Neurodegeneration with Brain Iron Accumulation (NBIA) is a rare group of genetic disorders characterized by abnormal iron accumulation in the brain. The prevalence of NBIA varies depending on the specific subtype. The most common subtype, called pantothenate kinase-associated neurodegeneration (PKAN), has an estimated prevalence of 1 to 3 cases per million individuals worldwide. Other subtypes, such as PLA2G6-associated neurodegeneration (PLAN) and beta-propeller protein-associated neurodegeneration (BPAN), have lower prevalence rates. NBIA is considered a rare condition, affecting a relatively small number of individuals globally.
Neurodegeneration with Brain Iron Accumulation (NBIA) is a rare group of genetic disorders characterized by abnormal iron accumulation in the brain, leading to progressive neurological symptoms. The prevalence of NBIA varies depending on the specific subtype.
One of the most common subtypes is called pantothenate kinase-associated neurodegeneration (PKAN), which accounts for approximately 50% of NBIA cases. PKAN has an estimated prevalence of 1 to 3 individuals per million worldwide. Another subtype, called PLA2G6-associated neurodegeneration (PLAN), has a prevalence of around 1 to 2 individuals per million. Other subtypes, such as beta-propeller protein-associated neurodegeneration (BPAN) and aceruloplasminemia, are even rarer.
Although NBIA is considered a rare disorder, it is important to note that prevalence rates may vary across different populations and geographic regions. Early diagnosis and proper management are crucial for individuals affected by NBIA, as it can significantly impact their quality of life.