Syringobulbia is a medical condition in which
syrinxes, or fluid-filled cavities, affect 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 conti ...
(usually the lower brainstem). The exact cause is often unknown, but may be linked to a widening of the
central canal
The central canal (also known as spinal foramen or ependymal canal) is the cerebrospinal fluid-filled space that runs through the spinal cord. The central canal lies below and is connected to the ventricular system of the brain, from which it r ...
of the
spinal cord
The spinal cord is a long, thin, tubular structure made up of nervous tissue that extends from the medulla oblongata in the lower brainstem to the lumbar region of the vertebral column (backbone) of vertebrate animals. The center of the spinal c ...
. This may affect one or more
cranial nerves
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 f ...
, resulting in various kinds of facial palsies. Sensory and motor nerve pathways may be affected by interruption or compression of nerves. This disorder is associated with
syringomyelia
Syringomyelia is a generic term referring to a disorder in which a cyst or cavity forms within the spinal cord. Often, syringomyelia is used as a generic term before an etiology is determined. This cyst, called a syrinx, can expand and elongate ...
, a syrinx limited to the spinal cord. It can be diagnosed using
magnetic resonance imaging
Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to generate pictures of the anatomy and the physiological processes inside the body. MRI scanners use strong magnetic fields, magnetic field gradients, and ...
. Symptoms may be treated with
tricyclic antidepressants
Tricyclic antidepressants (TCAs) are a class of medications that are used primarily as antidepressants. TCAs were discovered in the early 1950s and were marketed later in the decade. They are named after their chemical structure, which contains ...
.
Signs and symptoms
Syringobulbia usually causes
pain
Pain is a distressing feeling often caused by intense or damaging Stimulus (physiology), stimuli. The International Association for the Study of Pain defines pain as "an unpleasant sense, sensory and emotional experience associated with, or res ...
.
It may also cause a loss of sense of
temperature
Temperature is a physical quantity that quantitatively expresses the attribute of hotness or coldness. Temperature is measurement, measured with a thermometer. It reflects the average kinetic energy of the vibrating and colliding atoms making ...
.
Alveolar hypoventilation (insufficient breathing, a type of
central hypoventilation syndrome) may occur, with
hypercapnia
Hypercapnia (from the Greek ''hyper'', "above" or "too much" and ''kapnos'', "smoke"), also known as hypercarbia and CO2 retention, is a condition of abnormally elevated carbon dioxide (CO2) levels in the blood. Carbon dioxide is a gaseous pro ...
(excess blood CO
2),
stridor
Stridor () is an extra-thoracic high-pitched breath sound resulting from turbulent air flow in the larynx or lower in the bronchial tree. It is different from a stertor, which is a noise originating in the pharynx.
Stridor is a physical sig ...
(an unusual breathing sound), and irregular breathing.
Cause
Syringobulbia may be caused by a
birth defect
A birth defect is an abnormal condition that is present at birth, regardless of its cause. Birth defects may result in disabilities that may be physical, intellectual, or developmental. The disabilities can range from mild to severe. Birth de ...
,
trauma or
tumor
A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...
growth. The exact trigger is unknown, but may be linked to a widening of the
central canal
The central canal (also known as spinal foramen or ependymal canal) is the cerebrospinal fluid-filled space that runs through the spinal cord. The central canal lies below and is connected to the ventricular system of the brain, from which it r ...
of the
spinal cord
The spinal cord is a long, thin, tubular structure made up of nervous tissue that extends from the medulla oblongata in the lower brainstem to the lumbar region of the vertebral column (backbone) of vertebrate animals. The center of the spinal c ...
.
Mechanism
Syringobulbia affects the lower part of 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 conti ...
.
The
central canal
The central canal (also known as spinal foramen or ependymal canal) is the cerebrospinal fluid-filled space that runs through the spinal cord. The central canal lies below and is connected to the ventricular system of the brain, from which it r ...
of the
spinal cord
The spinal cord is a long, thin, tubular structure made up of nervous tissue that extends from the medulla oblongata in the lower brainstem to the lumbar region of the vertebral column (backbone) of vertebrate animals. The center of the spinal c ...
may be widened.
A fluid-filled lesion forms, known as a
syrinx
In classical Greek mythology, Syrinx () was an Arcadian nymph and a follower of Artemis, known for her chastity. Being pursued by Pan, she fled into the river Ladon, and at her own request was metamorphosed into a reed from which Pan then mad ...
.
This can vary in size significantly between patients.
Nerve fibres may be compressed where they cross the midline, or in other parts of the spinal cord.
Cranial nerves
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 f ...
may be affected.
Syringobulbia may be associated with
syringomyelia
Syringomyelia is a generic term referring to a disorder in which a cyst or cavity forms within the spinal cord. Often, syringomyelia is used as a generic term before an etiology is determined. This cyst, called a syrinx, can expand and elongate ...
, a syrinx limited to the spinal cord.
Diagnosis
Syringobulbia may be diagnosed using
magnetic resonance imaging
Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to generate pictures of the anatomy and the physiological processes inside the body. MRI scanners use strong magnetic fields, magnetic field gradients, and ...
.
Treatment
The pain caused by syringobulbia may be treated with
tricyclic antidepressants
Tricyclic antidepressants (TCAs) are a class of medications that are used primarily as antidepressants. TCAs were discovered in the early 1950s and were marketed later in the decade. They are named after their chemical structure, which contains ...
.
See also
*
Syrinx
In classical Greek mythology, Syrinx () was an Arcadian nymph and a follower of Artemis, known for her chastity. Being pursued by Pan, she fled into the river Ladon, and at her own request was metamorphosed into a reed from which Pan then mad ...
*
Syringomyelia
Syringomyelia is a generic term referring to a disorder in which a cyst or cavity forms within the spinal cord. Often, syringomyelia is used as a generic term before an etiology is determined. This cyst, called a syrinx, can expand and elongate ...
References
External links
*
{{CNS diseases of the nervous system
Gross pathology
Spinal cord disorders