Ataxic cerebral palsy is a type of cerebral palsy that occurs when the cerebellum or the frontal-bridge-cerebellar pathway is affected. The risk of developing pathology increases with prematurity, the action of teratogenic factors, birth trauma. The atactic form of paralysis is manifested by muscular hypotension, ataxia, cerebellar dysarthria, as well as intellectual disabilities of varying severity. Diagnosis of the disease involves an assessment of the neurological status, neuroimaging (cerebral ultrasound, CT or MRI), electrophysiological methods (ENMG, EEG). Treatment includes medications, comprehensive rehabilitation, orthopedic care.
G80.4 Ataxic cerebral palsy
Cerebral palsy occurs with a frequency of 2-3.6 cases per 1000 live-born full-term children, 9-40 cases per 1000 premature babies. The ataxic (atonic-astatic) form accounts for up to 9.2% of all cases of cerebral palsy, yielding to spastic diplegia (69.3%), hemiparetic form (16.3%). Cerebral palsy is considered the main cause of children’s neurological disability in the world, it requires large resources for lifelong rehabilitation and socialization of patients.
Ataxic cerebral palsy is a polyethological disease: in modern neurology, more than 400 stress factors that disrupt the normal formation of the nervous system in the fetus are known. The leading cause is considered to be damage to brain tissues in different periods of ontogenesis, manifested by polymorphic neurological symptoms. Predisposing factors of damage to the structures of the central nervous system are grouped into the following groups:
- Extragenital diseases of the mother. The probability of having a child with cerebral palsy increases if a pregnant woman has chronic cardiovascular (arterial hypertension, heart defects, anemia), endocrine pathologies (diabetes mellitus, thyroid dysfunction, obesity).
- Genetic anomalies. The ataxic form of the disease is characterized by a connection with undifferentiated genetic syndromes that appear sporadically or occur with a family predisposition.
- Teratogenic factors. Intrauterine viral infections, exposure to chemical toxins, and an unfavorable environmental situation have a negative impact on the formation of the central nervous system. Alcoholism of parents and occupational hazards are of great importance in the mechanism of development of ataxic paralysis.
- Pathology of pregnancy. Among the risk factors are disorders of placental circulation, uterine bleeding, presentation or placental abruption. All these prerequisites provoke intrauterine fetal hypoxia, which causes organic cerebral damage.
- Prematurity. Ataxic paralysis is diagnosed 5-10 times more often in prematurely born infants. The degree of risk also depends on body weight: in infants weighing 1.5-2.5 kg, cerebral palsy is detected in 5-15% of cases, and with extremely low weight (less than 1.5 kg) — in 25-30%.
- Birth injuries. Until recently, birth asphyxia was considered the main cause of atactic cerebral palsy, but a detailed history study found that 75% of children had other risk factors. Intracranial birth trauma is essential in the development of cerebral palsy.
The pathophysiological basis of the ataxic variant of the disease are disorders of muscle tone, chain-setting straightening reflexes. At the same time, such children retain unconditional posotonic reflexes. The main difference between cerebral palsy and other forms of central paresis is considered to be the time of the lesion — up to 80% of structural and functional disorders of the central nervous system occur antenatally, in the remaining 20% of cases predisposing factors act intranatally.
The type of changes in the nervous tissue depends on the leading cause of the pathology. With chronic fetal hypoxia, the synthesis of nucleic acids is inhibited, the processes of myelination of nerve fibers are disrupted, and the number of neurons decreases. With birth injuries, necrosis of cerebral tissues, intracerebral hemorrhages, circulatory disorders and cerebrospinal fluid dynamics occur. Acidosis, hypoglycemia, and other metabolic shifts also play a role in pathogenesis.
The ataxic variant of cerebral palsy is formed with gross violations of the frontal-pontine-cerebellar pathway, or with intrauterine organic brain disease with predominant involvement of the cerebellum. As a result of muscle dysfunctions, pathological synergies and installations are formed in the joints of the shoulder and pelvic girdle, which then develop into persistent contractures.
The ataxic type of the disease manifests itself with muscle hypotension, which makes children look sluggish, their voluntary movements are impoverished, there is often a weak cry, insufficiently active sucking of the mother’s breast. Against the background of a decrease in muscle tone, pathological installations of the forearms and hands gradually appear, the tension of the adductor muscles of the thighs and the muscles of the feet increases.
Tendon and periosteal reflexes in children suffering from ataxic paralysis are increased, and installation reflexes begin to form by 1.5-3 years. Labyrinthine, cervical tonic reflexes persist for a long time. Cerebellar disorders are manifested by tremor of the hands, especially with purposeful movements, instability in the standing position, shaky uncertain gait. Articulation also suffers in children.
When the frontal-bridge-cerebellar pathway is totally affected, gross disorders of psychomotor indicators are noted: newborns practically do not develop the functions of sitting, standing, walking. 15-20% of patients have convulsive and hypertensive syndrome. Children develop a severe delay in mental and speech development: the intelligence coefficient ranges from 20 to 50 units, which corresponds to an average degree of mental retardation.
The most serious consequences of ataxic cerebral palsy are gross cognitive disorders (more than 60% of patients), episyndrome (35%), neurotic symptoms (65%). More than 80% of children face asthenic syndrome, 70% of patients have vegetative dysfunctions. Concomitant damage to the cranial nerves is manifested by strabismus, hearing loss, pseudobulbar syndrome.
Over time, orthopedic pathologies increase in patients with atonic-asthenic cerebral palsy: deformities of the feet (equine, valgus, varus), contractures of the joints of the extremities. Severe motor deficiency limits self-service, labor activity. More severe violations are determined in those suffering from total organic damage to the structures of the frontal-pontine-cerebellar pathway.
During the initial examination of the patient, a child neurologist reveals a delay in psychorechological development, motor disorders — muscle hypotension, disorders of posture and voluntary movements, pathological contractures. To make a diagnosis, detailed information about the course of pregnancy, childbirth, and the neonatal period is required. To confirm the diagnosis of ataxic cerebral palsy, a set of studies is used, which includes:
- Electrophysiological diagnostics. To assess neuromuscular transmission, electroneuromyography and the study of evoked potentials are performed. In case of convulsive syndrome, electroencephalography, transcranial magnetic stimulation is necessary.
- Methods of neuroimaging. In young children, neurosonography is used, CT or MRI is prescribed for a more informative study of brain structures. According to the results of studies, congenital defects of the central nervous system, foci of hemorrhages or ischemia, signs of periventricular leukomalacia may be detected.
- Consultation of a geneticist. The ataxic form is similar in clinical symptoms to many hereditary pathologies, therefore, a detailed examination of the child in a genetic center is necessary, according to indications — specific biochemical, molecular genetic analyses.
Drug treatment for ataxic paralysis is limited, since no drugs have yet been developed to prevent or reduce the signs of the disease. Drugs are prescribed to eliminate complications, slow down the progression of cerebral palsy. All patients are shown vitamin and mineral complexes that have a restorative effect. Differentiated therapy, selected by a pediatric neurologist individually for a child, includes:
- neurometabolic drugs;
- means to improve cerebral hemodynamics;
- anticholinesterase drugs.
To eliminate contractures, restore mobility in the limbs, the help of pediatric orthopedists is required. Various orthoses are used to give the body a physiological position: tires, longettes, collars and rollers. In complicated forms of damage to the musculoskeletal system, orthopedic operations are recommended to stabilize the posture, correct dislocations, and eliminate deformities of the limbs.
Helping patients with ataxic cerebral palsy requires the participation of a multidisciplinary team of specialists, which includes pediatric neurologists, orthopedists, speech therapists, specialists in physiotherapy and physical therapy. Rehabilitation activities should preferably begin as early as possible, ideally before the 4-month age of the baby, and be carried out continuously throughout life. The integrated approach includes the following areas:
- Physical therapy. Special complexes of gymnastics, hardware kinesiotherapy, robotic mechanotherapy are aimed at strengthening the muscular corset, training coordination of movements, increasing the overall mobility of the patient.
- Massage. In children with signs of cerebral palsy, several types of massage are used for thorough study of skeletal muscles: segmental, periosteal, circular trophic, point.
- Voita-therapy. The method of reflex locomotion is aimed at the formation of motor skills based on simple physiological actions (crawling, turning over).
- Physical therapy. Methods of electromyostimulation, electrophoresis, acupuncture and magnetotherapy are widely used. Hydrokinesotherapy, mud treatment, paraffin applications show a good effect.
- Psychological and pedagogical correction. Children are assigned classes with a speech pathologist, sensory education, A. Peto’s method of coductive pedagogy or the Montessori system.
Prognosis and prevention
With an isolated lesion of the cerebellum, the prognosis is favorable, children have a sufficient level of motor development and social adaptation. Such patients, with proper rehabilitation, retain self-service skills, partial ability to work. Involvement of the entire cortical-bridge-cerebellar pathway in the process is characterized by an unfavorable prognosis, since patients have severe disabilities.
The complex of prevention of cerebral palsy includes the preservation of reproductive health of the population, careful preparation of the couple for conception, prevention of teratogenic effects on the fetus during pregnancy. An important role is played by the correct management of childbirth, the exclusion of obstetric injuries. Special attention is paid to pregnant women with extragenital pathologies or already having children with congenital cerebral anomalies.