Back to article
Larger     Normal     Smaller    

Hallervorden-Spatz disease

Definition

Hallervorden-Spatz disease is a movement disorder that is passed down through families (inherited).

Alternative Names

Pantothenate kinase-associated neurodegeneration

Causes, incidence, and risk factors

Hallervorden-Spatz disease usually begins in childhood.

Most cases of Hallervorden-Spatz disease are due to a defect in a gene that makes a protein called pantothenate kinase 2. Patients with this genetic defect have a buildup of iron in parts of the brain.

Symptoms

Signs and tests

The doctor or nurse will examine you and ask questions about your symptoms and medical history. The exam may show:

  • Abnormal postures and movements
  • Muscle rigidity
  • Tremors
  • Weakness

Genetic tests can look for the defective gene that causes the disease. However, this test is not yet widely available. It is only found at certain research laboratories.

Tests such as MRI can help rule out other movement disorders and diseases.

Treatment

There is no specific treatment for Hallervorden-Spatz disease. Treatment is focused on controlling the symptoms.

Expectations (prognosis)

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

Complications

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

Calling your health care provider

Call your health care provider if your child develops:

  • Increased stiffness in the arms or legs
  • Increasing problems at school
  • Unusual movements

Prevention

Genetic counseling is appropriate in families affected by this illness. There is no known way to prevent it.

References

Lang AE. Other movement disorders. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 428.

Review Date:8/28/2012

Reviewed by:Luc Jasmin, MD, PhD, Department of Neurosurgery at Cedars-Sinai Medical Center, Los Angeles, and Department of Anatomy at UCSF, San Francisco, CA. Review provided by VeriMed Healthcare Network. David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.

Related Items

Read More

A.D.A.M. qualityA.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is an independent audit to verify that A.D.A.M. follows rigorousstandards of quality and accountability. A.D.A.M. is among the first to achieve this important distinction for online health information andservices. Learn more about A.D.A.M.'s editorialpolicy, editorialprocess, and privacypolicy. A.D.A.M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health on the Net Foundation (www.hon.ch.)

The information provided herein should not be used during any medical emergency or for the diagnosis or treatmentof any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions.Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 2014 A.D.A.M., Inc. Any duplication ordistribution of the information contained herein is strictly prohibited.

A.D.A.M.

(C) Copyright 2014 Walgreen Co. All Rights Reserved.