Learn About Hallervorden-Spatz Disease

What is the definition of Hallervorden-Spatz Disease?

Neurodegeneration 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.

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What are the alternative names for Hallervorden-Spatz Disease?

Hallervorden-Spatz disease; Pantothenate kinase-associated neurodegeneration; PKAN; NBIA

What are the causes of Hallervorden-Spatz Disease?

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.

What are the symptoms of Hallervorden-Spatz Disease?

NBIA mainly causes movement problems. Other symptoms may include:

  • Dementia
  • Difficulty speaking
  • Difficulty swallowing
  • Muscle problems such as rigidity or involuntary muscle contractions (dystonia)
  • Seizures
  • Tremor
  • Vision loss, such as from retinitis pigmentosa
  • Weakness
  • Writhing movements
  • Toe walking
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What are the current treatments for Hallervorden-Spatz Disease?

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.

Who are the top Hallervorden-Spatz Disease Local Doctors?
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Highly rated in
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Munich Cluster For Systems Neurology

Munich, BY, DE 80336

Thomas Klopstock is in Munich, Germany. Klopstock is rated as an Elite expert by MediFind in the treatment of Hallervorden-Spatz Disease. He is also highly rated in 34 other conditions, according to our data. His top areas of expertise are Optic Nerve Atrophy, Leber Hereditary Optic Neuropathy, Pantothenate Kinase-Associated Neurodegeneration, and Hallervorden-Spatz Disease.

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Ludwig Maximilians Universität München

Munich, BY, DE 

Susanne Schneider is in Munich, Germany. Schneider is rated as an Elite expert by MediFind in the treatment of Hallervorden-Spatz Disease. She is also highly rated in 29 other conditions, according to our data. Her top areas of expertise are Hallervorden-Spatz Disease, Pantothenate Kinase-Associated Neurodegeneration, Drug Induced Dyskinesia, and Chorea.

 
 
 
 
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Elite
Highly rated in
17
conditions

Hazrat Rasool Hospital, Iran University Of Medical Sciences

Tehran, IR 

Mohammad Rohani is in Tehran, Islamic Republic of Iran. Rohani is rated as an Elite expert by MediFind in the treatment of Hallervorden-Spatz Disease. He is also highly rated in 17 other conditions, according to our data. His top areas of expertise are Hallervorden-Spatz Disease, Pantothenate Kinase-Associated Neurodegeneration, Drug Induced Dyskinesia, and Parkinson's Disease.

What is the outlook (prognosis) for Hallervorden-Spatz Disease?

NBIA gets worse and damages the nerves over time. It leads to a lack of movement, and often death by early adulthood.

What are the possible complications of Hallervorden-Spatz Disease?

Medicine used to treat symptoms can cause complications. Being unable to move from the disease can lead to:

  • Blood clots
  • Respiratory infections
  • Skin breakdown
When should I contact a medical professional for Hallervorden-Spatz Disease?

Call your provider if your child develops:

  • Increased stiffness in the arms or legs
  • Increasing problems at school
  • Unusual movements
How do I prevent Hallervorden-Spatz Disease?

Genetic counseling may be recommended for families affected by this illness. There is no known way to prevent it.

What are the latest Hallervorden-Spatz Disease Clinical Trials?
European Registry on Rare Neurological Diseases
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A Phase 2 Study of a Vitamin Metabolite for PKAN
What are the Latest Advances for Hallervorden-Spatz Disease?
Towards Precision Therapies for Inherited Disorders of Neurodegeneration with Brain Iron Accumulation.
NBIA Syndromes: A Step Forward from the Previous Knowledge.
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Down regulation of the expression of mitochondrial phosphopantetheinyl-proteins in pantothenate kinase-associated neurodegeneration: pathophysiological consequences and therapeutic perspectives.
Who are the sources who wrote this article ?

Published Date : August 02, 2020
Published 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.

What are the references for this article ?

Gregory 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.