ﺑﺎﺯﮔﺸﺖ ﺑﻪ ﺻﻔﺤﻪ ﻗﺒﻠﯽ
خرید پکیج
تعداد آیتم قابل مشاهده باقیمانده : 3 مورد
نسخه الکترونیک
medimedia.ir

Disorders affecting the spinal cord

Disorders affecting the spinal cord
Literature review current through: May 2024.
This topic last updated: Feb 22, 2024.

INTRODUCTION — Pathologies that affect the spinal cord are diverse. In addition to trauma, common etiologies of myelopathy include autoimmune, infectious, neoplastic, vascular, and hereditary-degenerative diseases. The relative incidence of each of these entities depends in large part upon the clinical setting. In a regional neuroscience center in the United Kingdom, the most common cause of spastic paraparesis or quadriparesis among 585 patients was cervical spondylotic myelopathy (24 percent), followed by tumor (16 percent), multiple sclerosis (MS; 18 percent), and motor neuron disease (4 percent) [1].

This topic will review some of the more common and important causes of nontraumatic spinal cord dysfunction. Clinical features of the more common of these disorders are summarized in the table (table 1). Traumatic spinal cord injury and the anatomy and clinical localization of spinal cord disease are discussed separately. (See "Acute traumatic spinal cord injury" and "Anatomy and localization of spinal cord disorders".)

DIAGNOSTIC APPROACH — The differential diagnosis of myelopathy is wide but can be significantly narrowed by the clinical syndrome, which is defined by features on examination. These are summarized in the table (table 2) and described in detail separately. (See "Anatomy and localization of spinal cord disorders", section on 'Spinal cord syndromes'.)

Other features in the examination and history can also limit the differential diagnosis and tailor the diagnostic workup. Clinical features of some of the more common causes of myelopathy are outlined in the table (table 1). These are described in detail in the sections below.

For patients with a clinical syndrome that suggests a localized process within the spinal cord (eg, transection syndrome, central cord syndrome, ventral cord syndrome, etc), an imaging study, usually magnetic resonance imaging (MRI), of the relevant section of the spinal cord is usually required [2,3]. Administration of gadolinium contrast is often helpful. When an infectious or inflammatory disorder is suspected, cerebrospinal fluid (CSF) examination may be helpful [4]. The role of positron emission tomography in evaluating patients with myelopathy is under investigation; it appears to be particularly sensitive for neoplastic disease [5].

In general, the pace at which spinal cord deficits appear dictates the urgency of the neurologic evaluation. Even when the deficits are not severe, acute myelopathic signs need to be evaluated urgently because neurologic deterioration can occur abruptly, and the clinical deficit at the time of intervention often dictates the chances of recovery. This is true particularly for compressive etiologies such as spinal cord metastases and epidural spinal abscess.

INFLAMMATORY DISEASES

Transverse myelitis — Transverse myelitis (TM) is a segmental spinal cord injury caused by acute inflammation [6-8]. TM is uncommon, with an approximate incidence of between one to five cases per million population annually [9].

Most cases of TM are idiopathic and presumably result from an autoimmune process; up to half of these patients have a preceding infection [10-12]. TM can also occur as part of other inflammatory demyelinating diseases, including multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), and acute disseminated encephalomyelitis (ADEM) [13]. (See "Transverse myelitis: Etiology, clinical features, and diagnosis", section on 'CNS demyelinating disorders'.)

TM is also associated with connective tissue diseases, including systemic lupus erythematosus, Sjögren's disease, antiphospholipid antibody syndrome, ankylosing spondylitis, and others. (See "Transverse myelitis: Etiology, clinical features, and diagnosis", section on 'Systemic autoimmune disorders'.)

The inflammation of TM is generally restricted to one or two segments, usually in the thoracic cord. Symptoms typically develop rapidly over several hours; approximately 37 percent of patients worsen maximally within 24 hours [14]. Occasionally patients worsen more slowly, over several weeks. Typically the inflammation is bilateral, producing weakness and multimodality sensory disturbance below the level of the lesion [9,11]. Unilateral syndromes (eg, Brown-Séquard) have been described as well. Almost all patients develop leg weakness of varying severity. Arm weakness also occurs if the lesion is in the cervical cord. In addition to diminished sensation, pain and tingling are common and frequently include a tight banding or girdle-like sensation around the trunk, which may be very sensitive to touch. Back and radicular pain are also common. Bowel and bladder dysfunction, reflective of autonomic involvement, also occur. (See "Transverse myelitis: Etiology, clinical features, and diagnosis", section on 'Clinical features'.)

MRI of the involved section of the spinal cord shows gadolinium-enhancing signal abnormality, usually extending over one or more cord segments (image 1) [9,12,15,16]. The cord often appears swollen at these levels. Cerebrospinal fluid (CSF) is abnormal in half of patients, with elevated protein level (usually 100 to 120 mg/100 mL) and moderate lymphocytosis (usually <100/mm3). Glucose levels are normal. Oligoclonal bands are usually not present in isolated TM, and when present suggest a higher risk of subsequent MS [9,17]. Other studies can help delineate the underlying cause (table 3). (See "Transverse myelitis: Etiology, clinical features, and diagnosis", section on 'Determining the cause of TM'.)

Patients presenting with acute transverse myelitis are typically treated with glucocorticoids and other immunosuppressive therapies. Most patients have at least a partial recovery. Long-term outcomes vary by the underlying condition. (See "Transverse myelitis: Treatment and prognosis".)

Sarcoidosis — The granulomatous inflammation of sarcoidosis can affect the spinal cord and produce an acute or subacute segmental myelopathy [10,18-20]. The lesions can be extramedullary or intramedullary, and can involve the cauda equina as well as the cord. MRI signal abnormalities are not specific; neurosarcoid lesions can appear similar to TM or can resemble a tumor. CSF evaluation usually shows elevated protein and/or pleocytosis. Hilar lymphadenopathy may suggest the diagnosis; however, serum and CSF angiotensin converting enzyme levels are neither sensitive nor specific for neurosarcoidosis. Patients with neurologic sarcoidosis are generally treated with corticosteroids and other immunomodulatory agents and can improve. (See "Neurologic sarcoidosis".)

Paraneoplastic syndromes — A number of distinct paraneoplastic syndromes involving the spinal cord have been described. These rare syndromes include:

Motor neuron syndrome – A subacute, progressive, painless, and often asymmetric lower motor neuron weakness, most often associated with lymphoma [21].

Acute necrotizing myelopathy – A rapidly ascending syndrome of sensory deficits, sphincter dysfunction, and flaccid or spastic paraplegia or quadriplegia [22].

Subacute sensory neuronopathy – An inflammatory disorder affecting the dorsal root ganglia, producing progressive loss of sensory modalities, leading to prominent ataxia [23]. This is most often associated with small cell lung cancer and anti-Hu antibodies.

Encephalomyelitis – A diffuse involvement of brain and spinal cord regions in which cerebral manifestations frequently overshadow the myelopathy. Several syndromes are described.

These are described separately. (See "Paraneoplastic syndromes affecting spinal cord, peripheral nerve, and muscle".)

INFECTIONS

Epidural abscess — Spinal epidural abscess is a rare disease, occurring in only 1 patient per 10,000 admitted to the hospital [24]. The infection can originate via contiguous spread from infections of skin and soft tissues or as a complication of spinal surgery and other invasive procedures, including indwelling epidural catheters. Other cases of epidural abscess arise from a remote site via the bloodstream. Diabetes, alcoholism, and human immunodeficiency virus (HIV) infection are risk factors.

The most common pathogen is Staphylococcus aureus, which accounts for approximately two-thirds of cases [24]. Damage to the spinal cord can be caused by direct compression of neural elements or arterial blood supply, thrombosis and thrombophlebitis of nearby veins, focal vasculitis, and/or bacterial toxins and mediators of inflammation. The classic clinical triad consists of fever, spinal pain, and neurologic deficits. However, only a few patients have all three components at presentation [25]. The rate of neurologic progression is highly variable.

Imaging of the spinal column is imperative on first suspicion of the disorder [25]. MRI is the preferred test and is highly sensitive for this diagnosis. Blood cultures and/or aspirate of abscess contents are ordered to identify the etiologic organism. Surgical decompression and drainage with systemic antibiotic therapy is the treatment of choice for most patients. Because the preoperative neurologic deficit is an important predictor of final neurologic outcome, early diagnosis and treatment are imperative.

This topic is discussed in detail separately. (See "Spinal epidural abscess".)

Acute viral myelitis — Two distinct syndromes of spinal cord involvement are associated with acute viral disease. In the first, the virus targets the gray matter of the spinal cord, producing acute lower motor neuron disease [26]. These viruses include:

Enteroviruses, such as poliovirus, coxsackie virus, and enterovirus 71 [27]

Flaviviruses, such as West Nile virus and Japanese encephalitis virus [28-30]

Viral invasion of the anterior horn cells occurs as part of an acute viral illness, usually with fever, headache, and meningismus, and produces asymmetrical flaccid weakness with reduced or absent reflexes and few sensory symptoms or signs. MRI often shows hyperintensities in the anterior horns of the spinal cord on T2-weighted imaging [27,31]. Cerebrospinal fluid (CSF) analysis demonstrates a moderate pleocytosis. These features help to distinguish this form of viral myelitis from Guillain-Barré syndrome, which usually produces symmetric deficits, with no MRI abnormalities, and is associated with elevated CSF protein levels without pleocytosis. (See "Guillain-Barré syndrome in adults: Pathogenesis, clinical features, and diagnosis".)

Focal or segmental depletion of spinal motor neurons has been described at autopsy, reflecting the observed pattern of neurologic deficits [32,33]. The prognosis for recovery is variable. Treatment is supportive. (See "Clinical manifestations and diagnosis of West Nile virus infection", section on 'Neuroinvasive disease' and "Enterovirus and parechovirus infections: Clinical features, laboratory diagnosis, treatment, and prevention", section on 'Acute paralysis and brainstem encephalitis'.)

A second form of viral myelitis has clinical and diagnostic test features that are similar to transverse myelitis. Associated viruses include cytomegalovirus, varicella zoster, herpes simplex virus, hepatitis C, and Epstein Barr virus [34-40]. The association between the myelitis and the virus is not always clear. In some cases, these may represent a postinfectious transverse myelitis. In others, a positive polymerase chain reaction (PCR) test in the CSF suggests that the myelitis is directly related to the viral infection. These patients are often treated with antiviral agents and corticosteroids.

AIDS myelopathy — HIV infection produces a vacuolar myelopathy, which is found in up to half of patients with acquired immunodeficiency syndrome (AIDS) at autopsy [25,41]. However, clinical manifestations occur when the pathology is advanced, and only approximately one-fourth of patients demonstrating vacuolar myelopathy at autopsy have symptoms during life [42]. AIDS myelopathy most often occurs in late stages of AIDS; most patients die within six months of developing symptoms of myelopathy. HIV-related dementia is present in more than half of patients and, with other disease complications, may obscure the myelopathy.

In typical cases, a slowly progressive spastic paraparesis is accompanied by loss of vibration and position sense and urinary frequency, urgency, and incontinence [43]. Upper-extremity function is usually normal. MRI of the spine is usually normal. CSF examination may show nonspecific abnormalities, such as protein elevation. Abnormal sensory evoked potentials may precede clinical symptoms of myelopathy. Aggressive antiretroviral therapy can lead to improvement of symptoms [44]. In one case series, the use of intravenous immunoglobulin appeared to be associated with neurologic improvement [45].

The pathogenesis of vacuolar myelopathy is unknown but may be related to abnormal transmethylation mechanisms induced by the virus and/or cytokines. In one series of 16 patients, there was no correlation between the viral load in the CSF and the presence or severity of myelopathy [46]. Pathologic descriptions include demyelination of the dorsal columns and the dorsal half of the lateral columns, with prominent vacuoles within the myelin sheaths. This pathologic appearance is similar to the changes seen in the subacute combined degeneration of the cord.

HTLV-I myelopathy — Human T-lymphotropic virus type I (HTLV-I) causes a progressive neurologic disease, which is called either HTLV-1-associated myelopathy (HAM) or tropical spastic paraparesis (TSP) [47,48]. This disorder is endemic in southern Japan, the Caribbean, South America, the Melanesian islands, Papua New Guinea, the Middle East, and central and southern Africa, with seroprevalences as high as 30 percent in southern Japan [49-51]. By contrast, it is quite rare in the United States and Europe. It is more common in women than men.

Primarily involving the thoracic cord, HAM/TSP is characterized by a slowly progressive spastic paraparesis and urinary disturbance. Pathologic studies demonstrate inflammation of the lateral corticospinal, spinocerebellar, and spinothalamic tracts, with relative sparing of the posterior columns [52]. HAM/TSP has also been associated with other nervous system pathology that results in less frequent symptoms, suggesting cerebral, cerebellar, cranial nerve, and peripheral nerve involvement [47,51].

MRI of the spinal cord may show spinal atrophy, particularly of the thoracic cord [48,53]. A brain MRI often shows subcortical, periventricular white matter lesions. CSF examination reveals a mild lymphocytosis and/or elevated protein concentration in some patients. Anti-HTLV-I antibodies are detected in the CSF with a high CSF:serum ratio. The virus can be cultured from CSF lymphocytes and proviral deoxyribonucleic acid (DNA) detected by PCR.

In general, neurologic deficits continue to progress slowly; steroids and other immunomodulatory treatment may slow progression, but this is not proven. This topic is discussed separately. (See "Human T-lymphotropic virus type I: Disease associations, diagnosis, and treatment" and "Human T-lymphotropic virus type I: Virology, pathogenesis, and epidemiology".)

Syphilis — Tabes dorsalis is a form of tertiary neurosyphilis in which the dorsal or posterior columns of the spinal cord are primarily affected. Patients present with a sensory ataxia and lancinating pains reflecting dorsal column and dorsal nerve root involvement. CSF examination may be normal or demonstrate elevated protein level, lymphocytosis, and/or a reactive Venereal Disease Research Laboratory (VDRL). Antibiotic treatment may reverse symptoms. (See "Neurosyphilis", section on 'Tabes dorsalis'.)

Syphilitic meningomyelitis and meningovascular myelitis represent an earlier form of syphilis infection in which focal inflammation of the meninges secondarily affects the adjacent spinal cord and/or the anterior spinal artery. In the former situation, a subacute progressive myelopathy develops [54]. In the latter, the clinical presentation may be one of a spinal cord infarction. (See "Neurosyphilis", section on 'Meningovascular syphilis' and "Spinal cord infarction: Epidemiology and etiologies".)

Tuberculosis — Tuberculosis can produce a myelopathy by different mechanisms. Infection of the vertebral body leads to tuberculous spondylitis or Pott's disease, which can lead to secondary cord compression. These patients present with back pain over the affected vertebra, low-grade fever, and weight loss, followed by a secondary compressive myelopathy [55]. Tuberculomas within the intramedullary, intradural, and extradural space can also produce myelopathy [56,57]. (See "Bone and joint tuberculosis" and "Central nervous system tuberculosis: An overview".)

Parasite infection — The parasites Schistosoma mansoni and Schistosoma haematobium typically infect the spinal cord, producing rapidly progressing symptoms of TM, including lower limb pain, weakness, and bowel and bladder dysfunction [58,59]. The lower thoracic region of the spinal cord is most frequently involved, followed by the lumbar and sacral regions. Spinal cord involvement can lead to permanent paralysis. CSF evaluation reveals pleocytosis and elevated protein; eosinophilia occurs in almost half of patients. MRI demonstrates signal change and swelling within the cord. Most patients are treated with glucocorticoids and praziquantel and achieve at least partial recovery. (See "Schistosomiasis: Epidemiology and clinical manifestations" and "Schistosomiasis: Treatment and prevention".)

Cysticercosis has been reported to cause a cyst within the spinal cord [60].

Others — Bacterial meningitis may be complicated by a myelopathy due to formation of an epidural abscess, myelitis, or vasculitic infarction [55,61,62].

Lyme disease rarely affects the spinal cord. However, cases have been described in which clinical and MRI features resembling acute TM have been attributed to Lyme disease [63,64]. CSF in these cases typically demonstrates a lymphocytic pleocytosis and elevated protein. (See "Clinical manifestations of Lyme disease in adults".)

VASCULAR DISEASE

Spinal cord infarction — Infarction of the spinal cord is rare compared with cerebral infarction. Spinal cord infarction is most frequently caused by surgical procedures and pathologies affecting the aorta [65]. Other causes of spinal cord infarction are diverse and include any etiology that also produces brain infarction (eg, atherosclerosis, embolism, and hypercoagulable and vasculitic disorders) [65-69]. Spinal cord infarction can also occur in the setting of severe systemic hypotension or cardiac arrest.

Symptoms are consistent with the functional loss within the anterior spinal artery territory and include paralysis, loss of bladder function, and loss of pain and temperature sensation below the level of the lesion. Position and vibratory sensation are spared. The onset of symptoms is sudden and is frequently associated with back pain.

MRI will demonstrate a T2 signal change consistent with cord ischemia, but may be normal in the first 24 hours [70,71]. Diffusion-weighted imaging (DWI) is more sensitive [72-74]. Cerebrospinal fluid (CSF) protein level may be elevated, but pleocytosis is rare.

Less than half of patients show substantial motor recovery following spinal cord infarction [65,75]. Treatment is generally supportive and focused on the underlying aortic pathology and/or secondary stroke prevention.

Spinal cord infarction is discussed in detail separately. (See "Spinal cord infarction: Epidemiology and etiologies" and "Spinal cord infarction: Treatment and prognosis".)

Vascular malformations — Vascular malformations of the spinal cord are classified into types according to their location and vascular pathology [76,77].

Dural arteriovenous fistula – Dural arteriovenous fistula is the most common type, making up approximately 70 percent of all lesions [69,78]. These exist on the dural surface and drain intradurally by retrograde flow through a single medullary vein to the anterior or posterior median vein, resulting in engorgement of the coronal venous plexus.

They usually present after the fifth decade of life and are more common in men. The most common clinical presentation is that of progressive, often stepwise myeloradiculopathy, probably related to venous hypertension. Some patients present with neurogenic claudication [79]. Symptoms may initially fluctuate, but eventually a permanent and progressive paraparesis with sensory disturbances and sphincter dysfunction occurs.

MRI with contrast-enhanced magnetic resonance angiography (MRA) can identify a dural arteriovenous fistula but has imperfect sensitivity [79-84]. The most common finding is a nonspecific hyperintense signal lesion on T2-weighted images. The more specific findings of intradural flow voids on T2-weighted images, and/or serpentine enhancement on MRA and T1 images, are seen in 85 to 100 percent or more of patients. If MRI/MRA cannot be performed, myelography with supine and prone images may demonstrate the serpentine vessels within the intradural space. When positive, MRA and/or myelography can help guide the performance of the spinal angiogram, which remains the gold-standard test, and is required prior to therapeutic intervention. Spinal angiography can be a difficult diagnostic procedure in this setting, often requiring multiple injections of segmental arteries in order to identify the feeding artery, especially when MRA does not provide specific guiding information. However, at least one observational study found that in experienced hands, the complication rate is low (1 to 2 percent) and did not (at least in this series) involve any neurologic morbidity [85].

Occlusion of the fistula by surgery or endovascular embolization can be helpful in stabilizing, even ameliorating, the neurologic deficits [83,86-88].

Intramedullary spinal arteriovenous malformations – Spinal arteriovenous malformations (AVMs) within the spinal cord are supplied by medullary arteries and drain through medullary veins [89,90].

The mean age at clinical presentation is the mid-20s, but close to 20 percent of the lesions are diagnosed in children under 16 years of age [89]. A myelopathy is produced by the mass effect of the lesion or by ischemia or hemorrhage into the cord. Some patients present instead with subarachnoid hemorrhage.

MRI is sensitive for intramedullary AVM, showing a cluster of low-intensity signal foci [77]. Contrast-enhanced MRA also helps localize the nidus and identify arterial supply and venous drainage.

These lesions are treated by endovascular occlusion, surgical resection, or both [91].

Intramedullary cavernous malformations – Spinal cord cavernous malformations may be isolated lesions; however, approximately 25 percent will have an intracranial cavernous malformation as well [69]. Ten percent of patients have a family history; in these patients, multiple cavernous malformations are more common.

Patients typically present with hemorrhage within the spinal cord (hematomyelia); recurrent hemorrhage rates may be as high as 10 percent per year [69]. Cavernous malformations that are discovered incidentally have a lower rate of hemorrhage (<1 percent per year).

These lesions are best visualized on MRI [69]. The lesions are typically well defined with heterogeneous T1- and T2-weighted signal intensity, giving a so-called "popcorn" appearance; a low signal intense rim on T2 images represents hemosiderin deposition.

Asymptomatic lesions are often observed, while surgical or other interventions are considered for those that have bled [69].

Spinal epidural hematoma — Spinal epidural hematoma can complicate procedures that involve a spinal dural puncture, usually in patients with thrombocytopenia or bleeding diathesis, including anticoagulant therapy [92]. Rarely, this occurs spontaneously; a predisposition to bleeding is a risk factor in these patients as well [93-95]. Some occur in the setting of minor trauma.

Patients typically present with local and/or radicular pain, followed by loss of sensory, motor, and bladder and bowel function [93-95]. The source of bleeding is usually venous rather than arterial, and symptoms typically present over days, although more abrupt presentations are also described.

MRI is a sensitive imaging modality for these lesions [94,96]. MRI findings vary according to the age of the clot. In the first 24 hours the hematoma is usually isointense on T1- and hyperintense on T2-weighted images; after 24 hours, it becomes mostly hyperintense on T1 and on T2.

The appropriate treatment for patients with significant and/or progressing neurologic deficits is prompt surgical intervention, usually a laminectomy, and evacuation of the blood. Timely decompression of the hematoma is essential to avoid permanent loss of neurologic function [97,98]. Many individuals with minor, stable neurologic deficits can be managed by observation and have a good prognosis for complete recovery [93,95].

TOXIC, METABOLIC DISORDERS

Subacute combined degeneration — Deficiency in vitamin B12 (cobalamin) leads to degeneration of the dorsal and lateral white matter of the spinal cord, producing a slowly progressive weakness, sensory ataxia, and paresthesias, and ultimately spasticity, paraplegia, and incontinence [99]. Not all patients with neurologic abnormalities will have anemia or macrocytosis [100]. Supplemental treatment with vitamin B12 can stop progression and will produce neurologic improvement in most patients [101]. (See "Clinical manifestations and diagnosis of vitamin B12 and folate deficiency" and "Clinical manifestations and diagnosis of vitamin B12 and folate deficiency", section on 'Clinical presentation'.)

Nitrous oxide abuse can also lead to subacute combined degeneration, by inactivation of vitamin B12 [102-105]. (See "Inhalant misuse in children and adolescents".)

Copper deficiency myeloneuropathy — A syndrome similar to the subacute combined degeneration of vitamin B12 deficiency can occur with acquired copper deficiency, which may be the result of gastrointestinal surgery, excessive zinc ingestion (eg, overuse of denture cream), and other causes [106-108]. Most patients also have hematologic abnormalities. Treatment can prevent progression, but patients with significant neurologic deficits at presentation often remain disabled. (See "Copper deficiency myeloneuropathy".)

Radiation myelopathy — Myelopathy is a serious complication of radiation therapy to the spinal cord [109,110]. White matter tracts in the lateral aspects of the cord are preferentially affected [111,112]. Two distinct clinical presentations are described:

A transient myelopathy occurring two to six months after irradiation is usually mild and resolves spontaneously over several months.

A late progressive myelopathy begins 6 to 12 months after irradiation. This begins insidiously and generally progresses inexorably, although some cases stabilize. MRI is important to exclude other etiologies and will typically show hyperintensity on T2 and fluid-attenuated inversion recovery (FLAIR) sequences. A high radiation dose is a risk factor [113]. Fractionation size, concomitant chemotherapy, and other comorbidities may play a role. There is no effective treatment.

This topic is discussed in detail separately. (See "Complications of spinal cord irradiation".)

Intrathecal chemotherapy — Myelopathy can occur as a complication of intrathecal chemotherapy, particularly methotrexate and cytarabine [114-117]. This appears to be an idiosyncratic reaction, as a wide range of dose and timing with respect to the administration of intrathecal chemotherapy has been reported. The clinical syndrome resembles subacute combined degeneration, and MRI frequently shows abnormalities in the dorsal columns on T2-weighted images. (See "Overview of neurologic complications of conventional non-platinum cancer chemotherapy", section on 'Transverse myelopathy' and "Overview of neurologic complications of conventional non-platinum cancer chemotherapy", section on 'Intrathecal cytarabine'.)

Individual cases of myelopathy with treatment with antitumor necrosis factors as well as intravenous administration of cisplatin, cladribine, and carmustine have also been reported, but these appear to be rare [114,118].

Electrical injury — High-voltage electrical injury can be associated with a variety of neurologic complications, including spinal cord injury. (See "Electrical injuries and lightning strikes: Evaluation and management".)

Different syndromes have been described:

A transient flaccid paralysis, called keraunoparalysis, is apparent immediately following the injury, affects the legs more than the arms, and typically resolves within the first 24 hours [119,120]. This is most often described in association with lightning strike. Peripheral vasoconstriction and sensory disturbances are commonly associated. The pathophysiology is uncertain.

Other patients develop a more enduring and sometimes permanent spinal cord injury after electrical injury [121-124]. In this situation, clinical signs of myelopathy may be present at the time of injury, or may develop after several days or weeks. Motor deficits are more prominent than sensory findings. Bladder and other sphincter dysfunction can occur. Spine MRI is typically normal. The clinical course and prognosis are not well characterized. Direct heat and electrical injury to neural elements, as well as a delayed microvascular disease, are proposed mechanisms.

Hepatic myelopathy — Progressive myelopathy is a rare neurologic complication of chronic liver disease with portal hypertension; the mechanism is unknown [125-129]. The myelopathy is predominantly or entirely motor in manifestation, reflected as a spastic paraparesis that progresses over months to paraplegia [130]. Deficits are limited to the lower extremities; sensory and bladder function is often unaffected. MRI and cerebrospinal fluid (CSF) studies are normal. Neuropathologic studies have demonstrated demyelination of the lateral corticospinal tracts with various degrees of axonal loss [127].

In contrast to hepatic encephalopathy, ammonia-lowering treatments have little or no effect on the myelopathy, but patients with early manifestations of spinal cord impairment may improve with liver transplantation [130].

Decompression sickness myelopathy — Impairment of spinal cord function can be a manifestation of decompression sickness, a complication of deep sea diving [131-135]. Symptoms usually develop during or immediately after ascent but may be delayed for several hours or a few days. The thoracic cord is most commonly involved, producing paraparesis of varying severity and a sensory level in the mid or low thoracic region. Lesions at higher spinal cord levels producing quadriplegia have also been described [134].

Early therapeutic recompression frequently reverses symptoms and signs [132]. Residual corticospinal and minor sensory signs may remain for months or indefinitely [135]. Both MRI and pathologic studies have shown multifocal white matter degeneration in the posterior and lateral columns of the spinal cord with secondary ascending and descending tract degeneration [128,134]. Gaseous occlusion of venous plexi within the spinal cord is one postulated mechanism of injury. (See "Complications of SCUBA diving", section on 'Decompression sickness'.)

Lathyrism and konzo — Two disorders of spastic paraparesis have been described, which occur in association with increased dietary intake of food plants with neurotoxic potential, as occurs in certain geographic regions during times of famine [136,137].

Neurolathyrism is associated with prolonged consumption of the grass or chickling pea (Lathyrus sativus) [138]. Exposed persons develop a slowly developing spastic paraparesis with cramps, paresthesias, and numbness, accompanied by bladder symptoms and impotence. Some patients have tremors and other involuntary movements in their arms. Pathologic studies have demonstrated a loss of myelinated fibers in the corticospinal and spinocerebellar tracts. The toxin appears to be the neuroexcitatory amino acid, beta-N-oxalylaminoalanine. There is no treatment.

Konzo, a disorder characterized by acute spastic paraparesis or quadriparesis, is linked to high exposure to cyanogenic compounds in diets containing insufficiently processed bitter cassava (Manihot esculenta) [139,140]. This disorder is less well characterized than lathyrism, and it may reflect a disorder of intracranial rather than intraspinal motor pathways.

NEOPLASMS — Both benign and malignant tumors can produce a myelopathy as a result of external compression or intramedullary growth.

Neoplastic epidural spinal cord compression – The most common syndrome is that of extradural spinal cord compression, as produced by metastases to the extradural space. Patients present with a progressive weakness below the level of the lesion with accompanying sensory loss and bladder dysfunction [141]. Pain at the site of involvement is typical. Progression to paraplegia can occur abruptly, as a result of vascular compression.

Because the prognosis for neurologic recovery depends on the severity of the deficit at the time of intervention (high-dose corticosteroids with radiation therapy and/or surgical decompression), diagnostic evaluation (with gadolinium-enhanced spinal MRI) must proceed promptly when this diagnosis is considered [142]. This topic is discussed in detail separately. (See "Clinical features and diagnosis of neoplastic epidural spinal cord compression" and "Treatment and prognosis of neoplastic epidural spinal cord compression".)

Intramedullary tumors – Intramedullary spinal cord tumors are typically primary central nervous system tumors (ependymoma, astrocytoma); metastases are less likely [143,144]. These produce a progressive myelopathy, often with central cord features. MRI with gadolinium will show the tumor [145]; biopsy with histologic examination is usually required for diagnosis. Because of their intramedullary location, management of these lesions is difficult. (See "Spinal cord tumors".)

Other causes of myelopathy in patients with cancer – Myelopathy can occur as a complication of radiation therapy (see 'Radiation myelopathy' above); as a complication of intrathecal chemotherapy, particularly methotrexate and cytarabine (see "Overview of neurologic complications of conventional non-platinum cancer chemotherapy", section on 'Transverse myelopathy' and "Overview of neurologic complications of conventional non-platinum cancer chemotherapy", section on 'Intrathecal cytarabine'); and as a paraneoplastic syndrome. (See 'Paraneoplastic syndromes' above and "Paraneoplastic syndromes affecting spinal cord, peripheral nerve, and muscle", section on 'Myelopathy'.)

INHERITED AND DEGENERATIVE CONDITIONS

Amyotrophic lateral sclerosis — Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder that produces progressive weakness, usually with mixed upper and motor neuron signs [146,147]. Symptoms begin insidiously in older adults (usually >60 years) and progress inexorably. In typical patients, there is asymmetric limb weakness with a mixture of upper and lower motor neuron features. Sensory and sphincter disturbances are usually absent. MRI is normal. Electromyography typically shows denervation in clinically affected muscles. (See "Clinical features of amyotrophic lateral sclerosis and other forms of motor neuron disease" and "Diagnosis of amyotrophic lateral sclerosis and other forms of motor neuron disease".)

Unusual variants of ALS with atypical symptoms can present a diagnostic challenge. Primary lateral sclerosis is a rare variant of ALS with primarily upper motor neuron features [148]. Muscle stiffness leading to overt and progressive spasticity without associated muscle weakness or atrophy typifies the disorder. In approximately two-thirds of patients, there is an ascending pattern with spasticity spreading in a rather stereotyped fashion from the legs to the arms and finally to involve the bulbar musculature [149]. Without positive test findings, this is a diagnosis of exclusion. There is no treatment. (See "Clinical features of amyotrophic lateral sclerosis and other forms of motor neuron disease", section on 'Primary lateral sclerosis'.)

One familial form of ALS, the recessively inherited D90A mutation, can also present with atypical features, including a long preparetic phase of lower extremity discomfort, followed by a slowly ascending asymmetric paresis that is often accompanied by bladder symptoms [150-153]. (See "Familial amyotrophic lateral sclerosis".)

Hereditary spastic paraplegias — Hereditary spastic paraplegia (HSP) is a large group of inherited neurologic disorders, in which the prominent feature is a progressive spastic paraparesis [154]. HSP is classified according to the mode of inheritance, the genetic locus when known, and whether the spastic paraplegia syndrome occurs alone or is accompanied by additional neurologic or systemic abnormalities ("pure" versus "complicated") [155-159]. There have been no recent epidemiologic studies, but previously the incidence has been reported to be between 1 in 10,000 and 1 in 100,000.

Genetically diverse, with at least 28 genetic loci for HSP identified, the final common pathway for these disorders is a degeneration of the corticospinal tracts [155]. Inheritance is usually autosomal dominant, but recessive and X-linked variants (eg, Pelizaeus-Merzbacher disease) are known.

The typical patient with pure HSP will have a slowly progressive spastic paraparesis [155]. The age of onset can vary from infancy to the eighth decade. The first symptoms are often gait disturbance or urinary urgency. Examination reveals spasticity, hyperreflexia, extensor plantar responses, and impaired vibration and/or joint position sense. Weakness may be demonstrated but is rarely the major cause of disability. Upper limb involvement is generally limited to hyperreflexia.

HSP is a clinical diagnosis, based in large part on the family history [155]. MRI may be normal or may show atrophy in the spinal cord. Thinning of the corpus callosum on brain MRI is seen in half of patients with the autosomal recessive form of HSP [160]. Otherwise, the diagnosis is based on careful exclusion of other etiologies. Treatment for HSP is limited to symptomatic management [155].

HSP is discussed in more detail separately. (See "Hereditary spastic paraplegia".)

Adrenoleukodystrophy — Adrenomyeloneuropathy, a variant of adrenoleukodystrophy, an X-linked recessive disorder, is characterized by a slowly progressive spastic paraparesis and mild polyneuropathy [161,162]. As opposed to the more common and severe phenotype of adrenoleukodystrophy, adrenomyeloneuropathy generally presents in adult men or in female carriers of the mutation. Sensory and sphincter disturbances are typically absent. There may be mild adrenal insufficiency. MRI is typically normal. In the absence of a family history, the finding of a neuropathy on electrophysiologic testing may be a clue to the disorder. The diagnosis is made by the finding of increased very long chain fatty acids in plasma, red blood cells, or cultured skin fibroblasts. (See "Clinical features, evaluation, and diagnosis of X-linked adrenoleukodystrophy".)

Friedreich ataxia — Friedreich ataxia is an autosomal recessive degenerative disorder of uncertain pathogenesis that typically presents in adolescence [163]. The neuropathologic changes in Friedreich ataxia include degeneration of the posterior columns and the spinocerebellar tracts of the spinal cord and loss of the larger sensory cells of the dorsal root ganglia. These findings correspond to the clinical manifestations of progressive ataxia of all four limbs and gait, weakness, absent reflexes with extensor plantar responses, loss of position and vibration sense, and sparing of pain and temperature. Cardiomyopathy and diabetes mellitus are part of the syndrome. Patients with late-onset disease are more likely to have retained reflexes, spasticity, and no cardiomyopathy [164]. MRI may show atrophy of the cervical cord. Disease severity and rate of progression are highly variable. There is no treatment. This topic is discussed separately. (See "Friedreich ataxia".)

OTHERS

Syringomyelia — Syringomyelia is a fluid-filled, gliosis-lined cavity within the spinal cord. Most lesions are between C2 and T9; however, they can descend further down or extend upward into the brainstem (syringobulbia). A syrinx can represent a focal dilation of the central canal, or it may lie separately, within the spinal cord parenchyma [165].

Syringomyelia most commonly occurs in the setting of the Chiari malformation type I [166]. (See "Chiari malformations".)

Other causes of syringomyelia are [165-169]:

Other congenital malformations (eg, Klippel-Feil syndrome, and tethered spinal cord) (see "Approach to neck stiffness in children", section on 'Congenital')

Postinfectious

Postinflammatory (transverse myelitis [TM] and multiple sclerosis [MS])

Spinal neoplasms (especially ependymoma and hemangioblastoma) (see "Spinal cord tumors")

Post-traumatic (see "Chronic complications of spinal cord injury and disease", section on 'Syringomyelia')

A syrinx can be asymptomatic and discovered incidentally on spinal cord imaging. Other patients present with progressive central cord deficits that can include a prominent central pain syndrome in a segmental distribution [170]. (See "Anatomy and localization of spinal cord disorders".)

MRI will typically identify the intramedullary cavity; gadolinium administration will increase the sensitivity of finding an associated tumor. Surgical decompression with fenestration and/or shunt placement is recommended for patients with neurologic deterioration or intractable central pain [168-170]. Neurologic deficits usually stabilize after intervention and sometimes improve.

Cervical spondylotic myelopathy — In many case series, cervical spondylotic myelopathy is the most common cause of myelopathy, particularly in older adults [1]. Degenerative changes in the vertebral bodies, discs, and connecting ligaments encroach on the cervical canal, producing a progressive myelopathy [171]. Symptoms begin insidiously, usually with a spastic gait. Sensory loss, muscle weakness, and atrophy in the hands also cause functional impairment over time. Some patients may develop or present with an acute myelopathy characterized by a central cord syndrome, often in the setting of mild neck trauma.

The diagnosis is made by correlating clinical features with evidence of cervical spondylosis and cord compression on MRI [172]. Treatment options include cervical immobilization and surgical decompression. When and if to operate remains controversial. This topic is discussed separately. (See "Cervical spondylotic myelopathy".)

Ossification of the posterior longitudinal ligament — Ossification of the posterior longitudinal ligament (OPLL) is a condition of abnormal calcification of the posterior longitudinal ligament, usually in the cervical spine [173-178]. Its pathogenesis is not known, but it is more common in individuals in East Asia, including Japan, and in males (roughly 2:1 male:female ratio) [179]. OPLL can be associated with idiopathic skeletal hyperostosis, ankylosing spondylitis, and other spondyloarthropathies, or may occur as an isolated condition. (See "Diffuse idiopathic skeletal hyperostosis (DISH)", section on 'Differential diagnosis'.)

Symptomatic patients typically present in the fifth to sixth decades of life with progressive myelopathic symptoms, but they are also at risk for and can present with acute spinal cord injury [180]. It is diagnosed by characteristic findings on imaging findings (computed tomography [CT] or MRI); significant compressive symptoms typically require surgical decompression.

Surfers' myelopathy — A syndrome of acute low back pain followed by progressive lower extremity numbness and weakness has been described in at least 26 patients while surfing [181-186]. Patients are generally young (ages 15 to 46 years), mostly male, are often surfing for the first time, and have suffered no apparent trauma. MRI shows restricted diffusion in the lower thoracic spinal cord to the conus medullaris. The pathogenesis is unclear; however, the MRI findings along with reports of individuals lying prone on the surfboard with possible lumbar hyperextension for prolonged periods of time suggest that vascular compression may play a role. The severity of the neurologic deficits and degree of recovery is variable.

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

Basics topics (see "Patient education: Paraplegia and quadriplegia (The Basics)" and "Patient education: Friedreich ataxia (The Basics)")

SUMMARY

Causes of myelopathy – Pathologies that affect the spinal cord are diverse. Common etiologies of myelopathy include autoimmune, infectious, neoplastic, vascular, and hereditary-degenerative diseases. (See 'Inflammatory diseases' above and 'Infections' above and 'Vascular disease' above and 'Toxic, metabolic disorders' above and 'Neoplasms' above and 'Inherited and degenerative conditions' above.)

Clinical localization – The neurologic examination will elicit the deficits and define a specific spinal cord syndrome (table 2). The approach to localization and the features of specific spinal cord syndromes are described separately. (See "Anatomy and localization of spinal cord disorders", section on 'Clinical localization' and "Anatomy and localization of spinal cord disorders", section on 'Spinal cord syndromes'.)

Diagnostic approach – The spinal cord syndrome, along with the clinical setting, course of presentation, and findings on a neuroimaging study (usually MRI), will identify the likely pathogenesis in most cases (table 1). (See 'Diagnostic approach' above.)

  1. Moore AP, Blumhardt LD. A prospective survey of the causes of non-traumatic spastic paraparesis and tetraparesis in 585 patients. Spinal Cord 1997; 35:361.
  2. Offenbacher H. The diagnostic impact of magnetic resonance imaging on the evaluation of suspected spinal cord disease. Wien Klin Wochenschr 1992; 104:589.
  3. Do-Dai DD, Brooks MK, Goldkamp A, et al. Magnetic resonance imaging of intramedullary spinal cord lesions: a pictorial review. Curr Probl Diagn Radiol 2010; 39:160.
  4. Pardo CA. Clinical Approach to Myelopathy Diagnosis. Continuum (Minneap Minn) 2024; 30:14.
  5. Flanagan EP, Hunt CH, Lowe V, et al. [(18)F]-fluorodeoxyglucose-positron emission tomography in patients with active myelopathy. Mayo Clin Proc 2013; 88:1204.
  6. Brinar VV, Habek M, Brinar M, et al. The differential diagnosis of acute transverse myelitis. Clin Neurol Neurosurg 2006; 108:278.
  7. Budka H. Neuropathology of myelitis, myelopathy, and spinal infections in AIDS. Neuroimaging Clin N Am 1997; 7:639.
  8. Cree BA, Wingerchuk DM. Acute transverse myelitis: is the "idiopathic" form vanishing? Neurology 2005; 65:1857.
  9. Jeffery DR, Mandler RN, Davis LE. Transverse myelitis. Retrospective analysis of 33 cases, with differentiation of cases associated with multiple sclerosis and parainfectious events. Arch Neurol 1993; 50:532.
  10. de Seze J, Lanctin C, Lebrun C, et al. Idiopathic acute transverse myelitis: application of the recent diagnostic criteria. Neurology 2005; 65:1950.
  11. Kaplin AI, Krishnan C, Deshpande DM, et al. Diagnosis and management of acute myelopathies. Neurologist 2005; 11:2.
  12. Pidcock FS, Krishnan C, Crawford TO, et al. Acute transverse myelitis in childhood: center-based analysis of 47 cases. Neurology 2007; 68:1474.
  13. Ciccarelli O, Cohen JA, Reingold SC, et al. Spinal cord involvement in multiple sclerosis and neuromyelitis optica spectrum disorders. Lancet Neurol 2019; 18:185.
  14. Knebusch M, Strassburg HM, Reiners K. Acute transverse myelitis in childhood: nine cases and review of the literature. Dev Med Child Neurol 1998; 40:631.
  15. Bakshi R, Kinkel PR, Mechtler LL, et al. Magnetic resonance imaging findings in 22 cases of myelitis: comparison between patients with and without multiple sclerosis. Eur J Neurol 1998; 5:35.
  16. Choi KH, Lee KS, Chung SO, et al. Idiopathic transverse myelitis: MR characteristics. AJNR Am J Neuroradiol 1996; 17:1151.
  17. Cordonnier C, de Seze J, Breteau G, et al. Prospective study of patients presenting with acute partial transverse myelopathy. J Neurol 2003; 250:1447.
  18. Kumar N, Frohman EM. Spinal neurosarcoidosis mimicking an idiopathic inflammatory demyelinating syndrome. Arch Neurol 2004; 61:586.
  19. Junger SS, Stern BJ, Levine SR, et al. Intramedullary spinal sarcoidosis: clinical and magnetic resonance imaging characteristics. Neurology 1993; 43:333.
  20. Saleh S, Saw C, Marzouk K, Sharma O. Sarcoidosis of the spinal cord: literature review and report of eight cases. J Natl Med Assoc 2006; 98:965.
  21. Rosenfeld MR, Posner JB. Paraneoplastic motor neuron disease. Adv Neurol 1991; 56:445.
  22. Ojeda VJ. Necrotizing myelopathy associated with malignancy. A clinicopathologic study of two cases and literature review. Cancer 1984; 53:1115.
  23. Dalmau J, Graus F, Rosenblum MK, Posner JB. Anti-Hu--associated paraneoplastic encephalomyelitis/sensory neuronopathy. A clinical study of 71 patients. Medicine (Baltimore) 1992; 71:59.
  24. Darouiche RO. Spinal epidural abscess. N Engl J Med 2006; 355:2012.
  25. Woolsey RM, McGarry JD. AIDS related spinal cord syndromes. J Am Paraplegia Soc 1989; 12:6.
  26. Solomon T, Willison H. Infectious causes of acute flaccid paralysis. Curr Opin Infect Dis 2003; 16:375.
  27. Chen CY, Chang YC, Huang CC, et al. Acute flaccid paralysis in infants and young children with enterovirus 71 infection: MR imaging findings and clinical correlates. AJNR Am J Neuroradiol 2001; 22:200.
  28. John TJ. Spinal cord disease in West Nile virus infection. N Engl J Med 2003; 348:564.
  29. Kelley TW, Prayson RA, Isada CM. Spinal cord disease in West Nile virus infection. N Engl J Med 2003; 348:564.
  30. Jeha LE, Sila CA, Lederman RJ, et al. West Nile virus infection: a new acute paralytic illness. Neurology 2003; 61:55.
  31. Kraushaar G, Patel R, Stoneham GW. West Nile Virus: a case report with flaccid paralysis and cervical spinal cord: MR imaging findings. AJNR Am J Neuroradiol 2005; 26:26.
  32. Cao NJ, Ranganathan C, Kupsky WJ, Li J. Recovery and prognosticators of paralysis in West Nile virus infection. J Neurol Sci 2005; 236:73.
  33. Arya SC, Agarwal N. Spinal cord neuropathology in human West Nile virus infection. Arch Pathol Lab Med 2004; 128:1210; author reply 1210.
  34. Fux CA, Pfister S, Nohl F, Zimmerli S. Cytomegalovirus-associated acute transverse myelitis in immunocompetent adults. Clin Microbiol Infect 2003; 9:1187.
  35. Nakajima H, Furutama D, Kimura F, et al. Herpes simplex virus myelitis: clinical manifestations and diagnosis by the polymerase chain reaction method. Eur Neurol 1998; 39:163.
  36. Shyu WC, Lin JC, Chang BC, et al. Recurrent ascending myelitis: an unusual presentation of herpes simplex virus type 1 infection. Ann Neurol 1993; 34:625.
  37. Kincaid O, Lipton HL. Viral myelitis: an update. Curr Neurol Neurosci Rep 2006; 6:469.
  38. Aktipi KM, Ravaglia S, Ceroni M, et al. Severe recurrent myelitis in patients with hepatitis C virus infection. Neurology 2007; 68:468.
  39. Orme HT, Smith AG, Nagel MA, et al. VZV spinal cord infarction identified by diffusion-weighted MRI (DWI). Neurology 2007; 69:398.
  40. Young-Barbee C, Hall DA, LoPresti JJ, et al. Brown-Séquard syndrome after herpes zoster. Neurology 2009; 72:670.
  41. Di Rocco A. Diseases of the spinal cord in human immunodeficiency virus infection. Semin Neurol 1999; 19:151.
  42. Dal Pan GJ, Glass JD, McArthur JC. Clinicopathologic correlations of HIV-1-associated vacuolar myelopathy: an autopsy-based case-control study. Neurology 1994; 44:2159.
  43. Ford B, Tampieri D, Francis G. Long-term follow-up of acute partial transverse myelopathy. Neurology 1992; 42:250.
  44. Staudinger R, Henry K. Remission of HIV myelopathy after highly active antiretroviral therapy. Neurology 2000; 54:267.
  45. Cikurel K, Schiff L, Simpson DM. Pilot study of intravenous immunoglobulin in HIV-associated myelopathy. AIDS Patient Care STDS 2009; 23:75.
  46. Geraci A, Di Rocco A, Liu M, et al. AIDS myelopathy is not associated with elevated HIV viral load in cerebrospinal fluid. Neurology 2000; 55:440.
  47. Grindstaff P, Gruener G. The peripheral nervous system complications of HTLV-1 myelopathy (HAM/TSP) syndromes. Semin Neurol 2005; 25:315.
  48. Manns A, Hisada M, La Grenade L. Human T-lymphotropic virus type I infection. Lancet 1999; 353:1951.
  49. Oger J, Dekaban G. HTLV-I associated myelopathy: a case of viral-induced auto-immunity. Autoimmunity 1995; 21:151.
  50. Oger JJ, Werker DH, Foti DJ, Dekaban GA. HTLV-I associated myelopathy: an endemic disease of Canadian aboriginals of the Northwest Pacific coast? Can J Neurol Sci 1993; 20:302.
  51. Leite AC, Mendonça GA, Serpa MJ, et al. Neurological manifestations in HTLV-I-infected blood donors. J Neurol Sci 2003; 214:49.
  52. Nagai M, Osame M. Human T-cell lymphotropic virus type I and neurological diseases. J Neurovirol 2003; 9:228.
  53. Liu W, Nair G, Vuolo L, et al. In vivo imaging of spinal cord atrophy in neuroinflammatory diseases. Ann Neurol 2014; 76:370.
  54. Chilver-Stainer L, Fischer U, Hauf M, et al. Syphilitic myelitis: rare, nonspecific, but treatable. Neurology 2009; 72:673.
  55. Balériaux DL, Neugroschl C. Spinal and spinal cord infection. Eur Radiol 2004; 14 Suppl 3:E72.
  56. al-Deeb SM, Yaqub BA, Sharif HS, Motaery KR. Neurotuberculosis: a review. Clin Neurol Neurosurg 1992; 94 Suppl:S30.
  57. Bahemuka M, Murungi JH. Tuberculosis of the nervous system. A clinical, radiological and pathological study of 39 consecutive cases in Riyadh, Saudi Arabia. J Neurol Sci 1989; 90:67.
  58. Scrimgeour EM, Gajdusek DC. Involvement of the central nervous system in Schistosoma mansoni and S. haematobium infection. A review. Brain 1985; 108 ( Pt 4):1023.
  59. Ferrari TC. Spinal cord schistosomiasis. A report of 2 cases and review emphasizing clinical aspects. Medicine (Baltimore) 1999; 78:176.
  60. Gonçalves FG, Neves PO, Jovem CL, et al. Chronic myelopathy associated to intramedullary cysticercosis. Spine (Phila Pa 1976) 2010; 35:E159.
  61. Kastenbauer S, Winkler F, Fesl G, et al. Acute severe spinal cord dysfunction in bacterial meningitis in adults: MRI findings suggest extensive myelitis. Arch Neurol 2001; 58:806.
  62. Josephson SA, Pillai DR, Phillips JJ, Chou D. Neurolisteriosis presenting as cervical myelitis in an immunocompetent patient. Neurology 2006; 66:1122.
  63. Mantienne C, Albucher JF, Catalaa I, et al. MRI in Lyme disease of the spinal cord. Neuroradiology 2001; 43:485.
  64. Meurs L, Labeye D, Declercq I, et al. Acute transverse myelitis as a main manifestation of early stage II neuroborreliosis in two patients. Eur Neurol 2004; 52:186.
  65. Cheshire WP, Santos CC, Massey EW, Howard JF Jr. Spinal cord infarction: etiology and outcome. Neurology 1996; 47:321.
  66. Faig J, Busse O, Salbeck R. Vertebral body infarction as a confirmatory sign of spinal cord ischemic stroke: report of three cases and review of the literature. Stroke 1998; 29:239.
  67. Fairhead JF, Phillips D, Handa A. Embolic spinal cord infarction as a presentation of abdominal aortic aneurysm. J R Soc Med 2005; 98:59.
  68. Salvarani C, Brown RD Jr, Calamia KT, et al. Primary CNS vasculitis with spinal cord involvement. Neurology 2008; 70:2394.
  69. Zalewski NL. Vascular Myelopathies. Continuum (Minneap Minn) 2021; 27:30.
  70. Masson C, Pruvo JP, Meder JF, et al. Spinal cord infarction: clinical and magnetic resonance imaging findings and short term outcome. J Neurol Neurosurg Psychiatry 2004; 75:1431.
  71. Weidauer S, Nichtweiss M, Lanfermann H, Zanella FE. Spinal cord infarction: MR imaging and clinical features in 16 cases. Neuroradiology 2002; 44:851.
  72. Küker W, Weller M, Klose U, et al. Diffusion-weighted MRI of spinal cord infarction--high resolution imaging and time course of diffusion abnormality. J Neurol 2004; 251:818.
  73. Shinoyama M, Takahashi T, Shimizu H, et al. Spinal cord infarction demonstrated by diffusion-weighted magnetic resonance imaging. J Clin Neurosci 2005; 12:466.
  74. Thurnher MM, Bammer R. Diffusion-weighted MR imaging (DWI) in spinal cord ischemia. Neuroradiology 2006; 48:795.
  75. Pelser H, van Gijn J. Spinal infarction. A follow-up study. Stroke 1993; 24:896.
  76. Riche MC, Reizine D, Melki JP, Merland JJ. Classification of spinal cord vascular malformations. Radiat Med 1985; 3:17.
  77. Bemporad JA, Sze GS. MR imaging of spinal cord vascular malformations with an emphasis on the cervical spine. Magn Reson Imaging Clin N Am 2000; 8:581.
  78. Lev N, Maimon S, Rappaport ZH, Melamed E. Spinal dural arteriovenous fistulae--a diagnostic challenge. Isr Med Assoc J 2001; 3:492.
  79. Muralidharan R, Saladino A, Lanzino G, et al. The clinical and radiological presentation of spinal dural arteriovenous fistula. Spine (Phila Pa 1976) 2011; 36:E1641.
  80. Luetmer PH, Lane JI, Gilbertson JR, et al. Preangiographic evaluation of spinal dural arteriovenous fistulas with elliptic centric contrast-enhanced MR Angiography and effect on radiation dose and volume of iodinated contrast material. AJNR Am J Neuroradiol 2005; 26:711.
  81. Saraf-Lavi E, Bowen BC, Quencer RM, et al. Detection of spinal dural arteriovenous fistulae with MR imaging and contrast-enhanced MR angiography: sensitivity, specificity, and prediction of vertebral level. AJNR Am J Neuroradiol 2002; 23:858.
  82. Bemporad JA, Sze G. Magnetic resonance imaging of spinal cord vascular malformations with an emphasis on the cervical spine. Neuroimaging Clin N Am 2001; 11:viii, 111.
  83. Koch C. Spinal dural arteriovenous fistula. Curr Opin Neurol 2006; 19:69.
  84. Toossi S, Josephson SA, Hetts SW, et al. Utility of MRI in spinal arteriovenous fistula. Neurology 2012; 79:25.
  85. Chen J, Gailloud P. Safety of spinal angiography: complication rate analysis in 302 diagnostic angiograms. Neurology 2011; 77:1235.
  86. Watson JC, Oldfield EH. The surgical management of spinal dural vascular malformations. Neurosurg Clin N Am 1999; 10:73.
  87. Aghakhani N, Parker F, David P, et al. Curable cause of paraplegia: spinal dural arteriovenous fistulae. Stroke 2008; 39:2756.
  88. Kilic AK, Kurne AT, Saatci I, Tan E. A rare cause of radiculomyelitis: dural arteriovenous fistula. JAMA Neurol 2015; 72:217.
  89. Yasargil MG, Symon L, Teddy PG. Arteriovenous malformations of the spinal cord. In: Advances and Technical Standards in Neurosurgery, Symon L (Ed), Springer, Wien 1984. p.61.
  90. Aminoff MJ. Spinal vascular disease. In: Spinal Cord Disease: Basic Science, Diagnosis and Management, Critchley E, Eisen A (Eds), Springer, London 1992. p.423.
  91. Zozulya YP, Slin'ko EI, Al-Qashqish II. Spinal arteriovenous malformations: new classification and surgical treatment. Neurosurg Focus 2006; 20:E7.
  92. Furlan JC, Hawryluk GW, Austin J, Fehlings MG. Spinal haemorrhage during anticoagulant regimen for thromboprophylaxis: a unique form of central nervous system haemorrhage. J Neurol Neurosurg Psychiatry 2012; 83:746.
  93. Thiele RH, Hage ZA, Surdell DL, et al. Spontaneous spinal epidural hematoma of unknown etiology: case report and literature review. Neurocrit Care 2008; 9:242.
  94. Liu WH, Hsieh CT, Chiang YH, Chen GJ. Spontaneous spinal epidural hematoma of thoracic spine: a rare case report and review of literature. Am J Emerg Med 2008; 26:384.e1.
  95. SreeHarsha CK, Rajasekaran S, Dhanasekararaja P. Spontaneous complete recovery of paraplegia caused by epidural hematoma complicating epidural anesthesia: a case report and review of literature. Spinal Cord 2006; 44:514.
  96. Braun P, Kazmi K, Nogués-Meléndez P, et al. MRI findings in spinal subdural and epidural hematomas. Eur J Radiol 2007; 64:119.
  97. Vandermeulen EP, Van Aken H, Vermylen J. Anticoagulants and spinal-epidural anesthesia. Anesth Analg 1994; 79:1165.
  98. Lawton MT, Porter RW, Heiserman JE, et al. Surgical management of spinal epidural hematoma: relationship between surgical timing and neurological outcome. J Neurosurg 1995; 83:1.
  99. Hemmer B, Glocker FX, Schumacher M, et al. Subacute combined degeneration: clinical, electrophysiological, and magnetic resonance imaging findings. J Neurol Neurosurg Psychiatry 1998; 65:822.
  100. Lindenbaum J, Healton EB, Savage DG, et al. Neuropsychiatric disorders caused by cobalamin deficiency in the absence of anemia or macrocytosis. N Engl J Med 1988; 318:1720.
  101. Vasconcelos OM, Poehm EH, McCarter RJ, et al. Potential outcome factors in subacute combined degeneration: review of observational studies. J Gen Intern Med 2006; 21:1063.
  102. Diamond AL, Diamond R, Freedman SM, Thomas FP. "Whippets"-induced cobalamin deficiency manifesting as cervical myelopathy. J Neuroimaging 2004; 14:277.
  103. Doran M, Rassam SS, Jones LM, Underhill S. Toxicity after intermittent inhalation of nitrous oxide for analgesia. BMJ 2004; 328:1364.
  104. Ng J, Frith R. Nanging. Lancet 2002; 360:384.
  105. Sotirchos ES, Saidha S, Becker D. Neurological picture. Nitrous oxide-induced myelopathy with inverted V-sign on spinal MRI. J Neurol Neurosurg Psychiatry 2012; 83:915.
  106. Kumar N, Gross JB Jr, Ahlskog JE. Copper deficiency myelopathy produces a clinical picture like subacute combined degeneration. Neurology 2004; 63:33.
  107. Nations SP, Boyer PJ, Love LA, et al. Denture cream: an unusual source of excess zinc, leading to hypocupremia and neurologic disease. Neurology 2008; 71:639.
  108. Goodman BP, Bosch EP, Ross MA, et al. Clinical and electrodiagnostic findings in copper deficiency myeloneuropathy. J Neurol Neurosurg Psychiatry 2009; 80:524.
  109. Goldwein JW. Radiation myelopathy: a review. Med Pediatr Oncol 1987; 15:89.
  110. Schultheiss TE, Stephens LC. Invited review: permanent radiation myelopathy. Br J Radiol 1992; 65:737.
  111. Okada S, Okeda R. Pathology of radiation myelopathy. Neuropathology 2001; 21:247.
  112. Schultheiss TE, Stephens LC. The pathogenesis of radiation myelopathy: widening the circle. Int J Radiat Oncol Biol Phys 1992; 23:1089.
  113. Ang KK, Stephens LC. Prevention and management of radiation myelopathy. Oncology (Williston Park) 1994; 8:71.
  114. Schwendimann RN. Metabolic, nutritional, and toxic myelopathies. Neurol Clin 2013; 31:207.
  115. Cachia D, Kamiya-Matsuoka C, Pinnix CC, et al. Myelopathy following intrathecal chemotherapy in adults: a single institution experience. J Neurooncol 2015; 122:391.
  116. Chamberlain MC. Neurotoxicity of intra-CSF liposomal cytarabine (DepoCyt) administered for the treatment of leptomeningeal metastases: a retrospective case series. J Neurooncol 2012; 109:143.
  117. Chamberlain MC. Neurotoxicity of cancer treatment. Curr Oncol Rep 2010; 12:60.
  118. Goodman BP. Metabolic and toxic causes of myelopathy. Continuum (Minneap Minn) 2015; 21:84.
  119. ten Duis HJ, Klasen HJ, Reenalda PE. Keraunoparalysis, a 'specific' lightning injury. Burns Incl Therm Inj 1985; 12:54.
  120. Cherington M. Neurologic manifestations of lightning strikes. Neurology 2003; 60:182.
  121. Breugem CC, Van Hertum W, Groenevelt F. High voltage electrical injury leading to a delayed onset tetraplegia, with recovery. Ann N Y Acad Sci 1999; 888:131.
  122. Kalita J, Jose M, Misra UK. Myelopathy and amnesia following accidental electrical injury. Spinal Cord 2002; 40:253.
  123. Ko SH, Chun W, Kim HC. Delayed spinal cord injury following electrical burns: a 7-year experience. Burns 2004; 30:691.
  124. Lammertse DP. Neurorehabilitation of spinal cord injuries following lightning and electrical trauma. NeuroRehabilitation 2005; 20:9.
  125. Utku U, Asil T, Balci K, et al. Hepatic myelopathy with spastic paraparesis. Clin Neurol Neurosurg 2005; 107:514.
  126. Campellone JV, Lacomis D, Giuliani MJ, Kroboth FJ. Hepatic myelopathy. Case report with review of the literature. Clin Neurol Neurosurg 1996; 98:242.
  127. Sobukawa E, Sakimura K, Hoshino S, et al. Hepatic myelopathy: an unusual neurological complication of advanced hepatic disease. Intern Med 1994; 33:718.
  128. Conn HO, Rössle M, Levy L, Glocker FX. Portosystemic myelopathy: spastic paraparesis after portosystemic shunting. Scand J Gastroenterol 2006; 41:619.
  129. Holroyd KB, Berkowitz AL. Metabolic and Toxic Myelopathies. Continuum (Minneap Minn) 2024; 30:199.
  130. Nardone R, Buratti T, Oliviero A, et al. Corticospinal involvement in patients with a portosystemic shunt due to liver cirrhosis: a MEP study. J Neurol 2006; 253:81.
  131. Barratt DM, Van Meter K. Decompression sickness in Miskito Indian lobster divers: review of 229 cases. Aviat Space Environ Med 2004; 75:350.
  132. Aharon-Peretz J, Adir Y, Gordon CR, et al. Spinal cord decompression sickness in sport diving. Arch Neurol 1993; 50:753.
  133. Mastaglia FL, McCallum RI, Walder DN. Myelopathy associated with decompression sickness: a report of six cases. Clin Exp Neurol 1983; 19:54.
  134. McCormac J, Mirvis SE, Cotta-Cumba C, Shanmuganathan K. Spinal myelopathy resulting from decompression sickness: MR findings in a case and review of the literature. Emerg Radiol 2002; 9:240.
  135. Tournebise H, Boucand MH, Landi J, Theobald X. Paraplegia and decompression sickness. Paraplegia 1995; 33:636.
  136. Tshala-Katumbay D, Spencer PS. Toxic disorders of the upper motor neuron system. In: Motor Neuron Diseases, Eisen A, Shaw PM (Eds), Elsevier, Edinburgh 2006. p.353.
  137. Eeg-Olofsson KE, Tshala-Katumbay D. Konzo. In: Clinical Neurophysiology of Motor Neuron Diseases, Elsevier, Amsterdam 2005. p.675.
  138. Ludolph AC, Hugon J, Dwivedi MP, et al. Studies on the aetiology and pathogenesis of motor neuron diseases. 1. Lathyrism: clinical findings in established cases. Brain 1987; 110 ( Pt 1):149.
  139. Tshala-Katumbay D, Edebol Eeg-Olofsson K, Kazadi-Kayembe T, et al. Abnormalities of somatosensory evoked potentials in konzo--an upper motor neuron disorder. Clin Neurophysiol 2002; 113:10.
  140. Tshala-Katumbay D, Eeg-Olofsson KE, Kazadi-Kayembe T, et al. Analysis of motor pathway involvement in konzo using transcranial electrical and magnetic stimulation. Muscle Nerve 2002; 25:230.
  141. Helweg-Larsen S, Sørensen PS. Symptoms and signs in metastatic spinal cord compression: a study of progression from first symptom until diagnosis in 153 patients. Eur J Cancer 1994; 30A:396.
  142. Helweg-Larsen S, Sørensen PS, Kreiner S. Prognostic factors in metastatic spinal cord compression: a prospective study using multivariate analysis of variables influencing survival and gait function in 153 patients. Int J Radiat Oncol Biol Phys 2000; 46:1163.
  143. Shrivastava RK, Epstein FJ, Perin NI, et al. Intramedullary spinal cord tumors in patients older than 50 years of age: management and outcome analysis. J Neurosurg Spine 2005; 2:249.
  144. Graber JJ, Nolan CP. Myelopathies in patients with cancer. Arch Neurol 2010; 67:298.
  145. Sevick RJ, Wallace CJ. MR imaging of neoplasms of the lumbar spine. Magn Reson Imaging Clin N Am 1999; 7:539.
  146. Rowland LP. Diagnosis of amyotrophic lateral sclerosis. J Neurol Sci 1998; 160 Suppl 1:S6.
  147. Mitsumoto H, Chad DA, Pioro EP. Amyotrophic lateral sclerosis. In: Contemporary Neurology Series, 49, FA Davis Company, 1998.
  148. Eisen A. Primary lateral slcerosis. In: Handbook of Clinical Neurology, Vol 82, Eisen A, Shaw PM (Eds), Elsevier, Edinburgh 2007. p.315.
  149. Zhai P, Pagan F, Statland J, et al. Primary lateral sclerosis: A heterogeneous disorder composed of different subtypes? Neurology 2003; 60:1258.
  150. Andersen PM, Nilsson P, Keränen ML, et al. Phenotypic heterogeneity in motor neuron disease patients with CuZn-superoxide dismutase mutations in Scandinavia. Brain 1997; 120 ( Pt 10):1723.
  151. Andersen PM, Forsgren L, Binzer M, et al. Autosomal recessive adult-onset amyotrophic lateral sclerosis associated with homozygosity for Asp90Ala CuZn-superoxide dismutase mutation. A clinical and genealogical study of 36 patients. Brain 1996; 119 ( Pt 4):1153.
  152. Eisen A, Krieger C. Amyotrophic Lateral Sclerosis. A Synthesis of Research and Clinical Practice, Cambridge University Press, 1998.
  153. Weber M, Eisen A, Stewart HG, Andersen PM. Preserved slow conducting corticomotoneuronal projections in amyotrophic lateral sclerosis with autosomal recessive D90A CuZn-superoxide dismutase mutation. Brain 2000; 123 ( Pt 7):1505.
  154. Salinas S, Proukakis C, Crosby A, Warner TT. Hereditary spastic paraplegia: clinical features and pathogenetic mechanisms. Lancet Neurol 2008; 7:1127.
  155. Fink JK. Hereditary spastic paraplegia. Neurol Clin 2002; 20:711.
  156. Fink JK. Hereditary spastic paraplegia. Curr Neurol Neurosci Rep 2006; 6:65.
  157. Gould RM, Brady ST. Neuropathology: many paths lead to hereditary spastic paraplegia. Curr Biol 2004; 14:R903.
  158. McDermott CJ, Shaw PJ. Hereditary spastic paraplegia. Int Rev Neurobiol 2002; 53:191.
  159. Reid E. Many pathways lead to hereditary spastic paraplegia. Lancet Neurol 2003; 2:210.
  160. Boukhris A, Stevanin G, Feki I, et al. Hereditary spastic paraplegia with mental impairment and thin corpus callosum in Tunisia: SPG11, SPG15, and further genetic heterogeneity. Arch Neurol 2008; 65:393.
  161. Moser HW, Mahmood A, Raymond GV. X-linked adrenoleukodystrophy. Nat Clin Pract Neurol 2007; 3:140.
  162. Griffin JW, Goren E, Schaumburg H, et al. Adrenomyeloneuropathy: a probable variant of adrenoleukodystrophy. I. Clinical and endocrinologic aspects. Neurology 1977; 27:1107.
  163. Dürr A, Cossee M, Agid Y, et al. Clinical and genetic abnormalities in patients with Friedreich's ataxia. N Engl J Med 1996; 335:1169.
  164. Bhidayasiri R, Perlman SL, Pulst SM, Geschwind DH. Late-onset Friedreich ataxia: phenotypic analysis, magnetic resonance imaging findings, and review of the literature. Arch Neurol 2005; 62:1865.
  165. Milhorat TH. Classification of syringomyelia. Neurosurg Focus 2000; 8:E1.
  166. Brickell KL, Anderson NE, Charleston AJ, et al. Ethnic differences in syringomyelia in New Zealand. J Neurol Neurosurg Psychiatry 2006; 77:989.
  167. Larner AJ, Muqit MM, Glickman S. Concurrent syrinx and inflammatory central nervous system disease detected by magnetic resonance imaging: an illustrative case and review of the literature. Medicine (Baltimore) 2002; 81:41.
  168. Laxton AW, Perrin RG. Cordectomy for the treatment of posttraumatic syringomyelia. Report of four cases and review of the literature. J Neurosurg Spine 2006; 4:174.
  169. Batzdorf U. Primary spinal syringomyelia: a personal perspective. Neurosurg Focus 2000; 8:E7.
  170. Todor DR, Mu HT, Milhorat TH. Pain and syringomyelia: a review. Neurosurg Focus 2000; 8:E11.
  171. McCormick WE, Steinmetz MP, Benzel EC. Cervical spondylotic myelopathy: make the difficult diagnosis, then refer for surgery. Cleve Clin J Med 2003; 70:899.
  172. Baron EM, Young WF. Cervical spondylotic myelopathy: a brief review of its pathophysiology, clinical course, and diagnosis. Neurosurgery 2007; 60:S35.
  173. Choi BW, Song KJ, Chang H. Ossification of the posterior longitudinal ligament: a review of literature. Asian Spine J 2011; 5:267.
  174. Mochizuki M, Aiba A, Hashimoto M, et al. Cervical myelopathy in patients with ossification of the posterior longitudinal ligament. J Neurosurg Spine 2009; 10:122.
  175. Matsunaga S, Sakou T. Ossification of the posterior longitudinal ligament of the cervical spine: etiology and natural history. Spine (Phila Pa 1976) 2012; 37:E309.
  176. Saetia K, Cho D, Lee S, et al. Ossification of the posterior longitudinal ligament: a review. Neurosurg Focus 2011; 30:E1.
  177. Jeon TS, Chang H, Choi BW. Analysis of demographics, clinical, and radiographical findings of ossification of posterior longitudinal ligament of the cervical spine in 146 Korean patients. Spine (Phila Pa 1976) 2012; 37:E1498.
  178. Fujimori T, Le H, Hu SS, et al. Ossification of the posterior longitudinal ligament of the cervical spine in 3161 patients: a CT-based study. Spine (Phila Pa 1976) 2015; 40:E394.
  179. Wang MY, Thambuswamy M. Ossification of the posterior longitudinal ligament in non-Asians: demographic, clinical, and radiographic findings in 43 patients. Neurosurg Focus 2011; 30:E4.
  180. Onishi E, Sakamoto A, Murata S, Matsushita M. Risk factors for acute cervical spinal cord injury associated with ossification of the posterior longitudinal ligament. Spine (Phila Pa 1976) 2012; 37:660.
  181. Chang CW, Donovan DJ, Liem LK, et al. Surfers' myelopathy: a case series of 19 novice surfers with nontraumatic myelopathy. Neurology 2012; 79:2171.
  182. Lieske J, Cameron B, Drinkwine B, et al. Surfer's myelopathy-demonstrated by diffusion-weighted magnetic resonance imaging: a case report and literature review. J Comput Assist Tomogr 2011; 35:492.
  183. Takakura T, Yokoyama O, Sakuma F, et al. Complete paraplegia resulting from surfer's myelopathy. Am J Phys Med Rehabil 2013; 92:833.
  184. Fessa CK, Lee BS. An Australian case of surfer's myelopathy. Clin J Sport Med 2012; 22:281.
  185. Chung HY, Sun SF, Wang JL, et al. Non-traumatic anterior spinal cord infarction in a novice surfer: a case report. J Neurol Sci 2011; 302:118.
  186. Shuster A, Franchetto A. Surfer's myelopathy--an unusual cause of acute spinal cord ischemia: a case report and review of the literature. Emerg Radiol 2011; 18:57.
Topic 5093 Version 19.0

References

آیا می خواهید مدیلیب را به صفحه اصلی خود اضافه کنید؟