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Most ganglioneuromas are noncancerous, thus expected outcome is usually good. However, a ganglioneuroma may become cancerous and spread to other areas, or it may regrow after removal.
If the tumor has been present for a long time and has pressed on the spinal cord or caused other symptoms, it may have caused irreversible damage that cannot be corrected with the surgical removal of the tumor. Compression of the spinal cord may result in paralysis, especially if the cause is not detected promptly.
Because ganglioneuromas are benign, treatment may not be necessary, as it would expose patients to more risk than leaving it alone. If there are symptoms or major physical deformity, treatment usually consists of surgery to remove the tumor.
There is no standard treatment that has been established for NM thus treatments are almost always palliative.
Radiotherapy:
This method is used mostly for focal type of NM due to the nature of damage and success rate associated with the treatment. Radiotherapy targets and tumor and destroys the collective tissues of cancerous cells.
Systemic (intravenous or oral) chemotherapy and intrathecal chemotherapy: Intrathecal therapy is when injection is done directly to the spinal cord into the sub-arachnoid space to avoid the Blood-Brain-Barrier (BBB) and gain direct access to the CSF. Intrathecal Therapy is preferred since intravenous chemotherapy do not penetrate the BBB. The most common chemicals used are liposomal cytarabine (DepoCyte) and intrathecal methotrexate (MTX).
In combination, intrathecal chemotherapy most often comprises methotrexate, cytarabine, thiotepa and steroids. Ventriculoperitoneal shunts may also be applied with chemotherapy to avoid invasive surgery to gain access to the CSF.
An example of treatment:
Intrathecal MTX injection at a dose of 15 mg/day for 5 days every other week with hydrocortisone acetate injecting IT on day one to prevent arachnoiditis, the inflammation of the arachnoid. MTX administration is continued until neurological progression or relapse occurred. Systemic chemotherapy, radiotherapy, and surgery are performed depending on the need of the patient.
Risks of treatments:
Both Chemotherapy and Radiotherapy are harmful to the body and most definitely the brain. Caution must be utilized in treating patients with NM. Another factor that makes treatment difficult is that there is no suitable method to evaluate the disease progression.
Spinal tumors are neoplasms located in the spinal cord. Extradural tumors are more common than intradural neoplasms.
Depending on their location, the spinal cord tumors can be:
- Extradural - outside the dura mater lining (most common)
- Intradural - part of the dura
- Intramedullary - inside the spinal cord
- Extramedullary- inside the dura, but outside the spinal cord
Definitive treatment for ganglioglioma requires gross total surgical resection, and a good prognosis is generally expected when this is achieved. However, indistinct tumor margins and the desire to preserve normal spinal cord tissue, motor and sensory function may preclude complete resection of tumor. According to a series by Lang et al., reviewing several patients with resected spinal cord ganglioglioma, the 5- and 10-year survival rates after total resection were 89% and 83%, respectively. In that study, patients with spinal cord ganglioglioma had a 3.5-fold higher relative risk of tumor recurrence compared to patients with supratentorial ganglioglioma. It has been recognized that postoperative results correlate closely with preoperative neurological status as well as the ability to achieve complete resection.
With the exception of WHO grade III anaplastic ganglioglioma, radiation therapy is generally regarded to have no role in the treatment of ganglioglioma. In fact, radiation therapy may induce malignant transformation of a recurrent ganglioglioma several years later. Adjuvant chemotherapy is also typically reserved for anaplastic ganglioglioma, but has been used anecdotally in partially resected low grade spinal cord gangliogliomas which show evidence of disease progression.
Ganglioglioma is a rare, slow-growing primary central nervous system (CNS) tumor which most frequently occurs in the temporal lobes of children and young adults.
Extradural tumors are mostly metastases from primary cancers elsewhere (commonly breast, prostate and lung cancer). Intradural tumours can be classified as intramedullary (within the spinal parenchyma) or extramedullary (within the dura, but outside the spinal parenchyma). Extramedullary tumours are more common than intramedullary tumours. Common extramedullary tumours include meningiomas, schwannomas, extramedullary ependymomas, haemangioblastomas, while intramedullary tumours include astrocytomas and intramedullary ependymomas.
Dexamethasone (a potent glucocorticoid) in doses of 16 mg/day may reduce edema around the lesion and protect the cord from injury. It may be given orally or intravenously for this indication.
Surgery is indicated in localised compression as long as there is some hope of regaining function. It is also occasionally indicated in patients with little hope of regaining function but with uncontrolled pain. Postoperative radiation is delivered within 2–3 weeks of surgical decompression. Emergency radiation therapy (usually 20 Gray in 5 fractions, 30 Gray in 10 fractions or 8 Gray in 1 fraction) is the mainstay of treatment for malignant spinal cord compression. It is very effective as pain control and local disease control. Some tumours are highly sensitive to chemotherapy (e.g. lymphomas, small-cell lung cancer) and may be treated with chemotherapy alone.
Once complete paralysis has been present for more than about 24 hours before treatment, the chances of useful recovery are greatly diminished, although slow recovery, sometimes months after radiotherapy, is well recognised.
The median survival of patients with metastatic spinal cord compression is about 12 weeks, reflecting the generally advanced nature of the underlying malignant disease.
The precise causes of syringomyelia are still unknown although blockage to the flow of cerebrospinal fluid has been known to be an important factor since the 1970s. Scientists in the UK and America continue to explore the mechanisms that lead to the formation of syrinxes in the spinal cord. It has been demonstrated a block to the free flow of cerebrospinal fluid is a contributory factor in the pathogenesis of the disease. Duke University in America and Warwick University are conducting research to explore genetic features of syringomyelia.
Surgical techniques are also being refined by the neurosurgical research community. Successful procedures expand the area around the cerebellum and spinal cord, thus improving the flow of cerebrospinal fluid thereby reducing the syrinx.
It is also important to understand the role of birth defects in the development of hindbrain malformations that can lead to syringomyelia as syringomyelia is a feature of intrauterine life and is also associated with spina bifida. Learning when these defects occur during the development of the fetus can help us understand this and similar disorders, and may lead to preventive treatment that can stop the formation of some birth abnormalities. Dietary supplements of folic acid prior to pregnancy have been found to reduce the number of cases of spina bifida and are also implicated in prevention of cleft palate and some cardiac defects.
Diagnostic technology is another area for continued research. MRI has enabled scientists to see conditions in the spine, including syringomyelia before symptoms appear. A new technology, known as dynamic MRI, allows investigators to view spinal fluid flow within the syrinx. CT scans allow physicians to see abnormalities in the brain, and other diagnostic tests have also improved greatly with the availability of new, non-toxic, contrast dyes.
Surgery is not always recommended for syringomyelia patients. For many patients, the main treatment is analgesia. Physicians specializing in pain management can develop a medication and treatment plan to ameliorate pain. Medications to combat any neuropathic pain symptoms such as shooting and stabbing pains (e.g. gabapentin or pregabalin) would be first-line choices. Opiates are usually prescribed for pain for management of this condition. Facet injections are not indicated for treatment of syringomyelia.
Drugs have no curative value as a treatment for syringomyelia. Radiation is used rarely and is of little benefit except in the presence of a tumor. In these cases, it can halt the extension of a cavity and may help to alleviate pain.
In the absence of symptoms, syringomyelia is usually not treated. In addition, a physician may recommend not treating the condition in patients of advanced age or in cases where there is no progression of symptoms. Whether treated or not, many patients will be told to avoid activities that involve straining.
Since the natural history of syringomyelia is poorly understood, a conservative approach may be recommended. When surgery is not yet advised, patients should be carefully monitored. Periodic MRI's and physical evaluations should be scheduled at the recommendation of a qualified physician.
Spinal cord compression develops when the spinal cord is compressed by bone fragments from a vertebral fracture, a tumor, abscess, ruptured intervertebral disc or other lesion. It is regarded as a medical emergency independent of its cause, and requires swift diagnosis and treatment to prevent long-term disability due to irreversible spinal cord injury.
Surgery
Surgical intervention is warranted in patients who present with new onset neurological signs and symptoms or have a history of progressive neurological manifestations which can be related to this abnormality. The surgical procedure required for the effective treatment of diastematomyelia includes decompression (surgery) of neural elements and removal of bony spur. This may be accomplished with or without resection and repair of the duplicated dural sacs. Resection and repair of the duplicated dural sacs is preferred since the dural abnormality may partly contribute to the "tethering" process responsible for the symptoms of this condition.
Post-myelographic CT scanning provides individualized detailed maps that enable surgical treatment of cervical diastematomyelia, first performed in 1983.
Observation
Asymptomatic patients do not require surgical treatment. These patients should have regular neurological examinations since it is known that the condition can deteriorate. If any progression is identified, then a resection should be performed.
A 69-year-old male with tethered cord that was low lying and associated with Spina Bifida in the form of meningocele that was operated on as an infant, was studied in this research. He presented with worsened neurological deficits due to progressive lumbar stenosis at the L3-L4 level which was associated with the spinal discs degenerating. Extreme lateral inter body fusion (XLIF) was performed to allow for indirect spinal cord decompression and stability which allowed for neurological improvement. The role of the XLIF approach to this treatment was emphasized and compared to other surgical approaches. It was concluded that surgical decompression should be performed as soon as possible to prevent any further neurological damage. Also concluded was that the XLIF approach is safe and fast and is indicative of a good surgical option to obtain spinal cord indirect decompression and lumbar inter body fusion.
Because neurological deficits are generally irreversible, early surgery is recommended when symptoms begin to worsen. In children, early surgery is recommended to prevent further neurological deterioration, including but not limited to chronic urinary incontinence.
In adults, surgery to detether (free) the spinal cord can reduce the size and further development of cysts in the cord and may restore some function or alleviate other symptoms. Although detethering is the common surgical approach to TCS, another surgical option for adults is a spine-shortening vertebral osteotomy. A vertebral osteotomy aims to indirectly relieve the excess tension on the spinal cord by removing a portion of the spine, shortening it. This procedure offers a unique benefit in that the spinal cord remains fixated to the spine, preventing retethering and spinal cord injury as possible surgical complications. However, its complexity and limited “track record” presently keeps vertebral osteotomies reserved as an option for patients who have failed in preventing retethering after detethering procedure(s).
Other treatment is symptomatic and supportive. Medications such as NSAIDs, opiates, synthetic opiates, COX-2 inhibitors, and off-label applications of tricyclic antidepressants combined with anti-seizure compounds have yet to prove they are of value in treatment of this affliction's pain manifestations. There is anecdotal evidence that TENS units may benefit some patients.
Treatment may be needed in adults who, while previously asymptomatic, begin to experience pain, lower back degeneration, scoliosis, neck and upper back problems and bladder control issues. Surgery on adults with minimal symptoms is somewhat controversial. For example, a website from the Columbia University Department of Neurosurgery says, "For the child that has reached adult height with minimal if any symptoms, some neurosurgeons would advocate careful observation only." However, surgery for those who have worsening symptoms is less controversial. If the only abnormality is a thickened, shortened filum, then a limited lumbosacral laminectomy with division of the filum may be sufficient to relieve the symptoms.
This syndrome was first noticed in the late 19th century. While information has been available for years, little widespread blind research has been done. More research has been called for, and doctors have conducted many studies with good results. There is a low morbidity rate, and no complications have been documented other than those typical of any type of back surgery. The association of this condition with others has been noticed, and needs further research to understand such relationships. TCS is causally linked to Chiari malformation and any affirmative diagnosis of TCS must be followed by screening for Chiari's several degrees. TCS may also be related to Ehlers-Danlos syndrome, or Klippel-Feil syndrome, which should also be screened for upon a positive TCS diagnosis. Spinal compression and the resulting relief is a known issue with this disorder. Like with the early-onset form, this disease form is linked to the Arnold-Chiari malformation, in which the brain is pulled or lowers into the top of the spine.
There is no known treatment to reverse nerve damage due to myelomalacia. In some cases, surgery may slow or stop further damage. As motor function degenerates, muscle spasticity and atrophy may occur. Steroids may be prescribed to reduce swelling of the spinal cord, pain, and spasticity.
Research is underway to consider the potential of stem cells for treatment of neurodegenerative diseases. There are, however, no approved stem cell therapies for myelomalacia.
The treatment and prognosis of myelopathy depends on the underlying cause: myelopathy caused by infection requires medical treatment with pathogen specific antibiotics. Similarly, specific treatments exist for multiple sclerosis, which may also present with myelopathy. As outlined above, the most common form of myelopathy is secondary to degeneration of the cervical spine. Newer findings have challenged the existing controversy with respect to surgery for cervical spondylotic myelopathy by demonstrating that patients benefit from surgery.
Arachnoiditis is difficult to treat and treatment is generally limited to alleviation of pain and other symptoms. While arachnoiditis may not yet be curable and can be significantly life-altering, management of the condition, including with medication, physical therapy, and if appropriate, psychotherapy, can help patients cope with the difficulties it presents. Surgical intervention generally has a poor outcome and may only provide temporary relief, but some cases of surgical success have been reported. Epidural steroid injections to treat sciatic pain have been linked as a "cause" of the disease by the U.S. Food and Drug Administration as well as in other research, and are therefore discouraged as a treatment for Arachnoiditis as they will most likely worsen the condition. Some patients benefit from motorized assistance devices such as the Segway or standing wheelchairs, although these types of devices may be beyond the reach of those with limited means. Standing endurance and vibration tolerance are considered before considering such devices in any case.
The type of treatment needed for dogs diagnosed with CM/SM depends on the severity of the condition and the age of the dog. Young dogs with clinical signs should be considered for surgical removal to minimize the progression of the disease as the dog ages. Older dogs with little or no clinical signs may be treated medically, rather than surgically. However, severe cases of CM/SM may require surgery regardless of age. The goal of surgery is syrynx decompression through restoration of normal cerebrospinal fluid circulation.
The surgical treatment of CM in dogs is described as "foramen magnum decompression FMD". Despite an approximately 80% success rate with this surgical technique, there is a 25% to 50% relapse, primarily due to excessive scar tissue formation at the decompression site. A cranioplasty may be performed instead, in which a plate, constructed using titanium mesh and bone cement, is fixed to the back of the skull following a standard FMD procedure. The procedure had been effective in humans. The postoperative relapse rate associated with the titanium cranioplasty procedure is less than 7%.
Potential non-surgical treatments include:
- Education about the course of the condition and how to relieve symptoms
- Medicines to relieve pain and inflammation, such as acetaminophen, nonsteroidal anti-inflammatory drugs (NSAIDs)
- Exercise, to maintain or achieve overall good health, aerobic exercise, such as riding a stationary bicycle, which allows for a forward lean, walking, or swimming can relieve symptoms
- Weight loss, to relieve symptoms and slow progression of the stenosis
- Physical therapy, to provide education, instruction, and support for self-care; physical therapy instructs on stretching and strength exercises that may lead to a decrease in pain and other symptoms
Arachnoiditis is a chronic disorder with no known cure, and prognosis may be hard to determine because of an unclear correlation between the beginning of the disease and the appearance of symptoms. For many, arachnoiditis is a disabling disease that causes chronic pain and neurological deficits, and may also lead to other spinal cord conditions, such as syringomyelia.
Spinal disease (also known as a dorsopathy) refers to a condition impairing the backbone. These include various diseases of the back or spine ("dorso-"), such as kyphosis. Dorsalgia refers to those conditions causing back pain.
An example is scoliosis. Some other spinal diseases include Spinal Muscular Atrophy, Ankylosing Spondylitis, Lumbar Spinal Stenosis, Spina Bifida, Spinal tumors, Osteoporosis and Cauda Equina Syndrome.
Potential surgical treatments include:
- Anterior cervical discectomy and fusion - A surgical treatment of nerve root or spinal cord compression by decompressing the spinal cord and nerve roots of the cervical spine with a discectomy in order to stabilize the corresponding vertebrae.
- Laminoplasty - A surgical procedure relieve pressure on the spinal cord by cutting the lamina on both sides of the affected vertebrae (cutting through on one side and merely cutting a groove on the other) and then "swinging" the freed flap of bone open.
- Laminectomy - A surgical procedure in which the lamina of the vertebra is removed or trimmed to widen the spinal canal and create more space for the spinal nerves and thecal sac.
The effectiveness of non surgical treatments is unclear as they have not been well studied.
- Education about the course of the condition and how to relieve symptoms
- Medicines to relieve pain and inflammation, such as acetaminophen, nonsteroidal anti-inflammatory drugs (NSAIDs)
- Exercise, to maintain or achieve overall good health, aerobic exercise, such as riding a stationary bicycle, which allows for a forward lean, walking, or swimming can relieve symptoms
- Weight loss, to relieve symptoms and slow progression of the stenosis
- Physical therapy to support self-care. Also may give instructs on stretching and strength exercises that may lead to a decrease in pain and other symptoms.
- Lumbar epidural steroid or anesthetic injections have low quality evidence to support their use.
Treatment options are either surgical or non-surgical. Overall evidence is inconclusive whether non-surgical or surgical treatment is the better for lumbar spinal stenosis.