Vascular myelopathy
Encyclopedia
Vascular myelopathy refers to an abnormality of the spinal cord
in regards 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
(death of tissue) in the anterior artery can be caused by disease in vessels originating from the segmental arteries such as atheroma
(arterial wall swelling) or aortic dissection
(a tear in the aorta
).
of tissue in the anterior spinal artery or its branches. It is characterised by pain which radiates at onset and sudden quadraplegia (paralysis of all four limbs) or paraplegia
(paralysis of the lower body). Within days, flaccid limbs become spastic
and hyporeflexia
(underactive nerve responses) turns into hyperreflexia
(overactive nerve responses) and extensor plantar nerve responses. Sensory loss to pain and temperature also occurs up to the level of damage on the spinal cord, as damage to different areas will affect different parts of the body.
In diagnosis, other causes of abrupt paralysis should be excluded such as cord compression
, transverse myelitis
(infection of the spinal cord) and Guillain-Barré syndrome
. A specific cause of the infarction
should be looked for, such as diabetes, polyarteritis nodosa
(inflammatory damage of vessels) or systemic lupus erythematosus
. Neurosyphilis
is also a known cause. Other causes include:
Treatment is supportive and aims to relieve symptoms. The prognosis
is dependant upon individual circumstances and factors.
structures that are present are much less vulnerable to ischemia
since they have a better blood supply. When posterior spinal artery syndrome does occur, dorsal
columns are damaged and ischemia may spread into the posterior horns. Clinically the syndrome presents as a loss of tendon
reflexes and loss of joint
position sense.
.
s bypass the capillary
network (which has not yet developed) and thus creates a shunt. AVMs appear as a mass of convoluted, dilated vessels. In regards to the spinal cord, they are usually located in the thoracolumbar region (between the thoracic and lumbar regions, 60% of the time), as opposed to the upper thoracic (20%) and cervical regions (approximately 15%). Cervical malformations arise from the anterior spinal artery and lie within the cord, whereas thoracolumbar malformations can be internal, external or encompass both areas of the cord.
Malformations can be recognised as part of an acute illness or gradual onset disease. In diseases such as subarachnoid hemorrhage
, signs and symptoms include headache
, neck stiffness and back and leg pain. Extradural, subdural and intramedullary hematoma
s are all signs of acute cord compression. Gradual onset diseases are more common (85-90% of all diseases leading to a diagnosis of malformation) and are usually due to an increased venous pressure. Other factors such as thrombosis
or arachnoiditis
can be involved. A bruit
(unusual blood sounds) may be head overlying the spinal arteriovenous malformation. Very occasionally, nevus
(moles) or angiolipoma
s are found.
Myelography
is used to confirm the diagnosis of AVMs and it shows 'snake-like' vessels on the cord's surface. If the myelogram is positive, angiography is required to show the extent of malformation and the exact site of the shunt. Magnetic resonance imaging
(MRI) may show the appropriate area. If AVMs are left untreated, 50% of patients with gradual symptoms will be unable to walk within 3 years of onset. Operations can prevent progression and may improve any gait
or incontinence
.
Spinal cord
The spinal cord is a long, thin, tubular bundle of nervous tissue and support cells that extends from the brain . The brain and spinal cord together make up the central nervous system...
in regards 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
Artery of Adamkiewicz
In human anatomy, the Artery of Adamkiewicz is the largest anterior segmental medullary artery.It has several other names, including:* Adamkiewicz artery* great radicular artery of Adamkiewicz* major anterior segmental medullary artery...
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
Atherosclerosis
Atherosclerosis is a condition in which an artery wall thickens as a result of the accumulation of fatty materials such as cholesterol...
of spinal arteries is rare, necrosis
Necrosis
Necrosis is the premature death of cells in living tissue. Necrosis is caused by factors external to the cell or tissue, such as infection, toxins, or trauma. This is in contrast to apoptosis, which is a naturally occurring cause of cellular death...
(death of tissue) in the anterior artery can be caused by disease in vessels originating from the segmental arteries such as atheroma
Atheroma
In pathology, an atheroma is an accumulation and swelling in artery walls that is made up of macrophage cells, or debris, that contain lipids , calcium and a variable amount of fibrous connective tissue...
(arterial wall swelling) or aortic dissection
Aortic dissection
Aortic dissection occurs when a tear in the inner wall of the aorta causes blood to flow between the layers of the wall of the aorta and force the layers apart. The dissection typically extends anterograde, but can extend retrograde from the site of the intimal tear. Aortic dissection is a medical...
(a tear in the aorta
Aorta
The aorta is the largest artery in the body, originating from the left ventricle of the heart and extending down to the abdomen, where it branches off into two smaller arteries...
).
Anterior spinal artery syndrome
Anterior spinal artery syndrome is necrosisNecrosis
Necrosis is the premature death of cells in living tissue. Necrosis is caused by factors external to the cell or tissue, such as infection, toxins, or trauma. This is in contrast to apoptosis, which is a naturally occurring cause of cellular death...
of tissue in the anterior spinal artery or its branches. It is characterised by pain which radiates at onset and sudden quadraplegia (paralysis of all four limbs) or paraplegia
Paraplegia
Paraplegia is an impairment in motor or sensory function of the lower extremities. The word comes from Ionic Greek: παραπληγίη "half-striking". It is usually the result of spinal cord injury or a congenital condition such as spina bifida that affects the neural elements of the spinal canal...
(paralysis of the lower body). Within days, flaccid limbs become spastic
Spastic
The word spastic is used differently depending on location which has led to some controversy and misunderstanding. Derived via Latin from the Greek spastikos , the word originally referred to a change in muscles affected by the medical condition spasticity, which is seen in spastic diplegia and...
and hyporeflexia
Hyporeflexia
Hyporeflexia is the condition of below normal or absent reflexes. It can be tested for by using a reflex hammer. It is the opposite of hyperreflexia....
(underactive nerve responses) turns into hyperreflexia
Hyperreflexia
Hyperreflexia is defined as overactive or overresponsive reflexes. Examples of this can include twitching or spastic tendencies, which are indicative of upper motor neuron disease as well as the lessening or loss of control ordinarily exerted by higher brain centers of lower neural pathways...
(overactive nerve responses) and extensor plantar nerve responses. Sensory loss to pain and temperature also occurs up to the level of damage on the spinal cord, as damage to different areas will affect different parts of the body.
In diagnosis, other causes of abrupt paralysis should be excluded such as cord compression
Spinal cord compression
Spinal cord compression develops when the spinal cord is compressed by bone fragments from a vertebral fracture, a tumor, abscess, ruptured intervertebral disc or other lesion...
, transverse myelitis
Transverse myelitis
Transverse myelitis is a neurological disorder caused by an inflammatory process of the spinal cord, and can cause axonal demyelination. The name is derived from Greek referring to the "spinal cord", and the suffix -itis, which denotes inflammation...
(infection of the spinal cord) and Guillain-Barré syndrome
Guillain-Barré syndrome
Guillain–Barré syndrome , sometimes called Landry's paralysis, is an acute inflammatory demyelinating polyneuropathy , a disorder affecting the peripheral nervous system. Ascending paralysis, weakness beginning in the feet and hands and migrating towards the trunk, is the most typical symptom...
. A specific cause of the infarction
Infarction
In medicine, infarction refers to tissue death that is caused by a local lack of oxygen due to obstruction of the tissue's blood supply. The resulting lesion is referred to as an infarct.-Causes:...
should be looked for, such as diabetes, polyarteritis nodosa
Polyarteritis nodosa
Polyarteritis nodosa is a vasculitis of medium & small-sized arteries, which become swollen and damaged from attack by rogue immune cells. Polyarteritis nodosa is also called Kussmaul disease or Kussmaul-Maier disease...
(inflammatory damage of vessels) or systemic lupus erythematosus
Systemic lupus erythematosus
Systemic lupus erythematosus , often abbreviated to SLE or lupus, is a systemic autoimmune disease that can affect any part of the body. As occurs in other autoimmune diseases, the immune system attacks the body's cells and tissue, resulting in inflammation and tissue damage...
. Neurosyphilis
Neurosyphilis
Neurosyphilis is an infection of the brain or spinal cord caused by the bacterium Treponema pallidum. It usually occurs in people who have had untreated syphilis for many years, usually about 10 - 20 years after first infection.-Symptoms and signs:...
is also a known cause. Other causes include:
| Type of disease | Causes |
|---|---|
| Small blood vessel | Diabetes, polyarteritis nodosa Polyarteritis nodosa Polyarteritis nodosa is a vasculitis of medium & small-sized arteries, which become swollen and damaged from attack by rogue immune cells. Polyarteritis nodosa is also called Kussmaul disease or Kussmaul-Maier disease... , systemic lupus erythematosus Systemic lupus erythematosus Systemic lupus erythematosus , often abbreviated to SLE or lupus, is a systemic autoimmune disease that can affect any part of the body. As occurs in other autoimmune diseases, the immune system attacks the body's cells and tissue, resulting in inflammation and tissue damage... , neurosyphilis Neurosyphilis Neurosyphilis is an infection of the brain or spinal cord caused by the bacterium Treponema pallidum. It usually occurs in people who have had untreated syphilis for many years, usually about 10 - 20 years after first infection.-Symptoms and signs:... |
| Arterial compression or occlusion | Extradural tumor Tumor A tumor or tumour is commonly used as a synonym for a neoplasm that appears enlarged in size. Tumor is not synonymous with cancer... s, aortic dissection Aortic dissection Aortic dissection occurs when a tear in the inner wall of the aorta causes blood to flow between the layers of the wall of the aorta and force the layers apart. The dissection typically extends anterograde, but can extend retrograde from the site of the intimal tear. Aortic dissection is a medical... , disk fragmentation, aortic surgery Surgery Surgery is an ancient medical specialty that uses operative manual and instrumental techniques on a patient to investigate and/or treat a pathological condition such as disease or injury, or to help improve bodily function or appearance.An act of performing surgery may be called a surgical... |
| Embolic occlusion | Decompression sickness Decompression sickness Decompression sickness describes a condition arising from dissolved gases coming out of solution into bubbles inside the body on depressurization... , aortic arteriography |
| Hypotensive | Myocardial infarction Myocardial infarction Myocardial infarction or acute myocardial infarction , commonly known as a heart attack, results from the interruption of blood supply to a part of the heart, causing heart cells to die... |
Treatment is supportive and aims to relieve symptoms. The prognosis
Prognosis
Prognosis is a medical term to describe the likely outcome of an illness.When applied to large statistical populations, prognostic estimates can be very accurate: for example the statement "45% of patients with severe septic shock will die within 28 days" can be made with some confidence, because...
is dependant upon individual circumstances and factors.
Posterior spinal artery syndrome
Posterior spinal artery syndrome is much rarer than its anterior counterpart as the white matterWhite matter
White matter is one of the two components of the central nervous system and consists mostly of myelinated axons. White matter tissue of the freshly cut brain appears pinkish white to the naked eye because myelin is composed largely of lipid tissue veined with capillaries. Its white color is due to...
structures that are present are much less vulnerable to ischemia
Ischemia
In medicine, ischemia is a restriction in blood supply, generally due to factors in the blood vessels, with resultant damage or dysfunction of tissue. It may also be spelled ischaemia or ischæmia...
since they have a better blood supply. When posterior spinal artery syndrome does occur, dorsal
Dorsum (biology)
In anatomy, the dorsum is the upper side of animals that typically run, fly, or swim in a horizontal position, and the back side of animals that walk upright. In vertebrates the dorsum contains the backbone. The term dorsal refers to anatomical structures that are either situated toward or grow...
columns are damaged and ischemia may spread into the posterior horns. Clinically the syndrome presents as a loss of tendon
Tendon
A tendon is a tough band of fibrous connective tissue that usually connects muscle to bone and is capable of withstanding tension. Tendons are similar to ligaments and fasciae as they are all made of collagen except that ligaments join one bone to another bone, and fasciae connect muscles to other...
reflexes and loss of joint
Joint
A joint is the location at which two or more bones make contact. They are constructed to allow movement and provide mechanical support, and are classified structurally and functionally.-Classification:...
position sense.
Transient ischemic attack
Transient ischemic attacks (TIAs) rarely affect the spinal cord and usually affect the brain; however, cases have been documented in these areas. Spinal ateriovenous malformations are the main cause and are represented later in this article. That said, TIAs can result from emboli in calcific aortic disease and aortic coarctationAortic coarctation
Coarctation of the aorta, or aortic coarctation, is a congenital condition whereby the aorta narrows in the area where the ductus arteriosus inserts.-Types:There are three types:...
.
Spinal arteriovenous malformations
Spinal arteriovenous malformations (AVMs, or angiomatous malformations) are congenital (from birth) abnormalities of blood vessels. Arteries that directly communicate with veinVein
In the circulatory system, veins are blood vessels that carry blood towards the heart. Most veins carry deoxygenated blood from the tissues back to the heart; exceptions are the pulmonary and umbilical veins, both of which carry oxygenated blood to the heart...
s bypass the capillary
Capillary
Capillaries are the smallest of a body's blood vessels and are parts of the microcirculation. They are only 1 cell thick. These microvessels, measuring 5-10 μm in diameter, connect arterioles and venules, and enable the exchange of water, oxygen, carbon dioxide, and many other nutrient and waste...
network (which has not yet developed) and thus creates a shunt. AVMs appear as a mass of convoluted, dilated vessels. In regards to the spinal cord, they are usually located in the thoracolumbar region (between the thoracic and lumbar regions, 60% of the time), as opposed to the upper thoracic (20%) and cervical regions (approximately 15%). Cervical malformations arise from the anterior spinal artery and lie within the cord, whereas thoracolumbar malformations can be internal, external or encompass both areas of the cord.
Malformations can be recognised as part of an acute illness or gradual onset disease. In diseases such as subarachnoid hemorrhage
Subarachnoid hemorrhage
A subarachnoid hemorrhage , or subarachnoid haemorrhage in British English, is bleeding into the subarachnoid space—the area between the arachnoid membrane and the pia mater surrounding the brain...
, signs and symptoms include headache
Headache
A headache or cephalalgia is pain anywhere in the region of the head or neck. It can be a symptom of a number of different conditions of the head and neck. The brain tissue itself is not sensitive to pain because it lacks pain receptors. Rather, the pain is caused by disturbance of the...
, neck stiffness and back and leg pain. Extradural, subdural and intramedullary hematoma
Hematoma
A hematoma, or haematoma, is a localized collection of blood outside the blood vessels, usually in liquid form within the tissue. This distinguishes it from an ecchymosis, which is the spread of blood under the skin in a thin layer, commonly called a bruise...
s are all signs of acute cord compression. Gradual onset diseases are more common (85-90% of all diseases leading to a diagnosis of malformation) and are usually due to an increased venous pressure. Other factors such as thrombosis
Thrombosis
Thrombosis is the formation of a blood clot inside a blood vessel, obstructing the flow of blood through the circulatory system. When a blood vessel is injured, the body uses platelets and fibrin to form a blood clot to prevent blood loss...
or arachnoiditis
Arachnoiditis
Arachnoiditis is a neuropathic disease caused by the inflammation of the arachnoid, one of the membranes that surround and protect the nerves of the central nervous system, including the brain and spinal cord...
can be involved. A bruit
Bruit
Bruit is the term for the unusual sound that blood makes when it rushes past an obstruction in an artery when the sound is auscultated with the bell portion of a stethoscope.The term "bruit" simply refers to the sound...
(unusual blood sounds) may be head overlying the spinal arteriovenous malformation. Very occasionally, nevus
Nevus
Nevus is the medical term for sharply-circumscribed and chronic lesions of the skin. These lesions are commonly named birthmarks and moles. Nevi are benign by definition...
(moles) or angiolipoma
Angiolipoma
Angiolipoma is a subcutaneous nodule with vascular structure, having all other features of a typical lipoma. They are commonly painful....
s are found.
Myelography
Myelography
Myelography is a type of radiographic examination that uses a contrast medium to detect pathology of the spinal cord, including the location of a spinal cord injury, cysts, and tumors...
is used to confirm the diagnosis of AVMs and it shows 'snake-like' vessels on the cord's surface. If the myelogram is positive, angiography is required to show the extent of malformation and the exact site of the shunt. Magnetic resonance imaging
Magnetic resonance imaging
Magnetic resonance imaging , nuclear magnetic resonance imaging , or magnetic resonance tomography is a medical imaging technique used in radiology to visualize detailed internal structures...
(MRI) may show the appropriate area. If AVMs are left untreated, 50% of patients with gradual symptoms will be unable to walk within 3 years of onset. Operations can prevent progression and may improve any gait
Gait
Gait is the pattern of movement of the limbs of animals, including humans, during locomotion over a solid substrate. Most animals use a variety of gaits, selecting gait based on speed, terrain, the need to maneuver, and energetic efficiency...
or incontinence
Urinary incontinence
Urinary incontinence is any involuntary leakage of urine. It is a common and distressing problem, which may have a profound impact on quality of life. Urinary incontinence almost always results from an underlying treatable medical condition but is under-reported to medical practitioners...
.
See also
- Foix-Alajouanine syndromeFoix-Alajouanine syndromeFoix-Alajouanine syndrome is a disorder caused by an arteriovenous malformation of the spinal cord. The patients present with symptoms indicating spinal cord involvement , and pathological examination reveals disseminated nerve cell death in the spinal cord and abnormally dilated and tortuous vessels...
- Chronic cerebrospinal venous insufficiencyChronic cerebrospinal venous insufficiencyChronic cerebrospinal venous insufficiency is a term developed by Italian researcher Paolo Zamboni in 2008 to describe compromised flow of blood in the veins draining the central nervous system...
- Peripheral vascular disease