Disease Directory Neurodegeneration with Brain Iron Accumulation
Neurological

Neurodegeneration with Brain Iron Accumulation

Also known as: NBIA, PKAN (pantothenate kinase-associated), BPAN, MPAN, PLAN, Hallervorden-Spatz syndrome (historical)

Prevalence

1-9 per 1,000,000 (Orphanet)

Onset

Childhood, Adolescent, Adult

Type

Autosomal recessive or X-linked genetic (varies by subtype)

Gene

PANK2, PLA2G6, FA2H, C19orf12, WDR45

About Neurodegeneration with Brain Iron Accumulation

Neurodegeneration with brain iron accumulation (NBIA) is a group of rare genetic disorders characterized by progressive iron deposition in the basal ganglia. PKAN (PANK2 mutations) is the most common subtype, causing dystonia, parkinsonism, and retinal degeneration with the pathognomonic 'eye of the tiger' sign on brain MRI. Other subtypes include BPAN (WDR45), MPAN (C19orf12), and PLAN (PLA2G6), each with distinct clinical features and genetic causes.

Common Clinical Features

Progressive dystonia Parkinsonism Retinal degeneration Eye of the tiger sign on MRI Spasticity Neuropsychiatric symptoms Cerebellar ataxia

Clinical Trial Eligibility Tips

What to know before applying to Neurodegeneration with Brain Iron Accumulation trials.

Specify NBIA subtype (PKAN, BPAN, MPAN, PLAN) — trials are subtype-specific due to distinct genetics and pathophysiology

Brain MRI T2/T2* or susceptibility-weighted imaging (SWI) demonstrating basal ganglia iron deposition is required for enrollment

Iron chelation therapy (deferiprone) trials are the most common interventional approach for PKAN — prior chelation history must be disclosed

Pantothenate kinase (PANK2) enzyme activity and genotype classification (classic vs. atypical) affect PKAN trial eligibility

Patient Resources

Patient Organization

NBIA Disorders Association

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Natural History Registry

NBIA Disorders Registry

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Orphanet

European reference resource for rare diseases (ORPHA:385)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

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Find recruiting Neurodegeneration with Brain Iron Accumulation trials

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