Hereditary Olivopontocerebellar Atrophy: Symptoms & Treatment

Olivopontocerebellar atrophy is a progressive neurological disorder which is characterized by degeneration of brain cells (neurone) of certain part of brain. The degeneration occurs in cells of cerebellum, pons and inferior olives. Cerebellum is responsible for balance and voluntary movement and pons acts as a coordinator of signals released from different part of brain. As these parts of brain starts degenerating patient losses control over body movement which over a period of time worsens.

He also suffers from several other symptoms such as slurred speech, tremor in hands, neuropathy, involuntary bowel and bladder issues etc.

Olivopontocerebellar atrophy in majority of patients is hereditary. However, sometimes exposure to harmful chemicals is also said to be an underlying reason. The disease usually begins in people over the age of 50. There is no definitive cure olivopontocerebellar atrophy. Treatment is aimed at improving daily life of the person.

What Causes Olivopontocerebellar Atrophy?

Hereditary olivopontocerebellar atrophy as the name suggests is a genetic disease affecting brain. It is caused due to mutation in the genes. In this disease brain cell of particular part of brain namely the pons, cerebellum and inferior olive begins to degenerate and die.

The disease usually occurs among people over the age of 50 but sometimes it may also develop at an early age. Men are more affected with this condition than females. Almost 3 persons out of every one million individuals are known to suffer from this condition, thus it is considered to be a rare disease.

Olivopontocerebellar atrophy sometimes also develops without any known history of inheritance. In such cases the prime suspected causative factor is exposure to certain harmful chemicals and environmental toxins.

Signs And Symptoms Of Olivopontocerebellar Atrophy

The symptoms and sign of hereditary olivopontocerebellar atrophy are variable from one person to another.

Although it a progressive disease, in some people the symptoms may progress gradually while in some patients it may worsen very fast. Symptoms usually begin after the age of 50. But sometimes it may present in the early adulthood.

Here are the most common symptoms of this condition:

  • Ataxia is one of the prominent and first symptoms to develop in olivopontocerebellar atrophy. Ataxia is a medical term used for loss of control over body movements. People in the beginning often misinterpret ataxia as clumsiness but in fact it is an indicating symptom of olivopontocerebellar atrophy.
  • Patient finds difficult to keep balance of his body while walking.
  • Slurred speech
  • Tremors in hands
  • Stiffness of muscles
  • Difficulty in swallowing
  • Involuntary urination and stool.
  • Abnormal movement of eyes.

Treatment Of Hereditary Olivopontocerebellar Atrophy

Hereditary olivopontocerebellar atrophy cannot be cured. The main aim of management is to provide relief to the patient in his daily life. Thus treatment is focused in improving his activities with the help of several options. For example medicines are prescribed for controlling tremors. Speech therapy helps the patient to improve his speech.

Patient may also be recommended various other modes of treatment such as occupational therapy, physical therapy that will help to keep the balance of his body and prevent falls. Walking aids such as walkers and canes will be valuable for the patient to keep his balance while walking. Patient is also taught technique of swallowing food so that he does not suffer nutritional deficiencies.