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1 | .Thisincludes .TableIIdem- 3 4 http://links.lww.com/ . Persistent symptoms, 4 Disclosure of Potential . 2 Diagnosing cervical spondylotic mye- Patients may experience stable upper-extremity findings the inability to grip and releaserapidly the and hand the presence of theescape finger sign, in which the ulnardrift 2 into digits flexion and abduction during extension of the metacarpophalangeal and interphalangeal joints. Severe sequelae include loss of bowel and bladdertion, func- , and gait instability. Posi- tive Hoffman or Babinski signs are clinical indicators of without transience or fluctuation, distin- guish cervical spondylotic myelopathy from other disorders. Sequelae such as sensory loss, motor weakness, or gait instability may not be present in every patient. lopathy is largely reliant on historyical and examination. phys- However, other modalities are critical in making the diagnosis. Rhee etperformed al. a prospective case-control study on the prevalence of physical signsspondylotic in myelopathy. cervical Thirty-nine patients with cervical spondylotic myelopathy and 37 controls were included. Twenty-one percent of patients with cervical spondylotic myelop- athy did not have any myelopathicexamination signs on onstrates the common signs and symp- toms of cervical spondylotic myelopathy. periods with slow, stepwise decline in func- tion or rapid decline . 1 . Ono et al. used the 2 forms are provided with the online version of the article ( ervical spondylotic myelopa- thy is a condition of neuro- logic impairment resulting from the nar- ). There was no source of external funding for this study. The C term myelopathy hand when describing Conflicts of Interest JBJSREV/A386 Disclosure: Abstract » Many disorders present similar to cervical» spondylotic Mimickers myelopathy. can beathy differentiated through from a cervical detailed spondylotic history myelop- and physical» examination. Differentiating between etiologiesstudies is and aided adjunctive by studies electrodiagnostic resonance using images. radiographs and magnetic rowing secondary to the degeneration of cervical structures. This causes compression gradually. Severity varies across individuals. Progression is typically slow and generates changes in both the cord and the periphery. Cervical spondylotic myelopathy is the most common disorder causing dysfunction of the spinal cord. However, patients may present with similar symptoms caused by various other condi- tions. Pathologic conditions that affect the spinal cord, or neighboring structures, should not be overlooked when evaluating patients with myelopathy. Table I provides other conditions that must be considered. Cervical Spondylotic Myelopathy Cervical spondylotic myelopathy typically presents insidiously and comprises a broad spectrum of . Patients may initially present with axial neck pain or limitations in the neck range of motion Subtle signs such as decreased handterity dex- or mild balance impairment may go unnoticed in early disease. Difficulty opening jars, buttoning shirts, or writing may be clinical indicators of cervicaldylotic spon- myelopathy http://dx.doi.org/10.2106/JBJS.RVW.17.00176 · 2018;6(10):e9

JBJS REVIEWS COPYRIGHT © 2018 BY THE JOURNAL OF BONE AND JOINT SURGERY, INCORPORATED Investigation performed at the University of Toledo Medical Center, Toledo, Ohio Anthony Kouri, MD Mina Tanios, MD Joseph S. Herron, MD Maxwell Cooper, MD Mustafa Khan, MD

Myelopathy Mimickers of Cervical Spondylotic

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TABLE I Differential Diagnosis in Cervical Spondylotic Myelopathy

Mimicker History and Physical Examination Diagnosis

Multiple sclerosis Fatigue, diplopia, bladder disturbances, difficulty 2 lesions in the white matter, 2 episodes in the with gait, unilateral leg numbness disease course, oligoclonal bands in , raised IgG Amyotrophic lateral sclerosis Muscle , weakness, , Hoffman Widespread lower disease on EMG, sign, clonus, Babinski sign, foot drop, loss of bilateral changes within corticospinal tracts on dexterity, difficulty with gait MRI Parkinson disease Slowness of initiation, movement, and thought; On brain MRI, striatum and pallidum will appear postural and resting ; and extrapyramidal normal; on high-field T2-weighted sequences, an rigidity; symptoms will start in 1 limb and, over 2 to 5 altered nigral signal will be demonstrated; surface years, will involve both limbs EMG can be helpful in determining the frequency and amplitude of a Carpal tunnel syndrome Pain in hand, decreased sensation in median nerve Based on history and physical examination, in distribution, reduced grip strength, night equivocal cases, electrodiagnostic studies are symptoms, clumsiness, thenar atrophy in late stages helpful Cubital tunnel syndrome Decreased sensation in the ulnar nerve distribution, Based on history and physical examination, in intrinsic weakness and weakness of the small and equivocal cases, electrodiagnostic studies are ring finger flexor digitorum profundus, Wartenberg helpful; on electrodiagnostic studies, ulnar nerve sign velocity must be ,50 m/s at elbow level HIV Lower-extremity weakness, vague discomfort in SpinalMRIshows extensivecentral high-signalareas legs, gait , erectile dysfunction (men), bowel or on T2, mainly cervicodorsal bladder disturbance HTLV-1 Slowly progressive chronic spastic paraparesis, HTLV-1 antibodies in serum and cerebrospinal fluid spastic bladder Incoordination, pain, absent ankle jerks, impaired High signal on T2 MRI with enhancement of the vibratory sensation, impotence, Romberg sign, spinal surface that disappears Argyll-Robertson pupils, autonomic dysfunction Guillain-Barre´ syndrome Rapidly progressive distal weakness that spreads Illness 7 to 10 days prior; clinical diagnosis, but EMG proximally, severe weakness in shoulder abduction and nerve conduction study demonstrate severe and elbow extension, urinary retention, ileus, sinus neuropathy; cerebrospinal fluid may have raised tachycardia, hyporeflexia, and areflexia protein content Acute transverse Flaccidparaparesis of lowerextremities that can also Focal and central high-signal areas in T2 sequences, involve the arms, well-defined sensory level occupying more than two-thirds of the spinal cord axially, and extending over 3 to 4 segments deficiency Diminished proprioception, decreased vibration Vitamin B12 serum levels; MRI shows T2 sensation, motor weakness, , gait hyperintense signal alterations usually confined to disturbance, intellectual impairment, impaired posterior columns, but may involve lateral columns vision or brain stem Intrinsic weakness, cape-like sensory loss, loss of MRI demonstrating syrinx pain and temperature sensation, hyperreflexia, gait disturbance, diplopia, dizziness Radiation damage Lhermitte sign, Brown-Sequard-type symptoms, Spinal cord segment in irradiated zone, .6- ascending sensorimotor disturbance, tetraplegia month latency period, normal cerebrospinal fluid and , local analysis, local spinal edema, and contrast enhancement on T2 for at least 8 months after latency period Paraneoplastic etiology Insidiously progressive myelopathy; may occur prior Increased protein concentration in cerebrospinal to cancer diagnosis fluid; longitudinally extensive, symmetric, tract- specific signal changes on MRI Vascular etiology Asymmetric leg weakness, worse with walking or Flow voids on MRI, engorged draining medullary standing for long periods veins and feeding arteries within cerebrospinal fluid Hepatic etiology Progressive pure motor spastic paraparesis, minimal Diagnosis of exclusion; decompensated liver sensory deficit disease, post-liver transplant, post-shunt surgery Alcoholic etiology Paresthesias of feet with progressive spastic Alcoholism without substantial liver disease paraparesis, sometimes bladder dysfunction

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Amyotrophic lateral sclerosis is a TABLE II Clinical Presentation of Cervical Spondylotic term that encompasses several adult- Myelopathy onset conditions characterized by pro- Common symptoms gressive motor neuron degeneration. Clumsy or weak hands Amyotrophic lateral sclerosis is also a 11 Leg weakness or stiffness term for one specific disease . Amyo- Neck stiffness trophic refers to the Pain in shoulders or arms disease signs and symptoms. These Unsteady gait include the muscle atrophy, weakness, Common signs and fasciculations seen with this disease. Weakness of the hand musculature Lateral sclerosis refers to corticospinal Hyperreflexia tract hardening from gliosis causing Lhermitte sign (electric shock-like sensation down the center of the signs, including back following flexion of the neck) overactive tendon reflexes, the Hoffman 12 Sensory loss sign, clonus, and the Babinski sign . Lower motor neuron loss causes sub- stantial weakness. Often, the weakness Magnetic resonance imaging In .33% of patients with multiple from lower motor neuron loss is more (MRI) is the imaging standard for cer- sclerosis, moderate to severe lower- disabling than the upper motor neuron vical spondylotic myelopathy because extremity spasticity is seen8.The symptoms12. it evaluates the spinal soft tissues. It is symptom most routinely seen isfatigue, Initially, patients with amyotro- imperative to match the clinical findings which affects 80% of patients. The pa- phic lateral sclerosis have unilateral and with the MRI because it has been re- tients with who are focal symptoms. This begins in the arms ported that up to 57% of patients .65 most affected by fatigue are those with in two-thirds of patients, but can be seen years of age have degenerative disc dis- progressive disease and those who have in the legs as well. Early findings include ease and herniation with no symptoms5. difficulty with ambulation. Fatigue foot drop, difficulty walking, and loss of In diagnosing cervical spondylotic causes substantial morbidity, even in hand dexterity. As the disease progresses, myelopathy, electrodiagnostic studies those patients who are nonambulatory9. patients increasingly require the help of are not typically useful. However, they Chronic neuropathic pain can caregivers. Many patients with amyo- can help to rule out other diagnoses. evolve from sensory disturbances, al- trophic lateral sclerosis lose the ability to Evidence suggests that somatosensory- though this is uncommon. Trigeminal ambulate13. evoked potentials (in 61.9% of cases) also occurs. Optic neuritis is The diagnosis is 95% accurate and motor-evoked potentials (in 71.4% another common symptom and presents when made by experienced clinicians13. of cases) demonstrate changes in the with complete or partial vision loss. Electrodiagnostic studies confirm wide- presence of cervical spondylotic mye- No single diagnostic test exists for spread lower and lopathy findings on MRI6. multiple sclerosis8. To make the diag- exclude other diseases. To rule out upper nosis of multiple sclerosis, there must be motor neuron conditions, advanced Neuromuscular Mimickers 2 separate central white imaging is necessary, and brain and spinal Multiple sclerosis is a chronic auto- matter lesions along with chronic central MRI scans are best. Findings consistent inflammatory central nervous system nervous system . Central with amyotrophic lateral sclerosis dem- disease7,8. It destroys myelin and axons nervous system inflammation is deter- onstrated on a brain MRI scan include to varying degrees by attacking central mined by analyzing the cerebrospinal bilateral signal changes within the corti- nervous system myelinated axons8. The fluid. In addition to these findings, cospinal tracts14. disease course is highly varied, but typ- the patient must have had 2 different Parkinson disease, along with par- ically presents as episodes of reversible symptomatic episodes. kinsonian syndromes, has symptoms deficits, followed by progressive neuro- On cerebrospinal fluid analysis of that are often confused with cervical logic deterioration8. patients with multiple sclerosis, oligo- spondylotic myelopathy. Progressive Initial findings are sensory distur- clonal bands are present in .90% of supranuclear palsy, multiple system bances, most commonly paresthesias, patients and a raised immunoglobulin G atrophy, and corticobasal degeneration dysesthesias, diplopia, incontinence, and index is present in .60% of patients. are parkinsonian syndromes15.The vertigo. Unilateral leg numbness that One additional study that may prove underlying pathology of Parkinson ascends the pelvis, abdomen, or thorax helpful is visually evoked response test- disease is apoptosis in the basal gan- and spreads to the other leg is a common ing. Subclinical optic nerve involvement glia, specifically the substantia nigra’s finding. may be evident in some patients8-10. dopamine-secreting neurons. Also

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contributing is the presence of Lewy caused by median nerve compression at to symptoms of radiculopathy or mye- bodies in the remaining neurons16. the level of the carpal tunnel. Clinically, lopathy. A centrally located lesion may Parkinson disease has variable char- patients report pain in the hand, de- make the distal site more vulnerable to acteristics. Common symptoms include creased sensation in the thumb and compression. Patients with double- resting and postural tremors, extrapy- the index, long, and radial ring fingers, crush syndrome who undergo peripheral ramidal rigidity, and slowness of initia- and weakened grip strength. Night nerve decompression have poorer tion, movement, and thought. Idiopathic symptoms are typically present. Clum- results24,25. Parkinson disease presents later in life. siness while performing tasks isreported. It is important to continually Often, symptoms will start in 1 limb and, In late stages, thenar atrophy appears18. question whether the working diagnosis over the course of 2 to 5 years, will involve Diagnosis can often be made on of a peripheral nerve disorder explains both limbs16.The asymmetry seen in history and examination. Patients may the clinical findings, if a neurologic the early stages of idiopathic Parkinson have positive provocative signs, which decline is observed, or whether a super- disease persists as the disease progresses. can help to differentiate carpal tunnel imposed worsening myelopathy may Early in the disease course, postural syndrome from cervical spondylotic coexist. Early diagnosis is aided by as- reflexes remain intact; however, patients myelopathy. If examination is equivocal, sessing spinothalamic and posterior ultimately have difficulty with balance. electrodiagnostic studies can be helpful column sensation and assessing these A tremor is typically present in 40% of in establishing the site of compression19. sensory modalities for proximal and dis- patients initially, and many of those Ulnar nerve entrapment at the talportionsof thelimbs.Hyperreflexia of patients demonstrate an asymmetric rest elbow, called cubital tunnel syndrome, muscles distal and proximal should also tremor. The rigidity seen in Parkinson can mimic myelopathic hand, and is be assessed. Electrodiagnostic studies disease is described as a lead pipe, the second most common entrapment and MRI are helpful in making the meaning constant throughout full neuropathy. With cubital tunnel syn- diagnosis22,25. motion. When a postural tremor is drome, patients experience decreased overlaid on the rigidity, it becomes sensation in the ulnar nerve distribution. Infectious and Immune- ratchet-like, and is known as cogwheel As compression worsens, motor dys- Mediated Mimickers rigidity. Approximately half of all patients function begins. Intrinsic weakness and Timely diagnosis is imperative in infec- with Parkinson disease report slowing in weakness of the small and ring finger tious . In parainfectious their maneuvering. Additionally, writ- flexor digitorum profundus cause catch- myelopathy, infection and an associated ing becomes smaller and patients de- ing when reaching into pockets20.Thisis immune reaction cause neurologic velop difficulty with activities of daily from overpowering of the small finger damage. In the context of infectious living in the later stages of the disease. As extensor without intrinsic opposition, myelopathies, many different organisms Parkinson disease progresses, patients causing abduction, or the Wartenberg may be the source. These include Trepo- begin to have a nonspecific feeling tak- sign. In cervical spondylotic myelopathy, nema pallidum,Mycobacterium tuberculosis, ing longer to get around. This eventually insufficiency of finger adduction and/or herpes viruses, human immunodeficiency leads to aslowed gait. While ambulating, extension startsandisgreatestintheulnar virus (HIV), human T-cell lymphotropic a 1-sided decreased arm swing is often digits21. virus type I (HTLV-I), enteroviruses, present. Later in the disease course, Radiographs should be obtained fungi, and parasites. Some of the more shuffling develops. This is followed by to evaluate cervical spine degeneration. common organisms are discussed below. difficulty initiating gait and spontaneous Electrodiagnostic studies are helpful in HIV-associated vacuolar myelop- acceleration15. establishing the site of compression. For athy is the most common form of HIV In Parkinson disease, laboratory re- a finding to be positive for cubital tunnel myelopathy. Patients report lower- sults are normal. On a brain MRI scan, the syndrome, the ulnar nerve velocity extremity weakness, difficulty with striatum and pallidum will appear normal. should be ,50 m/s at the level of the balance and gait, and bladder dysfunc- However, on T2-weighted sequences, an elbow20. tion26. Some have indistinct discomfort altered nigral signal will be demonstrated. Peripheral nerve disorders, either in the lower extremities as well as par- Typically, electromyograms are not helpful peripheral nerve entrapment or poly- esthesias. A hallmark of HIV-associated for Parkinson disease. However, a surface neuropathy, can mask signs of mye- vacuolar myelopathy is erectile dys- electromyogram can be helpful if there is lopathy such as sensory loss, intrinsic function in men27. doubt concerning the frequency and am- muscle atrophy, and clumsiness22. This On examination, spastic parapare- plitude of a tremor17. combination of peripheral and central sis is present, with weakness that exceeds nerve disorders has been referred to as spasticity. Other symptoms that may Peripheral Nerve Entrapment double-crush syndrome or multifocal been seen are greater weakness in 1 leg, Carpal tunnel syndrome is the most neuropathy, in which the nerve is com- monoparesis, quadriparesis, gait ataxia, common entrapment neuropathy. It is pressed at multiple sites23. This can lead or . On examination,

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weakness may be present or absent. sensation. The course of disease com- most patients with Guillain-Barr´e syn- However, hyperreflexia of the lower prises 3 phases. The first stage is called drome have findings of hyporeflexia or extremities and a Babinski sign are typ- the pre-ataxic phase, or period of areflexia, normal or brisk reflexes are ically present28. lightning pain. Early findings include present in a small subset of patients37. Spinal MRI may be helpful in impotence and sphincter disturbances. Seventy-five percent of patients making a diagnosis. Diffuse central Examination demonstrates muscle have a preceding illness, which may be high-signal areas in T2 sequences in stretch reflex absence, sensory distur- mild or asymptomatic. Often, the illness the cervicodorsal region are seen. Brain bance, Argyll-Robertson pupils, and is respiratory or gastrointestinal in nature. MRI is often not helpful; however, a positive Romberg sign. The second Typically, the neuropathy begins 7 to 10 some abnormalities may be seen and phase is the ataxic phase, characterized days after infection37.Inmostcases,the include white matter changes similar by worsening tabetic pain and severe patient has an acute neuropathy, which to the findings in acute demyelinating lower-extremity ataxia. The third stage is peaks within 4 weeks. These patients encephalopathy28. called the terminal or paralytic stage. typically have weakness, diminished re- The incidence of HTLV-I has Spastic paraparesis, , and auto- flexes, and raised cerebrospinal fluid increased in recent years because the nomic dysfunction are typical charac- protein concentrations. disease has been found in many intra- teristics. This includes severe constipation Because of the rapid onset of the venous drug abusers. In addition to chronic and bladder incontinence33. neuropathy, and associated weakness progressive myelopathy, HTLV-1 has Syphilitic myelopathy appears as a in someone recovering from illness, pa- been associated with adult T-cell leuke- high signal on a T2-sequence MRI, with tients may be incorrectly diagnosed with mia and lymphoma29. spinal surface enhancement that disap- a psychological disorder. Patients typi- Tropical spastic paraparesis (TSP) pears, signifying that it is reversible33. cally reach a plateau phase, which can or HTLV-I-associated myelopathy Guillain-Barr´esyndromeisan vary in length of time. Following the (HAM) are terms that describe HTLV-I acute autoimmune disorder, in which plateau, recovery begins with return myelopathy. Often in HAM/TSP, the immune system attacks the periph- of proximal, then distal, strength over patients will have chronic spastic para- eral nervous system, resulting in neu- weeks or months. Despite modern that is gradually progressive and ropathy. It is the leading cause of flaccid advances, this remains a dangerous dis- spastic bladder30,31. Patients may also in the world today. Guillain- ease, with 4% to 15% of patients dying, experience paresthesias, decreased sen- Barr´e syndrome expresses in several dif- and up to 20% remaining disabled after sation, and pain. Chronic pyramidal ferent ways. It presents as a spectrum a year36,37. There is a large differential tract involvement is present. involving varying peripheral-nerve dis- diagnosis and the correct diagnosis is The HAM/TSP diagnosis is based orders and is characterized by the dis- reliant upon the clinician recognizing on laboratory and clinical evidence, tribution of weakness of the limbs or that the problem is an acute peripheral including HTLV-1 antibodies in serum cranial-nerve-innervated muscles, path- neuropathy, not central. and cerebrospinal fluid. A very effective ologic abnormalities, and findings of Electrodiagnostic studies can be method of diagnosing HAM/TSP is by autoantibodies on testing34,35. helpful in characterizing the neuropa- combining cerebrospinal fluid HTLV-I The onset of disease presents with thy. A lumbar puncture is used to rule DNA and the intrathecal synthesis of sensory changes in the legs, followed by out infection or malignancy, such as antibodies to HTLV-I32. rapidly progressive weakness distally lymphoma37. However, raised cerebro- Within the first year, the infectious that spreads proximally. Another com- spinal fluid protein content may be seen agent of syphilis, Treponema pallidum, mon finding is lumbar pain, which may with Guillain-Barr´e syndrome. can occupy the central nervous system. represent nerve root inflammation. The Acute is an Spinal syphilis has variable manifesta- weakness is typically pyramidal, with inflammatory disorder of the spinal tions. is the most common knee and hip flexion and ankle dorsi- cord. The term acute transverse myelitis form, followed by meningomyelitis, and flexion being severely affected. The is occasionally used synonymously with finally spinal vascular syphilis. Histori- weakness in the upper extremities is non-compressive myelopathy. How- cally, syphilis was the most common worse in shoulder abduction and elbow ever, the term non-compressive mye- cause of spinal cord disease. However, it extension. Sensation loss is typically lopathy covers a diverse group. At the has become much rarer since the advent minimal; however, vibratory sensation level of the spinal cord, the entire cross- of antibiotics33. and proprioception may be affected36. sectionalareaat1levelisinvolved, Tabes dorsalis presents with pain, In Guillain-Barr´e syndrome, autonomic including pyramidal tracts, spinothala- sensory disturbances, incoordination, involvement is common. This can cause mic tracts, and posterior columns. and visceral trophic abnormalities33. hypertension, ileus, postural hypoten- There are multiple different non- Findings commonly observed are absent sion, urinary retention, sinus tachycar- compressive causes of acute transverse ankle reflexes and decreased vibratory dia, and cardiac arrhythmia37. Although myelitis. These include but are not

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limited to paraneoplastic causes, ante- 4 segments. In 40% of cases, no MRI The classic symptoms associated rior spinal artery thrombosis, and findings are present41. with Chiari malformation include vasculitis in autoimmune diseases. upper-limb weakness and cape-like sus- Autoimmune diseases with associated pended sensory loss50. The hand weak- vasculitis include systemic eryth- Vitamin B12 deficiency can cause sev- ness begins in the intrinsic musculature ematosus, mixed connective tissue dis- eral different disorders42. Typical causes and spreads proximally. Although classic ease, and scleroderma. Other causes are of vitamin B12 deficiency include pan- findings, these are rare since the advent infections such as herpes zoster, rubella, creatic insufficiency, medications, and of MRI. HIV, Epstein-Barr virus, Mycoplasma malabsorption42-44. A rare, but notable, In the early stages, there may be pneumoniae, Echo-25 virus, and mumps38. cause of acute vitamin B12 inactivation progressively worsening neurologic def- It may follow cholera vaccination, is exposure45. icits. In some cases, there is an acute loss typhoid, and poliomyelitis and has been The neurologic symptoms present of spinal cord function50,51,53. The ear- reported with penicillin injections, her- in vitamin B12 deficiency include auto- liest symptoms are often gait disturbance oin abuse, sulfasalazine chemotherapy, nomic dysfunction, gait disturbance, par- or radiculopathy with associated numb- B-cell lymphoma, myelomonocytic esthesias, diminished proprioception and ness or weakness in one arm. The hands leukemia, and general anesthesia or vibratory sensation, weakness, hyperre- may be especially affected. The sensory acupuncture38. flexia, areflexia, intellectual or behavioral symptomsofcape-likesensorylossappear A typical presentation of acute impairment, and impaired vision44. late, typically once spinal cord dysfunc- transverse myelitis includes leg weakness Vitamin B12 deficiency primarily tion becomes obvious. On examination, that appears suddenly, diminished sen- causes deficiency of the posterior col- leg weakness may be present, as well as sation with loss of sphincter control, and umns, but also affects the anterolateral spasticity, hyperreflexia, and upper-limb pain with no obvious spinal cord com- and anterior portions of the spinal cord weakness and wasting. Patients may pression. The weakness is typically a as well. This is known as subacute com- experience sensory loss in dissociated progressive flaccid paraparesis, and it can bined degeneration. It is most commonly patterns, with preservation of touch and involve the upper extremities. seeninthecervicalandthoracic cord, and position sense, but loss of pain and Generally, by the second week of includes a diffuse, multifocal pattern of temperature sensation49. illness, pyramidal signs appear. Within demyelination and axonal loss44. Fur- 10 days of onset, most patients, about thermore, myelin sheath swelling and a Radiation Damage 80%, reach their clinical low-point. patchy myelopathic spongy vacuolation A rare but devastating complication of During the acute phase, patients’ neu- of affected regions of the cord are typi- radiation therapy is radiation-induced rologic function usually deteriorates cally seen46,47. myelopathy. It is seen with previous progressively. This typically occurs over On MRI, subacute combined exposure to head and neck irradiation54. a period of 4 to 21 days39. degeneration presents as a T2 hyperin- Radiation therapy can cause central The defined sensory level is be- tense signal that is usually confined to nervous system at 3 different tween T5 and T10 in 70% of cases. the posterior columns, but may include points in time. During radiation ther- There is a cervical sensory level in about the lateral columns and brainstem46. apy, patients can experience a transient 20% of cases. Adults with acute trans- increase in from blood- verse myelitis note paresthesias at the Syringomyelia brain-barrier disruption. This is an acute outset of the disorder. In a large percent- Syringomyelia is a rare condition, in radiation injury. Weeks to months after age of children (approximately 40%), an which fluid-filled areas along the spinal the administration of radiation therapy, illness occurs within 3 weeks of the onset cord develop slowly. This is also known early-delayed radiation injury can occur. of acute transverse myelitis40. as a syrinx. Causes may be congenital Finally, transient demyelination can The diagnosis of transverse myeli- or acquired. These include trauma, occur following cervical spine irradiation. tis is one of exclusion, and the time tuberculosis-associated chronic arach- This primarily affects the posterior col- course lasts from 4 hours to 4 weeks. noiditis, and tumors. Chiari malfor- umns, causing Lhermitte syndrome55. Transverse myelitis criteria includes mation causes most nontraumatic This final stage presents like chronic pro- spinal cord dysfunction bilaterally with a forms48,49. Chiari malformation is gressive Brown-S´equard syndrome and defined sensory level and no compres- a congenital abnormality in which lasts 3 months to 5 years54. sive history40. On MRI, there are find- the cerebellar amygdalae herniates The presentation is slowly pro- ings specific to acute transverse myelitis, through the foramen magnum. This gressive ascending sensorimotor distur- which makes it helpful for diagnosis. causes altered cerebrospinal fluid flow, bance including tetraplegia or paraplegia, On T2 axial cuts, central high-signal which can lead to syrinx formation50,51. with bowel and bladder disturbances55. areas occupy more than two-thirds of Patients may experience diplopia, head- Thesensory loss often resolves within2 to the spinal cord and extend over 3 to aches, dizziness, and arm weakness52. 36 weeks.

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Tomake the diagnosis of radiation- nal changes specific to tracts within the included in the evaluation. This is the induced myelopathy, the affected spinal spinal cord that enhance with adminis- gold standard57. cord segment must be in the irradiated tration of gadolinium. Often, increased zone, the symptoms must correspond cerebrospinal fluid protein concentra- Hepatic Etiology to the involved spinal cord segment, tion is present56. Hepatic myelopathy is a neurologic and there must be a latency period condition that is seen in those with of .6 months55. Cerebrospinal fluid Vascular Etiology chronic liver disease. In patients with analysis is typically normal54.MRI Spinal cord vascular malformations may evident liver failure or patients who shows a local spinal high T2-sequence cause myelopathy. This presents with have a surgical or spontaneous sys- signal at least 8 months after the end of nonspecific symptoms, often distal to temic portocaval shunt, a progressive the latency period. Subsequently, signal the malformation. Early treatment offers pure motor spastic paraparesis may intensity is normal but severe atrophy the best chance for recovery57. develop61. remains54. Spinal vascular malformations These patients typically have min- are categorized on the basis of anatomy imal sensory deficit or bowel and bladder Paraneoplastic Etiology and location. The most common are involvement. Many of these patients Paraneoplastic syndromes are symptoms dural arteriovenous fistula malforma- experience episodes of hepatic enceph- that occur in conjunction with a tumor tions58. An arteriovenous fistula mal- alopathy. Occasionally, following the or metastases. They are rare, but essen- formationisdural,orextraspinal,in creation of surgical shunts, myelopathy tial considerations when evaluating 75% of cases57. In 10% of cases, in- can develop61-64. myelopathy. Symptoms may occur traspinal arteriovenous fistula malfor- It is imperative to recognize the before any cancer is suspected. The mations are present. Twelve percent of circumstances under which hepatic myelopathy due to paraneoplastic etiol- spinal malformations are cavernous myelopathy occurs. Early recognition is ogy is normally insidiously progressive. malformations58. important because patient liver trans- Rarely, patients undergo a relapsing- Initial symptoms include gait dis- plantation can reverse the myelopa- remitting course56. orders, paresthesias, numbness, radicu- thy65. Most cases of hepatic myelopathy Paraneoplastic syndromes are lopathy, asymmetric lower-extremity are in patients who have decompensated caused by several factors, including weakness, and bleeding in 25% of cases. liver disease66. Hepatic myelopathy is a antibodies and neurochemical factors. The symptoms of myelopathy from diagnosis of exclusion and has also been Subacute myelopathy can be associ- spinal cord arteriovenous fistula mal- associated with congenital hepatic fibro- ated with antibodies present with lung, formations are exacerbated with walking sis67, acute hepatitis E, and childhood breast, prostate, ovarian, and thyroid or standing for long periods59. portal vein thrombosis68. cancers and Hodgkin lymphoma. For Another cause of vascular mye- The neuropathology of hepatic instance, patients with small-cell lung lopathy is acute vascular occlusion, myelopathy demonstrates demyelina- carcinoma can develop autoimmunity which causes an infarct57. The initial tion in the lateral corticospinal tracts70. to collapsing response mediator protein symptoms include severe motor and Evidence suggests that motor-evoked 5 (CRMP5). Autoimmunity to this sphincter dysfunction, pain, and tem- potentials may be used to make an early antibody leads to myelopathy and optic perature changes, with no loss of pro- diagnosis of hepatic myelopathy61,65. neuropathy, which mimics neuromye- prioception or vibratory sensation. litis optica. Other paraneoplastic con- These symptoms present within 4 Alcoholic Etiology ditions mimic myelopathy, but are due hours. Myelopathy associated with alcoholism to neurochemical mediators. One such In the acute stage, diagnosing occurs in patients who heavily consume neurochemical mediator is glutamate myelopathy due to cord ischemia is alcohol and have cirrhosis with porto- decarboxylase 65 (GAD65). Autoim- challenging because of a lack of diag- systemic shunting69,70. However, Sage munity to GAD65 causes stiff person nostic criteria. It is generally found in et al. proposed another syndrome of syndrome. The associated with patients .50 years of age59,60. To define progressive posterior and lateral column this condition may mimic spasticity. the spinal arteriovenous fistula malfor- dysfunction in alcoholics without sub- Additionally, anti-amphiphysin anti- mation nidus on MRI, flow voids must stantial liver disease71,72. Symptoms bodies, occasionally present in stiff per- be visible in the form of decreased signals typically begin with paresthesias of the son syndrome, cause rigidity and on T1 and T2-weighted images. On T2 feet and progress to a spastic parapa- , which may mimic the images, engorged draining medullary resis. Patients may also present with spasticity seen in cervical spondylotic veins and feeding arteries are demon- bladder dysfunction69. Abstention from myelopathy55,56. strated. In patients suspected of having alcohol usually prevents further pro- The pathognomonic finding on spinal arteriovenous fistula malforma- gression, but is not usually followed by MRI is longitudinal and symmetric sig- tions, spinal angiography must be improvement73.

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TABLE III Similarities and Differences Between Mimickers and Cervical Spondylotic Myelopathy

Mimicker Similarities to Cervical Spondylotic Myelopathy Differences from Cervical Spondylotic Myelopathy

Multiple sclerosis Bladder disturbances, difficulty with gait, Fatigue, diplopia, unilateral leg numbness, reversible hyperreflexia, arm numbness and pain deficits, vertigo, optic neuritis with loss of vision Amyotrophic lateral sclerosis Muscle atrophy, weakness, Hoffman sign, clonus, Foot drop, fasciculations, difficulty speaking or Babinski sign, loss of dexterity, difficulty with gait impaired voice, difficulty swallowing, severe unintentional weight loss Parkinson disease Gait slowing, poor balance, neck tightness, reduced Asymmetric tremor at rest, shuffling gait, arm swing extrapyramidal rigidity, impaired voice, soft speech, difficulty swallowing, reduced facial expression, small handwriting, involuntary movements Carpal tunnel syndrome Hand numbness or paresthesias in median Absence of upper motor neuron signs, symptoms distribution, reduced grip strength, thenar atrophy, isolatedto medianhand,positiveprovocativesigns on clumsiness examination Cubital tunnel syndrome Hand numbness or paresthesias in ulnar distribution, Absence of upper motor neuron signs, symptoms reduced grip strength, intrinsic muscle atrophy, isolated to ulnar hand or forearm, positive provocative Wartenberg sign signs on examination HIV Gait ataxia, bowel and bladder disturbance, Lower-extremity weakness, erectile dysfunction hyperreflexia (men), distal limb pain and numbness, slowly progressive spastic paraparesis HTLV-1 Paresthesias, difficulty walking, sensory disturbances, Slowly progressive chronic spastic paraparesis; painful spastic bladder, hyperreflexia stiffness and weakness of legs Treponema pallidum Incoordination, , paresthesias Absent ankle jerks, impaired vibratory sensation, impotence, Romberg sign, vision change, Argyll- Robertson pupils, autonomic dysfunction Guillain-Barre´ syndrome Difficulty with coordination, neck pain, severe Rapidly progressive distal weakness that spreads weakness in upper extremity, urinary retention proximally, ileus, sinus tachycardia, hyporeflexia and areflexia, weakness of facial muscles Acute transverse myelitis Well-defined sensory level, extremity weakness and Flaccid paraparesis of upper and lower extremities, paresthesias, hyperreflexia autonomic dysfunction, lower-extremity paresthesias, difficulty flexing legs and extending arms Vitamin B12 deficiency Diminished proprioception, motor weakness, Decreased vibration sensation, intellectual hyperreflexia, gait disturbance impairment, impaired vision Syringomyelia Intrinsic weakness, hyperreflexia, gait disturbance, Cape-like sensory loss, loss of pain and temperature loss of sensation in hands sensation, diplopia, trigeminal nerve sensory loss, dizziness, neuropathic arthropathy Post-radiation damage Lhermitte sign, weakness of extremities, muscle Brown-Sequard syndrome, ascending sensorimotor atrophy disturbance Paraneoplastic etiology Rigidityor myoclonus, bladderdysfunction, weakness, Optic neuropathy, rarely relapsing remitting course difficulty with ambulation Vascular etiology Gait disturbance, paresthesias, radiculopathy Asymmetric leg weakness worse with walking or standing for long periods Hepatic etiology Minimal sensory deficit, no bowel or bladder Progressive pure motor spastic paraparesis dysfunction Alcoholic etiology Muscle weakness, muscle, atrophy, hyperreflexia, Paresthesias of feet with spastic paraparesis of lower bladder dysfunction extremities, numbness of lower extremities

Differential Workup causes, electrodiagnostic studies, cere- Summary While evaluating a patient with myelo- brospinal fluid analysis, and laboratory The most common cause of non- pathic symptoms, one must rule out analysis. However, a thorough history and traumatic spastic paraparesis and quad- mimickers, especially in unusual or physical examination remain the most riparesis in the United States is cervical equivocal cases. A comprehensive critical components in the myelopathy spondylotic myelopathy. This is due to workup should include an MRI of the workup. Table III lists the similarities and degenerative changes of the cervical brain and spinal cord, spinal angiogra- differences between mimickers and cer- spinal column. Although this is the most phy in those with suspected vascular vical spondylotic myelopathy. common cause of myelopathy, several

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