sodium-benzoate and Dementia

Sodium benzoate, a D-amino acid oxidase (DAAO) inhibitor, improved cognitive function of early-phase Alzheimer's disease (AD) after 24-week treatment. This study examined benzoate treatment for behavioral and psychological symptoms of dementia (BPSD).. In a double-blind, 6-week trial, 97 patients with BPSD were randomized to receive placebo or benzoate (mean dose: 622.0 mg/day). The primary outcomes were ADAS-cog and BEHAVE-AD.. Two treatments showed similar safety and primary and secondary outcomes.. Compared to antecedent 24-week, higher-dose treatment for early-phase AD, benzoate appeared ineffective in this 6-week trial. Longer-duration, higher-dose trials are warranted to clarify its efficacy for BPSD.

Topics: Aged; Alzheimer Disease; Cholinesterase Inhibitors; Cognition; D-Amino-Acid Oxidase; Dementia; Double-Blind Method; Female; Humans; Male; Nootropic Agents; Sodium Benzoate; Treatment Outcome

In areas without expanded newborn screening, instead of presenting neonatally, patients with arginase deficiency typically present with spastic paraplegia in early childhood. Diagnosis of this rare neurometabolic disease poses the first challenge because it is often misdiagnosed as cerebral palsy during initial stages. We describe arginase deficiency in a 20-year-old woman with spastic paraplegia, progressive dystonia, dementia, peripheral neuropathy, epilepsy, liver cirrhosis, and non-B/non-C hepatocellular carcinoma. A novel homozygous mutation NM_000045.2 (ARG1):c.673del (p.Arg225GlyfsX5) was detected. We suggest that all children presenting with progressive neurodegeneration or spastic paraplegia in the absence of risk factors for cerebral palsy should be screened for inborn errors of metabolism, including arginase deficiency. For monitoring urea cycle defects, noninvasive imaging screening for liver fibrosis and hepatocellular carcinoma can help ensure early detection, with potential treatment implications.

Topics: Anticonvulsants; Arginase; Base Sequence; Carcinoma, Hepatocellular; Cerebral Palsy; Codon, Nonsense; Combined Modality Therapy; Contraindications; Delayed Diagnosis; Dementia; Diagnostic Errors; Disease Progression; Epilepsy; Fatal Outcome; Female; Humans; Hyperargininemia; Liver; Liver Cirrhosis; Liver Neoplasms; Molecular Sequence Data; Palliative Care; Phenotype; Radiography; Sequence Deletion; Sodium Benzoate; Ultrasonography; Valproic Acid; Young Adult