Spastic diplegia
Encyclopedia
Spastic diplegia, historically known as Little's Disease, is a form of cerebral palsy
(CP) that is a neuromuscular condition of hypertonia
and spasticity
in the muscles of the lower extremities of the human body, usually those of the legs, hips
and pelvis
. Doctor William John Little's first recorded encounter with cerebral palsy is reported to have been among children who displayed signs of spastic diplegia.
This condition is by far the most common type of CP, occurring in almost 70% of all cases.
inhibits the proper development of upper motor neuron
function, impacting the motor cortex
, the basal ganglia
and the corticospinal tract
. Nerve receptors in the spine
leading to affected muscles become unable to properly absorb gamma amino butyric acid (GABA), the amino acid that regulates muscle tone
in humans. Without GABA absorption to those particular nerve rootlets (usually centred, in this case, around the sectors L1-S1 and L2-S2), affected nerves (here, the ones controlling the legs) perpetually fire the message for their corresponding muscles to permanently, rigidly contract, and the muscles become permanently hypertonic
.
The abnormally high muscle tone that results creates lifelong difficulty with all voluntary and passive movement in the legs, and in general creates stress over time — depending on the severity of the condition in the individual, the constant spasticity ultimately produces pain, muscle/joint breakdown including tendinitis and arthritis
, premature physical exhaustion (i.e., becoming physically exhausted even when you internally know that you have more energy than you are able to use), contracture
s, spasm
s, and progressively worse deformities/mis-alignments of bone structure around areas of the tightened musculature as the person's years progress. Severe arthritis, tendinitis, and similar breakdown can start as early as the spastic diplegic person's mid-20s (as a comprison, typical people with normal muscle tone are not at risk of arthritis, tendinitis, and similar breakdown until well into their 50s or 60s, if even then).
No type of CP is officially a progressive condition
, and indeed spastic diplegia does not clinically "get worse" given the nerves, damaged permanently at birth, neither recover nor degrade. This aspect is clinically significant because other neuromuscular conditions with similar surface characteristics in their presentations, like most forms of multiple sclerosis
, indeed do degrade the body over time and do involve actual progressive worsening of the condition, including the spasticity
often seen in MS. But, in the case of spastic CP and spastic diplegia, the lack of progression of the condition itself is ultimately irrelevant, because the symptoms themselves cause compounded effects on the body that are typically just as stressful on the human body as a progressive condition is. But, even though muscle tightness is the symptom of spastic diplegia and not the cause, symptoms are typically seen as the primary area of focus for treatment, especially surgical treatment, except when a rhizotomy
is brought into consideration.
Unlike any other condition that may present with similar effects, spastic diplegia is entirely congenital in origin — that is, it is almost always acquired shortly before or during a baby's birth process. Things like exposure to toxins, traumatic brain injury
, encephalitis
, meningitis
, drowning, or suffocation do not tend to lead to spastic diplegia in particular or even cerebral palsy generally. Overall, the most common cause of spastic diplegia is Periventricular leukomalacia
, more commonly known as neonatal asphyxia or infant hypoxia — a sudden in-womb shortage of oxygen-delivery through the umbilical cord
. This sudden lack of oxygen is also almost always combined with premature birth
, a phenomenon that, even by itself, would inherently risk the infant developing some type of CP. On the other hand, the presence of certain maternal infections during pregnancy such as congenital rubella syndrome
can also lead to spastic diplegia, since such infections can have similar end results to infant hypoxia.
so that their clinicians can determine the best assistive devices for them, if any are necessary, such as a walker or crutch
es. The main difference between spastic diplegia and a normal gait pattern
is its signature "Scissor gait
" — a style that some able-bodied people might tend to confuse with the effects of drunkenness
, multiple sclerosis
, or another nerve disease
. The degree of spasticity in spastic diplegia (and, for that matter, other types of spastic CP) varies widely from person to person. No two people with spastic diplegia are exactly alike. Balance problems and/or stiffness in gait can range from barely noticeable all the way to misalignments so pronounced that the person needs crutches (typically forearm crutches/lofstrand crutches) or a cane / walking stick to assist in ambulation. Less often, spasticity is severe enough to compel the person to use a wheelchair
.
In general, however, lower-extremity spasticity in spastic diplegia is rarely so great as to totally prevent ambulation — most people with the condition can walk, and can do so with at least a basic amount of overall stability. However, from case to case, steeply varying degrees of imbalance, potential tripping over uneven terrain while walking, or needing to hold on to various surfaces or walls in certain circumstances to keep upright, are typically ever-present potential issues and are much more common occurrences amongst those with spastic diplegia than among those with a normal or near-normal gait pattern. Among some of the people with spastic diplegia who choose to be ambulatory on either an exclusive or predominant basis, one of the seemingly common lifestyle choices is for the person to ambulate within his or her home without an assistive device, and then to use the assistive device, if any, once outdoors. Others may use no assistive device in any indoor situation at all, while always using one when outdoors.
Above the hips, persons with spastic diplegia typically retain normal or near-normal muscle tone
and range of motion
, though some lesser spasticity may also affect the upper body, such as the trunk and arms, depending on the severity of the condition in the individual (the spasticity condition affecting the whole body equally, rather than just the legs, is spastic quadriplegia
, a slightly different classification). In addition, because leg tightness often leads to instability in ambulation, extra muscle tension usually develops in the shoulders, chest, and arms due to compensatory stabilization movements, regardless of the fact that the upper body itself is not directly affected by the condition.
, contact improvisation
, modern dance
, resistance training
, and other physical activity regimens are often utilized by those with spastic CP to help prevent contracture
s and reduce the severity of symptoms.
Major clinical treatments for spastic diplegia are:
in spastic cerebral palsy and was originally an acceptable and common term to use in both self-description and in description by others, it has since gained more notoriety as a pejorative
, in particular when used in pop culture to insult able-bodied people when they seem overly anxious or unskilled in sports (see also the article spazz).
In 1952, a UK
charitable organization
with a membership mainly of those with spastic CP was formed; this organization called itself The Spastics Society. However, the charity changed its name to Scope
in 1994 due to the term spastic's having become enough of a pejorative to warrant the name change.
Spastic diplegia's social implications tend to vary with the intensity of the condition in the individual. If its effects are severely disabling, resulting in very little physical activity for the person, social elements can also suffer. Workplace environments can also be limited, since most labor-intensive work requires basic physical agility that spastic diplegics may not possess. However, the degree of variability among individuals with spastic diplegia means that no greater or lesser degree of stigma or real-world limitation is standard. Lesser effects usually mean fewer physical limitations, better-quality exercise, and more real-world flexibility, but the person is still in general seen as different than the norm. How such a person chooses to react to outside opinion is of paramount importance when social factors are considered.
overall — some may even find themselves in situations of institutionalisation, and thus barely see the outside world at all.
From what is known, the incidence of spastic diplegia is higher in males than in females; the Surveillance of Cerebral Palsy in Europe (SCPE), for example, reports a M:F ratio of 1.33:1. Variances in reported rates of incidence across different geographical areas in industrialised countries are thought to be caused primarily by discrepancies in the criteria used for inclusion and exclusion.
When such discrepancies are taken into account in comparing two or more registers of patients with cerebral palsy and also the extent to which children with mild cerebral palsy are included, the incidence rates still converge toward the average rate of 2:1000.
In the United States, approximately 10,000 infants and babies are born with CP each year, and 1200–1500 are diagnosed at preschool age when symptoms become more obvious. It is interesting to note that those with extremely mild spastic CP may not even be aware of their condition until much later in life: Internet chat forums have recorded men and women as old as 30 who were diagnosed only recently with their spastic CP.
Overall, advances in care of pregnant mothers and their babies has not resulted in a noticeable decrease in CP; in fact, because medical advances in areas related to the care of premature babies has resulted in a greater survival rate in recent years, it is actually more likely for infants with cerebral palsy to be born into the world now than it would have been in the past. Only the introduction of quality medical care to locations with less-than-adequate medical care has shown any decreases in the incidences of CP; the rest either have shown no change or have actually shown an increase. The incidence of CP increases with premature or very low-weight babies regardless of the quality of care.
d and older adult
s. An especially puzzling aspect of this lies in the fact that cerebral palsy as defined by modern science was first 'discovered' and specifically addressed well over 100 years ago and that it would therefore be reasonable to expect by now that at least some empirical data on the adult populations with these conditions would have long since been collected, especially over the second half of the 20th century when existing treatment technologies rapidly improved and new ones came into being. The vast majority of empirical data on the various forms of cerebral palsy is concerned near-exclusively with children (birth to about 10 years of age) and sometimes pre-teens and early teens (11-13). Some doctors attempt to provide their own personal justifications for keeping their CP specialities purely paediatric, but there is no objectively apparent set of reasons backed by any scientific consensus
as to why medical science has made a point of researching adult cases of multiple sclerosis
, muscular dystrophy
and the various forms of cancer
in young and older adults, but has failed to do so with CP. There are a few orthopaedic surgeons and neurosurgeons who claim to be gathering pace with various studies as of the past few years , but these claims do not yet seem to have been matched by real-world actualisation in terms of easily-accessible and objectively-verifiable resources available to the general public on the internet and in-person, where many, including medical-science researchers and doctors themselves, would more than likely agree such resources would ideally belong.
Cerebral palsy
Cerebral palsy is an umbrella term encompassing a group of non-progressive, non-contagious motor conditions that cause physical disability in human development, chiefly in the various areas of body movement....
(CP) that is a neuromuscular condition of hypertonia
Hypertonia
Hypertonia a condition marked by an abnormal increase in muscle tension and a reduced ability of a muscle to stretch. It is caused by lesions to upper motor neurons in the central nervous system, which carry information from the central nervous system to the muscles and control posture, muscle...
and spasticity
Spasticity
Spasticity is a feature of altered skeletal muscle performance in muscle tone involving hypertonia, which is also referred to as an unusual "tightness" of muscles...
in the muscles of the lower extremities of the human body, usually those of the legs, hips
Hip (anatomy)
In vertebrate anatomy, hip refer to either an anatomical region or a joint.The hip region is located lateral to the gluteal region , inferior to the iliac crest, and overlying the greater trochanter of the femur, or "thigh bone"...
and pelvis
Pelvis
In human anatomy, the pelvis is the lower part of the trunk, between the abdomen and the lower limbs .The pelvis includes several structures:...
. Doctor William John Little's first recorded encounter with cerebral palsy is reported to have been among children who displayed signs of spastic diplegia.
This condition is by far the most common type of CP, occurring in almost 70% of all cases.
Background
Spastic diplegia's particular type of brain damageBrain damage
"Brain damage" or "brain injury" is the destruction or degeneration of brain cells. Brain injuries occur due to a wide range of internal and external factors...
inhibits the proper development of upper motor neuron
Upper motor neuron
Upper motor neurons are motor neurons that originate in the motor region of the cerebral cortex or the brain stem and carry motor information down to the final common pathway, that is, any motor neurons that are not directly responsible for stimulating the target muscle...
function, impacting the motor cortex
Motor cortex
Motor cortex is a term that describes regions of the cerebral cortex involved in the planning, control, and execution of voluntary motor functions.-Anatomy of the motor cortex :The motor cortex can be divided into four main parts:...
, the basal ganglia
Basal ganglia
The basal ganglia are a group of nuclei of varied origin in the brains of vertebrates that act as a cohesive functional unit. They are situated at the base of the forebrain and are strongly connected with the cerebral cortex, thalamus and other brain areas...
and the corticospinal tract
Corticospinal tract
The corticospinal or pyramidal tract is a collection of axons that travel between the cerebral cortex of the brain and the spinal cord....
. Nerve receptors in the spine
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...
leading to affected muscles become unable to properly absorb gamma amino butyric acid (GABA), the amino acid that regulates muscle tone
Muscle tone
In physiology, medicine, and anatomy, muscle tone is the continuous and passive partial contraction of the muscles, or the muscle’s resistance to passive stretch during resting state. It helps maintain posture, and it declines during REM sleep.-Purpose:Unconscious nerve impulses maintain the...
in humans. Without GABA absorption to those particular nerve rootlets (usually centred, in this case, around the sectors L1-S1 and L2-S2), affected nerves (here, the ones controlling the legs) perpetually fire the message for their corresponding muscles to permanently, rigidly contract, and the muscles become permanently hypertonic
Hypertonia
Hypertonia a condition marked by an abnormal increase in muscle tension and a reduced ability of a muscle to stretch. It is caused by lesions to upper motor neurons in the central nervous system, which carry information from the central nervous system to the muscles and control posture, muscle...
.
The abnormally high muscle tone that results creates lifelong difficulty with all voluntary and passive movement in the legs, and in general creates stress over time — depending on the severity of the condition in the individual, the constant spasticity ultimately produces pain, muscle/joint breakdown including tendinitis and arthritis
Arthritis
Arthritis is a form of joint disorder that involves inflammation of one or more joints....
, premature physical exhaustion (i.e., becoming physically exhausted even when you internally know that you have more energy than you are able to use), contracture
Contracture
A muscle contracture is a permanent shortening of a muscle or joint.. It is usually in response to prolonged hypertonic spasticity in a concentrated muscle area, such as is seen in the tightest muscles of people with conditions like spastic cerebral palsy....
s, spasm
Spasm
In medicine a spasm is a sudden, involuntary contraction of a muscle, a group of muscles, or a hollow organ, or a similarly sudden contraction of an orifice. It is sometimes accompanied by a sudden burst of pain, but is usually harmless and ceases after a few minutes...
s, and progressively worse deformities/mis-alignments of bone structure around areas of the tightened musculature as the person's years progress. Severe arthritis, tendinitis, and similar breakdown can start as early as the spastic diplegic person's mid-20s (as a comprison, typical people with normal muscle tone are not at risk of arthritis, tendinitis, and similar breakdown until well into their 50s or 60s, if even then).
No type of CP is officially a progressive condition
Progressive illness
The term progressive illness describes the course of an illness that gradually progresses and changes mode, generally to the worse. In contrast, non-progressive or chronic illnesses are relatively constant.-Examples:...
, and indeed spastic diplegia does not clinically "get worse" given the nerves, damaged permanently at birth, neither recover nor degrade. This aspect is clinically significant because other neuromuscular conditions with similar surface characteristics in their presentations, like most forms of multiple sclerosis
Multiple sclerosis
Multiple sclerosis is an inflammatory disease in which the fatty myelin sheaths around the axons of the brain and spinal cord are damaged, leading to demyelination and scarring as well as a broad spectrum of signs and symptoms...
, indeed do degrade the body over time and do involve actual progressive worsening of the condition, including the spasticity
Spasticity
Spasticity is a feature of altered skeletal muscle performance in muscle tone involving hypertonia, which is also referred to as an unusual "tightness" of muscles...
often seen in MS. But, in the case of spastic CP and spastic diplegia, the lack of progression of the condition itself is ultimately irrelevant, because the symptoms themselves cause compounded effects on the body that are typically just as stressful on the human body as a progressive condition is. But, even though muscle tightness is the symptom of spastic diplegia and not the cause, symptoms are typically seen as the primary area of focus for treatment, especially surgical treatment, except when a rhizotomy
Rhizotomy
A rhizotomy is a term chiefly referring to a neurosurgical procedure that selectively severs problematic nerve roots in the spinal cord, most often to relieve the symptoms of neuromuscular conditions such as spastic diplegia and other forms of spastic cerebral palsy...
is brought into consideration.
Unlike any other condition that may present with similar effects, spastic diplegia is entirely congenital in origin — that is, it is almost always acquired shortly before or during a baby's birth process. Things like exposure to toxins, traumatic brain injury
Traumatic brain injury
Traumatic brain injury , also known as intracranial injury, occurs when an external force traumatically injures the brain. TBI can be classified based on severity, mechanism , or other features...
, encephalitis
Encephalitis
Encephalitis is an acute inflammation of the brain. Encephalitis with meningitis is known as meningoencephalitis. Symptoms include headache, fever, confusion, drowsiness, and fatigue...
, meningitis
Meningitis
Meningitis is inflammation of the protective membranes covering the brain and spinal cord, known collectively as the meninges. The inflammation may be caused by infection with viruses, bacteria, or other microorganisms, and less commonly by certain drugs...
, drowning, or suffocation do not tend to lead to spastic diplegia in particular or even cerebral palsy generally. Overall, the most common cause of spastic diplegia is Periventricular leukomalacia
Periventricular leukomalacia
Periventricular leukomalacia is a form of white-matter brain injury, characterized by the necrosis of white matter near the lateral ventricles. It can affect newborns and fetuses; premature infants are at the greatest risk of the disorder...
, more commonly known as neonatal asphyxia or infant hypoxia — a sudden in-womb shortage of oxygen-delivery through the umbilical cord
Umbilical cord
In placental mammals, the umbilical cord is the connecting cord from the developing embryo or fetus to the placenta...
. This sudden lack of oxygen is also almost always combined with premature birth
Premature birth
In humans preterm birth refers to the birth of a baby of less than 37 weeks gestational age. The cause for preterm birth is in many situations elusive and unknown; many factors appear to be associated with the development of preterm birth, making the reduction of preterm birth a challenging...
, a phenomenon that, even by itself, would inherently risk the infant developing some type of CP. On the other hand, the presence of certain maternal infections during pregnancy such as congenital rubella syndrome
Congenital rubella syndrome
Congenital rubella syndrome can occur in a developing fetus of a pregnant woman who has contracted rubella during her first trimester. If infection occurs 0–28 days before conception, there is a 43% chance the infant will be affected. If the infection occurs 0–12 weeks after conception, there is a...
can also lead to spastic diplegia, since such infections can have similar end results to infant hypoxia.
Presentation
Individuals with spastic diplegia are very tight and stiff and must work very hard to successfully resist and "push through" the extra tightness they perpetually experience. Other than this, however, these individuals are almost always normal in every significant clinical sense. When they are younger, spastic diplegic individuals typically undergo gait analysisGait analysis
Gait analysis is the systematic study of animal locomotion, more specific as a study of human motion, using the eye and the brain of observers, augmented by instrumentation for measuring body movements, body mechanics, and the activity of the muscles. Gait analysis is used to assess, plan, and...
so that their clinicians can determine the best assistive devices for them, if any are necessary, such as a walker or crutch
Crutch
Crutches are mobility aids used to counter a mobility impairment or an injury that limits walking ability.- Types :There are several different types of crutches:...
es. The main difference between spastic diplegia and a normal gait pattern
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...
is its signature "Scissor gait
Scissor gait
Scissor gait is a form of gait abnormality primarily associated with spastic cerebral palsy. It is associated with an upper motor neuron lesion.-Presentation:This gait pattern is reminiscent of a marionette...
" — a style that some able-bodied people might tend to confuse with the effects of drunkenness
Drunkenness
Alcohol intoxication is a physiological state that occurs when a person has a high level of ethanol in his or her blood....
, multiple sclerosis
Multiple sclerosis
Multiple sclerosis is an inflammatory disease in which the fatty myelin sheaths around the axons of the brain and spinal cord are damaged, leading to demyelination and scarring as well as a broad spectrum of signs and symptoms...
, or another nerve disease
Neurological disorder
A neurological disorder is a disorder of the body's nervous system. Structural, biochemical or electrical abnormalities in the brain, spinal cord, or in the nerves leading to or from them, can result in symptoms such as paralysis, muscle weakness, poor coordination, loss of sensation, seizures,...
. The degree of spasticity in spastic diplegia (and, for that matter, other types of spastic CP) varies widely from person to person. No two people with spastic diplegia are exactly alike. Balance problems and/or stiffness in gait can range from barely noticeable all the way to misalignments so pronounced that the person needs crutches (typically forearm crutches/lofstrand crutches) or a cane / walking stick to assist in ambulation. Less often, spasticity is severe enough to compel the person to use a wheelchair
Wheelchair
A wheelchair is a chair with wheels, designed to be a replacement for walking. The device comes in variations where it is propelled by motors or by the seated occupant turning the rear wheels by hand. Often there are handles behind the seat for someone else to do the pushing...
.
In general, however, lower-extremity spasticity in spastic diplegia is rarely so great as to totally prevent ambulation — most people with the condition can walk, and can do so with at least a basic amount of overall stability. However, from case to case, steeply varying degrees of imbalance, potential tripping over uneven terrain while walking, or needing to hold on to various surfaces or walls in certain circumstances to keep upright, are typically ever-present potential issues and are much more common occurrences amongst those with spastic diplegia than among those with a normal or near-normal gait pattern. Among some of the people with spastic diplegia who choose to be ambulatory on either an exclusive or predominant basis, one of the seemingly common lifestyle choices is for the person to ambulate within his or her home without an assistive device, and then to use the assistive device, if any, once outdoors. Others may use no assistive device in any indoor situation at all, while always using one when outdoors.
Above the hips, persons with spastic diplegia typically retain normal or near-normal muscle tone
Muscle tone
In physiology, medicine, and anatomy, muscle tone is the continuous and passive partial contraction of the muscles, or the muscle’s resistance to passive stretch during resting state. It helps maintain posture, and it declines during REM sleep.-Purpose:Unconscious nerve impulses maintain the...
and range of motion
Range of motion
Range of motion , is the distance that a movable object may normally travel while properly attached to another object. It is also called range of travel, particularly when talking about mechanical devices and in mechanical engineering fields...
, though some lesser spasticity may also affect the upper body, such as the trunk and arms, depending on the severity of the condition in the individual (the spasticity condition affecting the whole body equally, rather than just the legs, is spastic quadriplegia
Spastic quadriplegia
Spastic quadriplegia, sometimes called spastic tetraplegia, is the version of spastic diplegia that affects all four limbs rather than just the legs. It is defined by spasticity, rather than the paralysis that defines quadriplegia...
, a slightly different classification). In addition, because leg tightness often leads to instability in ambulation, extra muscle tension usually develops in the shoulders, chest, and arms due to compensatory stabilization movements, regardless of the fact that the upper body itself is not directly affected by the condition.
Treatment
As a matter of everyday maintenance, muscle stretching, range of motion exercises, yogaYoga
Yoga is a physical, mental, and spiritual discipline, originating in ancient India. The goal of yoga, or of the person practicing yoga, is the attainment of a state of perfect spiritual insight and tranquility while meditating on Supersoul...
, contact improvisation
Contact improvisation
Contact improvisation is a dance technique in which points of physical contact provide the starting point for exploration through movement improvisation...
, modern dance
Modern dance
Modern dance is a dance form developed in the early 20th century. Although the term Modern dance has also been applied to a category of 20th Century ballroom dances, Modern dance as a term usually refers to 20th century concert dance.-Intro:...
, resistance training
Resistance training
Resistance training has two different meanings. A broader meaning that refers to any training that uses a resistance to the force of muscular contraction , and elastic or hydraulic resistance, which refers to a specific type of strength training that uses elastic or hydraulic tension to provide...
, and other physical activity regimens are often utilized by those with spastic CP to help prevent contracture
Contracture
A muscle contracture is a permanent shortening of a muscle or joint.. It is usually in response to prolonged hypertonic spasticity in a concentrated muscle area, such as is seen in the tightest muscles of people with conditions like spastic cerebral palsy....
s and reduce the severity of symptoms.
Major clinical treatments for spastic diplegia are:
- BaclofenBaclofenBaclofen is a derivative of gamma-aminobutyric acid . It is primarily used to treat spasticity and is under investigation for the treatment of alcoholism....
(and its derivatives), a gamma amino butyric acid substitute in oral (pill-based) or intrathecal form. Baclofen is essentially chemically identical to the GABA that the damaged, over-firing nerves cannot absorb, except that it has an extra chemical 'marker' on it that makes the damaged nerves 'think' it is a different compound, and thus those nerves will absorb it. Baclofen is noted for being the sole medication available for GABA-deficiency-based spasticity which acts on the actual cause of the spasticity rather than simply reducing symptomatology as muscle relaxantMuscle relaxantA muscle relaxant is a drug which affects skeletal muscle function and decreases the muscle tone. It may be used to alleviate symptoms such as muscle spasms, pain, and hyperreflexia. The term "muscle relaxant" is used to refer to two major therapeutic groups: neuromuscular blockers and spasmolytics...
s and painkillers do. The intrathecal solution is a liquid injected into the spinal fluid for trial, and if successful in reducing spasticity, thereafter administered via an intrathecal pumpIntrathecal pumpAn intrathecal pump is a medical device used to deliver very small quantities of medications directly to the spinal fluid of a human being. Medications such as baclofen, morphine, or ziconotide may be delivered in this manner to minimize the side effects often associated with the higher dosages...
, which has variously been proven potentially very dangerous on one or another level with long-term use (see article), including sudden and potentially lethal baclofen overdose, whereas the oral route, which comes in 10- or 20-milligram tablets and the dosage of which can be gently titrated either upward or downward, as well as safely ceased entirely, has not. - antispasmodicAntispasmodicAn antispasmodic is a drug or a herb that suppresses muscle spasms.-Smooth muscle spasm:One type of antispasmodics is used for smooth muscle contraction, especially in tubular organs of the gastrointestinal tract...
muscle relaxantMuscle relaxantA muscle relaxant is a drug which affects skeletal muscle function and decreases the muscle tone. It may be used to alleviate symptoms such as muscle spasms, pain, and hyperreflexia. The term "muscle relaxant" is used to refer to two major therapeutic groups: neuromuscular blockers and spasmolytics...
chemicals such as TizanidineTizanidineTizanidine is a drug that is used as a muscle relaxant. It is a centrally acting α2 adrenergic agonist. It is used to treat the spasms, cramping, and tightness of muscles caused by medical problems such as multiple sclerosis, spastic diplegia, back pain, or certain other injuries to the spine or...
and Botulinum toxinBotulinum toxinBotulinum toxin is a protein produced by the bacterium Clostridium botulinum, and is considered the most powerful neurotoxin ever discovered. Botulinum toxin causes Botulism poisoning, a serious and life-threatening illness in humans and animals...
, injected directly into the spastic muscles; Botox wears off every three months. - PhenolPhenolPhenol, also known as carbolic acid, phenic acid, is an organic compound with the chemical formula C6H5OH. It is a white crystalline solid. The molecule consists of a phenyl , bonded to a hydroxyl group. It is produced on a large scale as a precursor to many materials and useful compounds...
and similar chemical 'nerve deadeners', injected selectively into the over-firing nerves in the legs on the muscle end to reduce spasticity in their corresponding muscles by preventing the spasticity signals from reaching the legs; Phenol wears off every six months. - orthopedic surgeryOrthopedic surgeryOrthopedic surgery or orthopedics is the branch of surgery concerned with conditions involving the musculoskeletal system...
to release the spastic muscles from their hypertonic state, a usually temporary result because of the source of the spasticity's being in the nerves, not the muscles; spasticity can fully reassert itself as little as one year post-surgery. - Selective Dorsal RhizotomyRhizotomyA rhizotomy is a term chiefly referring to a neurosurgical procedure that selectively severs problematic nerve roots in the spinal cord, most often to relieve the symptoms of neuromuscular conditions such as spastic diplegia and other forms of spastic cerebral palsy...
, a neurosurgeryNeurosurgeryNeurosurgery is the medical specialty concerned with the prevention, diagnosis, treatment, and rehabilitation of disorders which affect any portion of the nervous system including the brain, spine, spinal cord, peripheral nerves, and extra-cranial cerebrovascular system.-In the United States:In...
directly targeting and eliminating ("cutting" or "lesioning") the over-firing nerve rootNerve rootA nerve root is the initial segment of a nerve leaving the central nervous system. Types include:* A cranial nerve root, the beginning of one of the twelve pairs leaving the central nervous system from the brain stem or the highest levels of the spinal cord;...
lets and leaving the properly-firing ones intact, thereby permanently eliminating the spasticity but compelling the person to spend months re-strengthening muscles that will have been severely weakened by the loss of the spasticity, due to the fact of those muscles not really having had actual strength to begin with.
Social implications
Although the term "spastic" technically describes the attribute of spasticitySpasticity
Spasticity is a feature of altered skeletal muscle performance in muscle tone involving hypertonia, which is also referred to as an unusual "tightness" of muscles...
in spastic cerebral palsy and was originally an acceptable and common term to use in both self-description and in description by others, it has since gained more notoriety as a pejorative
Pejorative
Pejoratives , including name slurs, are words or grammatical forms that connote negativity and express contempt or distaste. A term can be regarded as pejorative in some social groups but not in others, e.g., hacker is a term used for computer criminals as well as quick and clever computer experts...
, in particular when used in pop culture to insult able-bodied people when they seem overly anxious or unskilled in sports (see also the article spazz).
In 1952, a UK
United Kingdom
The United Kingdom of Great Britain and Northern IrelandIn the United Kingdom and Dependencies, other languages have been officially recognised as legitimate autochthonous languages under the European Charter for Regional or Minority Languages...
charitable organization
Charitable organization
A charitable organization is a type of non-profit organization . It differs from other types of NPOs in that it centers on philanthropic goals A charitable organization is a type of non-profit organization (NPO). It differs from other types of NPOs in that it centers on philanthropic goals A...
with a membership mainly of those with spastic CP was formed; this organization called itself The Spastics Society. However, the charity changed its name to Scope
Scope (British charity)
Scope is a London-based charity, which operates in England and Wales, focusing on people with cerebral palsy particularly, and disabled people in general. Its aim is that disabled people achieve equality....
in 1994 due to the term spastic's having become enough of a pejorative to warrant the name change.
Spastic diplegia's social implications tend to vary with the intensity of the condition in the individual. If its effects are severely disabling, resulting in very little physical activity for the person, social elements can also suffer. Workplace environments can also be limited, since most labor-intensive work requires basic physical agility that spastic diplegics may not possess. However, the degree of variability among individuals with spastic diplegia means that no greater or lesser degree of stigma or real-world limitation is standard. Lesser effects usually mean fewer physical limitations, better-quality exercise, and more real-world flexibility, but the person is still in general seen as different than the norm. How such a person chooses to react to outside opinion is of paramount importance when social factors are considered.
Prevalence
In the industrialized world, the incidence of overall cerebral palsy, which includes but is not limited to spastic diplegia, is about 2 per 1000 live births. Thus far, there is no known study recording the incidence of CP in the overall nonindustrialized world. Therefore, it is safe to assume that not all spastic CP individuals are known to science and medicine, especially in areas of the world where healthcare systems are less advanced than the standard. Many such individuals may simply live out their lives in their local communities without any medical or orthopedic oversight at all, or with extremely minimal such treatment, so that they are never able to be incorporated into any empirical data that orthopedic surgeons or neurosurgeons might seek to collect. It is shocking to note that — as with people with physical disabilityPhysical disability
A physical disability is any impairment which limits the physical function of one or more limbs or fine or gross motor ability. Other physical disabilities include impairments which limit other facets of daily living, such as respiratory disorders and epilepsy....
overall — some may even find themselves in situations of institutionalisation, and thus barely see the outside world at all.
From what is known, the incidence of spastic diplegia is higher in males than in females; the Surveillance of Cerebral Palsy in Europe (SCPE), for example, reports a M:F ratio of 1.33:1. Variances in reported rates of incidence across different geographical areas in industrialised countries are thought to be caused primarily by discrepancies in the criteria used for inclusion and exclusion.
When such discrepancies are taken into account in comparing two or more registers of patients with cerebral palsy and also the extent to which children with mild cerebral palsy are included, the incidence rates still converge toward the average rate of 2:1000.
In the United States, approximately 10,000 infants and babies are born with CP each year, and 1200–1500 are diagnosed at preschool age when symptoms become more obvious. It is interesting to note that those with extremely mild spastic CP may not even be aware of their condition until much later in life: Internet chat forums have recorded men and women as old as 30 who were diagnosed only recently with their spastic CP.
Overall, advances in care of pregnant mothers and their babies has not resulted in a noticeable decrease in CP; in fact, because medical advances in areas related to the care of premature babies has resulted in a greater survival rate in recent years, it is actually more likely for infants with cerebral palsy to be born into the world now than it would have been in the past. Only the introduction of quality medical care to locations with less-than-adequate medical care has shown any decreases in the incidences of CP; the rest either have shown no change or have actually shown an increase. The incidence of CP increases with premature or very low-weight babies regardless of the quality of care.
Difficulty in tracking CP progression through the later years
Unusually, cerebral palsy, including spastic cerebral palsy, is notable for a glaring overall research deficiency — the fact that it is one of the very few major groups of conditions on the planet in human beings for which medical science has not yet (as of 2011) collected wide-ranging empirical data on the development and experiences of young adults, the middle ageMiddle age
Middle age is the period of age beyond young adulthood but before the onset of old age. Various attempts have been made to define this age, which is around the third quarter of the average life span of human beings....
d and older adult
Senior citizen
Senior citizen is a common polite designation for an elderly person in both UK and US English, and it implies or means that the person is retired. This in turn implies or in fact means that the person is over the retirement age, which varies according to country. Synonyms include pensioner in UK...
s. An especially puzzling aspect of this lies in the fact that cerebral palsy as defined by modern science was first 'discovered' and specifically addressed well over 100 years ago and that it would therefore be reasonable to expect by now that at least some empirical data on the adult populations with these conditions would have long since been collected, especially over the second half of the 20th century when existing treatment technologies rapidly improved and new ones came into being. The vast majority of empirical data on the various forms of cerebral palsy is concerned near-exclusively with children (birth to about 10 years of age) and sometimes pre-teens and early teens (11-13). Some doctors attempt to provide their own personal justifications for keeping their CP specialities purely paediatric, but there is no objectively apparent set of reasons backed by any scientific consensus
Scientific consensus
Scientific consensus is the collective judgment, position, and opinion of the community of scientists in a particular field of study. Consensus implies general agreement, though not necessarily unanimity. Scientific consensus is not by itself a scientific argument, and it is not part of the...
as to why medical science has made a point of researching adult cases of multiple sclerosis
Multiple sclerosis
Multiple sclerosis is an inflammatory disease in which the fatty myelin sheaths around the axons of the brain and spinal cord are damaged, leading to demyelination and scarring as well as a broad spectrum of signs and symptoms...
, muscular dystrophy
Muscular dystrophy
Muscular dystrophy is a group of muscle diseases that weaken the musculoskeletal system and hamper locomotion. Muscular dystrophies are characterized by progressive skeletal muscle weakness, defects in muscle proteins, and the death of muscle cells and tissue.In the 1860s, descriptions of boys who...
and the various forms of cancer
Cancer
Cancer , known medically as a malignant neoplasm, is a large group of different diseases, all involving unregulated cell growth. In cancer, cells divide and grow uncontrollably, forming malignant tumors, and invade nearby parts of the body. The cancer may also spread to more distant parts of the...
in young and older adults, but has failed to do so with CP. There are a few orthopaedic surgeons and neurosurgeons who claim to be gathering pace with various studies as of the past few years , but these claims do not yet seem to have been matched by real-world actualisation in terms of easily-accessible and objectively-verifiable resources available to the general public on the internet and in-person, where many, including medical-science researchers and doctors themselves, would more than likely agree such resources would ideally belong.
See also
- Inclusion (disability rights)Inclusion (disability rights)Inclusion is a term used by people with disabilities and other disability rights advocates for the idea that all people should freely, openly and without pity accommodate any person with a disability without restrictions or limitations of any kind...
- Cerebral PalsyCerebral palsyCerebral palsy is an umbrella term encompassing a group of non-progressive, non-contagious motor conditions that cause physical disability in human development, chiefly in the various areas of body movement....
- SpasticitySpasticitySpasticity is a feature of altered skeletal muscle performance in muscle tone involving hypertonia, which is also referred to as an unusual "tightness" of muscles...
- Gamma amino butyric acid
- RhizotomyRhizotomyA rhizotomy is a term chiefly referring to a neurosurgical procedure that selectively severs problematic nerve roots in the spinal cord, most often to relieve the symptoms of neuromuscular conditions such as spastic diplegia and other forms of spastic cerebral palsy...
- TizanidineTizanidineTizanidine is a drug that is used as a muscle relaxant. It is a centrally acting α2 adrenergic agonist. It is used to treat the spasms, cramping, and tightness of muscles caused by medical problems such as multiple sclerosis, spastic diplegia, back pain, or certain other injuries to the spine or...
External links
- Movement Disorder Virtual University, run by the non-profit organization WE MOVE