Hallervorden-Spatz Disease Information Page
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What is Hallervorden-Spatz Disease?
Is there any treatment?
What is the prognosis?
What is Hallervorden-Spatz Disease?
Hallervorden-Spatz disease, also called Neurodegeneration with Brain Iron Accumulation, is a rare, inherited, neurological movement disorder characterized by progressive degeneration of the nervous system. Symptoms, which vary greatly among patients and usually develop during childhood, may include slow writhing, distorting muscle contractions of the limbs, face, or trunk, choreoathetosis (involuntary, purposeless jerky muscle movements), muscle rigidity (uncontrolled tightness of the muscles), spasticity (sudden, involuntary muscle spasms), ataxia (inability to coordinate movements), confusion, disorientation, seizures, stupor, and dementia. Other less common symptoms may include painful muscle spasms, dysphasia (difficulty speaking), mental retardation, facial grimacing, dysarthria (poorly articulated speech), and visual impairment.
Is there any treatment?
There is no cure for Hallervorden-Spatz disease, nor is there a standard course of treatment. Treatment is symptomatic and supportive, and may include physical or work-related therapy, exercise physiology, and/or speech pathology.
What is the prognosis?
The prognosis for individuals with Hallervorden-Spatz disease is poor. The course of the disorder is relentlessly progressive. Death usually occurs approximately 10 years after onset. In a few cases, survival may extend for several decades.
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