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Tetraplegia, also known as quadriplegia, is defined as the dysfunction or loss of
motor An engine or motor is a machine designed to convert one or more forms of energy into mechanical energy. Available energy sources include potential energy (e.g. energy of the Earth's gravitational field as exploited in hydroelectric power ge ...
and/or sensory function in the cervical area of the
spinal cord The spinal cord is a long, thin, tubular structure made up of nervous tissue, which extends from the medulla oblongata in the brainstem to the lumbar region of the vertebral column (backbone). The backbone encloses the central canal of the spi ...
. A loss of motor function can present as either
weakness Weakness is a symptom of a number of different conditions. The causes are many and can be divided into conditions that have true or perceived muscle weakness. True muscle weakness is a primary symptom of a variety of skeletal muscle diseases, i ...
or paralysis leading to partial or total loss of function in the arms, legs, trunk, and pelvis;
paraplegia Paraplegia, or paraparesis, is an impairment in motor or sensory function of the lower extremities. The word comes from Ionic Greek () "half-stricken". It is usually caused by spinal cord injury or a congenital condition that affects the neural ...
is similar but affects the
thoracic The thorax or chest is a part of the anatomy of humans, mammals, and other tetrapod animals located between the neck and the abdomen. In insects, crustaceans, and the extinct trilobites, the thorax is one of the three main divisions of the cr ...
, lumbar, and sacral segments of the spinal cord and arm function is spared. The paralysis may be
flaccid Flaccid paralysis is a neurological condition characterized by weakness or paralysis and reduced muscle tone without other obvious cause (e.g., trauma). This abnormal condition may be caused by disease or by trauma affecting the nerves associat ...
or spastic. A loss of sensory function can present as an impairment or complete inability to sense light touch, pressure, heat, pinprick/pain, and
proprioception Proprioception ( ), also referred to as kinaesthesia (or kinesthesia), is the sense of self-movement, force, and body position. It is sometimes described as the "sixth sense". Proprioception is mediated by proprioceptors, mechanosensory neuron ...
. In these types of spinal cord injury, it is common to have a loss of both sensation and motor control.


Signs and symptoms

Although the most obvious symptom is impairment of the limbs, functioning is also impaired in the trunk and pelvic organs. This can lead to loss or impairment of controlling
bowel The gastrointestinal tract (GI tract, digestive tract, alimentary canal) is the tract or passageway of the digestive system that leads from the mouth to the anus. The GI tract contains all the major organs of the digestive system, in humans and ...
and
bladder The urinary bladder, or simply bladder, is a hollow organ in humans and other vertebrates that stores urine from the kidneys before disposal by urination. In humans the bladder is a distensible organ that sits on the pelvic floor. Urine enters ...
, sexual function, digestion, breathing and other autonomic functions. Furthermore, sensation is usually impaired in affected areas. This may manifest as numbness, reduced sensation or
neuropathic pain Neuropathic pain is pain caused by damage or disease affecting the somatosensory system. Neuropathic pain may be associated with abnormal sensations called dysesthesia or pain from normally non-painful stimuli (allodynia). It may have continuous ...
. Secondarily, because of their depressed functioning and immobility, people with tetraplegia are often more vulnerable to pressure sores,
osteoporosis Osteoporosis is a systemic skeletal disorder characterized by low bone mass, micro-architectural deterioration of bone tissue leading to bone fragility, and consequent increase in fracture risk. It is the most common reason for a broken bone am ...
and fractures, frozen joints,
spasticity Spasticity () is a feature of altered skeletal muscle performance with a combination of paralysis, increased tendon reflex activity, and hypertonia. It is also colloquially referred to as an unusual "tightness", stiffness, or "pull" of muscles. ...
, respiratory complications, infections,
autonomic dysreflexia Autonomic dysreflexia (AD) is a potential medical emergency classically characterized by uncontrolled hypertension and bradycardia, although tachycardia is known to commonly occur. AD occurs most often in individuals with spinal cord injuries wit ...
,
deep vein thrombosis Deep vein thrombosis (DVT) is a type of venous thrombosis involving the formation of a blood clot in a deep vein, most commonly in the legs or pelvis. A minority of DVTs occur in the arms. Symptoms can include pain, swelling, redness, and en ...
, and cardiovascular disease. The severity of the condition depends on both the level at which the spinal cord is injured and the extent of the injury. An individual with an injury at C1 (the highest cervical vertebra, at the base of the skull) will probably lose function from the neck down and be ventilator-dependent. An individual with a C7 injury may lose function from the chest down but still retain use of the arms and much of the hands. An individual in between, with a C5 injury may lose some function from the chest down and fine motor skills in his/her hands but still have flexion and extension abilities of certain muscles around the back or arm area. The extent of the injury is also important. A complete severing of the spinal cord will result in complete loss of function from that vertebra down. A partial severing or even bruising of the spinal cord results in varying degrees of mixed function and paralysis. A common misconception with tetraplegia is that the victim cannot move legs, arms or any of the major function; this is often not the case. Some individuals with tetraplegia can walk and use their hands, as though they did not have a spinal cord injury, while others may use wheelchairs and they can still have function of their arms and mild finger movement; again, this varies based on the degree of damage to the spinal cord and is mostly seen with incomplete tetraplegia. It is common to have movement in limbs, such as the ability to move the arms but not the hands, or to be able to use the fingers but not to the same extent as before the injury. Furthermore, the deficit in the limbs may not be the same on both sides of the body; either left or right side may be more affected, depending on the location of the lesion on the spinal cord. Another important fact to keep in mind would be the possibility to see a person with quadriplegia moving any areas affected by the injury sporadically. One of the main causes for this would be myoclonus, or muscle spasms. "After a spinal cord injury, the normal flow of signals is disrupted, and the message does not reach the brain. Instead, the signals are sent back to the motor cells in the spinal cord and cause a reflex muscle spasm. This can result in a twitch, jerk or stiffening of the muscle."


Causes

Tetraplegia is caused by damage to the
brain A brain is an organ that serves as the center of the nervous system in all vertebrate and most invertebrate animals. It is located in the head, usually close to the sensory organs for senses such as vision. It is the most complex organ in ...
or the
spinal cord The spinal cord is a long, thin, tubular structure made up of nervous tissue, which extends from the medulla oblongata in the brainstem to the lumbar region of the vertebral column (backbone). The backbone encloses the central canal of the spi ...
at a high level. The injury, which is known as a lesion, causes the loss of partial or total function of all four limbs, meaning the arms and the legs. Typical causes of this damage are trauma (such as a traffic collision, diving into shallow water, a fall, a sports injury), disease (such as transverse myelitis,
Guillain–Barré syndrome Guillain–Barré syndrome (GBS) is a rapid-onset muscle weakness caused by the immune system damaging the peripheral nervous system. Typically, both sides of the body are involved, and the initial symptoms are changes in sensation or pain often ...
,
multiple sclerosis Multiple (cerebral) sclerosis (MS), also known as encephalomyelitis disseminata or disseminated sclerosis, is the most common demyelinating disease, in which the insulating covers of nerve cells in the brain and spinal cord are damaged. Thi ...
, or
polio Poliomyelitis, commonly shortened to polio, is an infectious disease caused by the poliovirus. Approximately 70% of cases are asymptomatic; mild symptoms which can occur include sore throat and fever; in a proportion of cases more severe sy ...
), or
congenital disorder A birth defect, also known as a congenital disorder, is an abnormal condition that is present at birth regardless of its cause. Birth defects may result in disabilities that may be physical, intellectual, or developmental. The disabilities can ...
s (such as
muscular dystrophy Muscular dystrophies (MD) are a genetically and clinically heterogeneous group of rare neuromuscular diseases that cause progressive weakness and breakdown of skeletal muscles over time. The disorders differ as to which muscles are primarily af ...
). Tetraplegia is defined in many ways; C1–C4 usually affects arm movement more so than a C5–C7 injury; however, all tetraplegics have or have had some kind of finger dysfunction. So, it is not uncommon to have a tetraplegic with fully functional arms but no nervous control of their fingers and thumbs. It is possible to have a broken neck without becoming tetraplegic if the vertebrae are fractured or dislocated but the spinal cord is not damaged. Conversely, it is possible to injure the spinal cord without breaking the spine, for example when a ruptured disc or bone spur on the vertebra protrudes into the spinal column.


Anatomy & Function

Since tetraplegia is defined as dysfunction in the cervical spinal cord, this section will focus on the anatomy of the cervical spinal cord. To understand how tetraplegia presents after injury, it is imperative to have a broad knowledge of the cervical spinal roots and it's many functions. In the cervical spine, nerve roots exit the spine above the associated
vertebra The spinal column, a defining synapomorphy shared by nearly all vertebrates,Hagfish are believed to have secondarily lost their spinal column is a moderately flexible series of vertebrae (singular vertebra), each constituting a characteristic ...
(i.e. the C6 nerve root exits above the C6 vertebra). By evaluating what
nerve root A nerve root (Latin: ''radix nervi'') is the initial segment of a nerve leaving the central nervous system. Nerve roots can be classified as: *Cranial nerve roots: the initial or proximal segment of one of the twelve pairs of cranial nerves leaving ...
of the cervical spine is injured, the affected muscle groups and dermatomes can be determined. This informs the evaluator as to what activities may be limited as a result of the injury. This is typically done at 72 hours post-injury; exams done prior to this time have been found to be inaccurate due to the presence of swelling and other confounding factors. For example, an injury at the C6 nerve root level will affect the function of the triceps (elbow extension) but the
biceps The biceps or biceps brachii ( la, musculus biceps brachii, "two-headed muscle of the arm") is a large muscle that lies on the front of the upper arm between the shoulder and the elbow. Both Muscle head, heads of the muscle arise on the scapul ...
(elbow flexion) will be spared; in this case, an injury at the C6 root level affects all function at that level and below whereas the C5 nerve root, which controls the biceps, is spared since it is above the C6 level in the spinal column. When classifying an individual's level of function, there are numerous functional assessment tools that may be used in a clinical setting and it is often up to the clinician's discretion as to which tools are used. A comprehensive list of these tools may be found on th
ShirleyRyan AbilityLab website


Diagnosis


Classification

Spinal cord injuries A spinal cord injury (SCI) is damage to the spinal cord that causes temporary or permanent changes in its function. Symptoms may include loss of muscle function, sensation, or autonomic function in the parts of the body served by the spinal co ...
are classified as complete and incomplete by the
American Spinal Injury Association The American Spinal Injury Association (ASIA), formed in 1973, publishes the International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI), which is a neurological exam widely used to document sensory and motor impairment ...
(ASIA) classification. The ASIA scale grades patients based on their functional impairment as a result of the injury, grading a patient from A to D. This has considerable consequences for surgical planning and therapy. After a comprehensive neurologic exam testing segments of the body corresponding to spinal nerve roots, the examiner will determine the patient's motor level and sensory level (i.e. motor level C6, sensory level C7). These levels are unique for the patient's left and right side. This level is assigned based on the lowest (closest to the patient's feet) intact motor and sensory level. After this assignment, a neurological level of injury (NLI) is determined. The NLI is the lowest segment with intact sensory and motor function provided there is normal sensory and motor function above this segment.


Complete spinal-cord lesions

As in the above ASIA chart, a complete spinal cord injury is any injury which has absent motor and sensory function in the sacral segments S4 and S5. This is verified during the physical exam by the absence of all three of: voluntary anal contraction, deep anal pressure, and pinprick+light touch sensation in the perineal area. S4 and S5 are both sacral nerve roots found at the lowest portion of the spinal cord. In simpler terms, "complete" is meant as a way to express that the spinal cord is injured such that no signal, motor or sensory, is carried to or from the level of injury to these lower levels of the spinal cord.


Incomplete spinal-cord lesions

Incomplete spinal cord injuries result in varied post injury presentations. There are three main syndromes described, depending on the exact site and extent of the lesion. # Central cord syndrome: an injury to the central area of the spinal cord, most often seen as a result of a fall with subsequent hyperextension injury. This typically presents with weakness greater in the upper limbs than in the lower limbs. #
Brown-Séquard syndrome Brown-Séquard syndrome (also known as Brown-Séquard's hemiplegia, Brown-Séquard's paralysis, hemiparaplegic syndrome, hemiplegia et hemiparaplegia spinalis, or spinal hemiparaplegia) is caused by damage to one half of the spinal cord, i.e. hem ...
: hemisection of the spinal cord with resultant loss in: a.) ipsilateral proprioception, vibration, and motor control below the level of injury b.) complete sensory loss at the level of injury c.) contralateral pain and temperature loss. # Anterior cord syndrome: a lesion of the anterior two-thirds of the spinal cord, most commonly due to ischemia. This typically presents with loss of pain, temperature, and motor function at and below the level of injury. # Cauda equina syndrome: a lesion of the lumbosacral nerve roots that may spare the spinal cord. As these nerve roots are lower motor neurons, a flaccid lower limb paralysis is typically seen along with loss of bowel and bladder reflexes, varying degrees of impairment of sensation, and loss of sacral reflexes ( bulbocavernosus reflex, anal wink). # Conus medullaris syndrome: a lesion similar to cauda equina syndrome however this lesion is typically found higher in the cord. This presents clinically similarly to cauda equina syndrome however there may be intact sacral reflexes. Unlike cauda equina, the unique location of this syndrome leads it to present with mixed upper and lower motor neuron signs. For most patients with ASIA A (complete) tetraplegia, ASIA B (incomplete) tetraplegia and ASIA C (incomplete) tetraplegia, the International Classification level of the patient can be established without great difficulty. The surgical procedures according to the International Classification level can be performed. In contrast, for patients with ASIA D (incomplete) tetraplegia it is difficult to assign an International Classification other than International Classification level X (others). Therefore, it is more difficult to decide which surgical procedures should be performed. A far more personalized approach is needed for these patients. Decisions must be based more on experience than on texts or journals. The results of tendon transfers for patients with complete injuries are predictable. On the other hand, it is well known that muscles lacking normal excitation perform unreliably after surgical tendon transfers. Despite the unpredictable aspect in incomplete lesions, tendon transfers may be useful. The surgeon should be confident that the muscle to be transferred has enough power and is under good voluntary control. Pre-operative assessment is more difficult to assess in incomplete lesions. Patients with an incomplete lesion also often need therapy or surgery before the procedure to restore function to correct the consequences of the injury. These consequences are hypertonicity/spasticity, contractures, painful hyperesthesias and paralyzed proximal upper limb muscles with distal muscle sparing. Spasticity is a frequent consequence of incomplete injuries. Spasticity often decreases function, but sometimes a patient can control the spasticity in a way that it is useful to their function. The location and the effect of the spasticity should be analyzed carefully before treatment is planned. An injection of
Botulinum toxin Botulinum toxin, or botulinum neurotoxin (BoNT), is a neurotoxic protein produced by the bacterium ''Clostridium botulinum'' and related species. It prevents the release of the neurotransmitter acetylcholine from axon endings at the neuromu ...
(Botox) into spastic muscles is a treatment to reduce spasticity. This can be used to prevent muscle shortening and early contractures. Over the last ten years, an increase in traumatic incomplete lesions is seen, due to the better protection in traffic.


Treatment

Upper limb paralysis refers to the loss of function of the elbow and hand. When upper limb function is absent as a result of a spinal cord injury it is a major barrier to regain autonomy. People with tetraplegia should be examined and informed concerning the options for reconstructive surgery of the tetraplegic arms and hands.


Prognosis

Delayed diagnosis of cervical spine injury has grave consequences for the victim. About one in 20 cervical fractures are missed and about two-thirds of these patients have further spinal-cord damage as a result. About 30% of cases of delayed diagnosis of cervical spine injury develop permanent neurological deficits. In high-level cervical injuries, total paralysis from the neck can result. High-level tetraplegics (C4 and higher) will likely need constant care and assistance in activities of daily living (ADLs), such as getting dressed, eating, and bowel/bladder care. Individuals with C5 injuries retain some function in their biceps, deltoids, and other muscles; they typically can perform many ADLs including feeding, bathing, and grooming but require total assistance with bowel/bladder care. The C6 level adds function in the extensor carpi radialis, longus, and other muscles allowing for wrist extension, scapular abduction, and wrist flexion; typically, these patients have modified independent feeding and grooming with adaptive equipment, independent with dressing, can use both a manual and power wheelchair but require assistance with some activities of daily living. The C7 level is where function is retained in the triceps allowing for arm extension; C7 is considered the key level at which most activities can be performed independently with a wheelchair and assistive devices; activities include feeding, grooming, dressing, light meal preparation, and transfers on level surfaces. Even in complete spinal cord injury, it is common for individuals to recover up to 1 level of motor function. Even with "complete" injuries, in some rare cases, through intensive rehabilitation, function can be regained through "rewiring" neural connections, as in the case of actor
Christopher Reeve Christopher D'Olier Reeve (September 25, 1952 – October 10, 2004) was an American actor, best known for playing the title character in the film ''Superman (1978 film), Superman'' (1978) and three sequels. Born in New York City and raised in P ...
. In the case of
cerebral palsy Cerebral palsy (CP) is a group of movement disorders that appear in early childhood. Signs and symptoms vary among people and over time, but include poor coordination, stiff muscles, weak muscles, and tremors. There may be problems with sen ...
, which is caused by damage to the motor cortex either before, during (10%), or after birth, some people with incomplete tetraplegia are gradually able to learn to stand or walk through physical therapy. Quadriplegics can improve muscle strength by performing resistance training at least three times per week. Combining resistance training with proper nutrition intake can greatly reduce co-morbidities such as obesity and type 2 diabetes.


Epidemiology

There are an estimated 17,700 spinal cord injuries each year in the United States; the total number of people affected by spinal cord injuries is estimated to be approximately 290,000 people. In the US, spinal cord injuries alone cost approximately US$40.5 billion each year, which is a 317 percent increase from costs estimated in 1998 ($9.7 billion). The estimated lifetime costs for a 25-year-old in 2018 is $3.6 million when affected by low tetraplegia and $4.9 million when affected by high tetraplegia. In 2009, it was estimated that the lifetime care of a 25-year-old rendered with low tetraplegia was about $1.7 million, and $3.1 million with high tetraplegia. There are about 1,000 people affected each year in the UK (~1 in 60,000—assuming a population of 60 million).


Terminology

The condition of paralysis affecting four limbs is alternately termed ''tetraplegia'' or ''quadriplegia''. Quadriplegia combines the
Latin Latin (, or , ) is a classical language belonging to the Italic languages, Italic branch of the Indo-European languages. Latin was originally a dialect spoken in the lower Tiber area (then known as Latium) around present-day Rome, but through ...
root ''quadra'', for "four", with the
Greek Greek may refer to: Greece Anything of, from, or related to Greece, a country in Southern Europe: *Greeks, an ethnic group. *Greek language, a branch of the Indo-European language family. **Proto-Greek language, the assumed last common ancestor ...
root πληγία ''plegia'', for "paralysis". Tetraplegia uses the Greek root τετρα ''tetra'' for "four". In the past, tetraplegia and quadriplegia were used interchangeably in the medical literature. Medical literature favors using tetraplegia as the standardized term as it is frowned upon to mix Greek and Latin roots, although quadriplegia remains in use. Tetraplegia, meaning the paralysis of four limbs, may be confused with tetraparesis, meaning the weakness of four limbs. In medicine, it is important to not use these terms when making a diagnosis. When diagnosing and classifying spinal cord injuries, the ASIA classification is used to make the distinction of weakness vs. no weakness as well as to classify neurologically complete vs. incomplete lesions . Use of tetraparesis is discouraged as it inaccurately describes an incomplete lesion and incorrectly implies tetraplegia applies only to cases of complete lesions.


See also

*
Clearing the cervical spine Clearing the cervical spine is the process by which medical professionals determine whether cervical spine injuries exist, mainly regarding cervical fracture. It is generally performed in cases of major trauma. This process can take place in the ...
* Hemiplegia *
Paraplegia Paraplegia, or paraparesis, is an impairment in motor or sensory function of the lower extremities. The word comes from Ionic Greek () "half-stricken". It is usually caused by spinal cord injury or a congenital condition that affects the neural ...
* Locked-in syndrome * Sexuality after spinal cord injury *
Spinal cord injury research Spinal cord injury research seeks new ways to cure or treat spinal cord injury in order to lessen the debilitating effects of the injury in the short or long term. There is no cure for SCI, and current treatments are mostly focused on spinal cord ...


References


Further reading

* *


External links

* {{Authority control Cerebral palsy and other paralytic syndromes Neurotrauma