Alexander Disease: Treatment, Cost and Side Effects

Alexander disease refers to a rare neurological condition, which was discovered by WS Alexander in 1949. The condition is usually fatal and there is no cure for the same.

A fatty layer, also known as myelin, normally covers nerve fibres. This sheath of myelin is essential for the proper regulation and functioning of the nervous system. However, in people with Alexander disease, the myelin sheath is either damaged or absent. This leads to a lot of health issues that degenerate over time. People suffering from the disease may also have strange protein deposits on the specialized cells supporting the brain and the spinal cord.

The disease is usually diagnosed at the time of birth, although there have been cases where the condition appears later on in life. The symptoms of the disease include spasms, feeding problems, learning disabilities, enlarged brain, failure to thrive, impaired mobility, mental regression and sleep disturbances.

The infants diagnosed with the condition slowly deteriorate and rarely survive to be a year old. If the disease is diagnosed between the ages of 4 and 10, the disease may progress gradually. Very few people affected with Alexander disease will survive into middle age, although it is not unheard of.

There are different forms of Alexander disease- these include; one, neonatal form in which serious disabilities or even death can occur in two years time. This form is marked by intellectual disability, hydrocephalus, seizures and severe motor. Two, Usually, the form of Alexander disease that occurs most commonly is infantile form where the onset takes place during the initial years of one’s life. There is a combination of both physical and mental delays which is succeeded by developmental milestone losses and abnormal rise in the size of the head and also the rate of seizures. Three, the juvenile form is most common and occurs between ages two and thirteen. Those who are suffering from this disorder experience trouble swallowing, bad coordination, trouble speaking and loss of motor skills. Four, the adult form is rare. It can occur anywhere between a person’s late teenage years to even later in life.

There is no cure for the disease and the treatment mainly looks to control the symptoms related to the condition. A patient suffering from the condition needs to be cared for, along with physical therapy in order to maintain the muscle mobility and overall movement. Often, speech therapy and a proper diet is also required. If any infection develops due to the Alexander disease, antibiotics may be prescribed to treat those infections.

Since Alexander disease is primarily a nerve disorder, patients will often suffer from severe epileptic fits and seizures. Antiepileptic drugs are prescribed in such cases, so as to control the frequency and intensity of the seizures.

As a side effect of Alexander disease, fluid may also form inside the brain of the patient. This condition is known as hydrocephalus and doctors usually perform an invasive surgery to relieve some of the fluids. A shunt is inserted into the brain, which then drains the fluid.

It is extremely rare for the condition to appear in people above the age of 65. However, even if the disease occurs, it is seldom diagnosed and the symptoms from the disease are confused with other diseases. Usually, Alexander disease in the elderly is often detected after the person passes away.

People diagnosed with Alexander disease qualify for the treatment. However, the form of treatment required depends on the kind of symptoms that a particular patient is going through. For instance, if the disease leads to an infection, antibiotics are used and if the patient suffers from seizures, antiepileptic drugs may be prescribed.

If the epilepsy and other symptoms associated with Alexander disease is being caused due to some other underlying condition, patients will not need treatment for the nerve disorder. However, they should seek medical help and get to the root of the issue.

Furthermore, if the Alexander disease is somehow diagnosed at the age of 65 or above, doctors may not start any treatment. For these patients, the main aim is to improve the quality of life and reduce the severity of the symptoms associated with the condition.

Brivaracetam is one of the most common drug for epilepsy. This medication may lead to drowsiness, nausea, fatigue, dizziness and vomiting. Similar drugs also cause additional disorders such as depression, loss appetite, unsteady walking. In children, drooling may also be caused due to antiepileptic medicine.

There are no guidelines as such, as the treatment does not cure the condition. The treatment will only reduce the intensity of some of the symptoms. Patients will need to be cared for throughout life, while the physical therapy is often necessary as well.

Recovery does not occur. In fact, the condition deteriorates over time and the disease progresses. This progression usually ends with the death of the patient. During this time, the treatment can only provide relief from some of the symptoms, but cannot completely remove any of them.

Price of treatment depends on the tenure and also on the individual patient. For instance, a patient may suffer from seizures or may not suffer from the same. The treatment cost will depend on the symptoms related to the disease. Generally, it takes anywhere between Rs. 10,000 to Rs. 50,000 in the treatment of Alexander disease.

Results of the treatment are not permanent and symptoms will recur once the treatment is stopped for a few days. Patients will need to undergo continuous treatment from the time that the disease is diagnosed, till the time of their death.

There are no alternative treatments for Alexander disease. Since the condition almost always leads to death, patients should opt for conventional forms of medication and therapy.