Hallervorden-Spatz Disease

Hallervorden-Spatz disease 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.

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 occupational therapy, exercise physiology, and/or speech pathology.

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.

Information provided by the
National Institute of Neurological Disorders and Stroke,
National Institutes of Health

Each year, Medical College of Wisconsin physicians care for more than 180,000 patients, representing nearly 500,000 patient visits. Medical College physicians practice at Children's Hospital of Wisconsin, Froedtert Memorial Lutheran Hospital, the Milwaukee VA Medical Center, and many other hospitals and clinics in Milwaukee and southeastern Wisconsin.