|
|
Lifespan’s A - Z Health Information Library |
||||||||||
Hallervorden-Spatz diseaseDefinitionHallervorden-Spatz disease is a movement disorder that is passed down through families (inherited). CausesHallervorden-Spatz usually begins in childhood. Most cases of Hallervorden-Spatz 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
Exams and TestsA nervous system (neurological) examination will show:
If other family members are affected, this may help confirm the diagnosis. 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. TreatmentThe goal of treatment is to control the symptoms. Although there is no specific treatment for Hallervorden-Spatz disease, taking certain vitamins (including pantothenate, Coenzyme Q10, and other antioxidants) may help. Outlook (Prognosis)Hallervorden-Spatz gets worse and damages the nerves over time. It leads to a lack of movement and often death by early adulthood. Possible ComplicationsMedication used to treat symptoms can cause complications. Being unable to move from the disease can lead to:
When to Contact a Medical ProfessionalCall your health care provider if your child develops:
PreventionGenetic counseling is appropriate in families affected by this illness. There is no known way to prevent it. ReferencesJankovic J. Movement disorders. In: Goetz CG, ed. Textbook of Clinical Neurology. 3rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 34. Review Date: 9/22/2008
Reviewed By: Daniel B. Hoch, PhD, MD, Assistant Professor of Neurology, Harvard Medical School Department of Neurology, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc. 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. Call 911
for all medical emergencies. Links to other sites are provided for
information only -- they do not constitute endorsements of those
other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the
information contained herein is strictly prohibited.
 |
||||||||||
|
|
