A central nervous system cyst is a type of

cyst A cyst is a closed sac, having a distinct envelope and cell division, division compared with the nearby Biological tissue, tissue. Hence, it is a cluster of Cell (biology), cells that have grouped together to form a sac (like the manner in which ...

that presents and affects part of the

central nervous system The central nervous system (CNS) is the part of the nervous system consisting primarily of the brain and spinal cord. The CNS is so named because the brain integrates the received information and coordinates and influences the activity of all par ...

(CNS). They are usually

benign Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous benign tumor, ''benign'' tumor in that a malign ...

and filled with either

cerebrospinal fluid Cerebrospinal fluid (CSF) is a clear, colorless body fluid found within the tissue that surrounds the brain and spinal cord of all vertebrates. CSF is produced by specialised ependymal cells in the choroid plexus of the ventricles of the bra ...

, blood, 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 ...

cells. CNS cysts are classified into two categories: cysts that originate from non-central nervous system tissue, migrate to, and form on a portion of the CNS, and cysts that originate within central nervous system tissue itself. Within these two categories, there are many types of CNS cysts that have been identified from previous studies.

Classification

Originating from non-central nervous system tissue

These classification of cysts are embedded in the

endoderm Endoderm is the innermost of the three primary germ layers in the very early embryo. The other two layers are the ectoderm (outside layer) and mesoderm (middle layer). Cells migrating inward along the archenteron form the inner layer of the gast ...

(inner layer) and the

ectoderm The ectoderm is one of the three primary germ layers formed in early embryonic development. It is the outermost layer, and is superficial to the mesoderm (the middle layer) and endoderm (the innermost layer). It emerges and originates from t ...

(outer layer) of the cranial or spinal cord

germ layers A germ layer is a primary layer of cells that forms during embryonic development. The three germ layers in vertebrates are particularly pronounced; however, all eumetazoans (animals that are sister taxa to the sponges) produce two or three pri ...

. They normally take over the

neuraxis The neuraxis or sometimes neuroaxis is the axis of the central nervous system. It denotes the direction in which the central nervous system lies. During embryological development, the neuraxis is bent by various flexures, contributing to the ma ...

, the axis of the central nervous system that determines how the nervous system is placed, which allows the cysts to infiltrate the CNS tissues. They are most commonly found in the area near the

pineal gland The pineal gland, conarium, or epiphysis cerebri, is a small endocrine gland in the brain of most vertebrates. The pineal gland produces melatonin, a serotonin-derived hormone which modulates sleep, sleep patterns in both circadian rhythm, circ ...

, the

chiasmatic cistern The chiasmatic cistern (cistern of chiasma, or suprasellar cistern) is formed as the interpeduncular cistern extends forward across the optic chiasm and onto the upper surface of the corpus callosum – the arachnoid stretches across from one cere ...

, and the

cerebellopontine angle The cerebellopontine angle (CPA) ( la, angulus cerebellopontinus) is located between the cerebellum and the pons. The cerebellopontine angle is the site of the cerebellopontine angle cistern one of the subarachnoid cisterns that contains cerebro ...

space. These common places generally house extensive and continuously growing cysts. Some examples of cysts originating from non-central nervous system tissue include: *

Teratomas A teratoma is a tumor made up of several different types of tissue, such as hair, muscle, teeth, or bone. Teratomata typically form in the ovary, testicle, or coccyx. Symptoms Symptoms may be minimal if the tumor is small. A testicular ter ...

cysts (containing multiple body tissue types) *

Dermoid A dermoid cyst is a teratoma of a cystic nature that contains an array of developmentally mature, solid tissues. It frequently consists of skin, hair follicles, and sweat glands, while other commonly found components include clumps of long hair, ...

(epidermoid/epidermoid tumor) *

Rathke's cleft cyst A Rathke's cleft cyst is a benign growth on the pituitary gland in the brain, specifically a mucin-filled cyst in the posterior portion of the anterior pituitary gland. It occurs when the Rathke's pouch does not develop properly and ranges in siz ...

s * Pineal cysts * Tumor-associated cysts * Epithelial cysts that originate from upper respiratory and intestinal tracks.

Originating from the central nervous system tissue

Human brain showning a colloid cyst in the third ventricle

This category of cysts takes over areas of

necrotic Necrosis () is a form of cell injury which results in the premature death of cells in living tissue by autolysis. Necrosis is caused by factors external to the cell or tissue, such as infection, or trauma which result in the unregulated dige ...

tissue in the brain from injuries, diseases, or abnormalities, which occur due to the central nervous system's nonregenerative nature. These cysts can affect all germ layers of the CNS, but are most common in the

arachnoid mater The arachnoid mater (or simply arachnoid) is one of the three meninges, the protective membranes that cover the brain and spinal cord. It is so named because of its resemblance to a spider web. The arachnoid mater is a derivative of the neural c ...

, and the ventricular space, which may block CSF pathways. These cysts can be static (stationary) or progressive. Some examples of cysts originating from the CNS tissue include: *

Arachnoid cyst Arachnoid cysts are cerebrospinal fluid covered by arachnoidal cells and collagen that may develop between the surface of the brain and the cranial base or on the arachnoid membrane, one of the three meningeal layers that cover the brain and the ...

s (Leptomeningeal cysts) *

Ependyma The ependyma is the thin Neuroepithelial cell, neuroepithelial (Simple columnar epithelium, simple columnar ciliated epithelium) lining of the ventricular system of the brain and the central canal of the spinal cord. The ependyma is one of the fou ...

l cysts * Cystic cerebellar astrocytomas *

Colloid cyst A colloid cyst is a non-malignant tumor in the brain. It consists of a gelatinous material contained within a membrane of epithelial tissue. It is almost always found just posterior to the foramen of Monro in the anterior aspect of the third ve ...

Signs and symptoms

Symptoms are assessed on a case-by-case basis.Sundaram C, Paul T R, Raju B V, Ramakrishna Murthy T, Sinha A K, Prasad V S, Purohit A K. Cysts of the central nervous system : a clinicopathologic study of 145 cases. Neurol India erial online2001 ited 2017 Apr 1149:237. Available from: http://www.neurologyindia.com/text.asp?2001/49/3/237/1247 Some cysts in the CNS can be

asymptomatic In medicine, any disease is classified asymptomatic if a patient tests as carrier for a disease or infection but experiences no symptoms. Whenever a medical condition fails to show noticeable symptoms after a diagnosis it might be considered asy ...

(producing or showing no symptoms), depending on their location in the brain or spinal cord. If the cysts develop in critical areas of the central nervous system, they can present one or more of the following symptoms: * Pressure in the spinal cord or brain * Rupture of nerves around the cyst * Weakness in specific parts of the body controlled by the cyst-infected brain region *

Inflammation Inflammation (from la, wikt:en:inflammatio#Latin, inflammatio) is part of the complex biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or Irritation, irritants, and is a protective response involving im ...

Hydrocephalus Hydrocephalus is a condition in which an accumulation of cerebrospinal fluid (CSF) occurs within the brain. This typically causes increased intracranial pressure, pressure inside the skull. Older people may have headaches, double vision, poor ...

* Brainstem

hemorrhage Bleeding, hemorrhage, haemorrhage or blood loss, is blood escaping from the circulatory system from damaged blood vessels. Bleeding can occur internally, or externally either through a natural opening such as the mouth, nose, ear, urethra, vag ...

Seizures An epileptic seizure, informally known as a seizure, is a period of symptoms due to abnormally excessive or neural oscillation, synchronous neuronal activity in the brain. Outward effects vary from uncontrolled shaking movements involving much o ...

* Visual disturbances and

hearing loss Hearing loss is a partial or total inability to Hearing, hear. Hearing loss may be present at birth or acquired at any time afterwards. Hearing loss may occur in one or both ears. In children, hearing problems can affect the ability to Language ...

* Headache * Difficulty with balance or walking In general, symptoms vary depending on the type of cyst and its location within the CNS.

Causes

Many CNS cysts form in the womb during the first few weeks of development as a result of

congenital defects A birth defect, also known as a congenital disorder, 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 r ...

. In adults cysts may also form due to a

head injury A head injury is any injury that results in trauma to the skull or brain. The terms ''traumatic brain injury'' and ''head injury'' are often used interchangeably in the medical literature. Because head injuries cover such a broad scope of inju ...

or trauma, resulting in necrotic tissues (dead tissue), and can sometimes be associated with cancerous tumors or infection in the brain. However, the underlying reasons for cyst formation are still unknown.

Diagnosis

The diagnostic process typically begins with a medical history workup followed by a medical examination by a physician. Imaging tests, such as

CT scan A computed tomography scan (CT scan; formerly called computed axial tomography scan or CAT scan) is a medical imaging technique used to obtain detailed internal images of the body. The personnel that perform CT scans are called radiographers ...

s and

MRIs Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to form pictures of the anatomy and the physiological processes of the body. MRI scanners use strong magnetic fields, magnetic field gradients, and radio wa ...

, help provide a clearer picture. The physician typically looks for fluid (or other bodily substance) filled sacs to appear in the scans, as is shown in the CT scan of a colloid cyst. A primary health care provider will refer an individual to a

neurologist Neurology (from el, νεῦρον (neûron), "string, nerve" and the suffix -logia, "study of") is the branch of medicine dealing with the diagnosis and treatment of all categories of conditions and disease involving the brain, the spinal c ...

neurosurgeon Neurosurgery or neurological surgery, known in common parlance as brain surgery, is the medical specialty concerned with the surgical treatment of disorders which affect any portion of the nervous system including the brain, spinal cord and peri ...

for further examination. Other diagnostic methods include

radiological In physics, radiation is the emission or transmission of energy in the form of waves or particles through space or through a material medium. This includes: * ''electromagnetic radiation'', such as radio waves, microwaves, infrared, visib ...

examinations and macroscopic examinations. After a diagnosis has been made,

immunohistochemistry Immunohistochemistry (IHC) is the most common application of immunostaining. It involves the process of selectively identifying antigens (proteins) in cells of a tissue section by exploiting the principle of antibodies binding specifically to an ...

may be used to differentiate between epithelial cysts and

arachnoid cyst Arachnoid cysts are cerebrospinal fluid covered by arachnoidal cells and collagen that may develop between the surface of the brain and the cranial base or on the arachnoid membrane, one of the three meningeal layers that cover the brain and the ...

s. These examinations are useful to get a general idea of possible treatment options, but can be unsatisfactory to diagnose CNS cysts. Professionals still do not fully understand how cysts form; however, analyzing the walls of different cyst types, using

electron microscope An electron microscope is a microscope that uses a beam of accelerated electrons as a source of illumination. As the wavelength of an electron can be up to 100,000 times shorter than that of visible light photons, electron microscopes have a hi ...

s and

light microscope The optical microscope, also referred to as a light microscope, is a type of microscope that commonly uses visible light and a system of lenses to generate magnified images of small objects. Optical microscopes are the oldest design of microsco ...

s, has proven to be the best diagnostic tool. This has led to more accurate cyst classification and correct course of action for treatments that are cyst specific. In the past, before imaging scans or tests were available, medical professionals could only diagnose cysts via

exploratory surgery Exploratory surgery is a diagnostic method used by doctors when trying to find a diagnosis for an ailment. With the invention of modern imaging techniques, exploratory surgery is becoming less common in humans. Due to the high cost and lower avail ...

Treatment

Treatment is often largely dependent on the type of cyst.

Asymptomatic In medicine, any disease is classified asymptomatic if a patient tests as carrier for a disease or infection but experiences no symptoms. Whenever a medical condition fails to show noticeable symptoms after a diagnosis it might be considered asy ...

cysts, termed pseudocysts, normally require active monitoring with periodic scans for future growth. Symptomatic (producing or showing symptoms) cysts may require surgical removal if they are present in areas where brain damage is unavoidable, or if they produce chronic symptoms disruptive to the quality of life of the patient. Some examples of cyst removal procedures include: permanent drainage, fenestration, and endoscopic cyst fenestration.

Permanent drainage

A neurosurgeon may open a portion of the body and insert a shunt into cerebral spinal fluid (CSF) filled cysts to allow drainage into CSF pathways. The fluid from the cyst is then drained into the abdomen, the body reabsorbs the fluid (reabsorption of fluid does not cause any harm). This type of surgical treatment is often performed to relieve pressure on the brain from a cyst within the cerebral cortex.

Fenestration

A neurosurgeon performs a

craniotomy A craniotomy is a surgical operation in which a bone flap is temporarily removed from the skull to access the brain. Craniotomies are often critical operations, performed on patients who are suffering from brain lesions, such as tumors, blood clots ...

as a means of entry to access the cyst. The cyst is then opened to release its contents, which are reabsorbed by the brain. This is commonly used with inflammatory cysts located in the ventricles, and can result in increased ventricular fluid flow within the brain.

Endoscopic cyst fenestration

A neurosurgeon performs a same day surgery to insert an

endoscope An endoscope is an inspection instrument composed of image sensor, optical lens, light source and mechanical device, which is used to look deep into the body by way of openings such as the mouth or anus. A typical endoscope applies several modern t ...

, which drains the cyst internally.

Epidemiology

Cysts derived from CNS tissues are very common in America. They are a subtype of cerebrovascular diseases, which are the third leading cause of death in America. Generally, CNS cysts are present in all geographic regions, races, ages, and sexes. However, certain types of CNS cysts are more prevalent in certain types of individuals than others. Some examples of incidence rates in specific types of cysts include: *

s are more prevalent in males than females *

s are more prevalent in adults *

Dermoid cyst A dermoid cyst is a teratoma of a cystic nature that contains an array of developmentally mature, solid tissues. It frequently consists of skin, hair follicles, and sweat glands, while other commonly found components include clumps of long hair, ...

s are more prevalent in children under 10 years of age *

Epidermoid cyst An epidermoid cyst or epidermal inclusion cyst is a benign cyst usually found on the skin. The cyst develops out of ectodermal tissue. Histologically, it is made of a thin layer of squamous epithelium. Signs and symptoms The epidermoid cyst may ...

s are more prevalent in middle-aged adults

References

External links

{{Congenital malformations and deformations of nervous system Congenital disorders of nervous system