MRCP*
Affiliation : * Division of Medical Genetics & Molecular Medicine and Academic Center for Medical Genetics, Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
A 34-year-old Thai woman developed acute left hemiparesis with dysarthria from subcortical infarction of the right MCA territory eighteen months after being diagnosed with Noonan syndrome, diabetes mellitus, dyslipidaemia, and hypertension. Further investigations suggested atherosclerosis as a cause. Modifying her risk factors was difficult, partly because of limited adherence. Three years later, she had another attack of ischaemic stroke in the same area. Unlike the three previously reported cases, the causation of strokes in this patient appeared to be a more ‘complex’ interaction between genetic defect and environment including possible subtle arterial abnormalities, metabolic risk factors, and mental insufficiency.
Keywords : Noonan syndrome, Cerebral infarction, Metabolic syndrome, Diabetes mellitus, Multifactorial causality
