Homogenates of brain tissue from the frontal cortex at autopsy in patients with amyotrophic lateral sclerosis (ALS) showed dramatically reduced levels of the enzyme thiamine pyrophosphatase (TPPase), the enzyme responsible for the conversion of thiamine pyrophosphate (TPP) to thiamine monophosphate (TMP). Additionally, free thiamine (vitamin B1) and TMP levels have been shown to be significantly reduced in the plasma and cerebral spinal fluid (CSF) of patients with ALS. These findings suggest that there is impaired thiamine metabolism in patients with ALS. Impaired thiamine metabolism decreases adenosine triphosphate (ATP) production and is a well-established cause of neurodegeneration. Decreased levels of TPPase, resulting in decreased levels of TMP in the cells of the frontal cortex, might account for the focal neurodegenerative changes observed in motor neurons in ALS. Benfotiamine, a safe, lipid-soluble, highly absorbable thiamine analogue, significantly raises free thiamine, TMP, and TPP levels in the blood. A case in which benfotiamine may have positively impacted the symptoms of a patient with ALS is presented. The use of benfotiamine in patients with ALS appears to be a promising therapeutic option. Considering the severity and the lack of satisfactory treatment options associated with this disease, more research on the effects of benfotiamine on the course of ALS is urgently needed.

Refeeding syndrome (RS) is a condition characterized by electrolyte derangements, thiamin deficiency, and organ dysfunction after the provision of nutrition to an individual who had been deprived of nutrients. Published guidelines outlining the recognition and definition of RS exist for adult and children, but there are limited newborn-specific guidelines because of a paucity of available literature and variation in reporting. We describe a small-for-gestational-age, full-term newborn with neonatal encephalopathy who underwent therapeutic hypothermia. Electrolyte derangements and lactic acidosis developed and persisted after the introduction of intravenous nutrition. After the exclusion of other etiologies, neonatal RS was suspected, and the newborn was treated with thiamin. We provide a brief review of the current literature on neonatal RS, present a case report consistent with neonatal RS, and provide suggestions for the utility of thiamin replacement in newborns with suspected neonatal RS.

BACKGROUND: We report a case of a patient who was 3 months post-sleeve gastrectomy and presented with acute stroke symptoms ultimately due to Wernicke\'s encephalopathy (WE) after bariatric surgery. A 20-year-old white female presented to an outside hospital 3 months after sleeve gastrectomy complaining of nausea and vomiting. She initially underwent a cholecystectomy and later became less responsive and required intubation. Magnetic resonance imaging changes, presumed to be an acute stroke, prompted her transfer to our facility. Intravenous (IV) thiamin was administered, and the patient\'s symptoms improved over the course of her hospital stay.
RESULTS: Thiamin levels were markedly low, and the patient rapidly improved with the administration of IV thiamin. The patient was discharged to inpatient rehabilitation.
CONCLUSIONS: Bariatric surgery is a less common cause of WE but can lead to acute WE due to malabsorption of thiamin. In patients undergoing bariatric surgery, clinicians should be vigilant about the potential for WE to occur. In addition, based on history, WE should be considered in the differential diagnosis for symptoms of acute ischemic stroke.

Dietary Reference Intakes (DRIs) are fundamental to inform national nutrition policy. However, a regular systematic review of the 51 nutrients that have DRIs has limited feasibility, and many DRIs have not been reviewed in >15 y.
To address this issue, individuals (nutrient review group) who were members of the Food and Nutrition Board developed a streamlined, evidence-based methodology that could be used to identify nutrients potentially in need of a systematic review.
The proposed methodology, termed an evidence scan, comprises several steps. First, an analytic framework is developed to identify markers of associations between intake of a nutrient and a corresponding clinical outcome. Next, the framework is used to direct the identification of keywords for a scan of published research that is potentially relevant to intake requirements or upper intake levels for a nutrient. Last, a panel of content experts selects the abstracts that are likely to be relevant and reviews the full publications. The results may be used to determine whether a revision of the nutrient\'s DRI is an immediate priority but would not supplant a comprehensive systematic evidence review.
To illustrate the process, 2 nutrients were selected as case studies: thiamin and phosphorus (DRIs were last set in 1998 and 1997, respectively). Using the evidence scan for thiamin, we identified 70 potentially relevant abstracts, of which 9 full publications were reviewed. For phosphorus, 127 potentially relevant abstracts were identified, and 29 full publications were reviewed.
From the review of these 2 nutrients, the nutrient review group concluded that there was insufficient new evidence to assign a high priority to a comprehensive systematic review for either thiamin or phosphorus. Evidence scanning is an efficient method of identifying DRI nutrients that are most in need of either a new or an updated systematic review.