What are the expected outcomes for individuals diagnosed with

What is the prognosis of Neurodegeneration with Brain Iron Accumulation?

What is the prognosis of Neurodegeneration with Brain Iron Accumulation?

Neurodegeneration with brain iron accumulation (NBIA) is a rare neurological disorder characterized by the progressive degeneration of the nervous system. It is inherited in nature.


Symptoms of neurodegeneration with brain iron accumulation can vary widely among patients and typically emerge during childhood. These symptoms may include slow, writhing muscle contractions affecting the limbs, face, or trunk, as well as choreoathetosis, characterized by involuntary, purposeless jerky muscle movements. Other features of the condition may encompass muscle rigidity, spasticity, ataxia (lack of coordination in movements), confusion, disorientation, seizures, stupor, and dementia.


Most individuals with NBIA undergo periods of rapid deterioration lasting 1-2 months, interspersed with relatively stable intervals. The progression of the disease correlates with the age at onset, with early symptoms indicating a more severe form of the condition. Early onset NBIA may result in limited mobility, often leading to wheelchair use by the teenage years. Life expectancy varies, but premature death is not uncommon in NBIA, often due to complications such as poor nutrition or aspiration pneumonia. However, with advancements in medical care and improved management, more individuals affected by NBIA are surviving into adulthood. In cases of atypical, late-onset NBIA, diagnosis may occur in adulthood, and many affected individuals can live well into adulthood.