Progressive bulbar palsy

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at October 6, 2016
StartDiseasesProgressive bulbar palsy

Motor neuron diseases, such as progressive bulbar palsy (PBP) involves neurodegeneration of the cells that control muscle activity in the body such as speaking, walking, breathing, and swallowing. Progressive bulbar palsy is a rare type of motor neuron disease that affects both the lower motor neurons and upper motor neurons. This condition may eventually progress into amyotrophic lateral sclerosis (ALS or Lou Gehrig's disease). Some consider it a subset of ALS; 25% of people with ALS have this condition at the beginning of their illness.

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Definition & Facts

Progressive bulbar palsy, more simply known as PBP, is a motor neuron disease that attacks the nerves responsible for controlling the bulbar muscles of the body. The medical and anatomical term bulb or “bulbar” is an outdated and antiquated term for the medulla oblongata which is located in the brainstem. The word “bulbar” is often associated with and refers to the nerves connected to the medulla.

Progressive bulbar palsy reduces a patient's control over his or her tongue, pharynx, and larynx. It can result in speech disorders such as dysarthria, weakened muscles of the jaw, atrophy of the tongue, and dysphagia, that is, difficulty swallowing.

This medical condition, progressive bulbar palsy, is common in adults but can also occur in children. Brown-Vialetto-Van Laere syndrome is one type of infantile progressive bulbar palsy that results in deafness. Fazio-Londe disease is another form of infantile progressive bulbar palsy but it does not affect hearing.

Progressive bulbar palsy was first discovered and recognized by French neurologists, Guillaume Duchenne and Jean Cruveilhier in the mid-1800's. Since then a series of scientists and researchers have changed the name of this disease multiple times and have connected the similarity of its symptoms to that of amyotrophic lateral sclerosis (ALS), which also atrophies and weakens the body's muscles.

Symptoms & Complaints

Chewing, talking, and swallowing are made increasingly difficult and oftentimes impossible for the body and mind as this medical condition progresses. Other parts of the body that are weakened by progressive bulbar palsy (PBP) occur mostly in the head and neck regions and can include: reduced gag reflexes (pharyngeal reflex), weakened palatal movements, fasciculations, and atrophy of the facial muscles and the tongue. 

Emotional lability (pseudobulbar affect) is another symptom in which a patient cries or laughs uncontrollably or otherwise shows intense, uncontrollable emotions in reaction to mundane events.

In extremely complicated and progressive cases, progressive bulbar palsy can cause individuals to lose total control of their mouths and result in the inability to chew food, resulting in weight loss. When the muscles of the face start to weaken, patients also exhibit difficulty with pronouncing words and may drool.

Progressive bulbar palsy increases the chances of individuals choking and contracting pneumonia. Because the gag reflexes deteriorate, patients more likely to choke on their own saliva or inhale fluids into their lungs in an attempt to remedy a coughing attack. 

Causes

The causes of progressive bulbar palsy remain largely unknown. Brown-Vialetto-Van Laere syndrome, an infantile version of this condition can be passed down through autosomal recessive inheritance. Motor neuron diseases are more common among those over the age of 40.

Diagnosis & Tests

Stroke and myasthenia gravis will need to be ruled out because they share similar symptoms to progressive bulbar palsy. The diagnostic process, as with other conditions, involves asking the patient about his or her family history and medical history as well as performing a physical examination.

  • An electromyogram (EMG) may be used to examine the bulbar or throat muscles to test their electrical activity. If bulbar palsy is present, the electromyogram will likely be able to detect it because muscles without nerve supply have different electrical activity.
  • Blood tests may test for the presence of creatine which is a byproduct of muscles breaking down and which sometimes shows up in those with motor neuron diseases.
  • Magnetic resonance imaging (MRI) are used to rule out diseases that affect the head, neck, and the spinal cord
  • Muscle or nerve biopsies can assist in confirming the presence of a neurological disorder
  • Nerve conduction studies may also be performed to assess how the nerves transmit signals.
  • A lumbar puncture (also known as a spinal tap) may be performed.

Treatment & Therapy

Treating progressive bulbar palsy involves treating symptoms and improving the patient's quality of life. When symptoms are detected, doctors will typically work with patients and in coordination with a variety of therapists (usually physical and occupational) to properly manage and control the disease to the best of their ability.

Physical therapy, occupational therapy, and speech therapy may attempt to improve posture, slow muscle atrophy, increase range of motion and circulation, improve speech, and improve a person's capacity to chew and perform bodily functions. Stretching and applying heat may help to relieve and reduce muscle ache and pains within the body. 

Prevention & Prophylaxis

Because its causes are unknown, progressive bulbar palsy is not preventable.