Stroke & Vascular Disorders
Ischemic stroke
Oct. 29, 2024
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Support: service@medlink.com
Editor: editor@medlink.com
ISSN: 2831-9125
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Spinal cord vascular disorders are rare, though potentially devastating, conditions that require prompt recognition and treatment. These vascular disorders are heterogenous in origin, with pathologies involving both the arterial and venous vessels. Understanding the clinical hallmarks and potential etiologies are necessary to provide effective intervention and timely management. Most treatment strategies are reliant on a multidisciplinary team.
• Spinovascular disorders are relatively rare and encompass a wide breadth of etiologic designations. | |
• The majority of acute spinovascular processes have back or radicular pain. | |
• Classic intramedullary spinal cord disorders follow well-defined neurovascular territories. | |
• The etiologies of these conditions are heterogenous, including embolic, iatrogenic, hypotensive, ischemic, hemorrhagic, and congestive pathologies. | |
• Treatment requires a multidisciplinary effort and may require an interventional approach. | |
• Owing to the rarity of the disease, much is not robustly known on the outcomes of those with a spinovascular condition. |
Although the presence of a spinal epidural hematoma (termed “spinal apoplexy”) was possibly first described in 1682 by GJ Duverney, intramedullary spinovascular disease was not readily identified until the 1800s. In 1817 and 1857, early cases reported spinovascular complications ranging from abdominal aortic aneurysm to the development of paraplegia (112). In 1881 and 1882, Polish pathologist Albert Adamkiewicz provided insight into the vascular anatomy of the spinal cord (71). His work allowed for further characterization of spinal cord vascular syndromes, including the first report of an anterior spinal artery syndrome in 1909 (117). In addition to large artery spinovascular complications, more intricate pathologies were identified. In 1912, Marie and Foix described the syndrome of "tephromalacie anterieure," or ischemic lesions in the anterior horn cells due to atherosclerotic disease (73). In 1926, Foix and Alajouanine subsequently described a congestive myelopathy from two cases of spinal dural arteriovenous fistulas (30). Embolization of spinal dural arteriovenous fistula was subsequently reported by Newton and Adams in 1968 (86). With the increasing interest in cardiovascular surgeries in the 1970s, reports emerged of incidences of anterior spinal cord syndrome related to hypoperfusion (113). More recent advances in neuroimaging have more readily identified spinovascular disorders, such as spinal dural arteriovenous fistula and stroke.
• The symptoms of a spinovascular syndrome are highly reliant on the location and etiology of the condition. | |
• Timing and onset of symptoms are of great importance in delineating etiology. |
The clinical presentation varies depending on the vascular territory involved. The following vascular syndromes have been identified: anterior spinal artery syndrome, posterior spinal arteries syndrome, transverse infarction of the spinal cord, central cord infarction, venous infarction, transient spinal ischemia, and lacunar infarction.
Anterior spinal artery infarct. This syndrome is classically heralded by pain in approximately a third of individuals. If pain is present, it can be diffuse, radicular, or girdle-like in distribution and with both neuralgiform and throbbing qualities. Symptoms usually occur suddenly but may progress over minutes to hours, peaking at 12 hours (139). Unique to the spinal cord level involved, pain may mimic other conditions, such as cluster headaches in cervical cord infarcts and ischemic heart disease in thoracic cord infarcts (22; 60). Clinical features of an anterior spinal artery infarct are dependent on level and completeness of the infarct. Typically, lower motor neuron flaccid paralysis occurs with hyporeflexia, though over time, spasticity may occur depending on involvement of the anterior horn cells. Pain and temperature sensation may be lost beginning one to two levels below the infarct owing to the anatomy of the anterior fasciculus (137; 91). A “man-in-a-barrel” semiology has also been reported (08). Autonomic symptoms, such as bradycardia and incontinence, are common and depend on the spinal segments involved. Incomplete anterior spinal cord artery infarcts may lead to variable symptoms (119). Due to anatomical variations in spinal vasculature, partial Brown-Sequard syndromes (also known as hemi-cord myelopathy) may occur (55).
Transient spinal cord symptoms similar to a hemispheric transient ischemic attack may be a heralding sign of impending spinal cord infarct (25). Transient ischemia of the cervical cord may cause "drop attacks," whereas spinal cord claudication may occur in the lumbar region, leading to painless weakness of the lower extremities precipitated by effort and relieved by rest (124).
Posterior spinal artery infarct. These lesions are rare and are often difficult to recognize in a timely manner. Like anterior spinal artery infarcts, patients usually have pain along the spine. Due to predominant dorsal column involvement, vibration and proprioception are absent below the level of the lesion. Accompanying the expected dorsal column dysfunction is typically variable weakness and autonomic dysfunction, likely related to anterior anastomoses with the anterior spinal artery (118).
Complete segmental infarct. The syndrome is characterized by a segmental complete spinal infarct analogous to transverse myelitis. Depending on the segment involved, flaccid quadriplegia or quadriparesis and total loss of sensation below the lesion, as well as sphincter dysfunction, may occur.
Central cord infarction. Ischemia in the arterial border zone between anterior spinal and posterior spinal arteries may result in this type of clinical infarct. The picture, however, is believed to be clinically indistinguishable from anterior spinal artery syndrome (116).
Venous infarction. Spinal venous infarcts are rare and often subacute. There may be some degree of motor and sensory loss but without a well-defined pattern. The level of sensory loss may not be as easily delineated as in arterial infarcts (116). Those with symptoms of portal venous hypertension may be more at risk (40).
Lacunar cord infarction. Though perhaps controversial and with some variability in the literature, another potential ischemic myelopathy is of lacunar origin presenting as a subacute progressive weakness referable to the anterior horns. Lacunar cord infarction is also referred to as "vascular myelopathy in old age." The usual findings are wasting and weakness of the small muscles of the hand as well as scattered pyramidal signs, mimicking motor neuron disease (29).
Hematomyelia. Nontraumatic hematomyelia can occur spontaneously or as a symptom of a secondary complication, such as hemophilia (17). The most common presentation is excruciating pain in the back of sudden onset, often with radicular radiation. Neurologic symptoms may occur acutely, subacutely or in a stepwise fashion (61).
Spinal epidural or subdural hematomas. Extramedullary hematomas are rare and often of unclear etiology. Clinically, they present with rapid radicular pain and progressive myelopathic symptoms (depending on location) over the course of hours (58).
Spinal subarachnoid hemorrhage. Clinically, spinal subarachnoid hemorrhage is usually manifest by excruciating pain and meningismus, radicular involvement, and variable weakness (120).
Spinal dural arteriovenous fistulas. Spinal dural arteriovenous fistulas are the most common spinal vascular anomaly. The commonest presenting symptom is subacute lower extremity weakness that worsens with exercise and occurs in a subacute or stepwise deteriorative manner. Sensory and autonomic involvement are variable (82; 69).
Intramedullary fistulas. People with intradural fistulas frequently have an acute hemorrhagic presentation, though a progressive syndrome may be present. The arms are usually affected (cervicothoracic location) (104).
Cavernous malformations. These lesions are typically present in the thoracic cord and are present with an acute hemorrhage or as a subacute progressive or recurrent myelopathy (18).
Ischemic infarcts. The outcomes of an ischemic spinal cord infarct depend on location, severity, and etiology. Nearly a quarter of those with an ischemic spinal cord infarct will die, and complete recovery is unfortunately rare (101). Most with motor symptoms will need assistance with ambulation, though again, this depends on the severity of the index event (21; 37). Rehabilitation has been shown to improve functional outcomes (85).
Long-term complications include persistent pain in as many as 90% of patients, independent of motor function recovery. The frequency and intensity of pain after spinal cord infarction seem higher than with other myelopathies and may have an impact on the quality of life (138). Further, a myriad of complications may emerge, such as autonomic dysfunction with urinary and bowel incontinence leading to urinary tract infections or sepsis, autonomic dysreflexia, sexual dysfunction, pressure sores, deep venous thromboses, and pulmonary emboli as a result of immobility, and mental health dysfunction may occur (138; 107; 101).
Hemorrhagic processes. The prognosis of spinal epidural and subdural hematomas mainly depends on the preoperative neurologic status, anticoagulation use, cervical level, and involvement of the bladder autonomics (125). An estimated 5% of those with a subdural spinal hematoma may die, and 30%, if treated in a timely manner, may make a full recovery (23; 125). Surgical and interventional treatments are preferred to conservative measures (141). Rapid interventional treatment is favored for those with intramedullary hemorrhage or symptomatic medullary cavernoma (123).
Vascular anomalies. Unfortunately, spinal dural arteriovenous fistulas are difficult to diagnose, and those with this condition tend to have a long delay in diagnosis. Rapid identification of the fistula is imperative to forestall the progressive congestive myelopathy (50; 103). Outcomes are dependent on the time to treatment and severity of the symptoms.
A 57-year-old man with a history of smoking, coronary atherosclerotic disease, chronic obstructive pulmonary disease, and nephrolithiasis underwent emergency Dacron graft replacement of a dissecting abdominal aortic aneurysm between the renal and distal common iliac arteries. Postsurgically, the patient was noted to have flaccid paraplegia with absent reflexes and a pin-prick sensory level below the umbilicus. His vibration sense was intact at the knees and ankles. MRI of the thoracic cord demonstrated an extensive cord infarction.
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• Vascular anatomy may help define a location of the insult but also define the potential etiology. A thorough history and review of events at the time of onset are key. |
Segmental vessels. There are approximately 31 pairs of segmental vessels arising from different sources (in craniocaudal order): vertebral arteries, ascending cervical arteries, deep cervical arteries, superior intercostal artery, aorta, iliolumbar artery, and lateral sacral arteries (116). These segmental arteries then divide into anterior and posterior rami. The posterior rami enter the intervertebral foramen and constitute either the anterior or posterior (or both) radicular arteries. Radicular arteries are also known as medullary or radiculomedullary arteries (116).
Anterior radicular (medullary) arteries supply the anterior spinal artery, which acts as an anastomotic channel between them, and are distributed unevenly throughout the cord. The cervical-upper thoracic region is highly vascularized, whereas the midthoracic area (T4 to T8) is poorly perfused because it is supplied by a single small radicular artery known as the "arteria radicularis magna" or artery of Adamkiewicz (usually T5 to T7 level, but variable). Posterior radicular (medullary) arteries are smaller in size. They supplement the posterior spinal arteries.
Longitudinal vessels. There are three longitudinal vessels: one anterior spinal artery and two posterior spinal arteries. The anterior spinal artery is an anastomotic channel that runs anteriorly in the midline. The anterior spinal artery constitutes the caudal continuation of the two vertebral arteries, at the level of the foramen magnum. It extends throughout the length of the cord in the anterior median fissure. The posterior spinal arteries run in the posterior lateral aspect of the cord. They usually originate from the vertebral arteries but occasionally come from the posterior inferior cerebellar arteries (124; 116).
Vasa coronae. These surface vessels originate from the anterior spinal artery and posterior spinal arteries, forming a vascular ring around the cord (116).
Penetrating vessels. They originate from the anterior spinal artery, posterior spinal arteries, and the vasa coronae to perfuse the central portions of the cord (116).
Central (sulcal) arteries. They are branches of the anterior spinal artery entering the cord through the anterior median fissure and give longitudinal branches. There are more than 200 sulcal arteries, which are unevenly distributed throughout the cord. These arteries mainly perfuse anterior horns, deep gray matter of posterior horn, and central white matter (116).
Posterior median septum arteries. These are branches of the posterior spinal arteries that supply the dorsal funiculi and the central gray matter. The remaining posterior one third of the cord is perfused by other penetrating branches of posterior spinal arteries (116).
Extrinsic veins. Spinal venous anatomy is more variable than its arterial counterpart. There are typically one or two anterior spinal veins and usually a single posterior spinal vein. In addition, posterolateral and anterolateral spinal veins form discontinuous channels.
Extrathecally, an internal venous plexus lies in the epidural space, and an external plexus drains the vertebral bodies (116).
Intrinsic veins. There are two anterior central veins (draining central white matter and medial anterior horn), two posterior central veins (median septum white matter and gray commissure), and an intrinsic venous drainage for the rest of the cord (116).
Spinal cord border zones. The lower thoracic and upper lumbar cord (T6 to L3) is considered the area most vulnerable to ischemia due to less vascular anastomoses compared with other levels. The artery of Adamkiewicz, essentially an end artery, is classically responsible for this segment’s perfusion (115).
In a transverse section, the anterior spinal artery territory (anterior two thirds) is believed to be more susceptible to ischemia than the posterior one third of the cord. The explanation may be the existence of more efficient functional anastomoses at the level of the posterior spinal arteries region (116).
The gray matter is considered more susceptible to ischemia than the white matter, probably due to the differences in metabolic demand (121).
Etiology of spinal cord infarcts. Spinal infarcts result from a heterogenous collection of pathologies and may be divided into non-iatrogenic, iatrogenic, venous infarcts, and spinal transient ischemic attack (See Table 1).
Noniatrogenic etiologies | |
• Diabetic arteriopathy (124) | |
- Associated renal transplants (13) | |
• Inflammatory or infectious | |
- Syphilis (11) | |
• Vasculitic | |
- Systemic lupus erythematous (109) | |
• Non-inflammatory arteriopathies dissection (eg, congenital afibrinogenemia) (59) | |
- Dissecting aortic aneurysm (109) | |
• Embolic | |
- Nucleus pulposus embolism (45) | |
• Hypoperfusional states (115) | |
- Cardiac arrest (16) | |
• Disc protrusion (11) | |
- Cervical hyperextension injuries | |
• Tumor (124) | |
- Radicular artery compression | |
• Recreational drugs | |
- Heroin (70) | |
• Hypercoagulable states | |
- Antiphospholipid syndrome (39) | |
• Anemia (16) | |
Iatrogenic causes | |
• Aortic surgery (risk increases if previous colectomy) (108) | |
- Aortic coarctation | |
• Endovascular aortic repair (34) | |
- Neoarsphenamine (116) | |
• Dural fistula embolization/surgery (if common vessels) (83) | |
Venous infarction | |
• Sepsis (116) | |
- Fibrocartilaginous (100; 01) | |
• Hypercoagulable states (109) | |
- Associated to malignancies (116) | |
• Intramedullary tumor (109) | |
Spinal transient ischemic attack | |
• Paget disease (flow diversion to bone) (92) |
• Ischemic spinal infarcts are rare but may account for up to 1% of all ischemic strokes. Other types of spinovascular conditions are equally rare but have less data defining incidence. |
Ischemic processes. The incidence of spinal cord infarction has been estimated to be 3.1 per 100,000 person-years and may account for up to 1% of all ischemic strokes (99; 95). Vascular surgery data show that a spinal cord infarct may occur in up to a third of persons who undergo elective thoracoabdominal aneurysm repair (78).
Spinal venous infarctions are probably rarer than the arterial infarctions, although the exact frequency is unknown (116).
Hemorrhagic processes. Owing to the rarity of the condition, the incidence of spontaneous spinal hemorrhagic processes is not well reported. Post-procedural epidural and subdural hematomas are well documented in the surgical literature, with one study reporting roughly a 1% incidence (88). Risk factors for this subset of conditions includes surgical intervention, anticoagulant use, coagulopathy, and trauma (11). In some cases, congenital processes, such as cavernous malformation, are another risk factor.
Vascular anomalies. These conditions are rare, with an estimated incidence of 0.234 per 100,000 person years (41). Risk factors include congenital and genetic syndromes in younger adults (usually early adulthood) or posttraumatic and severe arthritic conditions in older adults (83; 98). Spinal cavernomas may occur in genetic syndromes, such as familial cavernomas, and as a complication of spinal radiation (72).
• Preventing an ischemic spinal infarct relies on the same strategies as secondary stroke prevention. | |
• Intraoperative monitoring and careful monitoring of vital signs are important in preventing iatrogenic spinal cord infarcts during aortic interventions. |
No data are available regarding primary and secondary prevention of spinal cord infarctions in the general population other than treating any underlying disease that could represent a potential causative condition. There are data regarding the prevention of iatrogenic spinal cord infarctions that occur during abdominal aortic surgery, such as the prevention of hypotension, limiting prolonged aortic clamping times. There are suggestions about the importance of preventing hypotensive episodes during surgery, the potential of hypothermia, and the use of pharmacologic agents (115; 52). The use of somatosensory evoked potentials during surgery is a common practice to minimize ischemic injury in the spinal cord (54).
Traumatic epidural, subdural, and cord hemorrhages may be mitigated by correcting coagulation dysfunction and monitoring the hematological parameters in anticoagulated patients. Avoiding high-risk maneuvers in those cases, such as lumbar punctures, may lower the incidence.
Spinal cord vascular conditions require timely identification and treatment to prevent long-term morbidity and mortality. Conditions that may mimic spinovascular disorders include autoimmune conditions (eg, neuromyelitis optica) that affect the spinal cord in either a longitudinal extensive or transverse fashion. Infectious myelitis may have a similar presentation as well and is differentiated by a thorough clinical history and accompanying features (eg, cough, constitutional symptoms, high-risk exposures) and is confirmed by lumbar puncture microbial analysis. The umbrella term “Guillain-Barre–related syndromes” shares many symptoms of a spinovascular emergency, such as progressive or rapid-onset weakness and spinal pain.
A variety of conditions may be associated with or predispose to a spinovascular process; many of these were discussed earlier.
Additional considerations include familial cerebral cavernous malformation syndrome, which may similarly present with intramedullary cavernous malformation. Hereditary hemorrhagic telangiectasia may present rarely with a spinal dural arteriovenous fistula or other spinal malformation. Cobb syndrome is an exceedingly rare condition resulting in skin manifestations and spinal arteriovenous malformation.
• Spinal MRI, including T1, T2, hemosiderin sensitive sequences, and DWI, are recommended for noninvasively diagnosing a spinovascular condition. | |
• Conventional arteriography remains the gold standard in diagnosing spinovascular anomalies. |
The evaluation for an acute spinal cord infarction includes neuroimaging studies to exclude the presence of an acute extrinsic spinal cord compression or an intraspinal mass. An MRI of the spinal cord should provide the needed information.
A series on the use of MRI offered a diagnostic high yield; approximately 93% of T2-weighted images detect the ischemic lesion at the time of the onset of symptoms (31). T1-weighted images are less reliable because 70% appear isointense at onset and only 18% are hypointense. Diffusion-weighted MRI may be useful for the detection of early ischemic cord lesions, though the size of the spinal cord may limit robust determination (132). Classic T2 hyperintense findings of an anterior spinal artery distribution infarct include “owl-eyes” and “pencil-like” hyperintensity patterns (137). Signs of vertebral body infarction on MRI are a useful radiological marker of spinal cord infarction associated with fibrocartilaginous embolism (27).
Admitting laboratory tests should include a comprehensive metabolic, infectious, and autoimmune screening. CSF analysis should be completed if concern arises for an alternative diagnostic etiology to ischemia.
Spinal dural arteriovenous fistula may be apparent on sagittal MRI T2 sequences due to the presence of flow voids. A T1 hypointense but T2 hyperintense pattern along three to seven vertebral body lengths additionally suggests a spinal dural arteriovenous fistula (48). However, the gold standard in diagnosis remains a conventional arteriogram (83).
• Treatment of an acute spinal infarct depends on the etiology, though the use of antithrombotics, permissive hypertension, and lowering intrathecal pressures may aid in augmenting spinal blood flow. | |
• Intravenous thrombolytics may be beneficial in the acute management of an ischemic spinal cord stroke. | |
• Collaboration with neurointerventional and neurosurgical teams are important for the treatment of spinovascular anomalies. |
Owing to the rarity of spinovascular conditions, there is no specific approved treatment for acute spinal cord infarctions. Intravenous rtPA has been used anecdotally, but the safety and efficacy are not known (26). Additionally, treatment by intravenous thrombolysis is contraindicated in those with aortic dissection or those who have an index major surgical intervention, further limiting the sample size. The use of permissive hypertension and lower intrathecal pressures through a lumbar drain have shown some theoretical success.
Spinal cord infarcts that are referable to conventional cardiovascular risk factors should be optimized medically with the aid of statin therapy, antihyperglycemics, and antithrombotic medications.
Spinal epidural and subdural hematomas should be decompressed emergently. Occasionally, fresh frozen plasma needs to be given to correct underlying coagulopathies.
Spinal dural and intramedullary arteriovenous malformations can be treated by excision, surgically eliminating the transmission of arterial pressure into the venous system of the cord. Surgical interruption of the intradural draining vein can provide a cure due to retrograde thrombosis. Embolization of these lesions is an alternative (83).
Symptomatic cavernous malformations may benefit from surgery, but there are not enough data about the natural history of these lesions (77).
Much is not known on the outcomes of treatment of a spinal cord infarct because the majority of documentation are case reports. Surgical treatment of spinovascular anomalies is well-documented, with a success rate of over 90% in treating spinal arteriovenous malformation via venous disconnection; however, this ultimately depends on the level of disability preoperatively and the experience of the surgeon (24). Additionally, a meta-analysis of spinal cord infarct from a malignancy found 12-month survival to be 16.3%, likely owing to the aggressiveness of the underlying malignancy but also the functional limitations imposed by the infarct (93).
Pregnancy may predispose individuals to venous infarctions, but only isolated case reports can be found in the literature (100).
The main anesthetic concern is to prevent hypotension during surgical intervention.
All contributors' financial relationships have been reviewed and mitigated to ensure that this and every other article is free from commercial bias.
Eric Goldstein MD
Dr. Goldstein of Brown University has no relevant financial relationships to disclose.
See ProfileSteven R Levine MD
Dr. Levine of the SUNY Health Science Center at Brooklyn has no relevant financial relationships to disclose.
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ISSN: 2831-9125
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