Biotin-thiamine-responsive basal ganglia disease (BTBGD) is a rare disease that affects the nervous system, particularly the basal ganglia in the brain. It is a treatable neurometabolic disorder with autosomal recessive inheritance. First described in 1998 and then genetically distinguished in 2005, the disease is characterized by progressive brain damage that, if left untreated, can lead to coma and/or death. Commonly observed in individuals with BTBGD is recurring subacute encephalopathy along with confusion, seizures, and disordered movement (hypokinesia). BTBGD has several alternate names, including: BTRBGD Biotin-responsive basal ganglia disease (BBGD) Thiamine metabolism dysfunction syndrome 2 (biotin or thiamine-responsive type) (THMD2) Thiamine-responsive encephalopathy Thiamine transporter-2 deficiency

Reference
Wiki: Biotin-responsive basal ganglia disease