Olivopontocerebellar Atrophy

Olivopontocerebellar Atrophy

Olivopontocerebellar atrophy is a neurological disease where the nerves and tissues of the area of brain [cerebellum and pons] degenerate and shrink. This is characterized by difficulty to control muscle movements. It is a type of neurodegenerative syndrome, showing multiple involvement and presentation with cerebellar and extrapyramidal signs and symptoms.

Causes and risk factors

Olivopontocerebellar atrophy is a hereditary disease. It runs in the families. Laboratory workers who are exposed to toxic chemicals are at high risk of developing OPA. The disease is more common after 50 years of age.

Clinical presentation

Patient shows symptoms like clumsiness that slowly gets worse, problems with maintaining balance and gait [ataxia]. Difficulty in swallowing is experienced [dysphagia]. There is slurring of speech. Difficult walking and abnormal movements are observed [dysarthria]. Bowel or bladder problems, muscle spasms, muscle stiffness or rigidity with muscle weakness are seen. There is nerve damage [neuropathy], tremors. Sexual function problems may occur. Paresis or paralysis of voluntary muscles like respiratory muscles can occur.

Investigations

Medical history by the patient and clinical examination by the doctor helps in diagnosis. MRI of brain, CT scan of brain are done. Other blood tests can be done to rule out the chances of other medical conditions.

Treatment

There is no specific treatment or cure for this medical condition. Medications can be given for relieving tremors. Physiotherapy can be helpful. Occupational therapy is effective. Mechanical aids such as wheelchairs or crutches help in managing the condition.

Complications

Complications such as multiple organ atrophy, shivering, twitching of muscles, falling, bedridden conditions, choking, infection from inhaling food into the lungs [aspiration pneumonia], injury from falls, nutrition problems due to difficulty swallowing occur.

When to Contact a Doctor  

One must consult a doctor if patient shows clumsiness in movement, problems in balancing etc.

Systems involved

CNS

Organs involved

Brain, extremities, GIT, rectum and anal canal, muscles

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