What is it ?
Little’s syndrome is a synonym for infantile spastic diplegia.
Infantile spastic diplegia is the best known cerebral palsy. It is characterized by a muscular stiffness in the affected subject, particularly in the legs and in less importance in the arms and face. Hyperactivity in the tendons of the legs is also visible in this pathology.
This muscle stiffness in the affected person’s legs results in a discrepancy in the movements of the legs and arms.
In children with Little Syndrome, language and intelligence are usually normal.
This cerebral diplegia is usually very early in infants or young children.
Subjects with this condition have an increase in muscle tone resulting in muscle spasticity. This phenomenon is a high muscle tone and permanent resting muscles. Exaggerated reflexes are often the translation. This muscular spasticity particularly affects the muscles of the legs. The muscles of the arms, for their part, are less affected or not affected.Other signs may be significant of the disease. This is the case, for example, of a walk on the toes or a jarring walk.
These abnormalities in muscle tone are the result of disturbances in brain neurons or their abnormal development.
The exact cause of this neurological disorder is still little known. Nevertheless, some researchers have hypothesized a relationship with genetic mutations, congenital malformations of the brain, infections or fevers in the mother during pregnancy or even accidents during childbirth or very soon after the birth. birth.
To date, there are no curative treatments for the disease. In addition, drug alternatives exist depending on the signs, symptoms and severity of the disease.
Symptoms of Little Syndrome
Different forms of severity of the disease exist.
The symptoms of Little’s syndrome are therefore different from one patient to another.
In the context of cerebral palsy due to neurological abnormalities, symtoms appear very early in childhood. Associated clinical signs are muscular affections (particularly in the legs) disrupting muscle control and coordination.
The child with this pathology has a muscle tone greater than normal and exaggerated reflexes (consequence of the development of spasticity).
Other signs may also be signs of development of a spastic diplegia infantile. Including signs showing a delay in the motor skills of the child, walking on the toes, asymmetrical walking, etc.
In rare cases, these symptoms change during the life of the person. Nevertheless, generally these do not evolve in a negative way.
Added to these symptoms of motor skills, other abnormalities may be related to the disease in some cases:
– intellectual disability;
– learning difficulties;
– growth retardation;
– abnormalities in the spine;
– osteoarthritis (or osteoarthritis);
– an impaired vision;
– a loss of hearing;
– language difficulties;
– loss of urinary control;
– muscle contractures.
The origins of the disease
Infant spastic diplegia (or Little’s syndrome) is a cerebral palsy caused by an abnormal development of a part of the brain controlling motor function.
This deficiency in brain development can be caused before, during or very soon after birth.
In the majority of cases, the exact cause of the development of the pathology is unknown.
Hypotheses have, however, been made, such as:
– genetic abnormalities;
– congenital malformations in the brain;
– the presence of infections or fevers in the mother;
Other origins of the disease have also been highlighted:
– intracranial bleeding which can disrupt the normal circulation of blood in the brain or cause rupture of blood vessels. These bleeds are usually due to a fetal shock or to the formation of a blood clot within the placenta. Hypertension or the development of infections in the mother during pregnancy may also be the cause;
– Depletion of oxygen in the brain, resulting in cerebral asphyxia. This phenomenon usually occurs after a very stressful delivery. An interrupted or reduced oxygen supply therefore causes significant damage for the child: it is an ischemic hypoxic encephalopathy (HIE). The latter is defined by the destruction of the brain tissue. Unlike the previous phenomenon, ischemic hypoxic encephalopathy may be the result of hypotension in the mother. A rupture of the uterus, detachment of the placenta, abnormalities impacting the umbilical cord or head trauma at delivery can also be the cause;
– an abnormality in the white part of the cerebral cortex (part of the brain responsible for transmitting signals from the brain to the whole body) is also an additional source of disease development;
– abnormal development of the brain as a consequence of an interruption in the normal process of its development. This phenomenon is linked to mutations in the genes coding for the formation of the cerebral cortex. Infections, the presence of repeated fevers, trauma or unhealthy lifestyle during pregnancy can be an additional risk of abnormal brain development.
Risk factors for Little Syndrome
The risk factors for developing Little’s syndrome are mainly:
– abnormalities in certain genes that would be predisposing;
– congenital malformations in the brain;
– the development of infections and high fever in the mother;
– intracranial lesions;
– a depletion of oxygen at the cerebral level;
– developmental abnormalities of the cerebral cortex.
Additional medical conditions may be associated with an increased risk of developing cerebral palsy in children:
– a slight weight at birth;
– infections or high fevers during pregnancy;
– multiple pregnancies (twins, triplets, etc.);
– blood incompatibilities between the mother and the child;
– thyroid abnormalities, intellectual disability, excess protein in the urine or convulsions in the mother;
– a delivery in a seat;
– complications during childbirth;
– a low Apgar index (index of the state of health of the infant from birth);
– jaundice of the newborn.
Prevention and treatment of Little Syndrome
Diagnosis of infantile spastic diplegia should be done rather soon after the birth of the child for the well-being of the child and his family.
Surveillance of the disease very closely also needs to be done. This involves developing a monitoring of the child during his growth and development. If this follow-up of the child proves worrying results, a test of the development is possible.
This screening for the development of the child results in tests assessing any delays in the development of the child, such as delays in motor skills or movements.
In the event that the results of this second phase of diagnosis are found to be significant, the physician can then continue the diagnosis towards medical evaluations of the development.
The purpose of the medical diagnostic phase of development is to highlight specific abnormalities in the child’s development.
This medical diagnosis gathers certain tests of recognition of the anomalies specific to the disease, it is about:
– the blood analysis;
– the cranial scanner;
– the MRI of the head;
– the electroencephalogram (EEG);
In terms of treatment, there is currently no cure for the disease.
However, treatments can improve the living conditions of patients. These treatments must be prescribed at the earliest after the diagnosis of the disease.
The most common treatments are medications, surgeries, splints, and physical therapy (physiotherapy) and language therapy (speech therapy).
School help can also be offered to people with this syndrome.
The vital prognosis of patients with this disease varies enormously depending on the signs and symptoms present in the person.
Indeed, some subjects are affected in a moderate way (no limitation in their movements, independence, etc.) and others more severely (inability to perform certain movements without assistance, etc.).