Preferred Label : Neurodegeneration with brain iron accumulation;
ICD-11 definition : Neurodegeneration with brain iron accumulation (NBIA, formerly Hallervorden-Spatz
syndrome) encompasses a group of rare neurodegenerative disorders characterized by
progressive extrapyramidal dysfunction (dystonia, rigidity, choreoathetosis), iron
accumulation in the brain and the presence of axonal spheroids, usually limited to
the central nervous system. NBIA can present as early onset with rapid progression:
classic pantothenate kinase-associated neurodegeneration (PKAN), infantile neuroaxonal
dystrophy (INAD) and atypical neuroaxonal dystrophy (atypical NAD); or later onset
with slower progression: atypical PKAN, neuroferritinopathy and aceruloplasminemia.
Idiopathic NBIA can have either type of onset and progression.;
ICD-11 synonym : Hallervorden-Spatz disease; pigmentary pallidal atrophy; NBIA - [Neurodegeneration with brain iron accumulation]; Pigmentary pallidal degeneration; pigmentary; degeneration, pallidal (progressive); pigmentary; degeneration, pallidal;
ICD-11 acronym : NBIA;
ICD-11 inclusion : Pigmentary pallidal degeneration;
Origin ID : 440483530;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
Neurodegeneration with brain iron accumulation (NBIA, formerly Hallervorden-Spatz
syndrome) encompasses a group of rare neurodegenerative disorders characterized by
progressive extrapyramidal dysfunction (dystonia, rigidity, choreoathetosis), iron
accumulation in the brain and the presence of axonal spheroids, usually limited to
the central nervous system. NBIA can present as early onset with rapid progression:
classic pantothenate kinase-associated neurodegeneration (PKAN), infantile neuroaxonal
dystrophy (INAD) and atypical neuroaxonal dystrophy (atypical NAD); or later onset
with slower progression: atypical PKAN, neuroferritinopathy and aceruloplasminemia.
Idiopathic NBIA can have either type of onset and progression.
