Spastic diplegia (Little’s disease) is the most common variant of cerebral palsy, characterized by spastic tetraparesis, more pronounced in the lower extremities. Along with motor disorders, there is a dysfunction of the cranial nerves (strabismus, pseudobulbar syndrome, hearing loss, facial paresis), speech disorders, in some cases – mild mental retardation. Diagnosis is carried out using EEG, neurosonography, brain MRI, ENMG. Treatment includes pharmacotherapy (nootropics, vascular, neuroprotectors, muscle relaxants), exercise therapy, massage, neuropsychic and speech correction.
ICD 10
G80.1 Spastic diplegia
General information
Spastic diplegia was first described in 1853 by the English obstetrician Little, who observed the disease in newborn children. Little’s disease is the most common clinical form of cerebral palsy, it is detected in half of cases of this pathology. Children with spastic diplegia make up 40-50% of all students in specialized schools. The disease affects newborns with perinatal trauma, premature babies, children who were affected by various adverse factors during intrauterine development. Spastic diplegia is detected in the first year of life, more often in the period from newborn to 6 months. The disease is often combined with hyperkinetic syndrome, which occurs in 20-25% of cases of cerebral palsy.
Causes
Among the causes provoking the development of cerebral palsy, modern neurology considers three factors to be the most significant in the etiology of Little’s disease:
- Prematurity. Premature babies are born as a result of premature birth due to fetoplacental insufficiency, Rh conflict, premature placental abruption, uterine abnormalities. Early childbirth causes diseases of the pregnant woman (heart defects, diabetes mellitus, kidney pathology), gestosis (late toxicosis).
- Birth trauma of a newborn. Traumatization of the child during natural childbirth is possible due to anomalies of labor activity (discoordinated, rapid childbirth), clinically narrow pelvis, pelvic presentation of the fetus. In most cases, complicated childbirth is observed against the background of existing intrauterine pathology.
- Asphyxia of a newborn. The absence of independent breathing at the time of birth is noted when amniotic fluid enters the respiratory tract, intrauterine infections, fetal development abnormalities, immunological incompatibility of mother and fetus. Asphyxia is possible with pneumopathy, including those associated with immaturity of the respiratory system in prematurity.
Pathogenesis
Spastic diplegia is formed as a result of damage to the precentral gyrus, which gives rise to pyramidal pathways that provide motor function. In 30-40%, Little’s disease is combined with damage to the cranial nerves. The development of pathological changes in these cerebral structures occurs mainly by an ischemic-hypoxic mechanism against the background of immaturity of brain tissues due to prematurity. The main pathogenetic link is fetal hypoxia in the prenatal period or during childbirth (asphyxia, birth trauma). The brain is the most sensitive structure of the human body to hypoxia, pathological changes in it develop after a few minutes of oxygen starvation. In more rare cases, spastic diplegia occurs as a result of direct damage to the above-mentioned parts of the brain during intracranial birth trauma.
Classification
According to the severity of clinical symptoms , spastic diplegia is classified into the following forms:
- Light — in the first half of the year, the child develops normally, in the future, spastic paresis is expressed in the lower extremities, movements in the hands are not limited. The patient is able to move without improvised means. Mental and mental development does not suffer.
- Moderate severity — pronounced spasticity in the legs makes movement of the patient possible only with the use of improvised means (crutches, sticks). The cognitive sphere suffers slightly, social adaptation is possible.
- Heavy — noticeable from the first days of life. There is a pronounced tetraparesis with an accent in the legs. Patients cannot move independently. Social adaptation is disrupted.
Symptoms
A typical manifestation of the disease is spastic tetraparesis with a predominant lesion of the lower extremities. Muscle hypertonus, which keeps the limbs bent and brought to the body, is necessary for the fetus during its stay in the uterine cavity. After birth, it gradually weakens, normally disappears by the age of 4-6 months. Pronounced spastic diplegia is characterized by hypertonicity exceeding the age norm, which does not decrease over time. Severe forms are noticeable from birth: during swaddling, children are sedentary, attempts at passive flexion / extension of the limbs meet strong resistance due to sharply increased muscle tone. With mild forms of the disease, the development of the child in the first six months corresponds to age norms, hypertension becomes clearly visible after 6 months of age.
Hypertonus is most pronounced in the flexor muscles of the feet, the adductor muscles of the thigh. The characteristic position of the legs of a sick child is formed: the hips are rotated inward, the knees are connected, the shins are pressed or crossed. The development of the motor sphere is delayed, children begin to walk by the age of 3-4. The typical gait is on socks with the legs bent out. When walking, the knees rub against each other. The upper limbs suffer much less, the tone is not so significantly increased, the movements are preserved.
Pathology of cranial nerves is represented by oculomotor disorders, strabismus, decreased visual acuity, hearing loss, smoothness of nasolabial folds, deviation of the tongue from the midline. Pseudobulbar paralysis is observed due to damage to the caudal group of nerves (lingual, vagus, sublingual). His clinic includes dysarthria, swallowing disorders, hoarseness of voice. Sensory disorders are absent, the function of the pelvic organs is preserved. Speech disorders in the form of delayed speech development, dysarthria are noted in 75-80% of patients.
In a quarter of cases, spastic diplegia occurs with hyperkinesis. Involuntary movements in the form of athetosis, choreoathetosis are localized in the distal parts of the upper extremities, increase with voluntary movements, emotional tension, weaken at rest, are absent in sleep. Mental development suffers less than with other variants of cerebral palsy. Oligophrenia is noted in 20-25% of patients, the severity does not go beyond debility.
Complications
The limitation of limb mobility caused by muscle hypertonicity leads to the formation of joint contractures that aggravate motor disorders. Contractures develop in the knee and ankle joints, and in severe form they can be observed in the joints of the upper extremities. The pathological installation of the legs causes an incorrect load on the vertebral column when walking, the formation of curvature of the spine (lumbar lordosis, scoliosis).
Diagnostics
Spastic diplegia is diagnosed by a pediatric neurologist taking into account the consulting opinions of related specialists (pediatrician, pediatric ophthalmologist, otolaryngologist). Great importance is attached to the presence in the anamnesis of indications for the pathology of the antenatal period, prematurity, birth trauma, asphyxia in childbirth. The list of diagnostic examinations includes:
- Neurological examination. It reveals an increase in muscle tone according to the central type, strengthening of tendon reflexes (especially knee, achilles), clonus of the feet, pyramidal stop signs of Babinsky, Rossolimo, hyperkinesis, pathology of cranial nerves.
- Ophthalmological examination. Diagnoses strabismus, oculomotor disorders. Visometry determines the decrease in visual acuity. Ophthalmoscopy may show signs of optic nerve atrophy.
- Consultation of an otolaryngologist with audiometry. It is necessary to determine the degree of hearing impairment.
- Electroencephalography. Reflects the state of bioelectric activity of the brain. Epileptiform changes are uncharacteristic.
- Electroneuromyography. It is prescribed to differentiate Little’s disease from neuromuscular diseases.
- Neurosonography. With the help of ultrasound, cerebral structures are visualized through an open fontanel. The technique allows to exclude congenital brain abnormalities, hydrocephalus, tumors, cerebral cysts, post-traumatic intracranial hematomas.
- MRI of the brain. It is carried out in diagnostically doubtful cases for children with an already closed fontanel. More accurately than ultrasound, it displays intracranial changes.
- Diagnosis of mental development. At an early age, it is carried out by a neurologist, later by a child psychiatrist. It is aimed at identifying DSD, oligophrenia.
Little’s disease is differentiated from other forms of cerebral palsy, abnormalities of the development of the central nervous system, progressive degenerative lesions of the central nervous system, hereditary diseases, dysmetabolic diseases. As part of the differential diagnosis, a consultation of a geneticist, a study of the biochemical composition of blood, urine may be required.
Treatment
Specific etiopathogenetic therapy of cerebral palsy has not been developed. Medication and rehabilitation treatment aimed at preventing complications, training in self-service skills, and improving social adaptation are being carried out. Medical treatment includes:
- Vascular agents (cinnarizine) — improve cerebral circulation, nutrition of brain tissues.
- Neurometabolites (glycine, pantothenic acid, thiamine, pyridoxine) — provide the substances necessary for the functioning of the central nervous system.
- Muscle relaxants (baclofen) — reduce muscle spasticity.
- Nootropics (piracetam) — activate cognitive functions.
- Botulinum toxin preparations are administered intramuscularly to relieve spastic tension, prevent contractures.
Rehabilitation therapy consists of:
- Physical therapy. It is performed by a physical therapy doctor and independently by the patient’s parents. It includes passive, active movements, classes on special simulators. It is necessary daily to maintain the volume of movements, prevent complications.
- Massage — improves blood supply to the affected muscles. Myofascial massage promotes muscle relaxation.
- Correction of speech disorders. Speech therapy classes on correction of dysarthria, speech therapy massage are conducted.
- Correction of mental disorders. Required in case of oligophrenia. It is carried out by neuropsychological correction, conducting classes with a psychologist, a defectologist, and game therapy. Older children are taught by correctional teachers in specialized schools.
Prognosis and prevention
In relation to the motor sphere, spastic diplegia has a more serious prognosis than the hemiplegic form of cerebral palsy. Only a quarter of patients are able to walk independently, half of patients move with the help of special aids. With severe tetraparesis, movement is possible only in a wheelchair. With mental preservation and minor changes in the muscles of the upper extremities, the social adaptation of patients approaches healthy people. In severe cases, patients require constant care. Proper regular rehabilitation measures, periodic course medication improve the prognosis. The preventive measures of the disease include careful management of pregnancy, prevention of intrauterine pathology, adequate choice of delivery methods, correct management of childbirth.