Spastic cerebral palsy
Encyclopedia
Spastic
cerebral palsy
is the type of cerebral palsy
wherein spasticity
is either the dominant or exclusive impairment present.
It is by far the most common type of overall cerebral palsy, occurring in 90% of all cases according to the Society for Cerebral Palsy in Europe (SCPE); even if the 90% assertion is an exaggeration, however, more conservative scientific estimates still place the prevalence of spasticity-dominant cerebral palsy at anywhere from 70-80% of all cases, leaving cases dominated by ataxic cerebral palsy, dyskinetic cerebral palsy and athetoid cerebral palsy trailing at 20-30%.
People with the spastic type of CP are hypertonic
and have what is essentially a neuromuscular mobility impairment (rather than hypotonia
or paralysis
) stemming from an upper motor neuron lesion in the brain; the corticospinal tract
or the motor cortex
may be secondarily affected. The upper motor neuron lesion in the brain in turn impairs the ability of some nerve receptors in the spine
to properly receive gamma amino butyric acid, leading to hypertonia
in the muscles signaled by those damaged nerves. The limbs and body areas in which hypertonia manifests can be any or even all of them, depending which specific nerve groupings within the spine are rendered unable to receive GABA. Thus, spastic CP is often designated by body topography.
In any manifestation of spastic CP, clonus
of the affected limb(s) may intermittently result, as well as muscle spasms, each of which results from the pain and/or stress of the tightness experienced, indicating especially hard-working and/or exhausted musculature. The spasticity itself can and usually does also lead to very early onset of muscle-stress symptoms like arthritis
and tendinitis, especially in ambulatory individuals in their mid-20s and early-30s. As compared to other types of CP, however, and especially as compared to hypotonic CP or more general paralytic mobility disabilities, spastic CP is typically more easily manageable by the person affected, and medical treatment can be pursued on a multitude of orthopedic
and neurological
fronts throughout life.
Physical therapy
and occupational therapy
regimens of assisted stretching, strengthening, functional tasks, and/or targeted physical activity and exercise are usually the chief ways to keep spastic CP well-managed, although if the spasticity is too much for the person to handle, other remedies may be considered, such as various antispasmodic
medications, botox, baclofen
, or even a neurosurgery
known as a selective dorsal rhizotomy (which eliminates the spasticity by eliminating the nerves causing it).
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.
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...
cerebral palsy
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....
is the type of cerebral palsy
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....
wherein 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...
is either the dominant or exclusive impairment present.
It is by far the most common type of overall cerebral palsy, occurring in 90% of all cases according to the Society for Cerebral Palsy in Europe (SCPE); even if the 90% assertion is an exaggeration, however, more conservative scientific estimates still place the prevalence of spasticity-dominant cerebral palsy at anywhere from 70-80% of all cases, leaving cases dominated by ataxic cerebral palsy, dyskinetic cerebral palsy and athetoid cerebral palsy trailing at 20-30%.
People with the spastic type of CP are 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...
and have what is essentially a neuromuscular mobility impairment (rather than hypotonia
Hypotonia
Hypotonia is a state of low muscle tone , often involving reduced muscle strength. Hypotonia is not a specific medical disorder, but a potential manifestation of many different diseases and disorders that affect motor nerve control by the brain or muscle strength...
or paralysis
Paralysis
Paralysis is loss of muscle function for one or more muscles. Paralysis can be accompanied by a loss of feeling in the affected area if there is sensory damage as well as motor. A study conducted by the Christopher & Dana Reeve Foundation, suggests that about 1 in 50 people have been diagnosed...
) stemming from an upper motor neuron lesion in the brain; 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....
or 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:...
may be secondarily affected. The upper motor neuron lesion in the brain in turn impairs the ability of some nerve receptors in the spine
Vertebral column
In human anatomy, the vertebral column is a column usually consisting of 24 articulating vertebrae, and 9 fused vertebrae in the sacrum and the coccyx. It is situated in the dorsal aspect of the torso, separated by intervertebral discs...
to properly receive gamma amino butyric acid, leading to 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...
in the muscles signaled by those damaged nerves. The limbs and body areas in which hypertonia manifests can be any or even all of them, depending which specific nerve groupings within the spine are rendered unable to receive GABA. Thus, spastic CP is often designated by body topography.
- Spastic hemiplegia is one side being affected. Generally, injury to muscle-nerves controlled by the brain's left side will cause a right body deficit, and vice versa. Typically, people that have spastic hemiplegia are the most ambulatory of all the forms, although they generally have dynamic equinus (a limping instability) on the affected side and are primarily prescribed ankle-foot orthoses to prevent said equinus.
- Spastic diplegiaSpastic diplegiaSpastic diplegia, historically known as Little's Disease, is a form of cerebral palsy 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...
is the lower extremities affected, with little to no upper-body spasticity. The most common form of the spastic forms (70-80% of known cases), most people with spastic diplegia are fully ambulatory, but are "tight" and have a scissors gait. Flexed knees and hips to varying degrees, and moderate to severe adduction (stemming from tight adductor muscles and comparatively weak abductor muscles), are present. Gait analysisGait analysisGait 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...
is often done in early life on a semi-regular basis, and assistive devices are often provided like walkers, crutches or canes; any ankle-foot orthotics provided usually go on both legs rather than just one. In addition, these individuals are often nearsighted. The intelligence of a person with spastic diplegia is unaffected by the condition. Over time, the effects of the spasticity sometimes produce hip problems and dislocations (see the main articleSpastic diplegiaSpastic diplegia, historically known as Little's Disease, is a form of cerebral palsy 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...
and 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...
for more on spasticity effects). In three-quarters of spastic diplegics, also strabismusStrabismusStrabismus is a condition in which the eyes are not properly aligned with each other. It typically involves a lack of coordination between the extraocular muscles, which prevents bringing the gaze of each eye to the same point in space and preventing proper binocular vision, which may adversely...
(crossed eyes) can be present as well. - Spastic monoplegia is one single limb being affected.
- Spastic triplegia is three limbs being affected.
- Spastic quadriplegiaSpastic quadriplegiaSpastic 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...
is all four limbs more or less equally affected. People with spastic quadriplegia are the least likely to be able to walk, or if they can, to desire to walk, because their muscles are too tight and it is too much of an effort to do so. Some children with spastic quadriplegia also have hemiparetic tremors, an uncontrollable shaking that affects the limbs on one side of the body and impairs normal movement. (This is not the same as clonus; see below.)
In any manifestation of spastic CP, clonus
Clonus
Clonus is a series of involuntary muscular contractions and relaxations. Clonus is a sign of certain neurological conditions, and is particularly associated with upper motor neuron lesions such as in stroke, multiple sclerosis, spinal cord damage and hepatic encephalopathy...
of the affected limb(s) may intermittently result, as well as muscle spasms, each of which results from the pain and/or stress of the tightness experienced, indicating especially hard-working and/or exhausted musculature. The spasticity itself can and usually does also lead to very early onset of muscle-stress symptoms like arthritis
Arthritis
Arthritis is a form of joint disorder that involves inflammation of one or more joints....
and tendinitis, especially in ambulatory individuals in their mid-20s and early-30s. As compared to other types of CP, however, and especially as compared to hypotonic CP or more general paralytic mobility disabilities, spastic CP is typically more easily manageable by the person affected, and medical treatment can be pursued on a multitude of orthopedic
Orthopedic surgery
Orthopedic surgery or orthopedics is the branch of surgery concerned with conditions involving the musculoskeletal system...
and neurological
Neurosurgery
Neurosurgery 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...
fronts throughout life.
Physical therapy
Physical therapy
Physical therapy , often abbreviated PT, is a health care profession. Physical therapy is concerned with identifying and maximizing quality of life and movement potential within the spheres of promotion, prevention, diagnosis, treatment/intervention,and rehabilitation...
and occupational therapy
Occupational therapy
Occupational therapy is a discipline that aims to promote health by enabling people to perform meaningful and purposeful activities. Occupational therapists work with individuals who suffer from a mentally, physically, developmentally, and/or emotionally disabling condition by utilizing treatments...
regimens of assisted stretching, strengthening, functional tasks, and/or targeted physical activity and exercise are usually the chief ways to keep spastic CP well-managed, although if the spasticity is too much for the person to handle, other remedies may be considered, such as various antispasmodic
Antispasmodic
An 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...
medications, botox, baclofen
Baclofen
Baclofen is a derivative of gamma-aminobutyric acid . It is primarily used to treat spasticity and is under investigation for the treatment of alcoholism....
, or even a neurosurgery
Neurosurgery
Neurosurgery 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...
known as a selective dorsal rhizotomy (which eliminates the spasticity by eliminating the nerves causing it).
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.
Scientific classifications
The scientific classifications for these types include:| Type | OMIM | Gene | Locus |
|---|---|---|---|
| CPSQ1 | GAD1 GAD1 Glutamate decarboxylase 1 , also known as GAD1, is a human gene.This gene encodes one of several forms of glutamic acid decarboxylase, identified as a major autoantigen in insulin-dependent diabetes. The enzyme encoded is responsible for catalyzing the production of gamma-aminobutyric acid from... |
2q31 | |
| CPSQ2 | ANKRD15 ANKRD15 KN motif and ankyrin repeat domain-containing protein 1 is a protein that in humans is encoded by the KANK1 gene.-Further reading:... |
9p24.3 | |
| CPSQ3 | AP4M1 AP4M1 AP-4 complex subunit mu-1 is a protein that in humans is encoded by the AP4M1 gene.-Clinical relevance:It is currently hypothesized that AP4-complex-mediated trafficking plays a crucial role in brain development and functioning.-Further reading:... |
7q22.1 |