Pantothenate kinase-associated neurodegeneration

Orpha code: 157850OMIM code: 234200

Definition

Pantothenate kinase-associated neurodegeneration (PKAN) is the most common type of neurodegeneration with brain iron accumulation (NBIA; see this term), a rare neurodegenerative disorder characterized by progressive extrapyramidal dysfunction (dystonia, rigidity, choreoathetosis), iron accumulation on the brain and axonal spheroids in the central nervous system.

Disease data
Classification

Disease

Synonyms
Hallervorden-Spatz syndrome
NBIA1
Neurodegeneracja z magazynowaniem żelaza w mózgu typu 1
PKAN
Zespół Hallervordena i Spatza
NBIA1
Neurodegeneration with brain iron accumulation type 1
PKAN
ORPHA code
157850
OMIM code
234200
ICD10 code
G23.0
ICD11 code
5C64.10

No additional description.

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