Infantile cerebral palsy
| Classification according to ICD-10 | |
|---|---|
| G80.- | Infantile cerebral palsy |
| G80.0 | Spastic tetraplegic cerebral palsy |
| G80.1 | Spastic diplegic cerebral palsy |
| G80.2 | Infantile hemiplegic cerebral palsy |
| G80.3 | Dyskinetic cerebral palsy |
| G80.4 | Atactic cerebral palsy |
| G80.8 | Other infantile cerebral palsy |
| G80.9 | Infantile cerebral palsy, unspecified |
| ICD-10 online (WHO version 2019) | |
The expression infantile cerebral palsy or cerebral palsy (from Latin cerebrum "brain" and Greek parese "paralysis", often abbreviated to ICP or CP ) in the narrower sense, more generally also called cerebral movement disorder, means movement disorders , the cause of which is brain damage in early childhood lies. The resulting disability is characterized by disorders of the nervous system and muscles in the area of voluntary motor skills . Spastic mixed forms and muscle hypertension are the most common , but athetotic or atactic forms also occur.
Synonyms
One speaks of “cerebral palsy”, “infantile paralysis”, “paralysis infantum”; after the English orthopedic surgeon William John Little also (obsolete) from the "Little'schen disease" ( Little's disease ) or ankylosing Little , too (also obsolete): "Foerster syndrome / diplegia / disease". The people who are affected by infantile cerebral palsy are also generally called spastics . The more general term “cerebral movement disorder” includes the different forms of disturbed movement and takes into account not only congenital but also acquired damage such as brain tumors, traumatic brain injuries or those following inflammatory or vascular diseases.
General
The human brain is, among other things, a control center that sends commands to the musculoskeletal system. This in turn reports actions carried out back to the brain. This is how the human motor cycle arises. Paresis is a disturbed circulation which, depending on which brain region is affected, leads to different types of disability. The cerebral movement disorder can be divided into three forms: spasticity, athetosis and ataxia, which however often occur in mixed forms.
In spasticity, muscle tension is increased (hypertonicity), as the interplay between tension and relaxation is disturbed. In athetosis, the muscle tension changes; sudden impulses lead to outward movements such as the arms and legs. The ataxia is characterized by a low basic tension, which makes it difficult to perform movements with confidence.
Cerebral palsy can be recognized soon after birth in severely affected children, and only after three to four months in others. In general, one speaks of cerebral palsy only after the end of the first year of life. There is no specific treatment for cerebral palsy; every child reacts differently.
causes
Clear causes of the brain development disorder are only found in around 50 percent of cases. Typically, an oxygen deficiency before, during or shortly after birth leads to the death of nerve cells. Accordingly, an Apgar score below 4 at the point in time five minutes after the birth represents a considerable risk factor for infantile cerebral palsy. Various infectious diseases during pregnancy such as toxoplasmosis , cytomegaly , rubella or, earlier, syphilis damage the brain of the unborn child. Malformations of the placenta can cause an insufficient supply of the developing brain with subsequent damage. Other causes during pregnancy can be damage from medication ( embryopathies ), alcohol ( alcohol embryopathy ) or carbon monoxide . After birth, cerebral haemorrhage, especially in premature babies, or damage from increased neonatal jaundice , which leads to bilirubin encephalopathy , are important causes. Beyond the neonatal period, traumatic brain injury or meningitis most commonly cause brain damage with subsequent cerebral movement disorders .
Only about two percent of the cases of infantile cerebral palsy can be traced back to genetic causes according to English and Swedish studies. In all of these cases the symptoms are symmetrical. As with paraplegic , a distinction is made between tetraplegic and paraplegic cerebral palsy with several types. The causative gene mutations are known for all of these types. Among other things, GABA biosynthesis ( glutamate decarboxylase ) is affected .
Epidemiology
Statistically, about 1 in 500 children born alive is diagnosed with cerebral palsy. Very small premature babies are about 100 to 300 times more likely to be affected than mature babies .
The incidence is 9 / 100,000 people per year.
Symptoms
The usual development of the central nervous system is prevented, development is inhibited, early childhood reflexes continue to exist and the development of physiological reflex pathways does not occur.
Different forms of movement and posture peculiarities can be distinguished, often mixed forms.
Slowed motor skills and weak muscles occur.
Spasticity
In the past, spasticity was also referred to as spastic paralysis. Brain damage includes both involuntary (extrapyramidal motor system) and voluntary movements ( pyramidal system ). Damage to this area affects the interplay of muscles between tension and relaxation. The muscle tone is increased (hypertonicity), which hardens the muscles and increases the reflex readiness. The co-contraction (= simultaneous tension of agonists and antagonists) is extremely high. This results in a very rigid posture that severely restricts the mobility of those affected and often leads to stereotypical movement patterns. The sense of balance is disturbed and fine motor skills are also impaired. Depending on the severity of the damage, there is either constantly increased muscle tension or, in milder cases of spasticity, the tone is only increased during activity. According to Udo Kalbe, spasticity is the most common form of cerebral movement disorders at 60 percent.
Ataxia
The term ataxia comes from the Greek (αταξία) and means disorder or without order. About 15 percent of people with cerebral movement disorders are affected.
Ataxia is a disturbed coordination of movement sequences. It is based u. a. too low a basic tension of the muscles, which is also known as hypotension. As a result, the movements are less precise, and sequences of movements usually appear jittery and inharmonious. The dosage and gradation as well as the rapid braking of movements are made more difficult by the low voltage, so that movement sequences cannot be coordinated properly. The ataxia also affects the equilibrium, which is considerably disturbed. It is still possible to learn to walk, but the gait seems unsteady. The ataxia is usually coupled with a tetraparesis, which means that the entire skeletal muscles are affected by the disorder.
Athetosis
The term athetosis is derived from the Greek word άθετος ('athetos') = 'not in its place' or 'without a fixed position'. About 10 percent of people with cerebral movement disorders are affected.
In athetosis, the (usually hypoxic) lesion affects the extrapyramidal system. In this disorder there is an alternating muscle tone and a balanced, normal co-contraction is missing. Muscle tension is too low at rest, while it alternates between hypotension and hypertension during activity. This creates - especially with sudden movement impulses - extending and bizarre screwed movements of the arms and legs, but especially of their distal sections. Not only are voluntary movements affected, but involuntary movements with typical characteristics also occur. The appearance of athetosis also means that it is difficult for the person affected to control the head. This makes it difficult for children to get up in an elevated position. Eating and speaking are hindered by the changing muscle tension. Another problem is maintaining balance, but this is a very important component in learning to walk. For this reason, walking independently is often only possible with mild forms of athetosis or at an advanced age. Although the damage affects the entire body, there is usually a significant difference between the sides, i.e. one side is more affected than the other.
Classification according to distribution pattern
The movement disorder can affect the legs (paraparesis, 40%), one half of the body (hemiparesis, 32%) or all four extremities (tetraparesis, 2%). The terms para-, hemi-, di- or tetraplegia, which are often used in connection with infantile cerebral palsy, are imprecise and inaccurate, as a plegia usually describes a complete paralysis in the context of a paraplegia .
Side effects
In addition to movement disorders, there are often other symptoms that are caused by the underlying brain damage. Between 30 and 50 percent of patients have symptomatic epilepsy . In addition to motor skills, mental development can also be delayed. Behavioral disorders may also occur. However, around half of those affected have normal or only slightly reduced intelligence . If the brain damage affects the corresponding regions, eye symptoms in the form of strabismus , speech disorders and hearing impairment can also occur. The disruption of the control of the muscles can ultimately lead to short stature and muscle wasting in pronounced cases .
Effects
The frequently occurring spastic paralysis results in restricted mobility of the affected extremities. The use of a wheelchair can be indicated to compensate for this. The occasional speech disorders can also impair participation in social life. In some cases, cognitive limitations also occur, which make independence and cope with everyday life more difficult. Others, on the other hand, have average cognitive performance and are usually well integrated into society. The independent cope with everyday life (e.g. eating and drinking, using the toilet, dressing and undressing) can be made more difficult by the restricted gross motor skills, but through training and constant repetition of individual targeted movements, the existing motor skills can sometimes be refined, so that often everyday life is made possible (if necessary with supportive assistance). In general, attention should always be paid to the support of the person concerned, as the development of skills (be it cognitive, physical or psychological) varies greatly from person to person.
Gait pattern
Characteristic of cerebral palsy is the stepper gear .
Mentality and psyche
Recent studies indicate that adults with cerebral palsy are more likely than other people to struggle with depression and anxiety, and that symptoms of fatigue syndrome are more common . Just like the sensation of pain, fatigue can also increase with age.
treatment
A multidisciplinary therapy from different medical and therapeutic areas is the focus of the treatment of infantile cerebral palsy. Basically, this should start as early as possible in the course of the disease. A causal therapy, i.e. therapy that heals the disease, is not possible due to the already existing and completed brain damage. Of particular importance is the elaboration of a rehabilitation plan that includes the various therapy options, in which, in particular, the therapy goals to be achieved should be explained and specified. The focus of the treatment is on supportive, conservative therapeutic measures such as physiotherapy , occupational therapy , speech therapy , which are supported by special drug therapies and conservative orthopedic contracture prevention through various orthopedic techniques. Operative measures should only be used if the course progresses, if all conservative measures have been exhausted and if the indication for surgery is very strict.
Conservative therapy
Conservative therapy encompasses a wide range of rehabilitation medicine. It should start as soon as possible after the diagnosis.
physical therapy
In earlier years, the physiotherapeutic treatment of cerebral movement disorders was aimed primarily at avoiding the development of so-called abnormal movement and substitute patterns by initiating the most normal movement sequences possible. Therapy based on both the Bobath and Vojta concepts aims to store movement sequences that are as normal as possible in the brain and use them for independent courses of action. According to the current state of knowledge, motor learning programs are conveyed using the physiotherapy methods. This is most effectively done through active, repeated practice of meaningful tasks. Accordingly, methods from adult rehabilitation such as treadmill training or forced training of the affected side in hemiparesis are increasingly being integrated into the treatment.
Occupational therapy
Occupational therapy consists primarily of instructions for self-help, special work and writing aids as well as targeted therapy for the mostly severely disturbed sensorimotor functions of the hands.
Speech therapy
Both swallowing disorders and speech disorders can make speech therapy necessary due to the disturbed movement sequences. As part of the accompanying general developmental delay, speech developmental delay is also treated through speech therapy.
Conductive promotion according to Petö
The conductive support according to Petö sees itself as a complex educational support and was developed at the end of 1940 by the Hungarian doctor András Petö in order to support cerebrally damaged children holistically in their motor and psychological development. It is suitable for children and adults. It consists of physiotherapeutic, educational, occupational therapy, psychological and speech therapy elements. The primary goal is to make the patient so mobile that he can lead an independent and independent life. In Germany, the Federal Joint Committee rejected inclusion in the reimbursable part of the catalog of medical aids in a final statement because it had not been proven that Petö is better than what the health insurances are already paying. On September 29, 2009, the Federal Social Court took into account the complexity of the conductive support and placed it under the jurisdiction of the social welfare agency as an integration assistance service.
Medical therapy
Various drugs are primarily intended to reduce the increased tension in the spastic muscles. These include benzodiazepines , baclofen, and anticholinergics . To treat the spasticity, botulinum toxin (also known as "botox") can be injected locally into the affected muscles , which reduces muscle tension over a period of about three months. In the case of accompanying mental disorders and states of restlessness in the context of an accompanying mental developmental disorder, various psychotropic drugs such as B. sedating neuroleptics are used.
Orthopedic technology
Orthopedic technology primarily provides aids for contracture prophylaxis and joint stabilization through functional splints, standing, walking, sitting and gripping aids. The production of custom-fit corsets should at least prevent a worsening of a secondary curvature of the spine.
Operative therapy
Surgical therapy focuses on the correction and prophylaxis of contractures and deformities, as well as the greatest possible restoration of muscle balance to further prevent pathological movement patterns. Various surgical techniques are available for this purpose: The aim of a tendon lengthening (tenotomy), muscle notching ( myotomy ) and relocation of the muscle origin is to eliminate the contracture while preserving the tension of the muscles. An Achilles tendon lengthening and hip flexion contracture are also used. A nerve transection ( neurotomy ) is used to treat the most severe spastic contractures, especially those who are unable to walk. This irreversibly converts a spastic paresis into a flaccid paralysis. A bone remodeling ( osteotomy ) is used when deformities of the joints have already occurred and a simple tendon lengthening does not bring any further therapeutic benefit. Joint stiffening ( arthrodesis ) leads to permanent corrections in the area of unstable joints. To treat the spasticity, a drug pump for intrathecal administration of baclofen can also be implanted into the CSF space . The effect unfolds directly on the spinal cord, where the spasticity is caused by the disinhibition of the moto-neurons. This happens after a successful test of an input of baclofen via a lumbar puncture. However, the prerequisite is a certain size of the person concerned.
School aspect
For children with cerebral palsy, enrolling in a regular school is usually a difficult and bureaucratic process. Many teachers are not trained to take children with a physical disability , even if their cognitive abilities are comparable to those of a regular child. Therefore z. For example, in Switzerland it is not mandatory for any teacher to take in a child with cerebral palsy. In many cases it is up to the parents to ensure that their own child is accepted into a regular class.
If a child with physical difficulties is accepted into the regular class, their everyday school life can be characterized by hurdles. It should be noted that the same is usually expected of a child with a disability as a child without restrictions. There is now the possibility of an integration helper for disabled children. However, the application is sometimes associated with a bureaucratic effort.
Popular culture
The actor RJ Mitte , who himself suffered from a mild form of infantile cerebral palsy , was best known for his role as a young person suffering from ICP in the television series Breaking Bad . In the sitcom Speechless , a family confronted with the illness of their eldest son is at the center. Leading actor Micah Fowler is affected by the disease himself.
literature
- Adriano Ferrari, Giovanni Cioni (Ed.): Infantile Cerebralparese. Spontaneous course and orientation aids for rehabilitation (= rehabilitation and prevention. Volume 39). Springer, Berlin a. a. 1998, ISBN 3-540-62028-1 .
- Renate Holtz: Therapy and everyday aids for cerebral palsy children. 2nd, revised and expanded edition. Pflaum, Munich a. a. 2004, ISBN 3-7905-0912-4 .
- Berta Bobath , Karel Bobath: The motor development in cerebral palsy. 6th, unchanged edition. Thieme, Stuttgart 2005, ISBN 3-13-539006-3 .
- Margret Feldkamp, Hans-Henning Matthiaß: Diagnosis of infantile cerebral palsy in infancy and childhood, 2nd edition Thieme, Stuttgart 1988
- Florian Heinen (ed.): Botulinum toxin in children with cerebral palsy. 2nd Edition. Science publishing house Wellingsbüttel, Hamburg 1999, ISBN 3-926774-28-2 .
- Freeman Miller, Steven J. Bachrach: Cerebral Palsy. A Complete Guide for Caregiving. 2nd Edition. Johns Hopkins University Press, Baltimore MD 2006, ISBN 0-8018-8354-7 .
Web links
Individual evidence
- ↑ a b c D. Karch: Physiotherapy on a neurophysiological basis according to Bobath and Vojta in children with cerebral movement disorders (with special consideration of infantile cerebral palsy). (PDF) German Ges. F. Social paediatrics and adolescent medicine e. V., accessed September 15, 2013 .
- ^ Kari Kveim Lie, Else-Karin Grøholt, Anne Eskild: Association of cerebral palsy with Apgar score in low and normal birthweight infants . In: British Medical Journal . No. 341 , October 7, 2010, p. c4990 ( bmj.com [accessed December 17, 2014]).
- ^ Infantile cerebral palsy. In: Online Mendelian Inheritance in Man . (English)
- ^ Infantile cerebral palsy. In: Online Mendelian Inheritance in Man . (English)
- ^ Thames Valley Children's Center - Cerebral Palsy - Causes and Prevalence. (No longer available online.) Archived from the original on August 23, 2007 ; Retrieved June 11, 2007 .
- ↑ Karl F. Masuhr, Marianne Neumann: Dual series of neurology . 6th edition. Georg Thieme Verlag, 2007, ISBN 978-3-13-135946-9 , p. 160 .
- ^ Janice Wood, Risk of Depression & Anxiety Higher in Adults with Cerebral Palsy. January 7, 2019, accessed April 3, 2019 (American English).
- ^ Cerebral Palsy (CP) and aging | Disability charity Scope UK. May 6, 2016, accessed April 4, 2019 .
- ↑ Conductive promotion according to Petö - summary report of the subcommittee “Medicines and Aids” of the Federal Joint Committee on the deliberations according to § 138 SGB V full text. (PDF; 1.7 MB) accessed on August 14, 2008
- ↑ BSG September 29, 2009 - B 8 SO 19/08 R, grounds for judgment of February 1, 2010, lexetius.com
