BACK TOTOP Browse A-ZSearchBrowse A-ZABCDEFGHIJKLMNOPQRSTUVWXYZ0-9 E-mail FormEmail ResultsName:Email address:Recipients Name:Recipients address:Message: Print-FriendlyBookmarksbookmarks-menuNeurodegeneration with brain iron accumulation (NBIA)Hallervorden-Spatz disease; Pantothenate kinase-associated neurodegeneration; PKAN; NBIANeurodegeneration with brain iron accumulation (NBIA) are a group of very rare nervous system disorders. They are passed down through families (inherited). NBIA involves movement problems, dementia, and other nervous system symptoms. Causes Symptoms of NBIA begin in childhood or adulthood.There are 10 types of NBIA. Each type is caused by a different gene defect. The most common gene defect causes the disorder called PKAN (pantothenate kinase-associated neurodegeneration).People with all forms of NBIA have a buildup of iron in the basal ganglia. This is an area deep inside the brain. It helps control movement. Symptoms NBIA mainly causes movement problems. Other symptoms may include:Dementia DementiaDementia is a loss of brain function that occurs with certain diseases. It affects memory, thinking, language, judgment, and behavior.Read Article Now Book Mark Article Difficulty speaking Difficulty swallowing Muscle problems such as rigidity or involuntary muscle contractions (dystonia) DystoniaUncontrolled or slow movement is a problem with muscle tone, usually in the large muscle groups. The problem leads to slow, uncontrollable jerky mov...Read Article Now Book Mark Article Seizures SeizuresA seizure is the physical changes in behavior that occurs during an episode of abnormal electrical activity in the brain. The term "seizure" is often...Read Article Now Book Mark Article Tremor TremorA tremor is a type of shaking movement. A tremor is most often noticed in the hands and arms. It may affect any body part, including the head or vo...Read Article Now Book Mark Article Vision loss, such as from retinitis pigmentosa Retinitis pigmentosaRetinitis pigmentosa is an eye disease in which there is damage to the retina. The retina is the layer of tissue at the back of the inner eye. This...Read Article Now Book Mark Article Weakness Writhing movements Toe walking Exams and Tests The health care provider will perform a physical exam and ask about symptoms and medical history.Genetic tests can look for the defective gene that causes the disease. However, these tests aren't widely available.Tests such as an MRI scan can help rule out other movement disorders and diseases. The MRI usually shows iron deposits in the basal ganglia, and are called the "eye of the tiger" sign because of the way the deposits look in the scan. This sign suggests a diagnosis of PKAN.MRIA magnetic resonance imaging (MRI) scan is an imaging test that uses powerful magnets and radio waves to create pictures of the body. It does not us...Read Article Now Book Mark Article Treatment There is no specific treatment for NBIA. Medicines that bind iron may help slow the disease. Treatment is mainly focused on controlling the symptoms. The most commonly used medicines to control symptoms include baclofen and trihexyphenidyl. Outlook (Prognosis) NBIA gets worse and damages the nerves over time. It leads to a lack of movement, and often death by early adulthood. Possible Complications Medicine used to treat symptoms can cause complications. Being unable to move from the disease can lead to:Blood clots Blood clotsBlood clots are clumps that occur when blood hardens from a liquid to a solid. A blood clot that forms inside one of your veins or arteries is calle...Read Article Now Book Mark Article Respiratory infections Skin breakdown When to Contact a Medical Professional Call your provider if your child develops: Increased stiffness in the arms or legs Increasing problems at school Unusual movements Prevention Genetic counseling may be recommended for families affected by this illness. There is no known way to prevent it.Open ReferencesReferencesGregory A, Hayflick S, Adam MP, et al. Neurodegeneration with brain iron accumulation disorders overview. 2013 Feb 28 [updated 2019 Oct 21]. In: Adam MP, Ardinger HH, Pagon RA, et al, eds. GeneReviews [Internet]. Seattle, WA: University of Washington; 1993-2020. PMID: 23447832 pubmed.ncbi.nlm.nih.gov/23447832/.Jankovic J. Parkinson disease and other movement disorders. In: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, eds. Bradley's Neurology in Clinical Practice. 7th ed. Philadelphia, PA: Elsevier; 2016:chap 96.NBIA Disorders Association. Overview of NBIA disorders. www.nbiadisorders.org/about-nbia/overview-of-nbia-disorders. Accessed November 3, 2020.AllVideoImagesTogRelated Information Muscle cramps(Symptoms) Review Date: 8/2/2020 Reviewed By: Amit M. Shelat, DO, FACP, FAAN, Attending Neurologist and Assistant Professor of Clinical Neurology, Renaissance School of Medicine at Stony Brook University, Stony Brook, NY. Review provided by VeriMed Healthcare Network.Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- © 1997- All rights reserved. A.D.A.M. content is best viewed in IE9 or above, Firefox and Google Chrome browser.Content is best viewed in IE9 or above, Firefox and Google Chrome browser.