A japanese patient with neonatal biotin-responsive basal ganglia disease

HIGHLIGHTS

  • who: Mizuki Kobayashi from the (UNIVERSITY) have published the paper: A Japanese patient with neonatal biotin-responsive basal ganglia disease, in the Journal: (JOURNAL) of 29/09/2022
  • what: The authors report a neonatal case of BBGD in which treatment with biotin and thiamine improved clinical symptoms and reduced further deterioration.

SUMMARY

    Biotin-responsive basal ganglia disease (BBGD) is an autosomal recessive disorder that causes catastrophic subacute metabolic encephalopathy1-3. BBGD includes a wide variety of neurological phenotypes, including early-infantile Leigh-like encephalopathy, classic recurrent subacute encephalopathy, and adult-onset . . .

     

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