We report a family in which a mother and son were affected with diabetes mellitus and myopathy characterized by ragged red fibers and suggestive of mitochondrial disease. Mitochondrial DNA (mtDNA) analysis of DNA isolated from peripheral blood showed a T-->C point mutation at nucleotide position 14709, in the transfer RNA gene for glutamic acid. We review the association of diabetes and mtDNA mutations. This child's case is unusual because of the early onset of diabetes, which is more typical of mtDNA deletions.

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Epilepsia partialis continua, or "Kozhevnikov syndrome," is a rare condition characterized by persistent localized motor seizures usually localized in a distant limb. It is most often seen in children under 16 years old without gender preference. We report a 12-year-old girl with epilepsia partialis continua who presented to the emergency room after the onset of seizures. Routine electroencephalography (EEG) was performed with no clear ictal localization. Magnetic resonance imaging (MRI) was unremarkable. Ictal single photon emission computed tomography (SPECT) images with Tc-99m exametazime fused with MRI images revealed a parietal lobe epileptic focus within the superior segment of the postcentral gyrus. This case illustrates a rare disorder with an unusual epileptic localization identified by SPECT-MRI fusion.

We have recently documented that oral Linomide (quinoline-3-carboxamide) prevents autoimmune insulitis, islet destruction, and diabetes in NOD mice treated at an early stage (5 wk of age) of the disease. In this report, we show that treatment of female NOD mice with advanced disease (age 23-24 wk) by syngeneic islet transplantation and oral Linomide administration results in prevention of graft insulitis and diabetes in the Linomide group up to 40 wk (diabetes at 40 wk: isograft recipients with Linomide n = 0 of 6; isograft recipients alone n = 5 of 6; p < 0.0001). The extent of protection from glucose intolerance by the combination of transplantation with Linomide was superior to that of Linomide alone [blood glucose (mean +/- SD) 60 min post-i.p. injection of 1 g/kg body weight glucose: Linomide plus isograft 6.7 +/- 1 mmol/L; Linomide alone 18.7 +/- 6.3 mmol/L; p < 0.0001]. Thus, Linomide should be considered a potential immunoregulatory modality in patients undergoing pancreatic islet or whole organ transplantation.