Pseudobulbar Affect and ALS

Pseudobulbar Affect (PBA) is seen in a number of patients diagnosed with ALS and is thought to be the result of nerve degeneration in the limbic region of the brain. Medication can be used to help improve symptoms especially the earlier stages of the disease where the individual may be taking part in many social situations.

How ALS Affects The Body

Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease that destroys the nerves in the body and causes a wide range of different symptoms. The primary effect of ALS is to weaken muscles and slowly paralyze the body.

In the later stages of the condition, patients will have difficulty breathing and swallowing and will typically require medical intervention.

While gradual loss of motor movement are the main symptoms of the disease, ALS patients will often experience secondary symptoms. These sometimes occur because of nerve damage in areas that are not associated with the muscles.

One of these secondary symptoms associated with ALS is called the Pseudobulbar Affect (PBA) which, according to statistics, can be present in anywhere between 15% and 45% of individuals with the disease.

Here we take a closer look at PBA, what potentially causes it and how it can be managed effectively.

What is Pseudobulbar Affect (PBA)?

The Pseudobulbar Affect in ALS patients is characterized by periods of uncontrolled laughing or crying that has no relation, or seems out of proportion, to how the individual actually feels. In some individuals, attacks of PBA can be extremely severe, take a long while to subside and may even happen several times in a day.

For the person caring for an individual with a PBA outburst, it can be quite difficult to cope with behavior like this unless there is an understanding of the symptom and how it forms part of their ALS diagnosis . In the early stages of the disease, PBA may cause embarrassment in social situations and could even be a primary symptom in the detection of the disease.

PBA is not unique to ALS alone, however, and occurs in a number of illnesses where neurological damage is present. It does not affect all patients with ALS either and much depends on the progression of the disease and which nerves it attacks.

Why does Pseudobulbar Affect Occur?

It’s thought that the Pseudobulbar Affect is caused by damage to the nerves serving the limbic region in the brain. This is an area that controls our emotional response as well as the muscles we use in primitive vocalization.

Because ALS is not uniform in how it attacks the body, much depends on whether this area is affected in the individual which is why it doesn’t always present as a secondary symptom.  

Symptoms and Diagnosis of Pseudobulbar Affect

The affect can often be confused with other conditions such as bipolar disorder if someone has not yet been diagnosed with ALS.

The main symptoms of the Pseudobulbar Affect are:

• Episodes when the individual is laughing or crying uncontrollably. The expression is generally not related to the emotions that they are feeling. For example, a person may start laughing when they are experiencing something sad.
• When an individual cries it may be for a prolonged period and can be difficult to stop.
• If individuals are discussing subjects that have some component of seriousness or fun, their response may be an overreaction or inappropriate for the situation.

If a condition such as ALS has not been diagnosed, PBA can obviously have a profound impact on the individual and those around them. An individual may suddenly start crying but not understand why they are doing so.

Their crying or laughing may be part of a whole range of other symptoms and the usual diagnostic tool for the person to have are a neurological exam and complete a questionnaire relating to their condition.

It’s important to separate PBA from any other neurological condition before any management attempts are undertaken. While medication can be used, understanding that it is a symptom of ALS can help individuals manage their response in less severe cases.

Management of Pseudobulbar Affect

There are several interventions used when it comes to the Pseudobulbar Affect in ALS patients. The most recent of these is the drug Nuedexta which was approved in 2011 in the US. This combines dextromethorphan and quinidine and has been shown to have some effectiveness in managing the associated symptoms of the Pseudobulbar Affect.

Other options include tricyclic antidepressants, some serotonin uptake inhibitors, valproate and lithium.

Part of the management of PSA in individuals with ALS is understanding what is causing it and coping with those symptoms. It can be difficult for those caring for a loved one with ALS to see them displaying the sudden emotional and uncontrollable outbursts that are typical of Pseudobulbar Affect.