PANDAS is a now-recognized neuropsychiatric syndrome that can be caused by a previous infection with group A beta-hemolytic streptococci in children and adolescents. Symptoms resemble those of obsessive-compulsive disorder or Tourette’s syndrome. Good success in preventing chronicity of the disease has been achieved with antibiotic treatment.
What is PANDAS?
PANDAS is the abbreviation for the English name of the syndrome Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections. This name stands for a neuropsychiatric syndrome that can be triggered by infection with group A beta-hemolytic streptococci in childhood and adolescence. The most significant bacterial species in this group is Streptococcus pyogenes. Streptococcus pyogenes triggers scarlet fever or purulent tonsillitis among others. It has been noted that in a few cases, the neuropsychiatric syndrome PANDAS suddenly breaks out following these infectious diseases. If left untreated, this condition can become chronic. PANDAS syndrome presents with symptoms similar to obsessive-compulsive disorder or tics (Tourette’s syndrome). It is an autoimmunological disorder attributed to a cross-reaction of antibodies attacking the surface of bacteria with specific structures of the brain, the basal ganglia. Group A beta-hemolytic streptococci generally have the property of being able to trigger rheumatic fever and other rheumatic-related diseases after infection. The mechanism of development of these sequelae of infection is similar in all diseases of the rheumatic circle. The antibodies often also attack the body’s own structures, especially collagen IV. Resulting diseases include rheumatic heart inflammation, inflammation of the joints (arthritis), skin inflammation (erythema), muscle inflammation, or chorea minor.
Causes
The cause of the occurrence of PANDAS, as mentioned above, is due to a cross-reaction of the antibody against the bacterial surface with endogenous structures of the brain. These are structures of the basal ganglia. The basal ganglia are responsible for the coordination of movements. If they are damaged, there are disturbances in motor function that correspond to the clinical picture of chorea minor. In contrast to chorea minor, psychiatric symptoms dominate in PANDAS. Imaging studies have found that there are changes in parts of the brain, in the striatum. PANDAS is therefore an autoimmune disease in which the basal ganglia are attacked by the body’s own immune system. According to current knowledge, this disease is triggered by group A beta-hemolytic streptococci. However, PANDAS belongs to a group of syndromes grouped under PANS that show similar symptoms. All disorders in this group are thought to be caused by basal ganglia antibodies, although other infections and noninfectious processes may also play a role in PANS. Only in the subset of PANS, from PANDA, is the specific trigger of the disease antibody to group A beta-hemolytic streptococci.
Symptoms, complaints, and signs
PANDAS syndrome is characterized by disturbances in motor function that may occur suddenly after the completion of an infection such as scarlet fever, purulent tonsillitis, or acute otitis media. The condition begins abruptly with behavioral and thought disturbances or tics. Food intake is often refused. Other symptoms include anxiety, aggression, irritability, depression, retardation in behavioral development in children and adolescents, difficulty concentrating, deterioration in school performance, deterioration in handwriting, sleep disturbances, bed-wetting, memory impairment, and other psychiatric abnormalities. A unique feature of PANDAS is that the combination of all symptoms cannot be better explained by other disorders such as chorea minor, Tourette’s syndrome, or lupus erythematosus. Most importantly, the symptoms must occur following infection with group A beta-hemolytic streptococci.
Diagnosis and course of the disease
The diagnosis of PANDAS is based on the nature of the symptoms associated with the causative infection.Differentially, the symptoms of PANDAS can still be compared with the symptoms of obsessive-compulsive disorder without infectious background (OCD). Thus, in PANDAS, such symptoms as frequent urination, separation anxiety, impulsivity, hyperactivity, poor writing, and deterioration of school performance come to the fore. These characteristics are closely related to the mode of action of the basal ganglia. Finally, imaging techniques provide information about the damaged areas in the brain (striatum).
Complications
In PANDAS, affected individuals primarily suffer from various mental disorders. The symptoms can vary in severity. However, in most cases, there are various motor function disorders and further inflammation in the ears. The affected persons suffer from various behavioral disorders and thought disorders and thus also from delayed development. Therefore, if PANDAS is not treated at a young age, it can lead to significant complications and difficulties in the patient’s adulthood. Affected individuals also continue to suffer from bedwetting and concentration disorders. Sleep disturbances and persistent aggression or irritability may also occur, leading to social discomfort. In many cases of PANDAS, the parents of the children also suffer from severe psychological complaints and depression. School performance may deteriorate as a result of the disease. In many cases, patients are also dependent on the help of those around them in their daily lives. The treatment of PANDAS can be carried out with the help of medication. However, psychological treatments are also necessary to completely limit the symptoms. In most cases, the life expectancy of patients is not affected by the disease.
When should one go to the doctor?
If children or adolescents show disturbances in motor function during their developmental process, there is cause for concern. The symptoms appear suddenly after an infectious disease has been cured and place a heavy burden on the child’s health. In case of earache, a noise in the ear, fever, general malaise and lack of interest, a doctor should be consulted. If behavioral abnormalities, personality peculiarities, or disturbances in thinking performance are evident, the observations should be discussed with a physician. If the mental performance level drops, there is nocturnal wetting or tics develop, the child needs medical help. Compulsive actions, an aggressive demeanor, easy excitability, and inner restlessness are signs of impaired health. If the general condition deteriorates, the child forgets already acquired skills or appears very stressed, a doctor should be consulted. Characteristic of the disease is the deterioration of the child’s handwriting. If this is noticed, consultation with a physician should be sought immediately. Loss of appetite, a withdrawal or refusal to engage in playful activities are considered unusual. If this development persists, it is advisable to seek medical attention. An anxious demeanor, decreased self-confidence, and sleep disturbances should also be presented to a doctor. If the child loses an independence that has already been achieved, this should be understood as an alarming signal. A visit to the doctor is recommended as soon as possible.
Treatment and therapy
PANDAS is a syndrome that is currently still in the research stage. Therefore, there are no strategies for causative treatment yet. Currently, mainly symptomatic treatment is taking place. Thus, behavioral therapies are offered to treat behavioral structures. At the same time, drug therapies with psychotropic drugs are also used. However, it is questionable whether these therapies can prevent a chronification of the disease. Hopeful approaches for a curative therapy are emerging in the context of studies where, with the help of antibiotic treatment, a far-reaching improvement and suppression of the symptoms was achieved. These therapies are based on the assumption that by fighting the group A beta-hemolytic streptococci, the antibody effect against the body’s own structures is also reduced. As long as there are no irreversible changes in the brain, the disease can subsequently heal completely.
Outlook and prognosis
The acronym “PANDAS” stands for “Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections.” The prognosis for this is currently poor. Neuropsychiatric symptoms often occur after a streptococcal infection. The onset of symptoms is abrupt. If these remain untreated, and the streptococcal infection is not recognized as a type A infection, chronic PANDAS progresses. Experts believe that this type of streptococcus causes changes in the brains of those affected. Meanwhile, medical professionals aim to treat any streptococcal disease as early and comprehensively as possible to avoid such complications. PANDAS is characterized by sudden and drastic behavioral changes, obsessive-compulsive disorders and motor disturbances. Adults with such behaviors are thought to potentially suffer from the lifelong consequences of PANDAS syndrome left undetected and untreated. In most cases, the dramatic consequences of streptococcal infection remain for life. The problem is that, apart from preventive treatment of the disease, there are still too few treatment options for the PANDAS syndrome that has already developed. This is still under investigation. In the past, the association with surviving streptococcal infection was not recognized. Therefore, there was no preventive or symptomatic treatment approach at all that was effective. It may be possible to improve the prognosis of PANDAS sufferers in the future. Research into a causative intravenous immunoglobulin therapy is nearing completion.
Prevention
The best prevention of PANDAS is prevention of infection with beta-hemolytic streptococci. These infections are contagious. The main route of transmission of these diseases is droplet infection via contact with the mouth and throat or through the air. Thus, transmission of the germs can be limited during times of increased risk of infection by avoiding large gatherings of people. In the event of an outbreak of the disease, immediate antibiotic treatment is necessary. The usual hygiene measures must be strictly observed.
Aftercare
In most cases, the affected person has few or even limited measures and options for direct aftercare in the case of PANDAS. In the first place, the affected person should see a doctor very early in the course of this disease to avoid complications or other complaints in the further course, so that a doctor should be contacted at the first signs and symptoms of the disease. It is also not possible for the disease to heal itself. The treatment of PANDAS is usually carried out with the help of various medications. The affected person should always pay attention to a correct dosage and also to a regular intake, in order to alleviate the complaints permanently and correctly. When taking antibiotics, it should also be noted that they should not be taken together with alcohol, so as not to weaken their effect. Also in case of questions or side effects, a doctor should be consulted first. In general, the person affected by PANDAS should prevent contact with other people, so that no infections occur among fellow human beings. The further course is strongly dependent on the time of diagnosis, so that no general prediction can be made in this case.
What you can do yourself
People who have PANDAS should take it easy and otherwise follow the doctor’s instructions regarding wound care, personal hygiene and taking medication. The disease usually resolves relatively quickly, provided it is well cured and the patient is not exposed to further physical exertion. Patients should contact their doctor if, despite everything, complications arise or unusual symptoms appear that were not noticed before. In addition, general measures apply, such as drinking plenty of fluids and eating a gentle diet. Fever and chills indicate that the infection is spreading and should therefore be clarified by a doctor. Parents of affected children should also ensure that the child does not have contact with other children, as there is a relatively high risk of infection in PANDAS. In addition, PANDAS requires close medical monitoring.If the child is already very weak, emergency medical services should be contacted if the physical or mental condition continues to decline. If in doubt, the child must be taken to the hospital for treatment. The physician can provide additional tips for effective self-help measures for PANDAS.
