This article looks at the symptoms and typical progression of bulbar-onset ALS. It also discusses causes, treatment options, when to contact a doctor, and more.
Bulbar-onset ALS typically affects speech and swallowing. The most common symptoms include:
- speech that’s slurred, slow, or weak
- coughing or choking while swallowing
- increased saliva in the mouth
- a gurgling voice after swallowing
- food or liquids spilling out of the mouth or coming out of the nose while eating
- regurgitation
- shortness of breath during eating
- vocal cord spasms
- involuntary tongue twitching
- nasal-sounding voice
Speech problems may begin around 3 years before a diagnosis of bulbar-onset ALS. Over time, there is a progressive loss of motor function due to central nervous system damage and loss of bulbar motor neurons.
Around 30% of people experience bulbar symptoms at ALS onset. This includes a reduced ability to speak and swallow safely. Individuals progressively become weaker and lose motor and respiratory function.
The standard way of assessing bulbar progression and dysfunction is with the ALS functional rating scale-revised (ALSFRS-R). Your doctor will be able to explain more about ALSFRS-R and how they monitor symptoms as they progress.
Learn more about how ALS symptoms progress.
Researchers do not know the exact cause of bulbar-onset ALS.
However, scientific evidence suggests that there are genetic and environmental factors that may come into play. In 1993, researchers discovered that the SOD1 gene was associated with some cases of familial ALS. Since then, they have identified over a dozen genetic mutations associated with ALS.
Research on gene mutations has revealed that changes in the processing of RNA molecules may lead to motor neuron degeneration related to ALS. RNA molecules are involved with gene activity. Other gene mutations indicate defects in the structure and shape of motor neurons.
Currently, researchers are studying the effects of environmental factors, such as exposure to toxins and infectious agents, such as viruses, as well as diet, physical trauma, and occupational factors.
Learn more about what causes ALS.
Treatment for bulbar-onset ALS involves an interdisciplinary team of doctors, occupational and speech therapists, nutritionists, social workers, and nurses.
The following are different treatments involved in the care of someone with bulbar-onset ALS.
Medication
The Food and Drug Administration (FDA) has approved various medications for ALS treatment. This includes the oral medication riluzole (Rilutek), the intravenous medication edaravone (Radicava), and the powder medication AMX0035 (RELYVRIO).
Both medications are used to slow the condition’s progression.
Your doctor may recommend other medications to help manage other ALS symptoms.
Speech therapy
Working with a speech therapist can help people with bulbar-onset ALS restore their communication function. It can also help assess swallowing and provide tips for safer swallowing.
Computer-based speech synthesizers may be suitable for helping people communicate in nonverbal ways. Similarly, a brain-computer interface allows people with bulbar-onset ALS to communicate using only brain activity.
Nutrition therapy
Nutritionists can help people with bulbar-onset ALS plan their meals and provide education on how to avoid foods that are difficult to swallow.
Caregivers can use suction devices to help remove excess fluids or saliva. In cases where the individual can no longer eat, a doctor may recommend inserting a feeding tube. Using a feeding tube helps reduce the risk of choking.
Respiratory support
As motor function decreases, individuals with bulbar-onset ALS may experience shortness of breath while eating or during physical activity. Noninvasive ventilation treatments, such as a breathing mask over the nose and mouth, can help provide respiratory support, especially at night.
As the condition progresses, a breathing tube through the mouth or front of the neck (tracheostomy) may be necessary to help support breathing.
Symptoms of bulbar-onset ALS usually progress over time. They do not come on suddenly. If you notice changes in your speech or ability to swallow foods or liquids, it’s a good idea to contact your doctor. These are often the first symptoms of bulbar-onset ALS.
Your doctor may want to evaluate your speech and swallowing abilities and conduct further testing to see what’s causing your symptoms.
Contact your doctor if you already have bulbar-onset ALS and are starting to develop shortness of breath, trouble breathing, or any other new symptoms.
There are several tests doctors use to diagnose bulbar-onset ALS. Some examples include:
- Cranial nerve exam: This can help detect bulbar upper motor neuron and lower motor neuron signs, such as weakness, muscle tone, and reflexes.
- Needle EMG: This test helps determine acute and chronic nerve supply deprivation of certain muscles.
- Videofluoroscopic swallowing exam: This is considered the gold standard for dysphagia testing, allowing doctors to directly visualize swallowing efficiency and safety.
- Swallow test: This is a screening test for dysphagia that involves giving the person 3 ounces of water and assessing whether they can swallow without coughing or stopping.
- MRI: A clinical MRI of the bulbar regions may be useful for ruling out other neurological conditions.
Your doctor will be able to explain the tests they order in more detail and answer any questions you may have.
Find out more about how doctors diagnose ALS.
The life expectancy for a person with bulbar-onset ALS is around 2 years or less after diagnosis.
Bulbar-onset ALS is more severe than limb-onset ALS. It generally leads to a faster decline in functioning.
It’s important to note that life expectancy differs for each person. Your doctor will be able to discuss your outlook with you with reference to your individual circumstances.
It’s not possible to prevent bulbar-onset ALS.
However, you can monitor for any signs or symptoms of the condition. Early recognition and treatment of the condition may help you to manage your symptoms and slow down the progression of the condition.
Bulbar-onset ALS is a progressive condition that affects an individual’s ability to speak, swallow, and breathe. It’s less common than limb-onset ALS but more aggressive.
Symptoms of the condition vary among individuals but eventually lead to inability to speak, trouble swallowing, vocal cord spasms, and involuntary tongue twitching.
Researchers believe the cause of bulbar-onset ALS is due to a combination of genetic and environmental factors. There is no way to prevent bulbar-onset ALS. However, early diagnosis and treatment can help with slowing down the condition and managing symptoms.
