Diabetic Dead-in-Bed Syndrome: A Possible Link to a Cardiac Ion Channelopathy.
Sudden unexpected nocturnal death among patients with diabetes occurs approximately ten times more commonly than in the general population. Malignant ventricular arrhythmia due to Brugada syndrome has been postulated as a cause, since a glucose-insulin bolus can unmask the Brugada electrocardiographic signature in genetically predisposed individuals. In this report we present a 16-year-old male with insulin-dependent diabetes who died suddenly at night. His diabetes had been well controlled, without significant hypoglycaemia. At autopsy, he had a full stomach and a glucose level of 7 mmol/L in vitreous humor, excluding hypoglycaemia. Genetic analysis of autopsy DNA revealed a missense mutation, c.370A>G (p.Ile124Val), in the GPD1L gene. A parent carried the same mutation and has QT prolongation. Mutations in this gene have been linked to Brugada syndrome and sudden infant death. The patient may have died from a ventricular arrhythmia, secondary to occult Brugada syndrome, triggered by a full stomach and insulin. The data suggest that molecular autopsies are warranted to investigate other cases of the diabetic dead-in-bed syndrome.
Cardiac Inherited Disease Group, Auckland City Hospital, Auckland 1148, New Zealand ; Green Lane Paediatric and Congenital Cardiac Services, Starship Children's Hospital, Auckland 1148, New Zealand ; Department of Child Health, University of Auckland, Private Bag 92019, Auckland 1142, New Zealand.
Jonathan R
JR
Skinner
Diagnostic Genetics, LabPlus, Auckland City Hospital, Auckland 1148, New Zealand ; Department of Pathology, University of Melbourne, Parkville, VIC 3010, Australia.
Renate
R
Marquis-Nicholson
School of Biological Sciences, University of Auckland, Private Bag 92019, Auckland 1142, New Zealand ; INRA, UMR 1198, Biologie du Développement et Reproduction, 78350 Jouy-en-Josas, France.
Alix
A
Luangpraseuth
Diabetes Service, North Shore Hospital, Takapuna, Auckland 0622, New Zealand.
Rick
R
Cutfield
Cardiac Inherited Disease Group, Auckland City Hospital, Auckland 1148, New Zealand.
Jackie
J
Crawford
Cardiac Inherited Disease Group, Auckland City Hospital, Auckland 1148, New Zealand ; Diagnostic Genetics, LabPlus, Auckland City Hospital, Auckland 1148, New Zealand ; School of Biological Sciences, University of Auckland, Private Bag 92019, Auckland 1142, New Zealand.
Donald R
DR
Love