Hallervorden-Spatz Disease – Symptoms and Causes

Hallervorden-Spatz cannot be cured but symptoms can be taken care of at the best homeopathic hospital in Hyderabad. The best homeopathic doctors in Hyderabad are very capable.

Hallervorden-Spatz disease (HSD) is also referred to as:

  • Neuro-degeneration with brain iron accumulation (NBIA)
  • pantothenate kinase-associated neuro-degeneration (PKAN)

It is an inherited neurological disorder. It does cause problems with movement. HSD is of course a very rare but serious condition that worsens over time and can be fatal.  There is the best homeopathic hospital in Hyderabad that can treat well.

What are the symptoms of Hallervorden-Spatz disease?

HSD does lead to a wide variety of symptoms that vary based on the severity of the disease and how long tend to progress.

Distorting muscle contractions are common symptom of HSD. They may occur in one’s face, trunk, and limbs. Unintentional, jerky muscle movements are other symptoms.

One may also experience involuntary muscle contractions that tend to cause abnormal posture or slow, recurring movements. This is referred to as dystonia. Best homeopathic doctors in Hyderabad are well-versed to treat Hallervorden-Spatz disease.

HSD may also make it difficult to coordinate movements. This is called ataxia. Eventually, it may cause an inability to walk after 10 or more years of initial symptoms.

Other symptoms of HSD include:

  • rigid muscles
  • writhing movements
  • tremors
  • seizures
  • disorientation
  • confusion
  • stupor
  • dementia
  • weakness
  • drooling
  • difficulty swallowing, or dysphagia

Less common symptoms include:

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

Causes of Hallervorden-Spatz disease

HSD happens to be a genetic disease. It is normally caused by an inherited defect in one’s pantothenate kinase 2 (PANK2) gene.

The PANK2 protein does control one’s body’s formation of coenzyme A. This molecule does help one’s body convert fats, some amino acids, and carbohydrates into energy.

In some cases, HSD is not due to PANK2 mutations. Several other gene mutations have also been observed in association with Hallervorden-Spatz disease, but they are less common than the PANK2 gene mutation.

HSD also involves a buildup of iron in certain parts of one’s brain. This buildup does cause the symptoms of the disease and it does become worse over some time. Best homeopathic hospital in Hyderabad can provide excellent care for this health issue.

Risk factors of Hallervorden-Spatz disease

HSD is inherited in case both parents have the disease-causing gene which is passed onto their child. If only one parent had the gene then also one can be a carrier of the disease to the off-springs. HSD generally develops in childhood. Late-onset HSD may not show up until adulthood.

How is Hallervorden-Spatz disease diagnosed?

If a person suspects HSD, it is better to discuss the health issue with the doctor. Details of personal and family medical history will be taken. A physical examination will be carried out.

A neurological exam is required to check for:

  • tremors
  • weakness
  • muscle rigidity
  • abnormal movement or posture

The doctor may suggest an MRI scan to rule out other neurological or movement disorders.

Screening for HSD is not very typical, but it can be obtained ifa person has the symptoms. In case the family is at a risk of the disease, it is advisable to have the baby genetically tested with an amniocentesis while in utero.

Treatment of Hallervorden-Spatz disease 

At present there is no cure for HSD. The doctor will treat the symptoms.Treatment does vary from person to person. It may include therapy, medication, or even both.

Therapy

Physical therapy can also help prevent as well as reduce muscle rigidity. It may also help reduce one’s muscle spasms and other muscular issues.

Occupational therapy can help a person develop skills for daily life. It can also help to retain one’s current abilities.Speech therapy helps manage dysphagia or speech impairment.

Medication

The doctor may prescribe one or more types of medication such as:

Hallervorden-Spatz Disease – Symptoms and Causes

  • methscopolamine bromide for drooling
  • baclofen for dystonia
  • memantine, rivastigmine, or donepezil (Aricept) to treat the symptoms of dementia
  • benztropine, which is an anti-cholinergic drug used to treat muscle rigidity and tremors
  • bromocriptine, pramipexole, or Levodopa to treat dystonia and Parkinson-like symptoms

 

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