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Bulbar muscle weakness, or bulbar palsy, refers to a set of signs and symptoms linked to the impaired function of the lower cranial nerves, typically caused by damage. It is characterised by weakness of certain facial muscles, including the tongue, jaw, and muscles involved in breathing, speaking, and swallowing. Bulbar palsy is often a symptom of an underlying condition, such as amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, or other neurodegenerative, autoimmune, or genetic diseases. In some cases, bulbar muscle weakness can lead to respiratory failure and require supportive treatments such as speech therapy, medication, and physical therapy.
| Characteristics | Values |
|---|---|
| Definition | Impaired function of the lower cranial nerves |
| Causes | Neurodegenerative diseases, autoimmune diseases, genetic causes |
| Symptoms | Muscle weakness, difficulty swallowing, impaired speech, drooling, weakness of jaw and facial muscles |
| Treatment | Non-invasive ventilation, tracheostomy, speech therapy, medications, muscle relaxants, physical therapy |
| Progression | Death occurs 1 to 3 years from the start of the disorder due to complications such as aspiration pneumonia |
| Specific conditions | Amyotrophic lateral sclerosis (ALS), Guillain-Barré syndrome, Kennedy disease, pseudobulbar palsy |
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What You'll Learn
Bulbar palsy
The condition is sometimes confused with pseudobulbar palsy, which is caused by damage to the upper motor neurons in the corticobulbar tract, a neural pathway that connects the brain's motor cortex to the brainstem. While the two conditions share many of the same symptoms, pseudobulbar palsy is often characterised by the atypical expression of emotion displayed by unusual outbursts of laughing or crying, whereas, with bulbar palsy, an individual's emotions usually remain unaffected.
Non-progressive bulbar palsy, on the other hand, refers to bulbar palsy that does not worsen and is considered very uncommon. It is often the result of structural brain damage from trauma, stroke, or congenital disorders rather than a degenerative condition. Other common causes of bulbar palsy include autoimmune diseases like Guillain-Barré syndrome, where the immune system attacks the myelin sheath or axons of peripheral nerves, leading to loss of sensation and muscle weakness. Kennedy disease, a rare X-linked recessive neuromuscular disorder primarily affecting males, is another genetic cause of bulbar palsy.
There is currently no cure for bulbar palsy, so treatment is limited to medical management and support. Treatment options include medication such as intravenous immunoglobulin and steroids, as well as physical therapy to improve posture, slow muscle atrophy, and aid joint mobility. In cases of airway obstruction due to bulbar palsy, intubation may be used. If bulbar weakness leads to respiratory failure, non-invasive ventilation or a tracheostomy (breathing tube) may be recommended depending on the severity of the condition.
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Myasthenia gravis
There are three main types of myasthenia gravis: autoimmune, neonatal, and congenital. In autoimmune MG, the body creates antibodies that block, alter, or destroy the receptors for acetylcholine, a neurotransmitter that carries signals between nerves and muscles. This prevents the muscle from contracting. The thymus gland, which controls immune function, may also be associated with autoimmune MG. In neonatal MG, the fetus acquires antibodies from the mother, while congenital myasthenic syndrome is caused by mutations in genes required for the normal function of the neuromuscular junction.
Bulbar myasthenia gravis is a subtype of generalized MG where the bulbar muscles in the head and neck are affected. Symptoms of bulbar MG include dysphagia (difficulty swallowing), dysarthria (slurred or unclear speech due to weak muscles), and facial weakness. About 15% of people with MG initially experience bulbar symptoms, such as difficulty speaking and eating. Diagnosis of bulbar MG typically involves a physical exam to test muscle strength, blood tests to look for specific antibodies, and electrodiagnostic tests such as an electromyograph (EMG).
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Amyotrophic lateral sclerosis (ALS)
Bulbar palsy is a set of signs and symptoms linked to the impaired function of the lower cranial nerves, which are typically caused by damage. One of the most common causes of bulbar palsy is amyotrophic lateral sclerosis (ALS), a rare neurological disorder characterised by the gradual deterioration and death of both upper and lower motor neurons. Motor neurons are nerve cells in the brain and spinal cord that control voluntary muscle movement and breathing.
As the disorder progresses, muscle weakness and atrophy spread to other parts of the body. People with ALS may develop problems with speaking, chewing, walking, breathing, and moving their hands and arms. They usually remain able to reason, remember, and understand, but they are aware of their progressive loss of function, which can cause anxiety and depression in the person with ALS and their loved ones. Although not as common, people with ALS may also experience problems with language or decision-making. Some also develop a form of dementia known as FTD-ALS. Most people with ALS die from being unable to breathe on their own (respiratory failure), usually within three to five years of symptoms first appearing. However, about one in ten people survive for ten years or more.
Progressive bulbar palsy is a specific form of ALS where degeneration occurs primarily in the brainstem, affecting speech and swallowing first. Other clinical signs associated with bulbar palsy include nasal speech that lacks modulation, difficulty pronouncing certain consonants, wasting of the tongue, drooling, and weakness of the jaw and facial muscles. In contrast, pseudobulbar palsy is a clinical syndrome similar to bulbar palsy, but the damage is located in the upper motor neurons of the corticobulbar tracts in the mid-pons. This is usually caused by a stroke.
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Fazio Londe syndrome
Fazio-Londe syndrome (FLS), also known as Fazio-Londe disease (FLD) or progressive bulbar palsy of childhood, is a rare inherited motor neuron disease that affects children and young adults. It is characterised by progressive paralysis of muscles innervated by cranial nerves, particularly the tongue, face, and pharyngeal muscles.
The syndrome is caused by a genetic mutation in the SLC52A3 gene on chromosome 20, which results in abnormalities in the intestinal riboflavin transporter (hRFT2). This mutation leads to a deficiency in riboflavin, which is essential for nerve function. The disease is inherited in an autosomal recessive manner, meaning that an individual must inherit the mutated gene from both parents to be affected.
The most common symptoms of Fazio-Londe syndrome include bulbar palsy, hearing loss, facial weakness, and difficulty breathing. Other symptoms may include difficulty swallowing, nasal regurgitation of liquids, and slurred or unclear speech. In some cases, the disease may progress rapidly, leading to death within 9 months to 3 years. However, with early diagnosis and treatment, the progression of the disease can be slowed, and symptoms can be managed.
Fazio-Londe syndrome is often considered the same disease entity as Brown-Vialetto-Van Laere syndrome (BVVL), another rare neurological disorder characterised by sensorineural deafness, bulbar palsy, and respiratory compromise. Both conditions have been found to respond positively to riboflavin supplementation, which has proven to be a life-saving treatment.
The Italian pathologist Eugenio Fazio (1849-1902) and the French physician Paul Frederic Louis Londe (1864-1944) are credited with the discovery of this syndrome.
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Pseudobulbar palsy
The main symptoms of pseudobulbar palsy include difficulty swallowing and speaking, and rapid, dramatic changes in mood. Other symptoms include drooling or excessive drooling, differences in the rhythm and sound of speech (which may be slurred), tongue weakness or paralysis, facial muscle weakness, difficulty chewing, outbursts of uncontrolled or inappropriate laughing or crying, muscle spasms in the jaw, exaggerated facial reflexes, and increased reflexes and spasticity in the tongue and the bulbar region. About 85% of children with pseudobulbar palsy experience cognitive impairment and seizures.
The condition is usually caused by bilateral damage to the corticobulbar pathways, which are upper motor neuron pathways that course from the cerebral cortex to nuclei of cranial nerves in the brain stem. It is often the result of a stroke, but it can also be caused by other neurological and cerebrovascular conditions, including amyotrophic lateral sclerosis (ALS), Parkinson's disease, progressive supranuclear palsy, multiple sclerosis (MS), and brain trauma.
There is no specific treatment for pseudobulbar palsy, but therapies can help manage its features. Treating the underlying disease may eventually reduce the symptoms of pseudobulbar palsy. Possible pharmacological interventions include tricyclic antidepressants, serotonin reuptake inhibitors, dextromethorphan and quinidine sulfate, and Nuedexta, an FDA-approved medication for pseudobulbar affect.
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Frequently asked questions
Bulbar muscle weakness, also known as bulbar palsy, refers to weakness of certain facial and neck muscles, including the tongue, jaw, and muscles involved in breathing, swallowing, and speaking.
Bulbar muscle weakness can be caused by various conditions, including:
- Neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS) or Fazio Londe syndrome.
- Autoimmune diseases like Guillain-Barré syndrome or myasthenia gravis.
- Genetic causes such as Kennedy disease.
Symptoms of bulbar muscle weakness can include:
- Nasal speech that lacks modulation.
- Difficulty pronouncing certain consonants.
- Wasting of the tongue.
- Drooling.
- Weakness of the jaw and facial muscles.
- Dysphagia (difficulty swallowing).
- Dysarthria (slurred or unclear speech).
- Respiratory failure.
