Noonan syndrome, metabolic syndrome and stroke-in-the-young: coincidence, causal or contribution?
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.
Eu-ahsunthornwattana
Jakris
J
Division of Medical Genetics & Molecular Medicine and Academic Center for Medical Genetics, Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. jakrise@gmail.com
Trachoo
Objoon
O
Dejsuphong
Donniphat
D
Tunteeratum
Atchara
A
Srichan
Kanoknan
K
Sura
Thanyachai
T
D000328:Adult; D015897:Comorbidity; D048909:Diabetes Complications / Q000473:pathology; D004401:Dysarthria; D005260:Female; D006429:Hemiplegia / Q000209:etiology*; D006801:Humans; D020244:Infarction, Middle Cerebral Artery / Q000473:pathology* / Q000503:physiopathology; D018810:Magnetic Resonance Angiography; D008297:Male; D024821:Metabolic Syndrome / Q000150:complications*; D009634:Noonan Syndrome / Q000503:physiopathology*