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.

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.

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.

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.

Tethered spinal cord can be caused by various conditions but the main cause is when tissue attachments limit the movement of the spinal cord in the spinal column which causes abnormal stretching of the cord. The tethered spinal cord syndrome is correlated with having the causes:

- Spina bifida

- Occulta

- Mylomeningocele

- Meningocele

- History of spinal trauma

- History of spinal surgery

- Tumor(s) in the spinal column

- Thickened and/or tight filum terminale

- Lipoma(s) in the spinal column

- Dermal Sinus Tract (congenital deformity)

- Diastematomyelia (split spinal cord)

Tethered spinal cord is a disorder and not a mechanism so it does not spread to other people and there are no measures that can be done to prevent it beforehand. The only preventative measure that is successful is to surgically untether the spinal cord though there might already be irreversible damage.

Diastematomyelia (occasionally diastomyelia) is a congenital disorder in which a part of the spinal cord is split, usually at the level of the upper lumbar vertebra.

Diastematomyelia is a rare congenital anomaly that results in the "splitting" of the spinal cord in a longitudinal (sagittal) direction. Females are affected much more commonly than males. This condition occurs in the presence of an osseous (bone), cartilaginous or fibrous septum in the central portion of the spinal canal which then produces a complete or incomplete sagittal division of the spinal cord into two hemicords. When the split does not reunite distally to the spur, the condition is referred to as a diplomyelia, or true duplication of the spinal cord.

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.

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.

Arachnoid inflammation can lead to many painful and debilitating symptoms which can vary greatly in each case, and not all people experience all symptoms. Chronic pain is common, including neuralgia, while numbness and tingling of the extremities can occur with spinal cord involvement, and bowel, bladder, and sexual functioning can be affected if the lower part of the spinal cord is involved. While arachnoiditis has no consistent pattern of symptoms, it frequently affects the nerves that supply the legs and lower back. Many patients experience difficulty sitting for long (or even short) periods of time due to discomfort or pain, or because of efferent neurological or other motor symptoms, such as difficulties controlling limbs. Difficulty sitting can be problematic for patients who have trouble standing or walking for long periods, as wheelchairs are not always helpful in such cases.

The most obvious clinical sign of syringomyelia is pain. Dogs with CM alone do not seem to have signs, but some appear to have facial pain. Common symptoms in human patients include, severe headache and neck pain, dizziness, vertigo, disequilibrium, visual disturbances, ringing in the ears, difficulty swallowing, palpitations, sleep apnea, muscle weakness, impaired fine motor skills, chronic fatigue and painful tingling of the hands and feet, pruritus.

The following are clear signs of Kyphoscoliosis:

- Abnormal hunch along with a presence of S or C-like shape.

- Uneven lengths of arms and legs

- Presence of associated disorders likes hypertension, neurological disorders

- Abnormal gait

Scoliosis refers to yet another form of abnormal curvature in which the person’s spine takes an “S” or “C” shape. Scoliosis too has similar forms of treatments available as Kyphosis including bracing, physical therapy and various types of surgeries. Typically, a human spine is straight but in Scoliosis patients; there may be a curve of ten degrees in either direction, left or right.

Arachnoiditis is an inflammatory condition of the arachnoid mater or 'arachnoid', one of the membranes known as meninges that surround and protect the nerves of the central nervous system, including the brain and spinal cord. The arachnoid can become inflamed because of adverse reactions to chemicals, infection from bacteria or viruses, as the result of direct injury to the spine, chronic compression of spinal nerves, complications from spinal surgery or other invasive spinal procedures, or the accidental intrathecal injection of steroids intended for the epidural space. Inflammation can sometimes lead to the formation of scar tissue and adhesion that can make the spinal nerves "stick" together, a condition where such tissue develops in and between the leptomeninges. The condition is extremely painful, especially when progressing to adhesive arachnoiditis. Another form of the condition is arachnoiditis ossificans, in which the arachnoid becomes ossified, or turns to bone, and is thought to be a late-stage complication of the adhesive form of arachnoiditis.

Chiari-like malformation (CM) is the most common cause of foramen magnum obstruction and syringomyelia in dogs. Syringomyelia (SM) is a disease of the spinal cord typified by fluid filled cavities, or syrinxes, within the spinal cord substance. The disease is caused by the obstruction of cerebrospinal fluid (CSF), in the nervous system. A situation of high pressure in the spinal cord compared to low pressure outside, leads to fluid accumulation, which eventually forms cavities. CM is a condition characterized by the mismatch of size between the brain and the skull. The skull is too small causing part of the brain to descend out of the skull through the opening at its base, crowding the spinal cord. The cause of CM is not yet fully understood. CM is rare in most breeds but reportedly has become very widespread in the Cavalier King Charles Spaniel and the Griffon Bruxellois (Brussels Griffon). As many as 95% of Cavalier King Charles Spaniels may have CM. It is worldwide in scope and not limited to any country, breeding line, or kennel, and experts report that it is believed to be inherited in the Cavalier King Charles Spaniel. CM is so widespread in the Cavalier that it may be an inherent part of the CKCS's breed standard. This disease not only affects thousands of dogs, but a similar condition affects over three hundred thousand children yearly. Therefore, canines are an appropriate model for the treatment of the human condition.

Syringobulbia is a medical condition in which syrinxes, or fluid-filled cavities, affect the brainstem. This defect normally results from congenital abnormality, trauma or tumor growth.

It mostly occurs within the lower brainstem as a slit-like gap. This may affect one or more cranial nerves, 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, a syrinx limited to the spinal cord.

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.

Symptoms associated with scoliosis can include:

- Pain in back, shoulders, and neck and buttock pain nearest bottom of the back

- Respiratory and/or cardiac problems in severe cases

- Constipation due to curvature causing "tightening" of stomach, intestines, etc.

- Limited mobility secondary to pain or functional limitation in adults

- Painful menstruation

The signs of scoliosis can include:

- Uneven musculature on one side of the spine

- Rib prominence or a prominent shoulder blade, caused by rotation of the rib cage in thoracic scoliosis

- Uneven hips, arms or leg lengths

- Slow nerve action

- Heart and lung problems in severe cases

- Calcium deposits in the cartilage endplate and sometimes in the disc itself

People who have reached skeletal maturity are less likely to have a worsening case. Some severe cases of scoliosis can lead to diminishing lung capacity, pressure exerted on the heart, and restricted physical activities.

Recent longitudinal studies reveal that the most common form of the condition, "late-onset idiopathic scoliosis", causes little physical impairment other than back pain and cosmetic concerns, even when untreated, with mortality rates similar to the general population. Older beliefs that untreated idiopathic scoliosis necessarily progresses into severe (cardiopulmonary) disability by old age have been refuted by later studies.

Basilar invagination is invagination (infolding) of the base of the skull that occurs when the top of the C2 vertebra migrates upward. It can cause narrowing of the foramen magnum (the opening in the skull where the spinal cord passes through to the brain). It also may press on the lower brainstem.

This is similar to Chiari malformation. That, however, is usually present at birth.

If there are symptoms, people with empty sella syndrome can have headaches, as symptoms, which subsides when lying down. Additional symptoms are as follows:

- Abnormality (middle ear ossicles)

- Cryptorchidism

- Dolichocephaly

- Arnold-Chiari type I malformation

- Meningocele

- Patent ductus arteriosus

- Muscular hypotonia

- Platybasia

Cleidocranial dysostosis is a general skeletal condition so named from the collarbone (cleido-) and cranium deformities which people with it often have.

People with the condition usually present with a painless swelling in the area of the clavicles at 2–3 years of age. Common features are:

- Clavicles (collarbones) can be partly missing leaving only the medial part of the bone. In 10% cases, they are completely missing. If the collarbones are completely missing or reduced to small vestiges, this allows hypermobility of the shoulders including ability to touch the shoulders together in front of the chest. The defect is bilateral 80% of the time. Partial collarbones may cause nerve damage symptoms and therefore have to be removed by surgery.

- The mandible is prognathic due to hypoplasia of maxilla (micrognathism) and other facial bones.

- A soft spot or larger soft area in the top of the head where the fontanelle failed to close, or the fontanelle closes late.

- Bones and joints are underdeveloped. People are shorter and their frames are smaller than their siblings who do not have the condition.

- The permanent teeth include supernumerary teeth. Unless these supernumeraries are removed they will crowd the adult teeth in what already may be an underdeveloped jaw. If so, the supernumeraries will probably need to be removed to make space for the adult teeth. Up to 13 supernumarary teeth have been observed. Teeth may also be displaced. Cementum formation may be deficient.

- Failure of eruption of permanent teeth.

- Bossing (bulging) of the forehead.

- Open skull sutures, large fontanelles.

- Hypertelorism.

- Delayed ossification of bones forming symphysis pubis, producing a widened symphysis.

- Coxa vara can occur, limiting abduction and causing Trendelenburg gait.

- Short middle fifth phalanges, sometimes causing short and wide fingers.

- Vertebral abnormalities.

- On rare occasions, brachial plexus irritation can occur.

- Scoliosis, spina bifida and syringomyelia have also been described.

Other features are: parietal bossing, basilar invagination (atlantoaxial impaction), persistent metopic suture, abnormal ear structures with hearing loss, supernumerary ribs, hemivertebrae with spondylosis, small and high scapulae, hypoplasia of illiac bones, absence of the pubic bone, short / absent fibular bones, short / absent radial bones, hypoplastic terminal phalanges.

There are two types of ESS: "primary" and "secondary".

- Primary ESS happens when a small anatomical defect above the pituitary gland increases pressure in the sella turcica and causes the gland to flatten out along the interior walls of the sella turcica cavity. Primary ESS is associated with obesity and increase in intracranial pressure in women.

- Secondary ESS is the result of the pituitary gland regressing within the cavity after an injury, surgery, or radiation therapy. Individuals with secondary ESS due to destruction of the pituitary gland have symptoms that reflect the loss of pituitary functions, such as intolerance to stress and infection.

Source:

- severe headache

- visual loss (due to papilledema)

- vomiting

- bilateral Babinski sign

- drowsiness (after several hours of the above symptoms)

- gait change (rotation of feet when walking)

- impaction/constipation

- back flexibility

The clinical presentation varies depending on the stage of the disease from mild swelling to severe swelling and moderate deformity. Inflammation, erythema, pain and increased skin temperature (3–7 degrees Celsius) around the joint may be noticeable on examination. X-rays may reveal bone resorption and degenerative changes in the joint. These findings in the presence of intact skin and loss of protective sensation are pathognomonic of acute Charcot arthropathy.

Roughly 75% of patients experience pain, but it is less than what would be expected based on the severity of the clinical and radiographic findings.

Dissociated sensory loss is a pattern of neurological damage caused by a lesion to a single tract in the spinal cord which involves "selective" loss of fine touch and proprioception "without" loss of pain and temperature, or vice versa.

Understanding the mechanisms behind these selective lesions requires a brief discussion of the anatomy involved.

Loss of pain and temperature are due to damage to the lateral spinothalamic tracts, which cross the central part of the cord close to the level where they enter it and travel up the spinal column on the opposite side to the one they innervate (i.e. they "ascend contralaterally"). Note that a lesion of the lateral spinothalamic tract at a given level will not result in sensory loss for the dermatome of the same level; this is due to the fibers of the tract of Lissauer which transmit the neuron one or two levels above the affected segment (thus bypassing the segmental lesion on the contralateral side).

Loss of fine touch and proprioception are due to damage to the dorsal columns, which do not cross the cord until the brainstem, and so travel up the column on the same side to the one they innervate (i.e. they "ascend ipsilaterally").

This means that a lesion of the dorsal columns will cause loss of touch and proprioception below the lesion and on the same side as it, while a lesion of the spinothalamic tracts will cause loss of pain and temperature below the lesion and on the opposite side to it.

Dissociated sensory loss always suggests a focal lesion within the spinal cord or brainstem.

The location of cord lesions affects presentation—for instance, a central lesion (such as that of syringomyelia) will knock out second order neurons of the spinothalamic tract as they cross the centre of the cord, and will cause loss of pain and temperature without loss of fine touch or proprioception.

Other causes of dissociated sensory loss include:

- Diabetes mellitus

- Syringomyelia

- Brown-Séquard syndrome

- Lateral medullary syndrome aka Wallenberg's syndrome

- Anterior spinal artery thrombosis

- Tangier disease

- Subacute combined degeneration

- Multiple sclerosis

- Tabes dorsalis

- Friedreich's ataxia (or other spinocerebellar degeneration)