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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
- Involuntary muscle contractions (dystonia)
- Movement problems
- Seizures
- Spasticity
- Tremor
- Vision changes
- Weakness
- Writhing movements
Signs and tests
The doctor will perform a physical exam and ask questions about the patient's medical history. It is important to get a complete medical and family history, since the condition can run in families.
A neurological examination will 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
The goal of treatment is to control the symptoms. There is no specific treatment for Hallervorden-Spatz disease.
Support Groups
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:
- Respiratory infections
- Skin breakdown
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 A. Other movement disorders. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 434.
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