What is Hallervorden Spatz Disease?

By  ,  Onlymyhealth editorial team
Sep 18, 2014
Quick Bites

  • Hallervorden-Spatz disease is neurological disorder.
  • It is a genetic disorder which can worsen with time.
  • The disorder has an effect on the movement.
  • The condition can also lead to death.

What is Hallervorden-Spatz disease (HSD)?

Hallervorden-Spatz disease (HSD) is neurological disorder which is usually inherited. The disorder usually effects the movement. It is a rare disease and can worsen over time. The condition can also lead to death.

Hallervorden-Spatz Disease

What Causes HSD?

The condition is sometimes referred as pantothenate kinase-associated neurodegenration and is genetic disease. It is caused by an inherited defect which takes place in the pantothenate kinase 2 gene. It is the PANK2 protein that controls the formation of coenzyme A. This is the molecule which helps the body convert the fats, some amino acids and carbohydrates into energy.

Who is at Risk for HSD?

If a person has a parent suffering from the condition, they may be at a risk of developing the condition. The disorder usually develops in the childhood and its late-onset may not be seen until adulthood.

Hallervorden-Spatz Disease

What are the Symptoms of HSD?

HSD is also known as neurodegenration with brain iron accumulation. It can cause wide variety of symptoms that vary based on the severity of HSD and how long it takes taken to progress.
Other symptoms include:

  • Rigid muscles
  • writhing movements
  • confusion
  • disorientation
  • stupor
  • seizures
  • dementia
  • weakness
  • tremors
  • drooling
  • difficulty swallowing (dysphagia)

There are some less common symptoms also:

  • vision changes
  • poorly articulated speech
  • facial grimacing
  • painful muscle spasms

Image courtesy: Getty Images

Read more on Understand Hallervorden-Spatz Disease.

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