Little’s syndrome is a synonym for infantile spastic diplegia.

Infantile spastic diplegia is the most well-known cerebral palsy. It is characterized by muscle stiffness in the affected subject, particularly in the legs and to a lesser extent in the arms and face. Hyperactivity in the tendons of the legs is also visible in this pathology.

This muscle stiffness in the legs of the affected person results in a discrepancy in the movements of the legs and arms.

In children with Little’s syndrome, language and intelligence are generally normal. (1)

This cerebral diplegia usually starts very early in infants or young children.

People with this condition have an increase in muscle tone leading to muscle spasticity. This phenomenon is a high and permanent muscle tone of the muscles at rest. Exaggerated reflexes are often the result. This muscle spasticity particularly affects the muscles of the legs. The muscles of the arms, for their part, are less affected or not affected.

Other signs can be significant of the disease. This is the case, for example, of a walk on the toes or a discordant walk.

These abnormalities in muscle tone are the result of disorders in the neurons of the brain or their abnormal development.

Little is known about the exact cause of this neurological disorder. Nevertheless, some researchers have hypothesized a relationship with genetic mutations, congenital malformations of the brain, infections or fevers in the mother during her pregnancy or even accidents during childbirth or very soon after birth. birth. (3)

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. (3)

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, the symptoms appear very early in childhood. The associated clinical signs are muscle disorders (particularly in the legs) which disrupt muscle control and coordination.

The child suffering from this pathology presents a muscle tone higher than normal and exaggerated reflexes (consequence of the development of spasticity).

Other signs may also be signs of developing infantile spastic diplegia. In particular signs showing a delay in the child’s motor skills, walking positioned on the toes, asymmetrical walking, etc.

In rare cases, these symptoms change over the course of a person’s life. However, generally these do not evolve in a negative way. (3)

In addition to these symptoms of motor skills, other abnormalities may be related to the disease in some cases: (3)

– intellectual disability;

– learning difficulties;

– convulsions;

– stunted growth;

– abnormalities in the spine;

– osteoarthritis (or arthritis);

– impaired vision;

– hearing loss;

– language difficulties;

– loss of urinary control;

– muscle contractures.

Infantile spastic diplegia (or Little’s syndrome) is a cerebral palsy caused by abnormal development of a part of the brain that controls motor skills.

 This impairment 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.

However, assumptions have been made, such as: (1)

– genetic abnormalities;

– congenital malformations in the brain;

– the presence of infections or fevers in the mother;

– fetal damage;

— etc.

Other origins of the disease have also been highlighted: (1)

– intracranial bleeding which can disrupt the normal circulation of blood in the brain or cause the rupture of blood vessels. This bleeding is usually due to fetal shock or the formation of a blood clot in the placenta. High blood pressure or the development of infections in the mother during pregnancy may also be the cause;

– oxygen depletion in the brain, leading to cerebral asphyxia. This phenomenon usually occurs after a very stressful childbirth. An interrupted or reduced oxygen supply therefore leads to significant damage to the child: it is a hypoxic ischemic encephalopathy (EHI). The latter is defined by the destruction of brain tissue. Unlike the previous phenomenon, hypoxic ischemic encephalopathy can be the result of hypotension in the mother. A rupture of the uterus, a detachment of the placenta, anomalies impacting the umbilical cord or a head trauma during childbirth 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 cause of the development of the disease;

– abnormal development of the brain, the consequence of an interruption in the normal process of its development. This phenomenon is linked to mutations in the genes encoding the formation of the cerebral cortex. Infections, the presence of repeated fevers, trauma or a poor lifestyle during pregnancy can be an additional risk of abnormal brain development.

The main risk factors for developing Little’s syndrome are: (1)

– abnormalities in the levels of certain genes which are said to be predisposing;

– congenital malformations in the brain;

– the development of infections and high fevers in the mother;

– intracranial lesions;

– oxygen depletion in the brain;

– developmental abnormalities of the cerebral cortex.

Additional medical conditions may be the subject of an increased risk of developing cerebral palsy in children: (3)

– premature birth;

– a light weight at birth;

– infections or high fevers during pregnancy;

– multiple pregnancies (twins, triplets, etc.);

– blood incompatibilities between mother and child;

– abnormalities in the thyroid, intellectual disability, excess protein in the urine or convulsions in the mother;

– a breech birth;

– complications during childbirth;

– a low Apgar index (index of the state of health of the infant from birth);

– jaundice of the newborn.

The diagnosis of infantile spastic diplegia should be made sooner after the birth of the child for the well-being of the child and his family. (4)

Very close disease surveillance should also be carried out. This implies developing monitoring of the child during his growth and development. If this follow-up of the child turns out to have worrying results, a developmental screening test is possible.

This screening concerning the development of the child results in tests evaluating the possible 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 proceed with the diagnosis towards developmental medical evaluations.

The objective of the developmental medical diagnosis phase is to highlight specific abnormalities in the development of the child.

This medical diagnosis includes certain tests for the recognition of abnormalities specific to the disease, they are: (3)

– blood analysis;

– the cranial scanner;

– MRI of the head;

– the electrencephalogram (EEG);

– electromyography.

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 as soon as possible after the diagnosis of the disease.

The most common treatments are drugs, surgery, splinting, and physical (physiotherapy) and language (speech therapy) therapy.

School aids can also be offered to people with this syndrome.

The vital prognosis of patients with this disease varies greatly 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.) (3).