Diplegia, when used singularly, refers to paralysis affecting symmetrical parts of the body. This is different from hemiplegia which refers to spasticity restricted to one side of the body, paraplegia which refers to paralysis restricted to the legs and hip, and quadriplegia which requires the involvement of all four limbs but not necessarily symmetrical. [1] Diplegia is the most common cause of crippling in children, specifically in children with cerebral palsy. [2] Other causes may be due to injury of the spinal cord. There is no set course of progression for people with diplegia. Symptoms may get worse but the neurological part does not change. The primary parts of the brain that are affected by diplegia are the ventricles, fluid filled compartments in the brain, and the wiring from the center of the brain to the cerebral cortex. [3] There is also usually some degeneration of the cerebral neurons, [2] as well as problems in the upper motor neuron system. [1] The term diplegia can refer to any bodily area, such as the face, arms, or legs.
Facial diplegia refers to people with paralysis of both sides of their face. Bilateral occurs when the onset of the second side occurs within one month of the onset of the first side. [4] Facial diplegia occurs in 50% of patients with Guillain–Barré syndrome. Facioscapulohumeral muscular dystrophy (FSHD) is the second most common adult-onset muscular dystrophy with facial weakness being a distinct feature of FSHD in over 90% of cases. [5]
Facial paralysis is usually caused by traumatic, infectious, neurological, metabolic, toxic, vascular, and idiopathic conditions. [4] While over 50% of the cases of unilateral facial paralysis are caused by idiopathic conditions, less than 20% of bilateral cases are idiopathic. The most common infectious cause of facial diplegia is Lyme disease. [4]
The treatment for facial diplegia depends on the underlying cause. Some causes are usually treatable such as infectious, toxic, and vascular by treating the main problem first. After the underlying problem is cured, the facial paralysis usually will go away.[ citation needed ]
People with diplegia in their arms experience difficulties in reaching, pointing, grasping, releasing, manipulating objects and many other motor functions performed by the hands and arms. [6]
There are several ways of getting diplegia in the arms. It is very common for people with cerebral palsy to have diplegia of the arms. Although most people with cerebral palsy have diplegia in their legs, some people have diplegia in their arms. Other ways of getting paralysis of both arms is through a traumatic event or injury.[ citation needed ] Brachial amyotrophic diplegia, a regional variant of ALS, is a rare motor neuron disease characterized by diplegia in the arms. [7]
There are several different modes of treatment for people with paralysis in their upper limbs. For example, behavioral and environmental treatments may include physiotherapy, occupational therapy, motor learning, strength training, and neurodevelopment treatment. Another treatment may be through the use of splints and casts. Electrophysical agents may be used such as neuromuscular electrical stimulation (NMES). Sometimes pharmacological treatments are necessary such as Botulinum toxin type A. On more severe cases surgery of the upper limbs may be required. [6]
Diplegia of the legs consists of paralysis of both legs. There are 3 levels of severity. Mild diplegia means the person can usually walk but might walk a little differently, can usually play and run to a limited extent. Moderate diplegia means the person can usually walk but with a slight bend in the knees. They usually cannot run and have to use the handrails to go up and down steps. People with severe diplegia usually need crutches, a walker, or a wheelchair to be able to get around. [3]
Children with diplegia in the legs have a delayed growth in their leg muscles which causes the muscles to be short. This then causes the joints to become stiff and the range of motion to decrease as the child grows. “For the majority of children with diplegia, growth and development are not a problem. Children with diplegia are eventually able to walk, just normally later; they generally attend regular schools and become independently functioning adults.” [8]
The most common cause of diplegia in the legs is cerebral palsy. Paralysis of the legs may also be caused by trauma, injury, or genetics, but this is very rare. [3]
Usually occurs within 2 periods: [3]
Diplegia is usually not diagnosed before the age of 2 years yet the symptoms and signs of the earlier stages are typical and should enable the diagnosis to be made before the contractures have occurred. [9] Parents suspecting diplegia should take their child to the doctor to potentially get an earlier diagnosis.[ citation needed ]
This is broken up by age categories. Different ages require different forms of treatment which may include: therapy, bracing, walkers, wheelchairs, and surgery. Currently the treatments for children are concentrated primarily on independent walking but instead a more independence-oriented therapeutic approach would be more beneficial. [10] This way the child can still focus on walking but at the same time be taught to do things for themselves while using the best method of walking for them. This could include using a walker or wheelchair to get around and do things easier than focusing all the attention on walking so early. For people requiring surgery, distal hamstring lengthening is the most common operation performed because it reduces knee flexion and improves knee motion. [11]
“This first year sees the development of many milestones, such as head control, reaching out for a toy, sitting, starting to vocalize sounds, and finger feeding.” [8] Most parents want their children to excel very fast, but there is a wide upper and lower range of development time for premature babies so it's very hard to diagnose cerebral palsy or diplegia this early. The most common symptom of a child with diplegia is stiff lower extremities. This should become apparent by the six month mark which means he or she does not have severe diplegia. During this age if a child is not moving his legs on his own then it is recommended to do some exercise, especially gentle stretching with the child. [8]
“This is the age at which the characteristics of diplegia become more noticeable, mainly because, unlike other children at this age, the child with diplegia is not walking.” [8] By the age of three, it is important for the child to be in a specialized school environment so the child can participate in physical therapy and learn social skills. Parents should not force the child to sit, crawl, or walk a certain way during this age period. Let the child do what's comfortable for them and allow the therapist to correct this problem. If you want to help your child walk more, then push toys are recommended for walking aids. Regular exams should be done to make sure the child's legs are growing normally and he or she is not having any problems with the hip. [8]
“This is the age range at which the child with diplegia makes the most significant physical improvement in motor function.” [8] During this time period the child makes major improvements in motor function. He or she should be in a regular school and focus on cognitive issues not therapy. A child using a walking aid for mobility to move around with the other children is not a bad thing. If a child is not walking yet, then this is usually caused by a problem in balance, muscle coordination, spasticity, or leg alignment. Each of these reasons should be looked into closely so the problem can be addressed and fixed. [8]
“By the time a child reaches this age the rate of physical improvement has leveled off in areas such as balance and coordination, and it's a good idea to refocus the child’s attention away from additional physical improvement and toward intellectual learning.” [8] During this time period a child should lean away from physical therapy and do more outdoor or social exercises such as sports and adaptive P.E. Usually by age 8-10 a child has reached maximum walking ability. This will usually decrease a little when a child hits puberty and gains height and weight because walking becomes harder during this changing period. Any significant problems in walking should be addressed with surgery at this stage. [8]
“During this time period of a child’s development, a major issue is separating from the family.” [8] Parents should learn how to cope with their child growing up and give them more freedom and independence. Teenagers need to make their own decisions and learn from them. One way to do this is for parents to compromise and let the child make smaller decisions so they feel important. Parents should also understand that their child may regress in walking some from increase in height and weight. Going back to therapy during puberty is recommended so the teenager can adjust to the increase in height and weight and not regress as much. [8]
In 1890 Sachs and Peterson first referenced to the term diplegia, along with the word paraplegia, for their cerebral palsy classification. In 1955 the word diplegia was used in the clinical field to describe a patient whose limbs were affected in a symmetrical way. This included limbs on the same side of the body thus including hemiplegia. Later in 1956 diplegia was presented as a form of bilateral cerebral palsy affecting like parts on either side of the body. In 1965 Milani Comparetti distinguished diplegia from tetraplegia by considering the patient's upper limb's ability to express a sufficient support reaction. Thus diplegia usually refers to just symmetry of one body part or limb, as the legs, or arms. While tetraplegia or quadriplegia refers to paralysis of all 4, both arms and legs. [12]
Hemiparesis, also called unilateral paresis, is the weakness of one entire side of the body. Hemiplegia, in its most severe form, is the complete paralysis of one entire side of the body. Either hemiparesis or hemiplegia can result from a variety of medical causes, including congenital conditions, trauma, tumors, traumatic brain injury and stroke.
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 sensation, vision, hearing, and speech. Often, babies with cerebral palsy do not roll over, sit, crawl or walk as early as other children. Other symptoms include seizures and problems with thinking or reasoning. While symptoms may get more noticeable over the first years of life, underlying problems do not worsen over time.
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.
Tetraplegia, also known as quadriplegia, is defined as the dysfunction or loss of motor and/or sensory function in the cervical area of the spinal cord. A loss of motor function can present as either weakness or paralysis leading to partial or total loss of function in the arms, legs, trunk, and pelvis. The paralysis may be flaccid 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. In these types of spinal cord injury, it is common to have a loss of both sensation and motor control.
Hereditary spastic paraplegia (HSP) is a group of inherited diseases whose main feature is a progressive gait disorder. The disease presents with progressive stiffness (spasticity) and contraction in the lower limbs. HSP is also known as hereditary spastic paraparesis, familial spastic paraplegia, French settlement disease, Strumpell disease, or Strumpell-Lorrain disease. The symptoms are a result of dysfunction of long axons in the spinal cord. The affected cells are the primary motor neurons; therefore, the disease is an upper motor neuron disease. HSP is not a form of cerebral palsy even though it physically may appear and behave much the same as spastic diplegia. The origin of HSP is different from cerebral palsy. Despite this, some of the same anti-spasticity medications used in spastic cerebral palsy are sometimes used to treat HSP symptoms.
Monoplegia is paralysis of a single limb, usually an arm. Common symptoms associated with monoplegic patients are weakness, numbness, and pain in the affected limb. Monoplegia is a type of paralysis that falls under hemiplegia. While hemiplegia is paralysis of half of the body, monoplegia is localized to a single limb or to a specific region of the body. Monoplegia of the upper limb is sometimes referred to as brachial monoplegia, and that of the lower limb is called crural monoplegia. Monoplegia in the lower extremities is not as common of an occurrence as in the upper extremities. Monoparesis is a similar, but less severe, condition because one limb is very weak, not paralyzed. For more information, see paresis.
Periventricular leukomalacia (PVL) 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 neonatal encephalopathy which may lead to this condition. Affected individuals generally exhibit motor control problems or other developmental delays, and they often develop cerebral palsy or epilepsy later in life. The white matter in preterm born children is particularly vulnerable during the third trimester of pregnancy when white matter developing takes place and the myelination process starts around 30 weeks of gestational age.
Pachygyria is a congenital malformation of the cerebral hemisphere. It results in unusually thick convolutions of the cerebral cortex. Typically, children have developmental delay and seizures, the onset and severity depending on the severity of the cortical malformation. Infantile spasms are common in affected children, as is intractable epilepsy.
Hypertonia is a term sometimes used synonymously with spasticity and rigidity in the literature surrounding damage to the central nervous system, namely upper motor neuron lesions. Impaired ability of damaged motor neurons to regulate descending pathways gives rise to disordered spinal reflexes, increased excitability of muscle spindles, and decreased synaptic inhibition. These consequences result in abnormally increased muscle tone of symptomatic muscles. Some authors suggest that the current definition for spasticity, the velocity-dependent over-activity of the stretch reflex, is not sufficient as it fails to take into account patients exhibiting increased muscle tone in the absence of stretch reflex over-activity. They instead suggest that "reversible hypertonia" is more appropriate and represents a treatable condition that is responsive to various therapy modalities like drug or physical therapy.
Fazio–Londe disease (FLD), also called progressive bulbar palsy of childhood, is a very rare inherited motor neuron disease of children and young adults and is characterized by progressive paralysis of muscles innervated by cranial nerves. FLD, along with Brown–Vialetto–Van Laere syndrome (BVVL), are the two forms of infantile progressive bulbar palsy, a type of progressive bulbar palsy in children.
A selective dorsal rhizotomy (SDR), also known as a rhizotomy, dorsal rhizotomy, or a selective posterior rhizotomy, is a neurosurgical procedure that selectively cuts problematic nerve roots in the spinal cord. This procedure has been well-established in the literature as a surgical intervention and is used to relieve negative symptoms of neuromuscular conditions such as spastic diplegia and other forms of spastic cerebral palsy. The specific sensory nerves inducing spasticity are identified using electromyographic (EMG) stimulation and graded on a scale of 1 (mild) to 4. Abnormal nerve responses are isolated and cut, thereby reducing symptoms of spasticity.
Central facial palsy is a symptom or finding characterized by paralysis or paresis of the lower half of one side of the face. It usually results from damage to upper motor neurons of the facial nerve.
Triplegia is a medical condition characterized by the paralysis of three limbs. While there is no typical pattern of involvement, it is usually associated with paralysis of both legs and one arm—but can also involve both arms and one leg. Triplegia can sometimes be considered a combination of hemiplegia overlaying diplegia, or as quadriplegia with less involvement in one extremity.
Spastic quadriplegia, also known as spastic tetraplegia, is a subset of spastic cerebral palsy that affects all four limbs.
Scissor gait is a form of gait abnormality primarily associated with spastic cerebral palsy. That condition and others like it are associated with an upper motor neuron lesion.
Over time, the approach to cerebral palsy management has shifted away from narrow attempts to fix individual physical problems – such as spasticity in a particular limb – to making such treatments part of a larger goal of maximizing the person's independence and community engagement. Much of childhood therapy is aimed at improving gait and walking. Approximately 60% of people with CP are able to walk independently or with aids at adulthood. However, the evidence base for the effectiveness of intervention programs reflecting the philosophy of independence has not yet caught up: effective interventions for body structures and functions have a strong evidence base, but evidence is lacking for effective interventions targeted toward participation, environment, or personal factors. There is also no good evidence to show that an intervention that is effective at the body-specific level will result in an improvement at the activity level, or vice versa. Although such cross-over benefit might happen, not enough high-quality studies have been done to demonstrate it.
Athetoid cerebral palsy, or dyskinetic cerebral palsy, is a type of cerebral palsy primarily associated with damage, like other forms of CP, to the basal ganglia in the form of lesions that occur during brain development due to bilirubin encephalopathy and hypoxic–ischemic brain injury. Unlike spastic or ataxic cerebral palsies, ADCP is characterized by both hypertonia and hypotonia, due to the affected individual's inability to control muscle tone. Clinical diagnosis of ADCP typically occurs within 18 months of birth and is primarily based upon motor function and neuroimaging techniques. While there are no cures for ADCP, some drug therapies as well as speech, occupational therapy, and physical therapy have shown capacity for treating the symptoms.
Spastic cerebral palsy is the type of cerebral palsy characterized by spasticity or high muscle tone often resulting in stiff, jerky movements. Cases of spastic CP are further classified according to the part or parts of the body that are most affected. Such classifications include spastic diplegia, spastic hemiplegia, spastic quadriplegia, and in cases of single limb involvement, spastic monoplegia.
Spastic hemiplegia is a neuromuscular condition of spasticity that results in the muscles on one side of the body being in a constant state of contraction. It is the "one-sided version" of spastic diplegia. It falls under the mobility impairment umbrella of cerebral palsy. About 20–30% of people with cerebral palsy have spastic hemiplegia. Due to brain or nerve damage, the brain is constantly sending action potentials to the neuromuscular junctions on the affected side of the body. Similar to strokes, damage on the left side of the brain affects the right side of the body and damage on the right side of the brain affects the left side of the body. Other side can be effected for lesser extent. The affected side of the body is rigid, weak and has low functional abilities. In most cases, the upper extremity is much more affected than the lower extremity. This could be due to preference of hand usage during early development. If both arms are affected, the condition is referred to as double hemiplegia. Some patients with spastic hemiplegia only experience minor impairments, where in severe cases one side of the body could be completely paralyzed. The severity of spastic hemiplegia is dependent upon the degree of the brain or nerve damage.
Diane Louise Damiano is an American biomedical scientist and physical therapist specializing in physical medicine and rehabilitation approaches in children with cerebral palsy. She is chief of the functional and applied biomechanics section at the National Institutes of Health Clinical Center. Damiano has served as president of the Clinical Gait and Movement Analysis Society and the American Academy for Cerebral Palsy and Developmental Medicine.