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Learning Objectives: Describe the 5 main signs & symptoms of myelopathy: Para-/quadriparesis, sensory level, urinary incontinence, constipation, sexual dysfunction Identify & distinguish the different causes of myelopathy by their clinical presentations Describe the evaluation & management of spinal cord compression

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When approaching a patient with a neurologic complaint, it is important to think like a neurologist. The initial most important aspect of the neurologic evaluation is to anatomically localize the site of the lesion. Example: Is the lesion in the central or peripheral nervous system? If central, is it in the brain or spinal cord? Within the brain, is the lesion cortical or subcortical? If peripheral, is it in the nerve root, peripheral nerve, neuromuscular junction, or muscle? The process of "localizing the lesion" from the onset of the evaluation will serve as a guide to focus the neurologic assessment. Localizing the lesion ultimately lays the foundation for forming a differential diagnosis and developing a plan for evaluation, management, and counseling of the patient.

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Numbness requires clarification to determine whether the patient is describing a loss of sensation, or a positive phenomena, such as paresthesias (abnormal spontaneous sensation) or dysesthesias (abnormal sensation to stimulus). Destructive or compressive lesions of the CNS more often cause sensory loss whereas inflammatory or irritative lesions often result in positive phenomena. Cold feeling in the legs can be neurologic in nature but can also be due to peripheral vascular disease.

Hemibody symptoms can result from a contralateral cerebral hemisphere or brainstem lesion, or from a spinal cord lesion. However, bilateral symptoms are more likely due to spinal cord or peripheral nerve disorders. A complaint of tightness in a band around the chest or abdomen is characteristic of a spinal cord lesion. This usually correlates with findings of a sensory level on neurologic examination.

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Progressive symptoms suggest an expanding lesion. Gait difficulty can be due to weakness, sensory ataxia (proprioceptive loss), or cerebellar ataxia. Urinary incontinence can result from upper-motor neuron dysfunction (brain, spinal cord) or lower-motor neuron dysfunction (peripheral nerves). Diminished perianal and genital sensation indicates either spinal cord or sacral nerve root lesion.

The most likely lesion localization in a patient with gait difficulty, bilateral leg symptoms, urinary incontinence, decreased perianal and genital sensation is either the spinal cord or bilateral lumbosacral nerves. However, when associated with band-like tightness around the chest, the most likely localization is the spinal cord. If the upper extremities are not involved, the lesion is more likely below the level of the brachial plexus (C5-T1).

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Our patient has no family history of neurologic disease, no risk factors for infection or tumor, and no history of trauma.

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Our patients vital signs and general exam are normal. There are no signs of infection. In addition, there is no spinal tenderness to suggest a bone lesion (e.g., vertebral compression fracture either pathologic or osteoporotic, bone metastases, osteomyelitis) or paraspinal tenderness to suggest an epidural abscess, hematoma, or other mass lesion

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The initial portion of the neurologic examination shows bilateral lower extremity weakness and supports our suspicions of either a spinal cord lesion below the level of the cervical spinal cord, or lesions involving bilateral lumbosacral nerves (e.g., bilateral lumbosacral plexus, cauda equina). Further testing for the presence of a sensory level and reflex testing will help to confirm the lesion localization.

lesions in the bilateral medial cerebral hemispheres or brainstem can cause weakness of both legs, normal mental status and normal cranial nerve exam makes a lesion in either of these locations less likely.

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The presence of a sensory level strongly suggests a spinal cord lesion. Depending on the extent of nerve-fiber damage, a sensory level can be relative, meaning that the sensation is partially diminished, or absolute, meaning that the sensation is completely absent below the level of the lesion.

Our patients sensory level suggests a spinal cord lesion at the level of T3. However, a sensory level may be hung a few to several levels below the actual site of the lesion. For example, a cervical lesion may result in a hung sensory level in the upper-thoracic cord.

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Hyperreflexia, 3+ or 4+ reflexes, and Babinski sign indicates upper-motor neuron dysfunction. Babinski sign, or extensor plantar response, is tested by stroking the lateral aspect of the sole from the heel across the ball to the big toe. Toe flexion is the normal response. Clonus is a series of involuntary muscle contractions stimulated by sudden stretching of the muscle. It is most common at the ankle. It can be sustained (indefinite) or nonsustained (described by the number of beats).

Bilateral hyperreflexia and Babinski signs in lower extremities with normal reflexes in the upper extremities suggests a spinal cord lesion below the level of the nerves roots supplying the brachial plexus.

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Localization in clinical neurology refers to the process of determining an anatomical/structural location of the patients neurological problem. For example, is the problem in the brain, brainstem, cerebellum, spinal cord, roots, peripheral nerves, neuromuscular or muscle.

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The differential diagnosis is the cornerstone of clinical medicine: determining diagnostic possibilities based upon history and examination.

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The spinal cord is normally protected by the spine. Spinal cord compression results when the cord is externally compressed by bone (e.g., compression fracture, bony metastasis, Potts disease), tumor, infection or hemorrhage. Cord compression syndromes are typically associated with pain. Myelitis, or transverse myelitis (TM), is an inflammatory myelopathy. It typically develops over hours to days. The most common etiologies are listed on this slide. Post-infectious TM is thought to be immune-mediated after exposure to a foreign antigen. Spinal cord tumors can be intramedullary (within the cord), intraduralextramedullary (e.g., meningioma, neuro-fibroma), or extradural (usually bony metastasis). Intradural tumors are usually low-grade astrocytoma, ependymoma, or hemangioblastoma Arteriovenous malformations (AVM) are congenital abnormal tangles of arteries and veins. In the cord, AVMs can cause symptoms by three mechanisms bleeding, compression, or shunting of blood. Spinal cord AVMs are rare but must be considered in the differential diagnosis.

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Syringomyelia, or syrinx, is a cystic cavitation of the spinal cord, most common in the cervical cord. It is typically associated with congenital brainstem anomalies (Chiari malformation), remote spinal cord trauma, or tumors within the spinal cord. Compression of central cord structures by the enlarging syrinx can cause loss of pain & temperature sensation in cape-distribution (anterior white commissure - crossing spinothalamic tract fibers), atrophy and weakness (anterior horn cells), and spastic para- or quadriparesis (corticospinal tracts). Typically vibration and proprioception are intact due to sparing of the dorsal columns

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Neurosyphilis can result in tabes dorsalis many years after the initial syphilis infection if it is left untreated. Typically, the posterior spinal nerve roots in the lumbosacral region are involved causing lancinating pain radiating into the legs. Eventually, there is progressive degeneration of the posterior columns of the spinal cord.

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Vitamin B12 deficiency can cause a myelopathy that presents subacutely over weeks to months. Degeneration of both the lateral corticospinal tracts and the posterior columns occur so the myelopathy is referred to as subacute combined degeneration of the spinal cord. Loss of vibration and proprioception results in severe sensory ataxia evidenced by the presence of Romberg sign on exam. HIV vacuolar myelopathy also affects the posterior and lateral columns and presents similarly to Vitamin B12 deficiency.

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Spinal cord infarction is rare and generally occurs in the anterior spinal artery territory. The paired posterior spinal arteries are well-supplied by feeders so infarction is rare in the posterior cord. Acute paraparesis and loss of pain & temperature sensation below the lesion with intact vibration and proprioception is characteristic. Reflexes and tone may be decreased acutely (spinal shock). Causes include trauma, dissecting aortic aneurysm, hypotensive crisis (e.g., intraoperative blood loss, aortic cross-clamping).

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HTLV-1 (Human T-lymphotrophic virus 1) is a retrovirus that causes T-cell leukemia and T-cell lymphoma in adults. It is endemic in the Carribbean, Japan, and Africa. Infection of the spinal cord by HTLV-1 results in HTLV-1 associated myelopathy, a chronic progressive demyelinating inflammatory myelopathy primarily affecting the cortico-spinal tracts of the thoracic cord. Minor sensory loss may also be present.

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Lateral hemisection of the spinal cord due to trauma or compression leads to a characteristic presentation of Brown-Squard Syndrome: Ipsilaterally below the level of the lesion there is weakness (corticospinal tract dysfunction), hyperreflexia, and a sensory level to vibration & proprioception (posterior column dysfunction). Contralaterally below the level of the lesion there is a sensory level to pinprick (spinothalamic tract dysfunction). Remember that the spinothalamic tract fibers enter the spinal cord and cross over to the opposite side of the cord, then ascend to the brain, unlike both the corticospinal tract and posterior column fibers which decussate in the lower brainstem

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Evaluation is partially synonymous with plan. This refers to the clinicians plan for testing to definitively diagnose the etiology of the patient's complaint based upon the differential diagnosis.

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Spinal X-rays may be helpful in detecting causes of back pain involving bone such as vertebral compression fractures, bone metastasis, and degenerative joint disease. However, it does not allow visualization of disks, nerves, or soft tissue. Spinal MRI is needed if spinal cord compression is suspected. MRI is the preferred study for obtaining detailed images of the bony structures and soft tissues of the spine, the spinal cord, and the spinal nerve roots. If MRI can be obtained quickly, spinal X-rays are not usually necessary in evaluating suspected spinal cord compression.

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The sagittal view of our patients thoracic-spine MRI, showing the midline of the spinal cord, is shown here. On T1-weighted images CSF appears dark, while on T2-weighted images it appears white. Notice the posterior mass at the T3-T4 level evident on both images, resulting in severe compression of the spinal cord posteriorly. This mass is extramedullary (outside the spinal cord) and intradural, and enhances homogeneously with contrast. Its appearance is most consistent with a calcified meningioma (calcium is black on T2-weighted images).

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Review the final diagnosis with the learners.

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Management refers to therapeutic interventions (including medications, procedures, therapies, etc) to provide acute and/or long term treatment for the patients disease and risk factors. Management also includes rehabilitation and lifestyle modification.

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Mild cord compression may be asymptomatic or cause only minimal disruption of signal conduction. More significant compression may cause severe disruption or blockage of nerve signal transmission, resulting in severe muscle weakness, sensory loss, and bowel and bladder dysfunction. If the compression is not relieved, irreversible spinal cord damage can occur within 24 hours. Acute cord compression from any cause (e.g., trauma, acute disk herniation, tumor, abscess, hematoma) is an emergency and warrants immediate referral to neurosurgery for decompression.

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Counseling refers to physician discussion and education sessions with the patient, family and caregivers regarding the patients illness, diagnostic evaluation, treatment plan and long term prognosis. These counseling sessions should also include education regarding how the patients disease will affect their daily life and ability to perform activities of daily living and employment capability. When appropriate, counseling regarding advanced directives and goals of care should also be performed. The physician can also refer the patient and family to other resources and allied health disciplines as appropriate: social work, case management, chaplains, vocational rehabilitation, etc.

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Prognosis depends on the severity and duration of the neurologic deficit prior to decompression. Improvement can occur post-operatively with remyelination and resolution of edema and inflammation. Some neurologic deficits may be irreversible Autonomic hyperreflexia can occur with cervical and thoracic cord lesions. Stimuli below the lesion level that are easily tolerated in healthy people (e.g., overfull bladder) trigger excess sympathetic nerve activation resulting in blood pressure, heart rate, excessive sweating and flushing. Neurogenic bladder occurs due to dysynergy between the bladder detrusor and sphincter muscles, often resulting in a flaccid bladder requiring intermittent self-catheterization. Neuropathic pain, often described as burning, lancinating, stabbing, or electric shock-like, can be severe & difficult to treat.

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