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Start free with EleplanBeta-propeller protein-associated neurodegeneration
ORPHA:329284Disease
Also called BPAN · NBIA5 · Neurodegeneration with brain iron accumulation type 5 · SENDA · Static encephalopathy of childhood with neurodegeneration in adulthood
What it is
Beta-propeller protein-associated neurodegeneration (BPAN), also known as static encephalopathy of childhood with neurodegeneration in adulthood, is a rare form of neurodegeneration with brain iron accumulation (NBIA) characterized by early-onset developmental delay and further neurological deterioration in early adulthood.
Key facts
- Prevalence
- <1 / 1 000 000
- Age of onset
- Childhood
- Inheritance
- X-linked dominant
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Common30–79%
19- Abnormal autonomic nervous system physiology
- Abnormality of eye movement
- Bradykinesia
- Cerebellar atrophy
- Cerebral atrophy
- Dementia
- Dystonia
- Frontal release signs
- Global developmental delay
- Intellectual disability
- Iron accumulation in brain
- Iron accumulation in substantia nigra
- Parkinsonism
- Poor speech
- Progressive encephalopathy
- Rigidity
- Sleep abnormality
- Spastic paraparesis
- Tremor
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Gene
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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