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Myelopathy

Spinal Cord

Myelopathy is an umbrella term for various problems affecting the spinal region. 


Presentation

Myelopathy may present with neck stiffness along with severe pain in the either one or both sides of the neck. The pain and associated stiffness may radiate to the arms and shoulders. Individuals suffering from myelopathy, experience lack of control and significant loss of coordination while carrying out certain activities. In the initial stages, individuals may face problem while walking or with maintaining balance. There may be associated muscle weakness and individuals also report a certain degree of disability. Other symptoms include development of stabbing pain in the elbow, arm, fingers and wrist and hyperreflexia. Certain percentage of individuals also experience incontinence and sexual dysfunction.

Amyloidosis
  • Related to Rheumatoid arthritis Amyloidosis: Dialysis-associated Clinical Location: Usually cervical Onset: Neck & shoulder pain Progression: Myelopathy within 2 years Pathology: Disk-space narrowing; Vertebral subluxation Decompression Sickness Early[neuromuscular.wustl.edu]
Lower Extremity Pain
  • The number-one tipoff to my sensibilities is when a client presents with a history of same-sided somatic complaints of the neck, and upper- and lower-extremity pain or dysfunction, including sciatica.[massagetoday.com]
Back Pain
  • If you have had a long history of back pain, changes in coordination, recent weakness, and difficulty doing tasks that used to be easier because your body seemed more responsive in the past, you should see a doctor as these are definite warning signs.[watkinsspine.com]
  • We offer comprehensive back pain management plans to help you overcome spine problems, so you can enjoy life with less pain.[spinecenter.nm.org]
  • Myelopathy symptoms may include: Neck, arm, leg or lower back pain Tingling, numbness or weakness Difficulty with fine motor skills, such as writing or buttoning a shirt Increased reflexes in extremities or the development of abnormal reflexes Difficulty[hopkinsmedicine.org]
  • pain (50%); Paresthesias Age range: 4 to 83 years; Mean 32 to 44 years Clinical Spinal cord Isolated defect: Other parts of CNS intact Levels: Most common T2 to T6; Cervical up to C1 Sensory loss (96%): Segmental and below lesion GU Bladder dysfunction[neuromuscular.wustl.edu]
Impulsivity
  • Causes There are many causes of cervical myelopathy; anything that interrupts the normal flow of neural impulses through the spinal cord may cause a clinical myelopathy.[ansdocs.com]
  • Because the spinal cord carries nerve impulses to many regions in the body, patients with CSM can experience a wide variety of symptoms.[orthoinfo.aaos.org]
  • It affects the fibers of the spinal cord that transmit impulses to the arms, hands, and legs. As a result, it can cause weakness, numbness, tingling, or rarely, pain in these areas.[columbianeurosurgery.org]
Hyperreflexia
  • Clinical features vary depending on the level of the lesion and include local pain , stiffness, and impaired sensation, hypotonia , and hyporeflexia at the level of the lesion, and spasticity and hyperreflexia below the level of the injury level.[amboss.com]
  • Physical examination commonly elicits long tract signs such as spasticity, hyperreflexia, and abnormal reflexes such as Babinski or Hoffman's sign.[neurosurgery.ucla.edu]
  • A characteristic physical finding of CSM is hyperreflexia. The biceps and supinator reflexes (C5 and C6) may be absent, with a brisk triceps reflex (C7).[aafp.org]
  • […] shoulder Numbness or paresthesia in the upper extremities Sensory changes in the lower extremities Motor weakness in the extremities Gait difficulties ("spastic gait," hesitant and jerky) Myelopathic or "upper motor neuron" findings such as spasticity, hyperreflexia[emoryhealthcare.org]
Clonus
  • […] see: exam of spinal cord - presentation: ( Bertalanffy, et al ) - 61% presented with radicular symptoms - 16% had pure myelopathic symptoms - 23% had a combination of myelopathic and radiculopathy - upper motor neuron findings such as hyper-reflexia, clonus[wheelessonline.com]
  • Figure V is a video showing sustained clonus with forced ankle dorsiflexion. 3 beats of clonus or less is considered normal.[orthobullets.com]
  • […] or paresthesia in the upper extremities Sensory changes in the lower extremities Motor weakness in the extremities Gait difficulties ("spastic gait," hesitant and jerky) Myelopathic or "upper motor neuron" findings such as spasticity, hyperreflexia, clonus[emoryhealthcare.org]
  • On physical examination he has 5 of 5 motor strength in all muscles groups in his upper and lower extremities, a bilateral Hoffman sign, bilateral 3 patellar reflexes, 3 beats of clonus on the right, and no clonus on the left.[orthobullets.com]
  • She was diffusely hyper-reflexic with sustained right ankle clonus, positive crossed adductor, suprapatellar, and pectoralis reflexes, bilateral plantar extensor responses, and positive Hoffmann’s signs.[touchneurology.com]
Radiculomyelopathy
  • […] osteoarthritis of the cervical spine causing stenosis of the canal and sometimes cervical myelopathy due to encroachment of bony osteoarthritic growths (osteophytes) on the lower cervical spinal cord, sometimes with involvement of lower cervical nerve roots (radiculomyelopathy[merckmanuals.com]
  • J Bone Joint Surg (Br), 1995, 77, 956-961. [3] F ouyas IP, S tatham PFX, S andercock PAG: Cochrane review on the role of surgery in cervical spondylotic radiculomyelopathy.[em-consulte.com]
  • "Cochrane review on the role of surgery in cervical spondylotic radiculomyelopathy." Spine 27.7 (2002): 736-747. Shimomura, Takatoshi, et al.[physio-pedia.com]
Spastic Paraplegia
  • Hereditary spastic paraplegia (also called familial spastic paraplegia or Strumpell-Lorrain disease) is not a single disease, but rather a heterogeneous group of genetic disorders, the main feature of which is progressive spasticity in the lower limbs[massagetoday.com]
  • ., metastatic tumors), subacute combined degeneration of the spinal cord (vitamin B 12 deficiency), hereditary spastic paraplegia, normal pressure hydrocephalus and spinal cord infarction ( Table 4 ) . 10 Most of these conditions can easily be distinguished[aafp.org]
  • Budka H, Sluga E, Heiss WD, Spastic Paraplegia Associated with Addisons-Disease - Adult Variant of Adreno-Leukodystrophy, J Neurol , 1976;213:237–50. 7.[touchneurology.com]
  • Positive findings on neurologic examination included a left afferent papillary defect, spastic paraplegia with hyperreflexia, crossed adductor responses, ankle clonus, and bilateral Babinski signs.[jamanetwork.com]
Long Tract Signs
  • Physical examination commonly elicits long tract signs such as spasticity, hyperreflexia, and abnormal reflexes such as Babinski or Hoffman's sign.[neurosurgery.ucla.edu]
  • […] extremities; - Brown-Séquard syndrome : - ipsilateral motor deficits with contralateral sensory deficits - may be the least advanced form of the disease; - Brachialgia and cord syndrome: radicular pain in the upper extremity along with motor and/or sensory long-tract[wheelessonline.com]
  • tract signs, ambulatory Class IIIB Objective weakness, long tract signs, non-ambulatory Japanese Orthopaedic Association Classification A point scoring system (17 total) based on function in the following categories upper extremity motor function lower[orthobullets.com]
  • When the stenosis and myelopathy is severe, most patients will develop long tract signs (UMN) consisting of a wide-based gait, balance difficulties, and weakness.[uscspine.com]
Urinary Incontinence
  • Urinary frequency and urgency, occasional dysuria, and urinary incontinence developed in the few weeks before admission. Four months earlier, she had undergone an extensive evaluation.[jamanetwork.com]

Workup

The signs and symptoms of the condition are carefully observed and taken note of. This is followed by physical neurologic examination that would involve testing reflexes, including abdominal reflexes, along with sensory testing of the body. This will be followed by laboratory tests to analyze levels of vitamin B12 or heavy metals present in the blood. Levels of white blood cells and erythrocyte sedimentation rate would also be tested, to examine infection or inflammation as the source of myelopathy. 

Lumbar puncture test would also be done in order to rule out multiple sclerosis or meningitis. Imaging studies such as CT scan, radionuclide bone scan and MRI of spinal cord will also be required [7]. In conditions, when the diagnostic procedures results are inconclusive, then bone biopsies or cultures are indicated.

Treatment

Treatment is geared towards treating the underlying disease condition and effective management of symptoms. In case of fracture or dislocation of vertebrae, the patient is put on traction, followed by immobilization for several weeks [8]. Rehabilitation therapy would also be required and medications to relieve pain and stiffness would also be employed.

Steroid medications to relieve inflammation due to arthritis are also indicated. If the pain is severe, then steroid injections may be given. These are given in the epidural region and used only in cases when conservative treatment did not bring about the desired effect [9].

In instances, when medications and physical therapy do not yield positive results, then surgery would be opted for. Surgical procedures to relieve nerve compression would be carried out. In many cases, spinal fusion would also accompany the surgical procedure in order to reduce the risks of complications after surgery [10].

Prognosis

Prognosis depends on the causative factors. For example, in case of infection complete recovery is possible. When traction is imparted and there are no signs of residual damage to the spinal column, then too complete recovery is a possibility. Surgery may be required in certain cases to relieve the nerve under pressure. Failure to do so can lead to irreversible permanent damage in the long run. However, in many instances, other forms of treatment to manage the symptoms are required. Recovery following removal of herniated disc is favorable and prognosis is excellent if there is no residual damage to the spinal cord [6].

Etiology

Aging process that causes normal wear and tear in the spinal region is one of the major causes of myelopathy. In addition to aging, accidents or other traumatic events are other significant factors that can cause nerve deficit in the spinal cord. Other causes include spinal stenosis, development of tumor in the spinal column, degenerative disorders governing the disc and disease conditions such as multiple sclerosis. In many instances, inflammatory diseases such as rheumatoid arthritis can also give rise to the condition of myelopathy. Congenital abnormalities and infections can also lead to the disorder, but such cases are rare.

Epidemiology

The exact prevalence of myelopathy is unknown. However, the incidence rate of various causes that gives rise to the condition has been reported. Statistics have revealed, that in US, there are about 12,000 to 15,000 spinal cord injuries every year [3].

It was also estimated that, cervical myelopathy strikes approximately 50% of men and 33% women aged 60 years and above. This clearly explains that age is the major factor for development of myelopathy. Prevalence rate of tumors in the spinal region is reported to be 0.5 to 2.5 per 100, 000 individuals in US. In addition, it was also reported that, 5 to 10% individuals with cancer are also likely to develop tumor in the epidural region, which is known to cause about 25,000 cases of myelopathy.

Sex distribution
Age distribution

Pathophysiology

Aging causes degenerative changes to take place in the spine, paving way for development of myelopathy. Degenerative changes may exert pressure on the cartilage preventing the joints in the spine from working properly. This in turn favors the degeneration to continue causing further damage to the spinal region. Myelopathy can also occur due to herniated discs which also favor nerve compression [4].

Myelopathy that occurs due to trauma or accident, significantly affects the muscles and ligaments that support and cushion the spine. Accidents can also lead to bone dislocation which in turn compresses the neighboring nerve giving rise to myelopathy [5].

Prevention

It is not always possible to prevent the onset of myelopathy. Aging process cannot be controlled, but certain factors can be adopted to keep the spinal column and neighboring muscles strong. Steps to prevent accidents should also be taken. However, in unforeseen situations, individuals are advised to undergo complete treatment plan to avoid irreversible damage to the spinal cord.

Summary

Development of disorders in the spinal region that gradually inflict compression is the major reason for occurrence of myelopathy. Nerve compression by osteophytes or extruded disk in the cervical region is a common cause of myelopathy. It is known to be one of the major causes of neck and cervical pain. When nerves in the spine region get compressed due to accident or trauma, the condition is then referred to as spinal cord injury [1]. When myelopathy occurs due to infections or inflammations, the condition is known as myelitis and when the disease is vascular in nature, it is known as vascular myelopathy [2].

Patient Information

  • Definition: Myelopathy refers to diseases governing the spinal cord. There are several types of myelopathy, which have been named based on their causative factors. These include radiation myelopathy which occurs as a complication of radiation therapy, compressive myelopathy which develops due to pressure exerted from a mass or hematoma or carcinomatous myelopathy occurs due to development of cancerous tumor in the spinal column.
  • Cause: Various factors give rise to myelopathy. These include aging, various disease conditions such as arthritis, multiple sclerosis, inflammatory diseases, infections and tumor. Accidents and trauma are other potential factors that can give rise to myelopathy.
  • Symptoms: In the initial stages, symptoms are mild and often go unnoticed. Affected individuals complain of pain in one or both the arms which is often accompanied by stiffness. There is also significant pain in the fingers and wrist. Individuals also complain of loss of balance and may even suffer from incontinence.
  • Diagnosis: Preliminary physical examination is done followed by laboratory tests to assess complete blood count and sedimentation rate. This is done to analyze infections and inflammations as the causative factors. In addition, imaging studies such as CT scan and MRI are also indicated.
  • Treatment: Treatment is cause dependent and is majorly geared towards correcting the underlying condition in order to relieve the nerve compression. Conservative treatment approach is adopted initially followed by steroid injections and surgery if other methods fail.

References

Article

  1. Garland DE, Stewart CA, Adkins RH, et al. Osteoporosis after spinal cord injury. J Orthop Res. May 1992;10(3):371-8
  2. Rubin MN, Rabinstein AA. Vascular diseases of the spinal cord. Neurol Clin. Feb 2013;31(1):153-81.
  3. Ackery A, Tator C, Krassioukov A. A global perspective on spinal cord injury epidemiology. J Neurotrauma 2004; 21:1355.
  4. Chiles BW 3rd, Leonard MA, Choudhri HF, Cooper PR. Cervical spondylotic myelopathy: patterns of neurological deficit and recovery after anterior cervical decompression. Neurosurgery 1999; 44:762.
  5. Allen AR. Remarks on the histopathological changes in the spinal cord due to impact an experimental study. J Ner Ment Dis 1914; 41:141.
  6. Menter RR, Hudson LM. Spinal cord injury clinical outcomes. In: Stover S, ed. The Model Systems. New York, NY: Aspen Pubs; 1995:272.
  7. Antevil JL, Sise MJ, Sack DI, et al. Spiral computed tomography for the initial evaluation of spine trauma: A new standard of care? J Trauma 2006; 61:382.
  8. Cervical spine immobilization before admission to the hospital. Neurosurgery 2002; 50:S7.
  9. Bracken MB. Steroids for acute spinal cord injury. Cochrane Database Syst Rev 2012; 1:CD001046.
  10. Kadanka Z, Mares M, Bednarík J, et al. Predictive factors for mild forms of spondylotic cervical myelopathy treated conservatively or surgically. Eur J Neurol 2005; 12:16.

Symptoms

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