Pediatric Acute-onset Neuropsychiatric Syndrome: Causes, Symptoms & Treatment

Pediatric acute-onset neuropsychiatric syndrome

is defined as a neuropsychiatric syndrome. It is composed of numerous different symptoms.

What is pediatric acute-onset neuropsychiatric syndrome?

Pediatric Acute-onset Neuropsychiatric Syndrome is also known as PANS for short. It refers to a neuropsychiatric disorder that has a sudden onset. It first appears in childhood or adolescence. It is characterized by compulsive behavioral abnormalities and thought disorders. Likewise, tics as well as physical-neurological abnormalities are among the features of the syndrome. Pediatric Acute-onset Neuropsychiatric Syndrome was discovered during PANDAS research, which focused on the neuropsychiatric syndrome “Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections” (PANDAS). The research took place at the U.S. NIMH Institute in Bethesda, Maryland. NIMH stands for “National Institute for Mental Heath” and is considered the world’s largest institute for research into mental illness. It represents a sub-agency of the U.S. Department of Health and Human Services. Research into PANS is still ongoing and focuses on the syndrome’s development, treatment and prognosis.

Causes

To date, it has not been possible to elucidate the exact pathophysiologic causes of Pediatric Acute-onset Neuropsychiatric Syndrome. It is suspected in some cases that PANS is the result of infections with viruses or bacteria. Suspected bacteria include mycoplasma, streptococcus and borrelia. However, genetic alterations or metabolic disorders are also suspected to be responsible for PANS. For example, alterations in metabolites such as kynorenin and inflammatory markers such as TNF-a and neopterin have been described. Some scientists assume that certain brain structures are affected by immunological processes. This is especially true for the basal ganglia region. Researchers consider the neurological disease chorea minor as a prototypical pathogenic mechanism. However, in Pediatric Acute-onset Neuropsychiatric Syndrome, psychiatric symptoms are dominant. The syndrome is responsible for thought disorders, obsessive-compulsive symptoms, motor disorders, and behavioral changes. In addition, PANS may also be the trigger of tic and obsessive-compulsive disorders in adults.

Symptoms, complaints, and signs

Typically, Pediatric Acute-onset Neuropsychiatric Syndrome presents abruptly in children and adolescents. In this case, the symptoms correspond to basal ganglia dysfunction. In addition, many different complaints may present. These include aggression, rebellious behavior, irritability, anxiety, depression, regression in behavioral development, and deterioration in school or work performance. Furthermore, sleep problems may occur in which REM sleep or day-night rhythms are disturbed. Furthermore, motor or sensory abnormalities are noted, which include, for example, deterioration of handwriting. Sometimes somatic complaints such as bedwetting (enuresis) and an altered frequency of urination are observed. Neurological-psychiatric abnormalities such as memory disorders, cognitive deficits or separation anxiety are also possible. It is not uncommon for patients to suffer from tics or obsessive thoughts and to refuse to eat food.

Diagnosis and course of the disease

If Pediatric Acute-onset Neuropsychiatric Syndrome is suspected, a medical examination is necessary. The physician makes the diagnosis by looking at the symptoms that occur in PANS. Tics, thought disorders, compulsive behavior, and refusal to eat are considered characteristic. In addition, the disorder must occur in a child or adolescent. Another criterion is a recent infection. Furthermore, the symptoms run in episodes. To confirm the suspicion of PANS, at least two of the leading symptoms must occur in the patient. Another diagnostic criterion is that the symptoms cannot be explained by other diseases such as Tourette’s syndrome, chorea minor or lupus erythematosus.In adult individuals, a structurally analogous syndrome exists called PIBS (Post-inflammatory Brain Syndrome). Basically, the diagnostic criteria of PANS and PANDAS are very similar. However, Pediatric Acute-onset Neuropsychiatric Syndrome does not necessarily arise from streptococcal infection. PANS usually reaches its manifestation in children and adolescents. The disease may persist into adulthood. While some cases take an acute course, chronicity must be expected in most patients. In varying degrees of severity, PANS then persists throughout life.

Complications

In this disorder, patients suffer from a number of different complaints. As a rule, patients appear aggressive and permanently irritable. Since the disease already appears in children and adolescents, serious social discomfort and problems with the child’s development can occur in the process. Depression and other psychological upsets also often occur. The patients themselves suffer from confusion and often anxiety. Professional or school performance can also suffer from this disease. Furthermore, patients often continue to suffer from severe psychological symptoms even in adulthood. Due to the sleep disturbances, aggressive behavior is usually additionally favored. Bedwetting in adolescence may also occur. Furthermore, the disease leads to memory disorders or eating disorders. Also the parents and the relatives often suffer from psychological complaints or also from depressions with this illness. The quality of life is considerably reduced. The treatment of this disease depends on the cause. Although special complications do not occur, a complete cure cannot be guaranteed.

When should you see a doctor?

Because Pediatric Acute-onset Neuropsychiatric Syndrome is a congenital disorder that does not go away on its own, it must always be examined and treated by a doctor. Only medical treatment can limit the symptoms to such an extent that the affected person can lead an ordinary life. The doctor should be consulted if the affected child suffers from depression, anxiety or irritability at a young age. It can also cause severe sleep problems, which have a very negative effect on school performance. Often the children are aggressive or suffer from memory disorders due to Pediatric Acute-onset Neuropsychiatric Syndrome, so that the child’s development itself is also significantly slowed down and hindered. A doctor should also be consulted if the affected person suffers from tics or obsessive thoughts. Pediatric Acute-onset Neuropsychiatric Syndrome can be diagnosed and treated by a psychologist. The earlier the diagnosis of the condition occurs, the higher the chance of a complete recovery. Usually, the patient’s life expectancy is not negatively affected by Pediatric Acute-onset Neuropsychiatric Syndrome.

Treatment and therapy

There is no generally accepted treatment for the causes of Pediatric Acute-onset Neuropsychiatric Syndrome. The reason for this is that both PANS and its subgroups PANDAS and PITANDS are still in the research phase. For this reason, treatment of the syndrome is limited to symptoms. Antibiotic and immune-based treatment options are mentioned by the NIMH Institute as possible therapeutic options. If bacteria are the trigger of Pediatric Acute-onset Neuropsychiatric Syndrome, antibiotics can be administered as a preventive measure to counteract further episodes. However, antibiotics are not yet routinely administered because the relevant research has not yet been completed. Thus, only the study results of the PANDAS subgroup are available. To prevent the condition from becoming chronic, most physicians recommend starting treatment for PANS as early as possible. In Europe, Pediatric Acute-onset Neuropsychiatric Syndrome has been included in a guideline on Tourette syndrome and other tic disorders since 2011. The German Society for Child and Adolescent Psychiatry also emphasizes the need for treatment of PANS in children with recent onset of obsessive-compulsive disorder.

Outlook and prognosis

The rapid onset of pediatric acute-onset neuropsychiatric syndrome (PANS or PANDAS) affects children. It is a systemic neurologic disorder that progresses chronically. It may be triggered, for example, as a result of surviving an infection with streptococcus. The syndrome can also occur with fluctuating symptoms. However, it is always a lifelong disease. This affects the possibilities for a cure. This sudden-onset disease is accompanied by numerous neurological and psychological disorders. Nowadays, there are neuropsychiatric and immunological treatment approaches for Pediatric Acute-onset Neuropsychiatric Syndrome. Antibiotics are used to nip infections in the bud, or to treat them preventively. Researchers are still exploring this condition, its causative agents, and potential treatment approaches. Therefore, the prognosis for Pediatric Acute-onset Neuropsychiatric Syndrome cannot be adequately assessed at this time. It could be improved in the future. The earlier Pediatric Acute-onset Neuropsychiatric Syndrome is diagnosed, and its causative agents are identified, the better the prognosis. In some patients, treatment works well. They recover. However, if the causative agent is not known and treatment is not effective, the prognosis is worse. Provided further infections can be suppressed or prevented, the chronic course of Pediatric Acute-onset Neuropsychiatric Syndrome can often be slowed.

Prevention

To date, there are no recommended preventive measures against Pediatric Acute-onset Neuropsychiatric Syndrome. Thus, the exact causes of the syndrome are still unclear.

Follow-up

In most cases, those affected by Pediatric Acute-onset Neuropsychiatric Syndrome have only a few or limited aftercare measures available to them. Yet, affected individuals should seek medical attention for this condition at a very early stage to prevent other complications or medical conditions from developing. The earlier the diagnosis is made, the better the further course of the disease usually is. During the acute treatment and often for a long time afterwards, those affected are dependent on taking various medications in order to alleviate the symptoms of the disease permanently and, above all, correctly. The doctor’s instructions must always be followed, and those affected should also contact a doctor first if they have any questions or if they experience severe side effects. If the syndrome is treated with the help of antibiotics, alcohol must not be taken during the treatment. Children suffering from Pediatric Acute-onset Neuropsychiatric Syndrome must be particularly well supported and encouraged by their parents. Intensive support at school is particularly important in this regard, so that complications do not arise in adulthood.

What you can do yourself

In addition to medicinal measures for the many symptoms, it is also possible to contribute to an improvement in the quality of life on your own. In the first place here is the avoidance of any physical exertion, since the body is greatly weakened by the severe immune reaction. In addition, it is important to clarify any other symptoms with the doctor so that the therapy can be adjusted and intensified at an early stage in case of doubt. In particular, the appearance of fever or chills are clear signs of deterioration and require immediate treatment. In order to support the body in its self-healing process, it is also advisable to initially change the diet to a light diet and to consume sufficient fluids. Parents should pay particular attention to increasing the amount of fluids consumed by sick children, as they tend to dehydrate more quickly than adults. In the acute phase of the disease, contact with children or immunocompromised persons in particular must be avoided if possible due to the high risk of infection. However, even persons who do not belong to a risk group can easily become infected and should ensure appropriate hygiene. If symptoms such as behavioral problems remain after the disease has subsided, they can be treated successfully by behavioral therapy. Here, one’s own behavior patterns are analyzed, possible problems are uncovered and a change in thinking is promoted.