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An 18-year-old male patient with MELAS phenotype and 2 previous episodes of cerebral stroke, recurrent seizures and nephropathy, was treated with creatine monohydrate after the acute onset of psychomental regression and changing states of somnolence and aggressive and agitated behaviour. These symptoms disappeared completely after 4 weeks of treatment with creatine after which the patient regained all his previous mental abilites. Brain (white matter) proton magnetic resonance spectroscopy (chemical shift imaging) performed at 6 and 12 months of treatment showed lactic acid (Lac) accumulation and high creatine (Cr) levels in relation to choline-containing compounds (Cho). Urinary creatinine excretion as an indicator of the muscle and brain creatine pool increased upon short-term (12 days) high-dosage creatine supplementation (20 g per day) while plasma creatinine concentrations as possible indicators both of increasing creatine pool and of renal insufficiency increased during the course (28 months) of low-dosage creatine supplementation (5 g per day). Deterioration of renal function was finally indicated by urea retention and by impairment of renal creatinine clearance. These observations suggest that creatine supplementation may have a neuroprotective effect in patients with MELAS and episodes of acute mental deterioration. Adverse effects of creatine supplementation on renal function must be considered especially in patients with preexisting nephropathy.

In persistent hyperinsulinemic hypoglycemia of infancy, ketone body concentrations are abnormally low at times of hypoglycemia, depriving the brain of its most important alternative fuel. The neuroprotective effect of endogenous ketone bodies is evidenced by animal and human studies, but knowledge about exogenous supply is limited. Assuming that exogenous ketone body compounds as a dietetic food might replace this alternative energy source for the brain, we have monitored the fate of orally supplemented DL sodium ␤-hydroxybutyrate (␤-OHB) in two 6-mo-old infants with persistent hyperinsulinemic hypoglycemia for 5 and 7 mo, while on frequent tube-feedings and treatment with octreotide. Near total (95%) pancreatectomy had been ineffective in one patient and was refused in the other. In blood, concentrations of ␤-OHB increased to levels comparable to a 16-to 24-h fast while on DL sodium ␤-OHB 880 to 1000 mg/kg per day. In cerebrospinal fluid, concentrations of ␤-OHB increased to levels comparable to a 24-to 40-h fast, after single dosages of 4 and 8 g, respectively. High ratios of ␤-OHB to acetoacetate indicated exogenous origin of ␤-OHB. An increase of intracerebral concentrations of ␤-OHB could be demonstrated by repetitive single-voxel proton magnetic resonance spectroscopy by a clear doublet at 1.25 ppm. Oral DL sodium ␤-OHB was tolerated without side effects. This first report on oral supplementation of DL sodium ␤-OHB in two patients with persistent hyperinsulinemic hypoglycemia demonstrates effective uptake across the blood-brain barrier and could provide the basis for further evaluation of the neuroprotective effect of ␤-OHB in conditions with hypoketotic hypoglycemia. PHHI is the most common cause of recurrent hypoglycemia in infancy (1). PHHI is characterized by inappropriately high blood insulin levels in the presence of symptomatic hypoglycemia (2) and by a prompt responsiveness to glucagon (3).Pathogenetically, PHHI may result from different molecular defects in the regulation of ␤-cell-mediated insulin release with diffuse or focal ␤-cell hyperplasia (4, 5).Treatment with diazoxide, a potassium-channel opener at the pancreatic ␤-cell and inhibitor of insulin secretion, and octreotide, a long-acting somatostatin analog, is often hampered by poor effect or severe side effects (3, 6 -9), and pancreatectomy, at least in cases with diffuse hyperplasia, bears the risk of postoperative diabetes (10).In most hypoglycemic states, ketone bodies (␤-OHB and AA) are released from counterregulatory lipolysis, serving as an alternative, neuroprotective, energetic fuel for the brain (3,

Psychotropic medication treatment of individuals who have experienced prenatal alcohol exposure (PAE) has lagged behind psychosocial interventions. Multiple psychotropic medications are often prescribed for those diagnosed with a range of neurodevelopmental disabilities and impairments of PAE Treatment algorithm for fetal alcohol spectrum disorder (FASD)