Central pontine myelinosis is a rare neurological disease caused by damage to the protective myelin coatings of nerve cells in the brainstem. Aggressive treatment for low sodium levels is a major risk factor, and symptoms include slurred speech, motor difficulties, and cognitive impairments. There is no cure, but medications and therapy can help manage symptoms. Diagnosis involves physical evaluation and brain imaging.
Central pontine myelinosis is a rare neurological disease that results from acute damage to a part of the brainstem called the pons. Problems occur when the protective myelin coatings of the nerve cells in the pons begin to disintegrate. The exact causes of central pontine myelinosis are unclear, but people who receive aggressive treatment for very low sodium levels are at a higher risk of developing symptoms. Patients may experience slurred speech, mental slowness and confusion, motor difficulties, and other serious complications. There is no cure for the disorder, but medications and guided therapy can help many people regain some of their cognitive and physical abilities.
Myelin sheaths are composed of specialized cells that protect the delicate structures of the nervous system and facilitate rapid electrical signaling. In pons, myelin sheaths are vital for normal cognitive functioning and control of muscle movements throughout the body. Central pontine myelinosis is the degeneration of these important sheaths. In some cases, the destruction of myelin extends beyond the pons to damage other areas of the brain and central nervous system.
Receiving inpatient treatment for hyponatremia, or low blood sodium, is the major risk factor for developing central pontine myelinosis. Intravenous fluids and medications intended to restore normal sodium levels can have immediate and major impacts on the brain stem and rapidly lead to myelin degeneration. Patients suffering from long-term alcoholism, malnutrition, anorexia, or chronic liver failure are at increased risk for brainstem complications due to treatment of hyponatremia.
Symptoms of central pontine myelinosis can vary, but most patients first experience something called spastic tetraplegia. The muscles in the arms and legs become stiff, weak, and prone to sudden spasms. Additionally, many people with the condition develop slurred speech, vision changes, trouble swallowing, and poor balance. The disorder can also lead to deficits in concentration, memory, and information processing. It is also possible to suffer from delirium or go into a coma shortly after the first symptoms appear.
A neurologist can diagnose central pontine myelinosis by evaluating physical symptoms, reviewing a patient’s medical history, and looking at imaging scans of the brain. MRIs often reveal slight swelling or tissue damage on the bridge. Treatment for the condition is supportive in nature and typically involves the use of antipsychotics, antidepressants, muscle relaxants, and other medications to help with acute symptoms. Mentally stable patients are usually enrolled in physical therapy programs to help them rebuild muscle strength and improve coordination.
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