Pediatric cerebral palsy (BSF) is a complex of clinical syndromes manifested by cerebral-type movement disorders. BSF occurs due to damage to the brain in the prenatal, natal, and postnatal periods. In addition to movement disorders (paralysis, hyperkinesis), mental retardation, epileptic seizures, ataxia, hearing and vision disorders are also observed.
Although most clinical symptoms of BSF appear in early childhood, they do not worsen. This means that the formed neurological syndromes remain unchanged over the years. However, age-related functional disorders may occur or deepen.
History.
The term “cerebral palsy” originated in the mid-XNUMXth century. At the time, English surgeon Little was trying to surgically correct spastic paralysis in children. The main cause of cerebral palsy is brain injuries that occur during childbirth, Little wrote. He often uses the terms "cerebral palsy" and "spastic diplegia" in his work. Later, experts began to refer to non-violent and almost permanent paralysis in children under the general name of “Children’s cerebral palsy”. Spastic diplegia is called Little's disease.
Etiology.
Cerebral palsy in children is a disease of many etiologies.
Various infections (SMV, toxoplasmosis, etc.) and diseases (toxicosis, severe anemia, chronic intoxication, endocrinopathies), harmful habits (smoking, drinking, drug addiction), satellite diseases, maternal-child rhesus factor during pregnancy inconsistency, consumption of pharmacological drugs with embryotoxic effects may contribute to the development of BSF.
Brain injuries, cerebral ischemia, hypoxia and hemorrhage that occur during childbirth are also major etiological factors of this disease. It is also worth noting that the child is born prematurely and is underweight. This is because most children diagnosed with ‘childhood cerebral palsy’ are born prematurely and weigh less than 2000 g. For example, the risk of developing BSF in children born with a body weight of 1500 g is almost 30 times higher than in children born with normal body weight. The main reasons for low body weight are the above etiological factors. Children born to women who are pregnant early (under 16) or late (after 40) are also at higher risk of developing BSF.
Another etiological factor of BSF is a genetic factor.
The fact that the parents are close relatives and similar various hereditary factors cause the child to develop underdeveloped brain (brain dysgenesis). At such times, there is talk of BSF of genetic etiology.
Early postnatal diseases such as ischemic and hemorrhagic strokes, hypoxic-ischemic encephalopathies, brain injuries (including hematomas), meningoencephalitis (measles, rubella) are also considered etiological factors in the development of BSF. However, in the development of BSF, the emphasis is on prenatal and natal factors rather than postnatal factors.
Pathogenesis and pathomorphology
The pathogenesis of BSF depends in many respects on the period in which the etiological factor affects the child's brain, ie in the prenatal, natal or postnatal periods. Various infections and diseases during pregnancy negatively affect the blood supply to the baby through the placenta. During this period, the supply of nutrients and oxygen also decreases. As a result, embryogenesis is disrupted. The embryo is very sensitive to oxygen deficiency.
Therefore, embryonic hypoxia is considered to be a major etiopathogenetic factor in the development of BSF. It is hypoxia that causes ischemic encephalopathy and strokes in the brain. Due to hypoxia and ischemia, the central nervous system of the fetus lags behind in development and its anomalies (micropolygyria, paxigiria, agiria, porencephaly, microcephaly, hydrocephalus, agenesis of the body) are formed.
These anomalies occur mainly during early ontogenetic development. Pathomorphological changes occur differently in different parts of the brain. In most cases, leukomalacia are detected in the periventricular area through which the pyramidal pathways pass. This is due to disruption of the myelination process or destruction of the formed myelin sheath. Modern neuroimaging techniques almost always show periventricular leukomalacia, pyramidal neuronal degeneration, and necrotic foci in the subcortical area of the white matter in types of disease with cerebral palsy. These pathological changes develop under the influence of hypoxic-ischemic damage, microhemorrhage and cytokines. In the hyperkinetic type of BSF, pathomorphological changes are detected in the subcortical nuclei, and in the atactic type in the cerebral nuclei and pathways.
Clinic.
Clinical syndromes depend in many ways on which area of the brain the pathological process is located. Therefore, the following clinical types of the disease are distinguished.
1. Spastic diplegia (Little's disease) - A type of cerebral palsy in children with spastic paralysis (diplegia) in both legs. This condition is also called "paralyzed child" in the vernacular. Little disease, i.e. spastic diplegia, is the most common and first studied type of BSF. Such children are almost confined to wheelchairs. Spastic hypertonus is unevenly located in the muscles of both legs, that is, the tone of the muscles that bend the thigh forward, stretch the leg and bend the toe down sharply increases.
As a result, the legs are stretched at the knee joint and stretched forward, while the toes bend sharply downward and rotate inward. If you try to hold such a child by the armpits, the inward rotation of both legs will increase and the toes will sit on top of each other or cross in an X-shape. If you want to move the child, his heels do not touch the ground, but try to walk on tiptoe. In both legs, the periosteal and knee reflexes increase sharply, but the axillary reflex may be decreased (due to improper distribution of muscle tone). In such children, symptoms of spastic paralysis may also appear on the hands, but they are often mildly expressed. Sometimes the signs of apraxia in the hands are clearly expressed, choreo-atetoid hyperkinesis is detected. As a result, the child is unable to perform manual tasks (holding a spoon or bowl, wearing buttons, etc.), the calligraphy is severely impaired. If apraxia is observed in both the oral and facial muscles, it is difficult for the child to chew the food given into the mouth. They are also diagnosed with dysarthria. This pathological condition in both legs leads to the development of different degrees of muscle-joint contracture. Contractures develop not only in the ankle joints, but also in the spine, and are often manifested by kyphosis and kyphoscoliosis. As a result, the shape of the chest also changes. Some children are diagnosed with mental retardation, pseudobulbar palsy, cranial neuropathy, and dyslalia. They need constant care.
2. Hemiplegic (hemiparetic) type is also the most common type of BSF. This type of disease is often associated with a cerebral stroke or developmental delay of one of the cerebral hemispheres. Symptoms of unilateral paralysis manifest to varying degrees. When the child is 3-4 months old, it is noticeable that he is moving with only one hand. This condition is often felt by the child's mother or other relatives. The peculiarity of the hemiplegic type of BSF is that the paralysis is mainly pronounced in one hand and mild in the other. The distal part of the hand, i.e. the palm of the hand, suffers more. Later, before the child is 1 year old, the muscle tone in the paralyzed arm and leg begins to increase spasticly, the arm bends at the elbow joint. Such children walk late. In severe cases, the symptoms of the disease become known less than a month after birth, and over time, the symptoms of hemiplegia become more pronounced. Such a child can hardly move one side (especially his arm), can not turn to the side, and if he passes it, he will fall to the paralyzed side. Regardless of how the disease manifests itself, the paralyzed side lags behind in growth and development. In most cases, mental retardation, cortical dysarthria, pseudobulbar palsy, dyslalia, spinal deformity (scoliosis), joint contracture on the paralyzed side are detected. Such children usually start walking at the age of 3-4 years, and speech also develops late.
3. Bilateral hemiplegia It is the most severe type of BSF, manifested by bilateral spastic cerebral palsy. This type of disease often develops due to severe brain damage (injury) that occurs in the early ontogenetic period. Such babies are often born motionless, usually by cesarean section. From the first days of infancy, muscle hypotension is detected on both sides. The child becomes very weak and develops slowly. Before the age of one, the child begins to spasticly increase muscle tone on both sides, the joint reflexes intensify, that is, spastic tetraparesis is formed. Such children can neither sit nor stand. In them, conditioned and unconditional reflex activity is disrupted. When he tries to help the child, syncope develops in his arms and legs, muscle tone becomes more intense, and the limbs become stiff in a pathological state. In most cases, microcephaly is detected, in almost 90% of cases there is a delay in mental and emotional development. Epileptic seizures, cortical dysarthria, cranial neuropathies, pseudobulbar palsy, dyslalia are also detected. Such children become completely disabled.
4. Hyperkinetic type of BSF a little rare. Its development is associated with damage to the extrapyramidal nuclei. Hyperkinesis is most often manifested in the form of choreoathetosis and torsional dystonia, rarely in the form of athetosis and ballism. These symptoms usually appear after the child is one year old and get worse over a period of time. In some children, the hyperkinetic syndrome is mildly expressed and they may be self-limiting. In severe cases, dystonic conditions are formed due to hyperkinesis: the shape of the neck, arms and legs, as well as the body changes, that is, they twist to one side.
Because of hyperkinesis, voluntary movements become impossible: the child is unable to hold objects with his hands, and even if he does, they fly out of his hands, do not eat properly, cannot sit, and fall. Such children can become self-sufficient and sit upright by the age of 6-8. Special rehabilitation treatments will definitely speed up the process. Due to hyperkinesis and muscular dystonia, the function of the joints in the limbs is impaired, in which outbursts are observed (especially in large-amplitude hyperkinesis). Extrapyramidal dysarthria is almost always diagnosed. High mental functions, including mental activity, are preserved. Occasionally, mild cognitive impairment may be observed.
5. Atactic (brain) type is manifested by varying degrees of disturbances of coordination and balance. Cerebral atrophy is detected in these children. The main neurological symptoms are cerebral symptoms. A child who is not yet young is initially diagnosed with complete hypotension, or "sluggish child syndrome." Later, cerebral symptoms begin to become more pronounced. Symptoms include nystagmus, scanned speech, tension tremor, asymmetry, asynergy, static and dynamic ataxia. The child finds it very difficult, especially when performing locomotor tests. It is known that the brain plays an important role in the formation of standing and walking correctly. Therefore, the ability to walk in these children is formed by the age of 3 (sometimes later).
The manifestation of scanned speech along with dysarthria seriously impairs the development of higher mental functions. The child begins to lag behind in mental development. The cerebral type of BSF is very rare.
6. Atonic-astatic type inability to stand, walk, and complete atony of the muscles. Such children cannot stand, sit, hold their heads properly, or walk. Hence, asthma and abasia symptoms can be observed in them. Therefore, goal-directed voluntary actions in the child develop very slowly. In such children, the ability to sit is formed at the age of 2-3 years, and the ability to stand and walk is usually formed at the age of 7-9 years. Articulatory speech is very sluggish.
In some children, mental retardation is identified. Symptoms of cranial neuropathy (optic nerve atrophy, dizziness) may also be detected. There are also mixed types of BSF, in which case two or more of the above syndromes are detected in a single child. While spastic diplegia (Little's disease), hemiplegic type, and bilateral hemiplegia are often observed in early childhood, mixed types, i.e., hyperkinetic-dystonic, atactic, and atonic-astatic types, are more common in slightly older children. Vegetative and neuroendocrine disorders are also frequently detected in BSF. These are persistent tachycardia, vestibular dizziness, orthostatic hypotension, profuse sweating, xerophthalmia, dry mouth, hyperkeratosis, obesity, gynecomastia, genital malformation, enuresis and trophic ulcers. In mild forms of BSF, psychoemotional and cerebrasthenic symptoms are also more common. Clinical syndromes in BSF are not always fully formed as described above.
BSF may present with hydrocephalus, oligophrenia, and monoparesis or microcephaly of one arm, epileptic seizures and ataxia, or only oligophrenia, focal hyperkinesis, and cranial neuropathy. Sometimes the syndrome is manifested only by cranial neuropathies (dizziness, nystagmus, optic nerve atrophy, hemianopsia, deafness) or mental retardation.
Diagnosis and comparative diagnosis.
The main factor to consider when making a diagnosis of BSF is the stability of the clinical symptoms that occur, i.e., the severity. Hence, symptom stability is very specific for BSF. But it should be remembered that as the child grows older, new symptoms may be added for some time. Naturally, this indicates that the disease is not developing, but that new defects are emerging. Diagnosing BSF can be difficult until the child is 3 years old. Because until this period, clinical syndromes continue to form. In the diagnosis of BSF, the child is lagging behind in psychomotor and speech development, persistent neurological defects (spastic paralysis, dysarthria, dyslalia, dystonic hyperkinesis, cranial neuropathy, ataxia, astasia-abasia) and neuroimaging examination data (structural defects in the brain) taken into account. Of course, anamnestic data (especially prenatal and postnatal) are also very important. Children born with various neurological defects should have a neurological examination every 1–1,5 months. Symptoms of BSF usually begin to form at the age of 1-2 years.
During this period, it is very important that the neurologist immediately detects and monitors them. This is very important in the correction of subsequent functional disorders.
Comparative diagnosis is made with many hereditary-degenerative diseases that occur in early childhood. In particular, spastic paraplegias (Stryumpel's disease), spinal amyotrophs (Verdnig-Goffmann's disease), congenital myopathies, and hereditary neurometabolic syndromes almost always have to be compared with BSF.
Treatment.
Treatment and rehabilitation procedures are continued for a long time. A child diagnosed with BSF should be monitored regularly and receive routine treatment. Such children are in constant need of neurological, neuropsychological and orthopedic-surgical rehabilitation. They are also treated in special boarding schools, educated and brought up in special schools. It is also important to take children to health resorts with the necessary conditions, to conduct physiotherapeutic and reflexology treatments. Speech therapists treat children with speech impediments. Children with special needs should be monitored by a psychologist or psychiatrist.
Today, computerized devices have been developed to eliminate or reduce functional impairment in children with BSF. They are used to correct functional defects caused by various clinical syndromes. More sophisticated types of these devices are being developed. They can also be used at home. Manual labor is also important in the correction of functional disorders.
Drug treatment includes cerebral metabolites (citicoline, choline al- fosserate, pyriditol, pantogam, pantocalcin), amino acids (lecithin, methionine, glutamic acid), muscle tone-reducing drugs (midokalm, baclofen, antidote, sirdalud, levodopa) phenobarbital, finlepsin, depakin, convulsion, diazepam, amitriptyline), anticholinesterase drugs (galantamine, procerin, kalimin), microcirculation drugs (trental, xanthinol nicotinate), immunomodulators (immunomodulin, vitamin B1, Bimalin, timalin, timalin) PP, E, A, C) are recommended. Physiotherapeutic treatments include drug electrophoresis, muscle electrostimulation, mineral baths (iodine-bromine, oxygen, radon), hydrokinesis, underwater massage, swimming, mud and ozokerite treatment, general orthopedic massages. Musculoskeletal contractures are corrected by orthopedic-surgical methods.
Forecast.
The severity of the disease depends on the type of clinical syndromes and how well the treatment-rehabilitation procedures are performed.
