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What is Central Pontine Myelinolysis?
Central pontine myelinolysis or CPM is a rare neurological disorder that primarily affects the myelin sheath of our nerve cells (neurons) found in the brainstem. This is a demyelinating disease which was first described in the year 1959. The said disease affects specifically the central portion of the brainstem (pons). Once the pons is damaged, it is expected that the patient shall have problems with maintaining the sense of balance, movement, sense of touch and speaking. According to researches, there is no exact number for the incidence rate of central pontine myelinolysis. Though this is an uncommon disease; mortality cases are still reported.
Diagnostic examination for central pontine myelinolysis includes imaging tests. The CT scan and MRI scan are two conventional procedures for CPM. An MRI scan is much recommended and considered as the main diagnostic tool for central pontine myelinolysis. In MRI scan results, damage to the pons is more visible compared with CT scan results. A physical examination can help in attaining the final diagnosis as long as the major symptoms are present.
Symptoms
The basic presentation of the disease is greatly associated with neurological deficit. The following are observed in patients with CPM:
- Delirium. The patient shall be significantly in a confused state. This manifestation is related to severe hyponatremia (electrolyte imbalance which there is below normal sodium concentration in the blood). This electrolyte imbalance is also included as a manifestation of the disease.
- Neurological status changed. There is a regression of the client’s RLS (Reaction Level Scale). Tendency is that the client starts to have reduced levels of alertness, with poor responses and eventually may come to a state of lethargy and unconsciousness.
- Quadriparesis and spastic quadriplegia. The motor function is greatly affected once the brainstem is affected. Quadriparesis is the general weakness of the extremities while spastic quadriplegia includes spasticity in all limbs.
- Weakness. Significant body parts are affected of weakness such as the face and upper and lower extremities.
- Speech problems and difficulty of swallowing food. The speech change is related to the neurological deficit that comes about when the pons is affected. Chewing and swallowing food becomes a hard task or is an unattainable task as the fifth cranial nerve is affected (pons is the point of origin of the CNV– Trigeminal nerve).
- Balance problems. This is a common manifestation as the cranial nerve responsible for maintaining balance and coordination is affected.
- Locked-in syndrome. This is considered as a complication from central pontine myelinolysis. Locked-in syndrome is composed of paralysis and eventually death. The voluntary muscles become paralyzed except for the muscles that control eye movement.
Central Pontine Myelinolysis Causes
The condition is related to a number of precipitating factors which includes alcoholism and malnutrition. According to studies, there are high risk clients for CPM. It was noted that chronic alcoholism, underlying liver disease or history of liver transplant and malnutrition is related to central pontine myelinolysis. Other high risk populations are those diagnosed with HIV-AIDS, those who take diuretics and those who suffer from bulimia or anorexia. In addition, the following can cause central pontine myelinolysis:
Abrupt rise in serum sodium levels. Those who are currently undergoing treatment from hyponatremia are said to be at risk for developing central pontine myelinolysis. However, there are cases where some do not develop such disease but does make them at risk for CPM development.
Renal dialysis and severe burns. This shall result to CPM because of the prolonged exposure to serum hyperosmolality.
Pathophysiology
The pathophysiology of the disease is quite difficult to understand as research for this, until now, is on the works. Sodium concentration in the blood/serum is connected to the destruction of the neurons’ myelin sheath. As there is an abrupt rise in the sodium concentration, fluid shifting mechanism is triggered thus resulting to the pulling of significant amount of water from brain cells.
The mechanism therefore is believed to cause damage to our nerve cells, particularly the myelin sheath, and when the pons receives the principal damage, central pontine myelinolysis develops. It is also considered that the pons is a susceptible part of the brainstem to suffer from myelinolysis. In addition, the myelin sheath is an important component of the nervous system. This structural entity of our neurons is an insulating material that helps increase the speed of transmitting nerve impulses. As the myelin sheath is damaged, nerve impulse transmission becomes ineffective.
Picture : Central pontine myelinolysis, MRI FLAIR
Image source : wikipedia.org
Treatment for Central Pontine Myelinolysis
Though there is vast information on this article about the treatment of this disease, it is highly recommended that you do not cling on self medication. Make sure you go to your doctor and seek professional help; this is to avoid any complication to happen.
Central pontine myelinolysis is considered to require emergent attention once suspected or diagnosed. A holistic approach in the treatment course should be provided in order to attain recovery. It has been decades since the disease has been identified, still development of treatment is undergoing. Until now, research for CPM is being propagated. The National Institute of Neurological Disorders and Stroke (NINDS) is holding research for CPM by granting funds to medical establishments. The ultimate aim for such research is to find suitable treatment course plans and to finally find the cure from central pontine myelinolysis.
The population should be made aware of central pontine myelinolysis. Prevention can be attained when people know the existence of such fatal disease, thus decreasing its incidence rate. Informing others that chronic alcoholism is a major risk factor for central pontine myelinolysis would be of help. Alcoholics or treated alcoholics should be provided with vitamin supplements in order to avoid development of CPM. One can also prevent CPM when medical professionals are attentive and particular with their roles to high risk patients. For example; nurses or doctors who will administer sodium replacement to patients suffering from hyponatremia should be a hundred percent cautious in doing such task. Correct dosage and rate of sodium administration is an essential task that can avoid CPM.
The main aim in the treatment plan for CPM is to provide comfort and assistance to the patient. Rehabilitative treatment is required as there are a number of major motor defects gained from the disease. The affected should be enrolled in physical therapy and occupational therapy in order to gradually recover from the effects of CPM. There are others who would require long term management, depending on the extent of affectation. Others may suffer from chronic disability; which is why it’s essential for the next of kin or a close family member to assist in the whole treatment course for a wider support (emotional support is also crucial in the treatment course).
Prognosis
Death is a common outcome of central pontine myelinolysis. Complete recovery should still be expected but would require intensive management for a number of months. Development of complications is also a possibility. After treatment, there are others who would still manifest residual symptoms and difficulties recovering from the significant alterations acquired. Eventually, recovery can be attained by a patient but is entirely variable from one person to another.
References
http://www.nlm.nih.gov/
http://en.wikipedia.org/wiki/
http://emedicine.medscape.com/
http://www.rightdiagnosis.com/
