Bulbar palsy

Last updated
Bulbar palsy
Specialty Neurology
Symptoms Dysphagia, dysarthria, flaccid paralysis, muscle atrophy, drooling of saliva, reduced or absent gag reflex

Bulbar palsy refers to a range of different signs and symptoms linked to impairment of function of the glossopharyngeal nerve (CN IX), the vagus nerve (CN X), the accessory nerve (CN XI), and the hypoglossal nerve (CN XII). It is caused by a lower motor neuron lesion in the medulla oblongata, or from lesions to these nerves outside the brainstem, and also botulism. 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. When there is airway obstruction, intubation is used.

Contents

Signs and symptoms

Symptoms

Signs

In addition, there may be lower motor neuron lesions of the limbs.

The ocular muscles are spared and this differentiates it from myasthenia gravis.

Causes

Mechanism

Bulbar palsy involves problems with function of the glossopharyngeal nerve (CN IX), the vagus nerve (CN X), the accessory nerve (CN XI), and the hypoglossal nerve (CN XII). [1] These all emerge from pathways in the medulla oblongata. [1] A lower motor neuron lesion can impair their function. [5] [1]

Diagnosis

Differential diagnosis

In contrast, pseudobulbar palsy is a clinical syndrome similar to bulbar palsy but in which the damage is located in upper motor neurons of the corticobulbar tracts in the mid-pons (i.e., in the cranial nerves 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.

Treatment

In patients with airway obstruction due to bulbar palsy, intubation may be used. [2] This can be tracheal intubation or supraglottal intubation. [2]

See also

Related Research Articles

<span class="mw-page-title-main">Cranial nerves</span> Nerves that emerge directly from the brain and the brainstem

Cranial nerves are the nerves that emerge directly from the brain, of which there are conventionally considered twelve pairs. Cranial nerves relay information between the brain and parts of the body, primarily to and from regions of the head and neck, including the special senses of vision, taste, smell, and hearing.

<span class="mw-page-title-main">Vagus nerve</span> Cranial nerve X, for visceral innervation

The vagus nerve, also known as the tenth cranial nerve, cranial nerve X, or simply CN X, is a cranial nerve that carries sensory fibers that create a pathway that interfaces with the parasympathetic control of the heart, lungs, and digestive tract. It comprises two nerves—the left and right vagus nerves—but they are typically referred to collectively as a single subsystem. The vagus is the longest nerve of the autonomic nervous system in the human body and comprises both sensory and motor fibers. The sensory fibers originate from neurons of the nodose ganglion, whereas the motor fibers come from neurons of the dorsal motor nucleus of the vagus and the nucleus ambiguus. The vagus was also historically called the pneumogastric nerve.

<span class="mw-page-title-main">Medulla oblongata</span> Structure of the brain stem

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 (involuntary) functions, ranging from vomiting to sneezing. The medulla contains the cardiac, respiratory, vomiting and vasomotor centers, and therefore deals with the autonomic functions of breathing, heart rate and blood pressure as well as the sleep–wake cycle.

<span class="mw-page-title-main">Brainstem</span> Posterior part of the brain, adjoining and structurally continuous

The brainstem is the stalk-like part of the brain that interconnects the cerebrum and diencephalon with the spinal cord. In the human brain the brainstem is composed of the midbrain, the pons, and the medulla oblongata. The midbrain is continuous with the thalamus of the diencephalon through the tentorial notch.

<span class="mw-page-title-main">Glossopharyngeal nerve</span> Cranial nerve IX, for the tongue and pharynx

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 to the vagus nerve. Being a mixed nerve (sensorimotor), it carries afferent sensory and efferent motor information. The motor division of the glossopharyngeal nerve is derived from the basal plate of the embryonic medulla oblongata, whereas the sensory division originates from the cranial neural crest.

<span class="mw-page-title-main">Hypoglossal nerve</span> Cranial nerve XII, for the tongue

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 by the vagus nerve. CN XII is a nerve with a solely motor function. The nerve arises from the hypoglossal nucleus in the medulla as a number of small rootlets, passes through the hypoglossal canal and down through the neck, and eventually passes up again over the tongue muscles it supplies into the tongue.

<span class="mw-page-title-main">Pyramidal tracts</span> Include both the corticobulbar tract and the corticospinal tract

The pyramidal tracts include both the corticobulbar tract and the corticospinal tract. These are aggregations of efferent nerve fibers from the upper motor neurons that travel from the cerebral cortex and terminate either in the brainstem (corticobulbar) or spinal cord (corticospinal) and are involved in the control of motor functions of the body.

<span class="mw-page-title-main">Solitary nucleus</span> Sensory nuclei in medulla oblongata

The solitary nucleus is a series of sensory nuclei forming a vertical column of grey matter in the medulla oblongata of the brainstem. It receives general visceral and/or special visceral inputs from the facial nerve, glossopharyngeal nerve and vagus nerve ; it receives and relays stimuli related to taste and visceral sensation. It sends outputs to various parts of the brain. Neuron cell bodies of the SN are roughly somatotopically arranged along its length according to function.

<span class="mw-page-title-main">Nucleus ambiguus</span>

The nucleus ambiguus is a group of large motor neurons, situated deep in the medullary reticular formation named by Jacob Clarke. The nucleus ambiguus contains the cell bodies of neurons that innervate the muscles of the soft palate, pharynx, and larynx which are associated with speech and swallowing. As well as motor neurons, the nucleus ambiguus contains preganglionic parasympathetic neurons which innervate postganglionic parasympathetic neurons in the heart.

<span class="mw-page-title-main">Medial medullary syndrome</span> Medical condition

Medial medullary syndrome, also known as inferior alternating syndrome, hypoglossal alternating hemiplegia, lower alternating hemiplegia, or Dejerine syndrome, is a type of alternating hemiplegia characterized by a set of clinical features resulting from occlusion of the anterior spinal artery. This results in the infarction of medial part of the medulla oblongata.

<span class="mw-page-title-main">Posterior cranial fossa</span> Area of the cranium containing the brainstem and cerebellum

The posterior cranial fossa is the part of the cranial cavity located between the foramen magnum, and tentorium cerebelli. It is formed by the sphenoid bones, temporal bones, and occipital bone. It lodges the cerebellum, and parts of the brainstem.

<span class="mw-page-title-main">Fazio–Londe disease</span> Medical condition

Fazio–Londe disease (FLD), also called progressive bulbar palsy of childhood, is a very rare inherited motor neuron disease of children and young adults and is characterized by progressive paralysis of muscles innervated by cranial nerves.

Progressive bulbar palsy (PBP) is a medical condition. It belongs to a group of disorders known as motor neuron diseases. PBP is a disease that attacks the nerves supplying the bulbar muscles. These disorders are characterized by the degeneration of motor neurons in the cerebral cortex, spinal cord, brain stem, and pyramidal tracts. This specifically involves the glossopharyngeal nerve (IX), vagus nerve (X), and hypoglossal nerve (XII).

Pseudobulbar palsy is a medical condition characterized by the inability to control facial movements and caused by a variety of neurological disorders. Patients experience difficulty chewing and swallowing, have increased reflexes and spasticity in tongue and the bulbar region, and demonstrate slurred speech, sometimes also demonstrating uncontrolled emotional outbursts.

<span class="mw-page-title-main">Foix–Chavany–Marie syndrome</span> Medical condition

Foix–Chavany–Marie Syndrome (FCMS), also known as bilateral opercular syndrome, is a neuropathological disorder characterized by paralysis of the facial, tongue, pharynx, and masticatory muscles of the mouth that aid in chewing. The disorder is primarily caused by thrombotic and embolic strokes, which cause a deficiency of oxygen in the brain. As a result, bilateral lesions may form in the junctions between the frontal lobe and temporal lobe, the parietal lobe and cortical lobe, or the subcortical region of the brain. FCMS may also arise from defects existing at birth that may be inherited or nonhereditary. Symptoms of FCMS can be present in a person of any age and it is diagnosed using automatic-voluntary dissociation assessment, psycholinguistic testing, neuropsychological testing, and brain scanning. Treatment for FCMS depends on the onset, as well as on the severity of symptoms, and it involves a multidisciplinary approach.

<span class="mw-page-title-main">Dorsal nucleus of vagus nerve</span>

The dorsal nucleus of vagus nerve is a cranial nerve nucleus of the vagus nerve situated in the medulla oblongata of the brainstem ventral to the floor of the fourth ventricle. It contains nerve cell bodies of parasympathetic neurons of CN X that provide parasympathetic innervation to the gastrointestinal tract and lungs as well as other thoracic and abdominal organs. These functions include, among others, bronchoconstriction and gland secretion.

<span class="mw-page-title-main">Medullary pyramids (brainstem)</span> White matter structures within the brainstems medulla oblongata

In neuroanatomy, the medullary pyramids are paired white matter structures of the brainstem's medulla oblongata that contain motor fibers of the corticospinal and corticobulbar tracts – known together as the pyramidal tracts. The lower limit of the pyramids is marked when the fibers cross (decussate).

<span class="mw-page-title-main">Braak staging</span> Classification of disease severity

Braak staging refers to two methods used to classify the degree of pathology in Parkinson's disease and Alzheimer's disease. These methods are used both in research and for the clinical diagnosis of these diseases and are obtained by performing an autopsy of the brain.

Alternating hemiplegia is a form of hemiplegia that has an ipsilateral cranial nerve palsies and contralateral hemiplegia or hemiparesis of extremities of the body. The disorder is characterized by recurrent episodes of paralysis on one side of the body. There are multiple forms of alternating hemiplegia, Weber's syndrome, middle alternating hemiplegia, and inferior alternating hemiplegia. This type of syndrome can result from a unilateral lesion in the brainstem affecting both upper motor neurons and lower motor neurons. The muscles that would receive signals from these damaged upper motor neurons result in spastic paralysis. With a lesion in the brainstem, this affects the majority of limb and trunk muscles on the contralateral side due to the upper motor neurons decussation after the brainstem. The cranial nerves and cranial nerve nuclei are also located in the brainstem making them susceptible to damage from a brainstem lesion. Cranial nerves III (Oculomotor), VI (Abducens), and XII (Hypoglossal) are most often associated with this syndrome given their close proximity with the pyramidal tract, the location which upper motor neurons are in on their way to the spinal cord. Damages to these structures produce the ipsilateral presentation of paralysis or palsy due to the lack of cranial nerve decussation before innervating their target muscles. The paralysis may be brief or it may last for several days, many times the episodes will resolve after sleep. Some common symptoms of alternating hemiplegia are mental impairment, gait and balance difficulties, excessive sweating and changes in body temperature.

References

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  3. Jones, Bronwyn (2008). "19 - Abnormalities of Pharyngeal Function". Textbook of Gastrointestinal Radiology (3rd ed.). Saunders. pp. 253–269. doi:10.1016/B978-1-4160-2332-6.50024-5. ISBN   978-1-4160-2332-6.
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  5. "Bulbar Palsy". Palsy. Archived from the original on 2011-04-27.