Calcitriol Treatment Is Safe and Increases Frataxin Levels in Friedreich Ataxia Patients.

BACKGROUND: Calcitriol, the active form of vitamin D (also known as 1,25-dihydroxycholecalciferol), improves the phenotype and increases frataxin levels in cell models of Friedreich ataxia (FRDA). OBJECTIVES: Based on these results, we aimed measuring the effects of a calcitriol dose of 0.25 mcg/24h in the neurological function and frataxin levels when administered to FRDA patients for a year. METHODS: 20 FRDA patients where recluted and 15 patients completed the treatment for a year. Evaluations of neurological function changes (SARA scale, 9-HPT, 8-MWT, PATA test) and quality of life (Barthel Scale and Short Form (36) Health Survey [SF-36] quality of life questionnaire) were performed. Frataxin amounts were measured in isolated platelets obtained from these FRDA patients, from heterozygous FRDA carriers (relatives of the FA patients) and from non-heterozygous sex and age matched controls. RESULTS: Although the patients did not experience any observable neurological improvement, there was a statistically significant increase in frataxin levels from initial values, 5.5 to 7.0 pg/µg after 12 months. Differences in frataxin levels referred to total protein levels were observed among sex- and age-matched controls (18.1 pg/µg), relative controls (10.1 pg/µg), and FRDA patients (5.7 pg/µg). The treatment was well tolerated by most patients, and only some of them experienced minor adverse effects at the beginning of the trial. CONCLUSIONS: Calcitriol dosage used (0.25 mcg/24 h) is safe for FRDA patients, and it increases frataxin levels. We cannot rule out that higher doses administered longer could yield neurological benefits. © 2024 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

Behavior of Ni20Cr Alloy in Molten Nitrate Salts.

This study reports the behavior of the Ni20Cr alloy in molten nitrate salts. Its behavior was evaluated in the eutectic mixture called Solar Salt (binary salt) and in a ternary mixture (90% Solar Salt and 10% lanthanum nitrate). The addition of lanthanum nitrate was performed to determine if the presence of the La3+ cation could act as a corrosion inhibitor. Through mass loss and potentiodynamic polarization studies, the effects of both electrolytes on the corrosion resistance of the alloy at 300, 400, and 500 °C and at exposure times of 250, 500, 750, and 1000 h were determined. The results showed an increase in the corrosivity of the ternary salt, due to a decrease in its melting point and an increase in the concentration of nitrate ions. However, it was observed that the La3+ cations formed a protective layer (La2O3) on the alloy surface. In both corrosive media, the Ni20Cr alloy showed excellent corrosion resistance, due to its ability to form protective layers of Cr2O3, NiO, and NiCr2O4, in addition to the presence of a layer of La2O3 in the case of the ternary salt.

Hypomagnesemia, a Rare Cause of Reversible Ataxia.

Background: A 61-year-old male patient presented with cerebellar syndrome, which had progressively worsened for 10 days, followed by a tonic-clonic seizure. Phenomenology Shown: Blood analysis showed severe hypomagnesemia and a brain MRI showed T2 hyperintensity in the cerebellar hemispheres (Figure 1). Therefore, the final diagnosis was cerebellar syndrome and epileptic seizures secondary to severe hypomagnesemia. Educational Value: In cases of subacute onset of ataxia, the possibility of ataxia secondary to hypomagnesemia should be considered, as it can be diagnosed with a basic blood test and there are potentially life-threatening outcomes in the absence of treatment, with a reversible course following early supplementation.