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Tarlov cysts are likely highly underdiagnosed as it was Isadore Tarlov's later research that led him to the understanding of their symptomology. Symptoms are based on the locations of the cysts along the spine, and follow general pathology of spinal injury:
- Pain
- Paresthesia
- Spasticity, Hypertonia
- Muscular Dysfunction or Weakness
- Radiculopathy
Although they are most frequently reported along sacral regions, they are rarely seen in other locations along the spine. Women are more likely to exhibit symptoms They can also appear in clusters or bilaterally along the spine, thus symptoms can be unilateral, bilateral, or with symptoms more dominant on one side. The cases of reported symptomatic Tarlov cysts ranges from 15% to 30% of the overall reported Tarlov cyst case, depending on the source of literature. Nevertheless, these cysts are important clinical entities because of their tendency to increase in size over time, potentially causing complications and eroding the surrounding bone tissue. Patients with symptomatic Tarlov cysts near the sacrum (and not other locations of the spine) can be divided into 4 categories, according to their experienced symptoms:
- Group 1 - Pain on tailbones that radiates to the legs with potential weakness;
- Group 2 - Pain on bones, legs, groin area, sexual dysfunctions, and dysfunctional bladder;
- Group 3 - Pain that radiate from the cyst site across hips to the lower abdomen;
- Group 4 - No pain, just sexual dysfunction and dysfunctional bladder.
Below are a list of commonly reported symptoms associated with sacral Tarlov cysts:
Back pain, perineal pain, secondary Sciatica, secondary piriformis muscle dysfunction with tertiary sciatica, Cauda equina syndrome, neurogenic claudication (pain caused by walking), neurogenic bladder, dysuria, urinary incontinence, coccygodynia, sacral radiculopathy, radicular pain, headaches, retrograde ejaculation, paresthesia, hypesthesia, secondary pelvic floor dysfunction, vaginismus, motor disorders in lower limbs and the genital, perineal, or lumbosacral areas, sacral or buttocks pain, vaginal or penile paraesthesia, Persistent Genital Arousal Disorder (PGAD) characterized by unwanted, unrelenting genital sensory awareness, itch or pain that can persist for days, months, even years), sensory changes over buttocks, perineal area, and lower extremity; difficulty walking; severe lower abdominal pain, bowel dysfunction, intestinal motility disorders like constipation or bowel incontinence.
Patients with arachnoid cysts may never show symptoms, even in some cases where the cyst is large. Therefore, while the presence of symptoms may provoke further clinical investigation, symptoms independent of further data cannot—and should not—be interpreted as evidence of a cyst's existence, size, location, or potential functional impact on the patient.
Symptoms vary by the size and location of the cyst(s), though small cysts usually have no symptoms and are discovered only incidentally. On the other hand, a number of symptoms may result from large cysts:
- Cranial deformation or macrocephaly (enlargement of the head), particularly in children
- Cysts in the suprasellar region in children have presented as bobbing and nodding of the head called bobble-head doll syndrome.
- Cysts in the left middle cranial fossa have been associated with ADHD in a study on affected children.
- Headaches. A patient experiencing a headache does not necessarily have an arachnoid cyst.
- In a 2002 study involving 78 patients with a migraine or tension-type headache, CT scans showed abnormalities in over a third of the patients, though arachnoid cysts only accounted for 2.6% of patients in this study.
- A study found 18% of patients with intracranial arachnoid cysts had non-specific headaches. The cyst was in the temporal location in 75% of these cases.
- Seizures
- Hydrocephalus (excessive accumulation of cerebrospinal fluid)
- Increased intracranial pressure
- Developmental delay
- Behavioral changes
- Nausea
- Hemiparesis (weakness or paralysis on one side of the body)
- Ataxia (lack of muscle control)
- Musical hallucination
- Pre-senile dementia, a condition often associated with Alzheimer's disease
- In elderly patients (>80 years old) symptoms were similar to chronic subdural hematoma or normal pressure hydrocephalus:
- Dementia
- Urinary incontinence
- Hemiparesis
- Headache
- Seizures
Arachnoid cysts can be found on the brain or on the spine. Intracranial arachnoid cysts usually occur adjacent to the arachnoidal cistern. Spinal arachnoid cysts may be extradural, intradural, or perineural and tend to present with signs and symptoms indicative of a radiculopathy.
Arachnoid cysts may also be classified as primary (congenital) or secondary (acquired) and have been reported in humans, cats, and dogs.
Arachnoid cysts can be relatively or present with symptoms; for this reason, diagnosis is often delayed.
Symptoms are assessed on a case by case basis. Some cysts in the CNS can be asymptomatic (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
- Hydrocephalus
- Brainstem hemorrhage
- Seizures
- Visual disturbances and hearing Loss
- Headache
- Difficulty with balance or walking
In general, symptoms vary depending on the type of cyst and its location within the CNS.
This category of cysts takes over areas of necrotic 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, 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 cysts (Leptomeningeal cysts)
- Ependymal cysts
- Cystic cerebellar astrocytomas
- Colloid cysts
Pain is the most common symptom at presentation. The symptoms seen are due to spinal nerve compression and weakening of the vertebral structure. Incontinence and decreased sensitivity in the "saddle area" (buttocks) are generally considered warning signs of spinal cord compression by the tumor. Other symptoms of spinal cord compression include lower extremity weakness, sensory loss, numbness in hands and legs and rapid onset paralysis. The diagnosis of primary spinal cord tumors is very difficult, mainly due to its symptoms, which tend to be wrongly attributed to more common and benign degenerative spinal diseases.
Spinal cord compression is commonly found in patients with metastatic malignancy. Back pain is a primary symptom of spinal cord compression in patients with known malignancy. It may prompt a bone scan to confirm or exclude spinal metastasis. Rapid identification and intervention of malignant spinal tumors, often causing spinal cord compression, is key to maintaining quality of life in patients.
The diagnosis of primary spinal cord tumors is difficult, mainly due to their symptoms, which in early stages mimic more common and benign degenerative spinal diseases. MRI and bone scanning are used for diagnostic purposes. This assesses not only the location of the tumor(s) but also their relationship with the spinal cord and the risk of cord compression.
A small cyst that requires magnification to be seen, may be called a microcyst. Similarly, a cyst that is larger than usual or compared to others, may be called a macrocyst.
Syringomyelia is a generic term referring to a disorder in which a cyst or cavity forms within the spinal cord. This cyst, called a syrinx, can expand and elongate over time, destroying the spinal cord. The damage may result in loss of pain, paralysis, weakness, and stiffness in the back, shoulders, and extremities. Syringomyelia may also cause a loss of the ability to feel extremes of hot or cold, especially in the hands. It may also lead to a cape-like bilateral loss of pain and temperature sensation along the upper chest and arms. Each patient experiences a different combination of symptoms. These symptoms typically vary depending on the extent and, often more critically, to the location of the syrinx within the spinal cord.
Syringomyelia has a prevalence estimated at 8.4 cases per 100,000 people, with symptoms usually beginning in young adulthood. Signs of the disorder tend to develop slowly, although sudden onset may occur with coughing, straining, or myelopathy.
The aneurysmal bone cyst is a neoplastic cyst, more specifically, an aggressive lesion with radiographic cystic appearance.
Syringomyelia causes a wide variety of neuropathic symptoms due to damage of the spinal cord and the nerves inside. Patients may experience severe chronic pain, abnormal sensations and loss of sensation particularly in the hands. Some patients experience paralysis or paresis temporarily or permanently. A syrinx may also cause disruptions in the parasympathetic and sympathetic nervous systems, leading to abnormal body temperature or sweating, bowel control issues, or other problems. If the syrinx is higher up in the spinal cord or affecting the brainstem as in syringobulbia, vocal cord paralysis, ipsilateral tongue wasting, trigeminal nerve sensory loss, and other signs may occur. Rarely, bladder stones can occur in the onset of weakness in the lower extremities.
Classically, syringomyelia spares the dorsal column/medial lemniscus of the spinal cord, leaving pressure, vibration, touch and proprioception intact in the upper extremities. Neuropathic arthropathy, also known as a Charcot joint, can occur, particularly in the shoulders, in patients with syringomyelia. The loss of sensory fibers to the joint is theorized to lead to damage of the joint over time.
Dermoid cysts can appear in young children, often near the lateral aspect of the eyebrow (right part of the right eyebrow or left part of the left eyebrow). Depending on the perceived amount of risk, these are sometimes excised or simply kept under observation.
An inflammatory reaction can occur if a dermoid cyst is disrupted, and the cyst can recur if it is not completely excised. Sometimes complete excision is not practical if the cyst is in a dumbbell configuration, whereby it extends through a suture line in the skull.
If dermoid cysts appear on the medial aspect, the possibility of an encephalocele becomes greater and should be considered among the differential diagnoses.
Dermoid cysts develop during pregnancy. They occur when skin cells and things like hair, sweat glands, oil glands or fatty tissue get trapped in the skin as a baby grows in the womb.Dermoid cysts are present at birth (congenital) and are common. It can be months or years before a dermoid cyst is noticed on a child because the cysts grow slowly.
Dermoid cyst symptoms are minor and the cysts are usually painless. They are not harmful to a child’s health. If they become infected, the infection must be treated and the cyst should be removed. It is easier to remove cysts and prevent scars if the cyst is removed before it gets infected.
The signs and symptoms of diastematomyelia may appear at any time of life, although the diagnosis is usually made in childhood. Cutaneous lesions (or stigmata), such as a hairy patch, dimple, Hemangioma, subcutaneous mass, Lipoma or Teratoma override the affected area of the spine is found in more than half of cases. Neurological symptoms are nonspecific, indistinguishable from other causes of cord tethering. The symptoms are caused by tissue attachments that limit the movement of the spinal cord within the spinal column. These attachments cause an abnormal stretching of the spinal cord.
The course of the disorder is progressive. In children, symptoms may include the "stigmata" mentioned above and/or foot and spinal deformities; weakness in the legs; low back pain; scoliosis; and incontinence. In adulthood, the signs and symptoms often include progressive sensory and motor problems and loss of bowel and bladder control. This delayed presentation of symptoms is related to the degree of strain placed on the spinal cord over time.
Tethered spinal cord syndrome appears to be the result of improper growth of the neural tube during fetal development, and is closely linked to spina bifida.
Tethering may also develop after spinal cord injury and scar tissue can block the flow of fluids around the spinal cord. Fluid pressure may cause cysts to form in the spinal cord, a condition called syringomyelia. This can lead to additional loss of movement, feeling or the onset of pain or autonomic symptoms.
Cervical diastematomyelia can become symptomatic as a result of acute trauma, and can cause major neurological deficits, like hemiparesis, to result from otherwise mild trauma.
The following definitions may help to understand some of the related entities:
- Diastematomyelia (di·a·stem·a·to·my·elia) is a congenital anomaly, often associated with spina bifida, in which the spinal cord is split into halves by a bony spicule or fibrous band, each half being surrounded by a dural sac.
- Myeloschisis (my·elos·chi·sis) is a developmental anomaly characterized by a cleft spinal cord, owing to failure of the neural plate to form a complete neural tube or to rupture of the neural tube after closure.
- Diplomyelia (diplo.my.elia) is a true duplication of spinal cord in which these are two dural sacs with two pairs of anterior and posterior nerve roots.
Due to its classification, a dermoid cyst can occur wherever a teratoma can occur.
Adult presentation in diastematomyelia is unusual. With modern imaging techniques, various types of spinal dysraphism are being diagnosed in adults with increasing frequency. The commonest location of the lesion is at first to third lumbar vertebrae. Lumbosacral adult diastematomyelia is even rarer. Bony malformations and dysplasias are generally recognized on plain x-rays. MRI scanning is often the first choice of screening and diagnosis. MRI generally give adequate analysis of the spinal cord deformities although it has some limitations in giving detailed bone anatomy. Combined myelographic and post-myelographic CT scan is the most effective diagnostic tool in demonstrating the detailed bone, intradural and extradural pathological anatomy of the affected and adjacent spinal canal levels and of the bony spur.
Prenatal ultrasound diagnosis of this anomaly is usually possible in the early to mid third-trimester. An extra posterior echogenic focus between the fetal spinal laminae is seen with splaying of the posterior elements, thus allowing for early surgical intervention and have a favorable prognosis. Prenate ultrasound could also detect whether the diastematomyelia is isolated, with the skin intact or association with any serious neural tube defects. Progressive neurological lesions may result from the "tethering cord syndrome" (fixation of the spinal cord) by the diastematomyelia phenomenon or any of the associated disorders such as myelodysplasia, dysraphia of the spinal cord.
Thyroglossal duct cysts most often present with a palpable asymptomatic midline neck mass above the level of the hyoid bone. The mass on the neck moves during swallowing or on protrusion of the tongue because of its attachment to the tongue via the tract of thyroid descent. Some patients will have neck or throat pain, or dysphagia.
The persistent duct or sinus can promote oral secretions, which may cause cysts to become infected. Up to half of thyroglossal cysts are not diagnosed until adult life. The tract can lie dormant for years or even decades, until some kind of stimulus leads to cystic dilation. Infection can sometimes cause the transient appearance of a mass or enlargement of the cyst, at times with periodic recurrences. Spontaneous drainage may also occur. Differential diagnosis are ectopic thyroid, enlarged lymph nodes, dermoid cysts and goiter.
Thyroglossal cyst usually presents as a midline neck lump (in the region of the hyoid bone) that is usually painless, smooth and cystic, though if infected, pain can occur. There may be difficulty breathing, dysphagia (difficulty swallowing), or dyspepsia (discomfort in the upper abdomen), especially if the cyst becomes large.
The most common location for a thyroglossal cyst is midline or slightly off midline, between the isthmus of the thyroid and the hyoid bone or just above the hyoid bone. A thyroglossal cyst can develop anywhere along a thyroglossal duct, though cysts within the tongue or in the floor of the mouth are rare.A thyroglossal cyst will move upwards with protrusion of the tongue.Thyroglossal cysts are associated with an increased incidence of ectopic thyroid tissue. Occasionally, a lingual thyroid can be seen as a flattened strawberry-like lump at the base of the tongue.
In children, symptoms may include:
- Lesions, hairy patches, dimples, or fatty tumours on the lower back
- Foot and spinal deformities
- Weakness in the legs (loss of muscle strength and tone)
- Change in or abnormal gait including awkwardness while running or wearing the tips or side of one shoe
- Low back pain
- Scoliosis (abnormal curvature of the spine to the left or right)
- Urinary irregularities (incontinence or retention)
Tethered spinal cord syndrome may go undiagnosed until adulthood, when sensory, motor, bowel, and bladder control issues emerge. This delayed presentation of symptoms relates to the degree of strain on the spinal cord over time.
Tethering may also develop after spinal cord injury. Scar tissue can block the flow of fluids around the spinal cord. Fluid pressure may cause cysts to form in the spinal cord, a condition called syringomyelia. This can lead to additional loss of movement or feeling, or the onset of pain or autonomic nervous system symptoms.
In adults, onset of symptoms typically include:
- Severe pain (in the lower back and radiating into the legs, groin, and perineum)
- Bilateral muscle weakness and numbness
- Loss of feeling and movement in lower extremities
- Urinary irregularities (incontinence or retention)
- Bowel control issues
Neurological symptoms can include a mixed picture of upper and lower motor neuron findings, such as amyotrophy, hyperreflexia, and pathologic plantar response, occurring in the same limb. Profound sensory changes, such as loss of pain, temperature, and proprioceptive sensations, are common. Last, progressive symptoms of a neuropathic bladder are noted on over 70% of adult patients, versus only 20% to 30% of children. These symptoms include urinary frequency and urgency, feeling of incomplete voiding, poor voluntary control, and urge and stress incontinence. Chronic recurrent infections are common and occasionally lead to nephrolithiasis (kidney stones), renal failure, or renal transplantation. Female patients also give a history of ineffective labor and postpartum rectal prolapse, presumably due to an atonic pelvic floor.
Symptoms suggestive of cord compression are back pain, a dermatome of increased sensation, paralysis of limbs below the level of compression, decreased sensation below the level of compression, urinary and fecal incontinence and/or urinary retention. Lhermitte's sign (intermittent shooting electrical sensation) and hyperreflexia may be present.
An infected thyroglossal duct cyst can occur when it is left untreated for a certain amount of time or simply when a thyroglossal duct cyst hasn't been suspected. The degree of infection can be examined as major rim enhancement has occurred, located inferior to the hyoid bone. Soft tissue swelling occurs, along with airway obstruction and trouble swallowing, due to the rapid enlargement of the cyst.
With infections, there can be rare cases where an expression of fluid is projected into the pharynx causing other problems within the neck.
Diagnosis is by X-rays but preferably magnetic resonance imaging (MRI) of the whole spine. The most common causes of cord compression are tumors, but abscesses and granulomas (e.g. in tuberculosis) are equally capable of producing the syndrome. Tumors that commonly cause cord compression are lung cancer (non-small cell type), breast cancer, prostate cancer, renal cell carcinoma, thyroid cancer, lymphoma and multiple myeloma.
The blockage of cerebrospinal fluid (CSF) flow may also cause a syrinx to form, eventually leading to syringomyelia. Central cord symptoms such as hand weakness, dissociated sensory loss, and, in severe cases, paralysis may occur.
Syringomyelia is a chronic progressive degenerative disorder characterized by a fluid-filled cyst located in the spinal cord. Its symptoms include pain, weakness, numbness, and stiffness in the back, shoulders, arms or legs. Other symptoms include headaches, the inability to feel changes in the temperature, sweating, sexual dysfunction, and loss of bowel and bladder control. It is usually seen in the cervical region but can extend into the medulla oblongata and pons or it can reach downward into the thoracic or lumbar segments. Syringomyelia is often associated with Chiari malformation type I and is commonly seen between the C-4 and C-6 levels. The exact development of syringomyelia is unknown but many theories suggest that the herniated tonsils in Chiari malformation type I form a "plug" which does not allow an outlet of CSF from the brain to the spinal canal. Syringomyelia is present in 25% of patients with Chiari malformation.
Symptoms vary depending on whether the spinal cord, brain stem, nerves or their blood supply is affected by the pressure.
Symptoms become apparent when the neck is bent. They include:
- Posterior head pain
- Neck weakness
- Periods of confusion
- Dysarthria (difficulty swallowing or talking due to loss of muscle control)
- Dizziness
- Loss of sensation
- Cranial nerve disturbance
- Loss of the ability to know how joints are positioned
- Lhermitte's sign ('electric shock sensation' down spine and/or to the extremities when the neck is flexed forward)
- Weakness of the arms and legs
- Orthostatic hypotension
- Patients will go into a pool and notice that below their belly button the water is not as cold as it is above.
Complications from this can include hydrocephalus, pseudotumor cerebri or syringomyelia because it blocks the flow of fluid around the brain and spinal cord.
Any of the factors below may cause the spaces in the spine to narrow.
- Spinal ligaments can thicken ("ligamenta flava")
- Bone spurs develop on the bone and into the spinal canal or foraminal openings
- Intervertebral discs may bulge or herniate into the canal or foraminal openings
- Degenerative disc disease causes narrowing of the spaces
- Facet joints break down
- Compression fractures of the spine, which are common in osteoporosis
- Cysts form on the facet joints causing compression of the spinal sac of nerves (thecal sac)