Bulbar palsy
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Bulbar palsy refers to a range of different signs and symptoms linked to impairment of function of the
glossopharyngeal nerve The glossopharyngeal nerve (), also known as the ninth cranial nerve, cranial nerve IX, or simply CN IX, is a cranial nerve that exits the brainstem from the sides of the upper medulla, just anterior (closer to the nose) to the vagus nerve. ...
(CN IX), the
vagus nerve The vagus nerve, also known as the tenth cranial nerve, cranial nerve X, or simply CN X, is a cranial nerve that interfaces with the parasympathetic control of the heart, lungs, and digestive tract. It comprises two nerves—the left and righ ...
(CN X), the
accessory nerve The accessory nerve, also known as the eleventh cranial nerve, cranial nerve XI, or simply CN XI, is a cranial nerve that supplies the sternocleidomastoid and trapezius muscles. It is classified as the eleventh of twelve pairs of cranial nerv ...
(CN XI), and the
hypoglossal nerve The hypoglossal nerve, also known as the twelfth cranial nerve, cranial nerve XII, or simply CN XII, is a cranial nerve that innervates all the extrinsic and intrinsic muscles of the tongue except for the palatoglossus, which is innervated ...
(CN XII). It is caused by a
lower motor neuron lesion A lower motor neuron lesion is a lesion which affects nerve fibers traveling from the lower motor neuron(s) in the anterior horn/ anterior grey column of the spinal cord, or in the motor nuclei of the cranial nerves, to the relevant muscle(s). ...
in the
medulla oblongata The medulla oblongata or simply medulla is a long stem-like structure which makes up the lower part of the brainstem. It is anterior and partially inferior to the cerebellum. It is a cone-shaped neuronal mass responsible for autonomic (invol ...
, or from lesions to these nerves outside the
brainstem The brainstem (or brain stem) is the posterior stalk-like part of the brain that connects the cerebrum with the spinal cord. In the human brain the brainstem is composed of the midbrain, the pons, and the medulla oblongata. The midbrain is ...
. This may be caused by any of a number of genetic, vascular, degenerative, inflammatory, and other underlying conditions. It can be differentiated from
pseudobulbar palsy Pseudobulbar palsy is a medical condition characterized by the inability to control facial movements (such as chewing and speaking) and caused by a variety of neurological disorders. Patients experience difficulty chewing and swallowing, have in ...
. When there is
airway obstruction Airway obstruction is a blockage of respiration in the airway that hinders the free flow of air. It can be broadly classified into being either in the upper airway (UPA) or lower airway (LOA). Airway obstruction is a life-threatening condition ...
,
intubation Intubation (sometimes entubation) is a medical procedure involving the insertion of a tube into the body. Patients are generally anesthetized beforehand. Examples include tracheal intubation, and the balloon tamponade with a Sengstaken-Blake ...
is used.


Signs and symptoms


Symptoms

*
dysphagia Dysphagia is difficulty in swallowing. Although classified under "symptoms and signs" in ICD-10, in some contexts it is classified as a condition in its own right. It may be a sensation that suggests difficulty in the passage of solids or liq ...
(difficulty in swallowing). * difficulty in
chewing Chewing or mastication is the process by which food is crushed and ground by teeth. It is the first step of digestion, and it increases the surface area of foods to allow a more efficient break down by enzymes. During the mastication process, th ...
. * nasal regurgitation. * difficulty in handling secretions, including aspiration of liquids. *
difficulty breathing Shortness of breath (SOB), also medically known as dyspnea (in AmE) or dyspnoea (in BrE), is an uncomfortable feeling of not being able to breathe well enough. The American Thoracic Society defines it as "a subjective experience of breathing disc ...
(
airway obstruction Airway obstruction is a blockage of respiration in the airway that hinders the free flow of air. It can be broadly classified into being either in the upper airway (UPA) or lower airway (LOA). Airway obstruction is a life-threatening condition ...
). *
dysphonia A hoarse voice, also known as dysphonia or hoarseness, is when the voice involuntarily sounds breathy, raspy, or strained, or is softer in volume or lower in pitch. A hoarse voice, can be associated with a feeling of unease or scratchiness in the ...
(defective use of the voice, inability to produce sound due to laryngeal weakness). *
dysarthria Dysarthria is a speech sound disorder resulting from neurological injury of the motor component of the motor–speech system and is characterized by poor articulation of phonemes. In other words, it is a condition in which problems effective ...
(difficulty in articulating words due to a CNS problem), such as slurred speech.


Signs

*
flaccid paralysis Flaccid paralysis is a neurological condition characterized by weakness or paralysis and reduced muscle tone without other obvious cause (e.g., trauma). This abnormal condition may be caused by disease or by trauma affecting the nerves associa ...
, such as
soft palate The soft palate (also known as the velum, palatal velum, or muscular palate) is, in mammals, the soft tissue constituting the back of the roof of the mouth. The soft palate is part of the palate of the mouth; the other part is the hard palat ...
weakness (examined by asking the patient to say aah). *
muscle atrophy Muscle atrophy is the loss of skeletal muscle mass. It can be caused by immobility, aging, malnutrition, medications, or a wide range of injuries or diseases that impact the musculoskeletal or nervous system. Muscle atrophy leads to muscle weakness ...
, such as tongue atrophy with fasciculations. *nasal speech lacking in modulation and difficulty with all consonants. *
drooling Drooling, or slobbering, is the flow of saliva outside the mouth. Drooling can be caused by excess production of saliva, inability to retain saliva within the mouth (incontinence of saliva), or problems with swallowing (dysphagia or odynophagia ...
of saliva. * normal or absent
jaw jerk The jaw jerk reflex or the masseter reflex is a stretch reflex used to test the status of a patient's trigeminal nerve (cranial nerve V) and to help distinguish an upper cervical cord compression from lesions that are above the foramen magnum. The m ...
. * reduced or absent
gag reflex The pharyngeal reflex or gag reflex is a reflex muscular contraction of the back of the throat, evoked by touching the roof of the mouth, the back of the tongue, the area around the tonsils, the uvula, and the back of the throat. It, along with ot ...
. In addition, there may be
lower motor neuron Lower motor neurons (LMNs) are motor neurons located in either the anterior grey column, anterior nerve roots (spinal lower motor neurons) or the cranial nerve nuclei of the brainstem and cranial nerves with motor function (cranial nerve low ...
lesions of the limbs. The ocular muscles are spared and this differentiates it from
myasthenia gravis Myasthenia gravis (MG) is a long-term neuromuscular junction disease that leads to varying degrees of skeletal muscle weakness. The most commonly affected muscles are those of the eyes, face, and swallowing. It can result in double vision, ...
.


Causes

* Genetic:
Kennedy's disease Spinal and bulbar muscular atrophy (SBMA), popularly known as Kennedy's disease, is a debilitating neurodegenerative disorder resulting in muscle cramps and progressive weakness due to degeneration of motor neurons in the brainstem and spinal cor ...
,
acute intermittent porphyria Acute intermittent porphyria (AIP) is a rare metabolic disorder affecting the production of heme resulting from a deficiency of the enzyme porphobilinogen deaminase. It is the most common of the acute porphyrias. Signs and symptoms The clinical ...
. * Vascular causes: medullary infarction, such as
lateral Lateral is a geometric term of location which may refer to: Healthcare *Lateral (anatomy), an anatomical direction * Lateral cricoarytenoid muscle * Lateral release (surgery), a surgical procedure on the side of a kneecap Phonetics *Lateral co ...
or medial medullary infarction. * Degenerative diseases:
amyotrophic lateral sclerosis Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND) or Lou Gehrig's disease, is a neurodegenerative disease that results in the progressive loss of motor neurons that control voluntary muscles. ALS is the most commo ...
,
syringobulbia Syringobulbia is a medical condition in which syrinxes, or fluid-filled cavities, affect the brainstem (usually the lower brainstem). The exact cause is often unknown, but may be linked to a widening of the central canal of the spinal cord. Thi ...
,
Wolfram syndrome Wolfram syndrome, also called DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness), is a rare autosomal-recessive genetic disorder that causes childhood-onset diabetes mellitus, optic atrophy, and deafness as well as vario ...
. * Inflammatory/infective:
Guillain–Barré syndrome Guillain–Barré syndrome (GBS) is a rapid-onset muscle weakness caused by the immune system damaging the peripheral nervous system. Typically, both sides of the body are involved, and the initial symptoms are changes in sensation or pain oft ...
,
poliomyelitis Poliomyelitis, commonly shortened to polio, is an infectious disease caused by the poliovirus. Approximately 70% of cases are asymptomatic; mild symptoms which can occur include sore throat and fever; in a proportion of cases more severe sym ...
,
Lyme disease Lyme disease, also known as Lyme borreliosis, is a vector-borne disease caused by the '' Borrelia'' bacterium, which is spread by ticks in the genus '' Ixodes''. The most common sign of infection is an expanding red rash, known as erythema ...
. * Cancer: brainstem
glioma A glioma is a type of tumor that starts in the glial cells of the brain or the spine. Gliomas comprise about 30 percent of all brain tumors and central nervous system tumours, and 80 percent of all malignant brain tumours. Signs and symptoms ...
, malignant meningitis. * Toxic:
botulism Botulism is a rare and potentially fatal illness caused by a toxin produced by the bacterium ''Clostridium botulinum''. The disease begins with weakness, blurred vision, feeling tired, and trouble speaking. This may then be followed by weakne ...
, venom of
bark scorpion :''The striped bark scorpion and the closely related Baja California bark scorpion are also called bark scorpions.'' The Arizona bark scorpion (''Centruroides sculpturatus'', once included in '' Centruroides exilicauda'') is a small light brown ...
(species Centruroides), some neurotoxic
snake venom Snake venom is a highly toxic saliva containing zootoxins that facilitates in the immobilization and digestion of prey. This also provides defense against threats. Snake venom is injected by unique fangs during a bite, whereas some species are a ...
s. * Autoimmune:
myasthenia gravis Myasthenia gravis (MG) is a long-term neuromuscular junction disease that leads to varying degrees of skeletal muscle weakness. The most commonly affected muscles are those of the eyes, face, and swallowing. It can result in double vision, ...
.


Mechanism

Bulbar palsy involves problems with function of the
glossopharyngeal nerve The glossopharyngeal nerve (), also known as the ninth cranial nerve, cranial nerve IX, or simply CN IX, is a cranial nerve that exits the brainstem from the sides of the upper medulla, just anterior (closer to the nose) to the vagus nerve. ...
(CN IX), the
vagus nerve The vagus nerve, also known as the tenth cranial nerve, cranial nerve X, or simply CN X, is a cranial nerve that interfaces with the parasympathetic control of the heart, lungs, and digestive tract. It comprises two nerves—the left and righ ...
(CN X), the
accessory nerve The accessory nerve, also known as the eleventh cranial nerve, cranial nerve XI, or simply CN XI, is a cranial nerve that supplies the sternocleidomastoid and trapezius muscles. It is classified as the eleventh of twelve pairs of cranial nerv ...
(CN XI), and the
hypoglossal nerve The hypoglossal nerve, also known as the twelfth cranial nerve, cranial nerve XII, or simply CN XII, is a cranial nerve that innervates all the extrinsic and intrinsic muscles of the tongue except for the palatoglossus, which is innervated ...
(CN XII). These all emerge from pathways in the
medulla oblongata The medulla oblongata or simply medulla is a long stem-like structure which makes up the lower part of the brainstem. It is anterior and partially inferior to the cerebellum. It is a cone-shaped neuronal mass responsible for autonomic (invol ...
. A
lower motor neuron lesion A lower motor neuron lesion is a lesion which affects nerve fibers traveling from the lower motor neuron(s) in the anterior horn/ anterior grey column of the spinal cord, or in the motor nuclei of the cranial nerves, to the relevant muscle(s). ...
can impair their function.


Diagnosis


Differential diagnosis

In contrast,
pseudobulbar palsy Pseudobulbar palsy is a medical condition characterized by the inability to control facial movements (such as chewing and speaking) and caused by a variety of neurological disorders. Patients experience difficulty chewing and swallowing, have in ...
is a clinical syndrome similar to bulbar palsy but in which the damage is located in
upper motor neurons Upper motor neurons (UMNs) is a term introduced by William Gowers in 1886. They are found in the cerebral cortex and brainstem and carry information down to activate interneurons and lower motor neurons, which in turn directly signal muscles t ...
of the corticobulbar tracts in the mid-pons (i.e., in the
cranial nerve Cranial nerves are the nerves that emerge directly from the brain (including the brainstem), of which there are conventionally considered twelve pairs. Cranial nerves relay information between the brain and parts of the body, primarily to and ...
s IX-XII), that is the nerve cells coming down from the cerebral cortex innervating the motor nuclei in the medulla. This is usually caused by
stroke A stroke is a disease, medical condition in which poor cerebral circulation, blood flow to the brain causes cell death. There are two main types of stroke: brain ischemia, ischemic, due to lack of blood flow, and intracranial hemorrhage, hemorr ...
.


Treatment

In patients with
airway obstruction Airway obstruction is a blockage of respiration in the airway that hinders the free flow of air. It can be broadly classified into being either in the upper airway (UPA) or lower airway (LOA). Airway obstruction is a life-threatening condition ...
due to bulbar palsy,
intubation Intubation (sometimes entubation) is a medical procedure involving the insertion of a tube into the body. Patients are generally anesthetized beforehand. Examples include tracheal intubation, and the balloon tamponade with a Sengstaken-Blake ...
may be used. This can be
tracheal intubation Tracheal intubation, usually simply referred to as intubation, is the placement of a flexible plastic tube into the trachea (windpipe) to maintain an open airway or to serve as a conduit through which to administer certain drugs. It is frequentl ...
or supraglottal intubation.


See also

*
Progressive bulbar palsy Progressive bulbar palsy (PBP) is a medical condition. It belongs to a group of disorders known as motor neuron diseases. Lapiedra 2002 PBP is a disease that attacks the nerves supplying the bulbar muscles. These disorders are characterized by the ...


References


External links

{{Medical resources , DiseasesDB = 16043 , ICD10 = {{ICD10, G, 12, 2, LinkGroup, LinkMajor , ICD9 = {{ICD9, 335.22 , ICDO = , OMIM = 211530 , MedlinePlus = , eMedicineSubj = , eMedicineTopic = , MeshID = D010244 Neurological disorders