Spinal cord stroke

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Spinal cord stroke is a rare type of stroke with compromised blood flow to any region of spinal cord owing to occlusion or bleeding, leading to irreversible neuronal death. [1] It can be classified into two types, ischaemia and haemorrhage, in which the former accounts for 86% of all cases, a pattern similar to cerebral stroke. [2] [3] The disease is either arisen spontaneously from aortic illnesses or postoperatively. [4] It deprives patients of motor function or sensory function, and sometimes both. [5] Infarction usually occurs in regions perfused by anterior spinal artery, which spans the anterior two-thirds of spinal cord. [6] Preventions of the disease include decreasing the risk factors and maintaining enough spinal cord perfusion pressure during and after the operation. The process of diagnosing the ischemic and hemorrhagic spinal cord stroke includes applying different MRI protocols and CT scan. [7] [8] Treatments for spinal cord stroke are mainly determined by the symptoms and the causes of the disease. For example, antiplatelet and corticosteroids might be used to reduce the risk of blood clots in ischaemic spinal stroke patients, while rapid surgical decompression is applied to minimize neurological injuries in haemorrhagic spinal stroke patients instead. [9] Patients may spend years for rehabilitation after the spinal cord stroke. [3]

Contents

Signs and symptoms

Signs and symptoms are related to the portion of spinal cord affected, and appear below the level of lesion. [1] [5] Abrupt onset of pain at the back or neck marks the location of ischaemia or hemorrhage at the beginning, which radiates as the damage intensifies. [9] [10] Temporary paresis in limbs may occur days before the onset of spinal ischaemic stroke, though the relationship remains unclear. [1] [11] While it takes minutes for ischaemic spinal stroke to develop the symptoms, the time could be extended to days and weeks in hemorrhagic spinal stroke. [9] [10] Infarction occurs predominantly in arteries, and the watershed region, which refers thoracic spinal cord here, is highly susceptible to ischaemic attack. [4] Patients with a male gender, younger age, lower body mass index, hypertension, diabetes mellitus, renal insufficiency and chronic obstructive pulmonary disease are predisposed to higher risks of severe spinal cord stroke. [3] [12]

Symptoms of various types of spinal cord stroke. Kabir Spinal Cord Lesions and Syndromes.jpg
Symptoms of various types of spinal cord stroke.

Anterior spinal cord syndrome

A major feature is losing motor function such as voluntary movement, reflexes and coordination as a result of compromised anterior and lateral corticospinal tract, anterior grey matter and spinocerebellar tract. [5] [13] There is also a loss in nociception and thermosensation as a result of interrupted spinothalamic tract. [5]

Posterior spinal cord syndrome

Sensory functions namely vibration, fine touch, and proprioception are undermined, which are associated to dorsal column. [5] Unlike anterior spinal cord stroke, motor functions are not handicapped in posterior spinal cord stroke. [5]

Central spinal cord syndrome

In central spinal cord syndrome, impairment of motor function in the upper body is considerably more severe than that of lower body, which is related to hyperextension of corticospinal tracts and spinocerebellar tract in cervical spinal cord, accompanied by dysfunction in urinary bladder and sensational loss at a varying degree. [5] [14]

Brown-Séquard syndrome

Brown-Séquard syndrome is only the subtype that affects the spinal cord unilaterally, either anteriorly, posteriorly, or both. [2] Ipsilateral loss of vibration, fine touch, body position perception and fine movement control, as well as contralateral loss of axial muscles and movement coordination are found. [5] Spasticity followed by dysfunctinoal regulation of muscle tone also exists. [5]

Transverse

Death of cells at the complete transverse level is presented clinically as lower paraplegia or quadriplegia, sensory loss below the lesion, urinary incontinence, and disturbances in autonomous nervous system and hormonal system. [11]

Causes

Diseases in aorta are recognized as a widely seen contributor of spontaneous spinal cord ischaemia, represented by rupturing of thoracic aortic aneurysm, arterial occlusion by aortic intima separated from endothelial wall in aortic dissection, and aortic coarctation. [4] Embolism, meningeal inflammation at spinal cord, global ischaemia and abusing nicotinic drugs are also identified to factors. [2]

Artery of Adamkiewicz is supplied by posterior intercostal artery, and drains into anterior spinal artery. Artery of Adamkiewicz-01.jpg
Artery of Adamkiewicz is supplied by posterior intercostal artery, and drains into anterior spinal artery.

Aortic surgeries contribute to many iatrogenic spinal cord ischaemia, although its percentage is much lower than that of spontaneous type. [4] Thoracic endovascular aortic repair (TEVAR) was carried out to introduce a stent graft in order to treat thoracoabdominal aortic aneurysm, a condition of enlarged aorta with weakened vascular wall, as well as traumas and atherosclerosis. [12] Segmental medullary arteries, notably the artery of Adamkiewicz, could be excluded from circulation after blockage of intercostal arteries by the device, which directly branches from descending aorta. [12] Furthermore, during open repair, blood flow within aorta is halted by clamping to facilitate the sewing of interposition graft. [12] The reduced blood flow to anterior and posterior radicular artery could trigger spinal stroke. [12] Cases of spinal stroke following operations like aortography, spinal anesthesia and lumbar spine surgery are reported. [4]

Abnormalities in blood vessels including arteriovenous malformations, arteriovenous fistulas and cavernomas are preferably presented as ischaemia and occasionally hemorrhage. [2] [9] The direct fusion between arteries and veins increases blood pressure in radiculomedullary vein and coronal venous plexus, which is an important factor of venous congestive myelopathy and infarction. [6]

Prolonged compression on the blood network by vertebral diseases such as cervical spondylosis and protruded intervertebral disks can be attributed to acute ischaemia in spinal cord, yet the correlation is uncertain. [3] [11]

On the other hand, trauma, which generally originates from terminal vascular network, is a common cause of spinal cord hemorrhage for all four subtypes, namely haematomyelia, subarachnoid hemorrhage, subdural hemorrhage and epidural hemorrhage. [9] There is a correlation between anticoagulating drugs and hemorrhagic stroke. [9]

Causes are often not clearly defined in clinical settings. [3]

Mechanism

The pathophysiology of spinal stroke is similar to its counterpart in brain. Decreasing blood flow hampers oxygen and glucose delivery to neurones, causing a huge decline in ATP production and failure of calcium pump. [15] The rising intracellular calcium level activates a series of enzymes like phospholipase A2 (PLA2), COX-2, calcineurin, calpain, mitogen-activated protein kinase, nitric oxide synthase, matrix metalloproteinases (MMPs) to produce proinflammatory and proapoptotic chemicals. [15] There are also activation of cytokines and changes in transcription factors. [15] Meanwhile, glutamate is released to extracellular space and binds to its excitatory receptors, further exacerbating calcium influx and a cascade of events involving mitochondrial, cell membrane damage, and production of reactive oxygen species. [15] Such excitotoxicity is closely associated with the eventual neuronal cell death and loss of tract function. [15]

Prevention

Risk factors

Modifiable risk factors that contribute to the common strokes such as hypertension and heart disease, are found less commonly in the formation of spinal cord stroke. [3] On the other hand, diabetes mellitus, peripheral artery disease, smoking and cholesterol are associated more with such disease. [3] Prevention and treatment of these modifiable risk factors could reduce the likelihood of spinal cord stroke.

Intraoperative strategy

As the high difficulty for the detection during operation, somatosensory evoked potential monitoring or motor evoked potential monitoring is necessary to early detect the spinal cord ischaemia in anesthetized patients for quick intervention. [12] [16] Cerebrospinal fluid drainage is always used to decrease intraspinal pressure and increase blood flow to the spinal cord to avoid hypotension, thus reducing the risk of spinal cord ischaemia. [16]

Postoperative strategy

Probability of postoperative spinal cord stroke is linked to both aneurysm extent, particularly extent II (descending aorta at full length) and length of graft, which highlights the importance of postoperative management. [12] It aims to maintain enough spinal cord perfusion pressure, and make serial neurologic assessments to detect the disease. Similar to the intraoperative strategy, increasing the spinal cord perfusion as an immediate intervention may increase the chance of successful treatment. [17] Neurological examination should be conducted after anesthesia to test the motor function of the low extremity of patients. By using this method to detect whether patients have spinal cord ischaemia, doctors could decide whether rapid treatment should be provided. [12]

Diagnosis

Owl-eyes sign exhibits bilaterally symmetric circular to ovoid foci of T2-weighted signals in anterior horn cells. Owl's eye sign.jpg
Owl-eyes sign exhibits bilaterally symmetric circular to ovoid foci of T2-weighted signals in anterior horn cells.

Spinal stroke could be easily misdiagnosed because of its rarity. [10] Doctor will first assess the clinical symptoms of the patient, such as paralysis, sensory loss and urinary and bowel dysfunction, to determine whether it is possible for the spinal stroke. After that, different MRI protocols will be used, including axial and sagittal T1 and T2-weighted sequences and diffusion-weighted imaging (DWI).

Ischemic spinal cord stroke

As the non-contrast CT and spine CT angiography are ineffective in imaging modalities, doctors use MRI to confirm the diagnosis. MRI findings, including pencillike hyperintensities on T2-weighted sagittal images and "owl's eyes" or "snake eyes" sign on T2 axial images, indicate the infarction is predominately in the watershed area of the gray matter of ventral horn (anterior spinal artery infarct). [10] Also, posterior paramedian triangular hyperintensity in T2 hyperintensity indicates posterior spinal artery infarct. On a T1 sequence, we may also observe a cord expansion and a decreased signal. [18] However, traditional MRI may show no abnormality especially for those patients in the acute phase. [8] DWI is very sensitive for early detection of spinal cord infarction and shows a typical high signal intensity. [19]

Hemorrhagic spinal cord stroke

On axial imaging of MRI at the level of the denticulate ligaments, the inverted Mercedes-Benz sign denotes the form taken on by a spinal subdural hematoma. Spinal-subdural-haematoma-inverted-mercedes-benz-sign-1.jpg
On axial imaging of MRI at the level of the denticulate ligaments, the inverted Mercedes-Benz sign denotes the form taken on by a spinal subdural hematoma.

To identify the hematoma in the spinal cord, MRI with and without gadolinium enhancement is the preferred choice. [9] CT is also used to identify the hemorrhage and provide evidence for pathological analysis. Complete spinal MRI with MR angiography is used when patients with subarachnoid hemorrhage without the intracranial etiology. [20] As the evaluation of the need for intradural interrogation is important, it is necessary to differentiate between subdural and epidural hematomas. Based on the location of the hematoma, use both axial and sagittal images of MRI to identify the boundary between hematoma and fat. [7] An inverted Mercedes-Benz sign shows the spinal subdural hematoma on the axial image. [21]

Treatment

Given the rarity and heterogeneity of spinal cord stroke, symptomatic treatment of associated complications is applied, which is based on patients' own circumstances.

Ischemic spinal cord stroke

Although some literature suggest that thrombolysis could be the treatment for ischaemic spinal stroke, the associated risks are unknown due to the scarce data. [22] If the cause is global hypoperfusion, maintaining enough blood pressure to maintain adequate spinal perfusion is needed. [18] Also, anticoagulant and antiplatelet agents have been prescribed to prevent vascular occlusion or embolism. [8] Corticosteroids are prescribed in situations of vasculitis or aortitis. [18]

Hemorrhagic spinal cord stroke

Surgical decompression

The goal of treatment in an acute situation is to relieve pressure on the spinal cord. Several case studies show a substantial link between the time from bleeding to surgical decompression and neurological outcome, with the greatest results coming from individuals who had surgery within 12 hours after symptom onset. [23] Therefore, surgical decompression should be undertaken as soon as possible to limit neurological injury. [24]

Administration of large dose corticosteroids

While waiting for surgery, high-dose corticosteroids were administered in the acute phase. It could reduce oedema and secondary cord compression. [25]

Reversal of anticoagulation

As anticoagulation treatment with warfarin or heparin has been linked to spontaneous haematomyelia, reversal anticoagulation is used to reduce the risk of bleeding by using suitable antidotes. Protamine is used to reverse heparin and low-molecular-weight heparin. Vitamin K is a reversal agent for warfarin. [26]

Prognosis

It is possible that spinal cord ischaemia patients have a full recovery. Although the mortality rate after spinal cord ischaemia is relatively high (23%), 58% of the survivors were ambulating with or without gait assistance at their final follow-up appointment. Patients with total paraplegia and sensory loss at nadir can, however, progress significantly over months to years. [27]

Related Research Articles

<span class="mw-page-title-main">Arteriovenous malformation</span> Vascular anomaly

An arteriovenous malformation (AVM) is an abnormal connection between arteries and veins, bypassing the capillary system. Usually congenital, this vascular anomaly is widely known because of its occurrence in the central nervous system, but can appear anywhere in the body. The symptoms of AVMs can range from none at all to intense pain or bleeding, and they can lead to other serious medical problems.

<span class="mw-page-title-main">Foix–Alajouanine syndrome</span> Medical condition

Foix–Alajouanine syndrome, also called subacute ascending necrotizing myelitis, is a disease caused by an arteriovenous malformation of the spinal cord. In particular, most cases involve dural arteriovenous malformations that present in the lower thoracic or lumbar spinal cord. The condition is named after Charles Foix and Théophile Alajouanine who first described the condition in 1926.

<span class="mw-page-title-main">Transverse myelitis</span> Medical condition of the spinal cord

Transverse myelitis (TM) is a rare neurological condition wherein the spinal cord is inflamed. The adjective transverse implies that the spinal inflammation (myelitis) extends horizontally throughout the cross section of the spinal cord; the terms partial transverse myelitis and partial myelitis are sometimes used to specify inflammation that affects only part of the width of the spinal cord. TM is characterized by weakness and numbness of the limbs, deficits in sensation and motor skills, dysfunctional urethral and anal sphincter activities, and dysfunction of the autonomic nervous system that can lead to episodes of high blood pressure. Signs and symptoms vary according to the affected level of the spinal cord. The underlying cause of TM is unknown. The spinal cord inflammation seen in TM has been associated with various infections, immune system disorders, or damage to nerve fibers, by loss of myelin. As opposed to leukomyelitis which affects only the white matter, it affects the entire cross-section of the spinal cord. Decreased electrical conductivity in the nervous system can result.

<span class="mw-page-title-main">Cerebrovascular disease</span> Condition that affects the arteries that supply the brain

Cerebrovascular disease includes a variety of medical conditions that affect the blood vessels of the brain and the cerebral circulation. Arteries supplying oxygen and nutrients to the brain are often damaged or deformed in these disorders. The most common presentation of cerebrovascular disease is an ischemic stroke or mini-stroke and sometimes a hemorrhagic stroke. Hypertension is the most important contributing risk factor for stroke and cerebrovascular diseases as it can change the structure of blood vessels and result in atherosclerosis. Atherosclerosis narrows blood vessels in the brain, resulting in decreased cerebral perfusion. Other risk factors that contribute to stroke include smoking and diabetes. Narrowed cerebral arteries can lead to ischemic stroke, but continually elevated blood pressure can also cause tearing of vessels, leading to a hemorrhagic stroke.

<span class="mw-page-title-main">Aortic dissection</span> Injury to the innermost layer of the aorta

Aortic dissection (AD) occurs when an injury to the innermost layer of the aorta allows blood to flow between the layers of the aortic wall, forcing the layers apart. In most cases, this is associated with a sudden onset of severe chest or back pain, often described as "tearing" in character. Vomiting, sweating, and lightheadedness may also occur. Damage to other organs may result from the decreased blood supply, such as stroke, lower extremity ischemia, or mesenteric ischemia. Aortic dissection can quickly lead to death from insufficient blood flow to the heart or complete rupture of the aorta.

<span class="mw-page-title-main">Thrombolysis</span> Breakdown (lysis) of blood clots formed in blood vessels, using medication

Thrombolysis, also called fibrinolytic therapy, is the breakdown (lysis) of blood clots formed in blood vessels, using medication. It is used in ST elevation myocardial infarction, stroke, and in cases of severe venous thromboembolism.

<span class="mw-page-title-main">Stroke</span> Death of a region of brain cells due to poor blood flow

Stroke is a medical condition in which poor blood flow to the brain causes cell death. There are two main types of stroke: ischemic, due to lack of blood flow, and hemorrhagic, due to bleeding. Both cause parts of the brain to stop functioning properly.

<span class="mw-page-title-main">Intracranial hemorrhage</span> Hemorrhage, or bleeding, within the skull

Intracranial hemorrhage (ICH), also known as intracranial bleed, is bleeding within the skull. Subtypes are intracerebral bleeds, subarachnoid bleeds, epidural bleeds, and subdural bleeds.

<span class="mw-page-title-main">Cerebral infarction</span> Medical condition

Cerebral infarction is the pathologic process that results in an area of necrotic tissue in the brain. It is caused by disrupted blood supply (ischemia) and restricted oxygen supply (hypoxia), most commonly due to thromboembolism, and manifests clinically as ischemic stroke. In response to ischemia, the brain degenerates by the process of liquefactive necrosis.

<span class="mw-page-title-main">Anterior spinal artery syndrome</span> Human spinal cord disorder

Anterior spinal artery syndrome is syndrome caused by ischemia of the anterior spinal artery, resulting in loss of function of the anterior two-thirds of the spinal cord. The region affected includes the descending corticospinal tract, ascending spinothalamic tract, and autonomic fibers. It is characterized by a corresponding loss of motor function, loss of pain and temperature sensation, and hypotension.

<span class="mw-page-title-main">Endovascular aneurysm repair</span> Surgery used to treat abdominal aortic aneurysm

Endovascular aneurysm repair (EVAR) is a type of minimally-invasive endovascular surgery used to treat pathology of the aorta, most commonly an abdominal aortic aneurysm (AAA). When used to treat thoracic aortic disease, the procedure is then specifically termed TEVAR for "thoracic endovascular aortic/aneurysm repair." EVAR involves the placement of an expandable stent graft within the aorta to treat aortic disease without operating directly on the aorta. In 2003, EVAR surpassed open aortic surgery as the most common technique for repair of AAA, and in 2010, EVAR accounted for 78% of all intact AAA repair in the United States.

<span class="mw-page-title-main">Surfer's myelopathy</span> A spinal cord injury caused by hyperextension of the back

Surfer's myelopathy is a rare, nontraumatic spinal cord injury caused by hyperextension of the back injury and resulting in paraplegia. During hyperextension, a blood vessel leading to the spine, such as the anterior spinal artery, can become kinked, depriving the spinal cord of oxygen. Although the condition derives its name from the fact that the phenomenon is most often seen in those surfing for the first time, it can be caused by any activity in which the back is hyperextended. In some cases, the paralysis is permanent.

<span class="mw-page-title-main">Cervical artery dissection</span> Medical condition

Cervical artery dissection is dissection of one of the layers that compose the carotid and vertebral artery in the neck (cervix). They include:

<span class="mw-page-title-main">Leptomeningeal collateral circulation</span>

The leptomeningeal collateral circulation is a network of small blood vessels in the brain that connects branches of the middle, anterior and posterior cerebral arteries, with variation in its precise anatomy between individuals. During a stroke, leptomeningeal collateral vessels allow limited blood flow when other, larger blood vessels provide inadequate blood supply to a part of the brain.

Vascular myelopathy refers to an abnormality of the spinal cord in regard to its blood supply. The blood supply is complicated and supplied by two major vessel groups: the posterior spinal arteries and the anterior spinal arteries—of which the Artery of Adamkiewicz is the largest. Both the posterior and anterior spinal arteries run the entire length of the spinal cord and receive anastomotic (conjoined) vessels in many places. The anterior spinal artery has a less efficient supply of blood and is therefore more susceptible to vascular disease. Whilst atherosclerosis of spinal arteries is rare, necrosis in the anterior artery can be caused by disease in vessels originating from the segmental arteries such as atheroma or aortic dissection.

<span class="mw-page-title-main">Acute limb ischaemia</span> Occurs when there is a sudden lack of blood flow to a limb

Acute limb ischaemia (ALI) occurs when there is a sudden lack of blood flow to a limb, within 14 days of symptoms onset. It is different from another condition which is more chronic called critical limb ischemia (CLD). CLD is the end stage of peripheral vascular disease where there is still some collateral circulation (alternate circulation pathways} that bring some blood to the distal parts of the limbs. While limbs in both acute and chronic limb ischemia may be pulseless, a chronically ischemic limb is typically warm and pink due to a well-developed collateral artery network and does not need emergency intervention to avoid limb loss.

<span class="mw-page-title-main">Open aortic surgery</span> Surgical technique

Open aortic surgery (OAS), also known as open aortic repair (OAR), describes a technique whereby an abdominal, thoracic or retroperitoneal surgical incision is used to visualize and control the aorta for purposes of treatment, usually by the replacement of the affected segment with a prosthetic graft. OAS is used to treat aneurysms of the abdominal and thoracic aorta, aortic dissection, acute aortic syndrome, and aortic ruptures. Aortobifemoral bypass is also used to treat atherosclerotic disease of the abdominal aorta below the level of the renal arteries. In 2003, OAS was surpassed by endovascular aneurysm repair (EVAR) as the most common technique for repairing abdominal aortic aneurysms in the United States.

Remote ischemic conditioning (RIC) is an experimental medical procedure that aims to reduce the severity of ischaemic injury to an organ such as the heart or the brain, most commonly in the situation of a heart attack or a stroke, or during procedures such as heart surgery when the heart may temporary suffer ischaemia during the operation, by triggering the body's natural protection against tissue injury. Although noted to have some benefits in experimental models in animals, this is still an experimental procedure in humans and initial evidence from small studies have not been replicated in larger clinical trials. Successive clinical trials have failed to identify evidence supporting a protective role in humans.

Embolic stroke of undetermined source (ESUS) is an embolic stroke, a type of ischemic stroke, with an unknown origin, defined as a non-lacunar brain infarct without proximal arterial stenosis or cardioembolic sources. As such, it forms a subset of cryptogenic stroke, which is part of the TOAST-classification. The following diagnostic criteria define an ESUS:

A migrainous infarction is a rare type of ischaemic stroke which occurs in correspondence with migraine aura symptoms. Symptoms include headaches, visual disturbances, strange sensations and dysphasia, all of which gradually worsen causing neurological changes which ultimately increase the risk of an ischaemic stroke. Typically, women under the age of 45 who experience migraine with aura (MA) are at the greatest risk for developing migrainous infarction, especially when combined with smoking and use of oral contraceptives.

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  24. ↑ Akpınar, Aykut; Celik, Bahattin; Canbek, Ihsan; Karavelioğlu, Ergun (2016). "Acute Paraplegia due to Thoracic Hematomyelia". Case Reports in Neurological Medicine. 2016: 1–3. doi: 10.1155/2016/3138917 . ISSN   2090-6668. PMC   4960333 . PMID   27478663.
  25. ↑ Bracken, Michael B (2012-01-18). Cochrane Injuries Group (ed.). "Steroids for acute spinal cord injury". Cochrane Database of Systematic Reviews. 1 (9): CD001046. doi:10.1002/14651858.CD001046.pub2. PMC   6513405 . PMID   22258943.
  26. ↑ Frontera, Jennifer A.; Lewin III, John J.; Rabinstein, Alejandro A.; Aisiku, Imo P.; Alexandrov, Anne W.; Cook, Aaron M.; del Zoppo, Gregory J.; Kumar, Monisha A.; Peerschke, Ellinor I. B.; Stiefel, Michael F.; Teitelbaum, Jeanne S (February 2016). "Guideline for Reversal of Antithrombotics in Intracranial Hemorrhage: A Statement for Healthcare Professionals from the Neurocritical Care Society and Society of Critical Care Medicine". Neurocritical Care. 24 (1): 6–46. doi:10.1007/s12028-015-0222-x. ISSN   1541-6933. PMID   26714677. S2CID   21145026.
  27. ↑ Robertson, C. E.; Brown, R. D.; Wijdicks, E. F. M.; Rabinstein, A. A. (2012-01-10). "Recovery after spinal cord infarcts: Long-term outcome in 115 patients". Neurology. 78 (2): 114–121. doi:10.1212/WNL.0b013e31823efc93. ISSN   0028-3878. PMC   3466672 . PMID   22205760.
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