Non-ketotic hyperglycemia with hemichorea-Hemiballismus (HGSC) is a rare complication of non-equilibrated diabetic disease characterized by continuous rapid, non-rhythmic, involuntary unilateral movement the extremities, caused in most cases decreased activity contralateral subthalamic nucleus basal ganglia. It may reveal undiagnosed diabetes. We describe here case an 84-year-old Moroccan woman...

We report a recent diabetic who presented with hyperglycemic, hyperosmolar, non ketotic state with coma and multiple cranial nerve palsies due to an associated previously undetected sinus infection. This is a classic case revisited purely for its educational value in terms of clinical photographs, tissue biopsy and imaging findings. These findings reiterate the devastating neurological conseque...

OBJECTIVE: The aim of this work was to evaluate a protocol for investigation of Inborn Errors of Metabolism (IEM) in children who are acutely ill.METHODS: Forty six children with clinical suspicion of a metabolic disorder were studied during 2 years. They were selected through request for investigation of IEM from Pediatrics or Neonatal Intensive Care Units located in the metropolitan area of P...

Fasting ketosis caused no change in liver acetylcarnitine concentration but caused a decrease in muscle acetylcarnitine to one-half the control level. The combined acetyl coenzyme A and acetylcarnitine turnover rates for control and ketotic liver were, respectively, 4.66 and 10.90 mpmoles per g (fresh weight) per min; for normal and ketotic muscle the corresponding figures were 8.23 and 4.52 mp...

Dr. Andre C. Felicio – Disciplina Neurologia Rua Pedro de Toledo 650 04039-002 São Paulo SP Brasil. E-mail: [email protected] Hemichorea-hemiballism (HCHB) can be the solely presentation of a wide range of non-neurological clinical pictures, such as metabolic or hydro-electrolyte derangements. HCHB as the first presentation of type 2 diabetes mellitus has been rarely described. The case depict...

Background: Impaired glycogen release with fasting results in hypoglycemia in the glycogen storage diseases. A waxy-maize extended release cornstarch was introduced in the United States in 2012 to maintain glucose concentrations during the overnight period, but no studies have assessed the long-term safety and efficacy of this product in the ketotic forms of GSD. Objective: To assess long-term ...

It is recognized that ketone bodies, such as R-beta-hydroxybutyrate (beta-HB) and acetoacetate, are energy sources for the brain. As with glucose metabolism, monocarboxylate uptake by the brain is dependent on the function and regulation of its own transporter system. We concurrently investigated ketone body influx, blood flow, and regulation of monocarboxylate transporter (MCT-1) and glucose t...

Short-chain L-3-hydroxyacyl-CoA dehydrogenase (SCHAD) catalyzes the penultimate reaction in the mitochondrial fatty acid oxidation spiral, the NAD+dependent conversion of L-3-hydroxyacyl-CoA to 3-ketoacyl-CoA. The cDNA and genomic sequences for human SCHAD have been elucidated (1, 2). Northern blot analysis of SCHAD mRNA revealed a single transcript; expression was highest in skeletal and cardi...