PANDAS syndrome is a neuropsychic pathology of an autoimmune nature that develops in children due to BHSA. The symptom complex of the disease is extremely diverse – from sudden anxiety and neurosis-like obsessive states to hyperkinesis and involuntary shouting of words. The diagnosis is made on the basis of a psychiatric examination and laboratory confirmation of the presence of streptococcal infection. Treatment includes antibiotics, intravenous administration of immunoglobulin, psychotropic medications. Sometimes the symptoms completely regress without therapy.
ICD 10
F06.8 Other specified mental disorders caused by brain damage and dysfunction or somatic disease
General information
PANDAS is an English abbreviation in which P means pediatric, A – autoimmune, N – neuropsychiatric, D – disorder, A – associated, S – with streptococcal infection. The term was first used in 1998 by the American pediatrician S. Shvedo and his colleagues. According to some sources, the disease occurs in one child out of 200. The true prevalence of the condition is unknown. Prepubescent children (from 7 to 12 years old) usually suffer. Boys are more likely to get sick, the ratio with girls is 2.6-4.7:1.
Causes
It is assumed that the occurrence of pathology is associated with a previous infection caused by beta-hemolytic streptococcus group A (BHSA), for example, acute angina, scarlet fever or chronic tonsillitis. Due to the structural features of the cell wall, the bacterium is able to trigger autoimmune reactions, namely, the production of autoantibodies that attack the body’s own cells and tissues, including brain cells.
By itself, the presence of streptococcal bacteria in the human body does not ensure the development of the disease, much depends on the immunogenetic predisposition. The syndrome occurs only in individuals whose blood contains specific alloantigens D8/17 on B-lymphocytes. The same marker is found in patients with another autoimmune pathology associated with BHSA – acute rheumatic fever.
Pathogenesis
The pathological reactions of PANDAS syndrome are based on immune autoaggression. Along with other pathogenic properties (virulence, production of toxins), BHSA has the phenomenon of molecular mimicry. The structure of the bacterial wall of streptococcus is very similar to the structure of the membranes of some cells of the central nervous system.
Antibodies developed against BHSA, in addition to the pathogenic pathogen, attack the cells of the basal ganglia located in the caudate nucleus, pale ball, striatum, etc. These subcortical structures of the brain regulate motor (coordination of movements, tone of skeletal muscles) and vegetative (control over the frequency of breathing and heartbeat, activity of the gastrointestinal tract) functions.
Basal ganglia are directly involved in the processes of higher nervous activity – memory, emotional reactions, information processing speed. The defeat of these parts of the nervous system determines the main clinical picture of PANDAS syndrome. According to most experts, the penetration of autoantibodies into brain tissue is associated with high permeability of the blood-brain barrier in children.
Symptoms
The disease occurs and proceeds acutely, which distinguishes it from most neuropsychic disorders of childhood of a different etiology. The clinical picture varies significantly. The child becomes restless, irritable, emotionally unstable, his mood changes sharply and often, concentration of attention is lost, memorization of new information and learning ability deteriorates.
The most typical obsessive-compulsive states are: repetitive actions (blinking, tilting the head to the side, shrugging shoulders), the desire to perform motor rituals (high lifting of the leg when descending the stairs, repeatedly tying and untying shoelaces on shoes). Falling asleep is disturbed.
Unreasonable anxiety or fear is characteristic. Spontaneous uncontrolled shouting of words or repetitive clicking, smacking, involuntary muscle twitching, tics, sweeping irregular movements of the limbs or head (hyperkinesis) are often found. Possible paroxysmal frequent sneezing, hiccups, sniffling for several hours.
A distinctive feature of PANDAS syndrome is the spontaneous disappearance of symptoms during sleep. The average duration of the attack is 10-12 weeks, after which recovery occurs. Sometimes there is a tendency to a wave-like flow, especially often this is observed in chronic tonsillitis, the exacerbation of which leads to a relapse of neuropsychiatric manifestations.
Complications
Specific complications that pose a threat to the patient’s life are extremely rare in PANDAS syndrome. Clinical symptoms of the disease significantly worsen the quality of life of the child. Due to the lag in their studies, children are sometimes left for the second year or temporarily transferred to home schooling. The disease has a particularly strong effect on socialization in society.
Some disorders (severe anxiety, compulsions) can become the basis for the development of neurotic or mental disorders. Involuntary movements or increased motor activity in some cases lead to injuries. Suicidal tendencies or excessively aggressive behavior are possible, in which the patient becomes dangerous to himself and others, which requires isolation of the patient and constant monitoring.
Diagnostics
Children with PANDAS syndrome are supervised by pediatricians, child psychiatrists and neurologists. Diagnosis is difficult due to the diversity of the clinical picture. Take into account the age (7-12 years), the presence of signs of streptococcal infection (hyperemia of the pharynx, plaque on the tonsils, enlargement and soreness of the cervical lymph nodes) or an indication of a sore throat. To clarify the diagnosis, an additional examination is prescribed, including:
- Blood test. In the general analysis, there may be signs of inflammation – leukocytosis, an increase in ESR. Biochemical analysis reveals serological markers of streptococcal infection – elevated titers of antistreptolysin-O (ASLO), anti-DNase B and rheumatoid factor (RF).
- Immunological and bacteriological studies. To confirm the presence of BHSA in the body, immunochromatographic rapid diagnostics (streptatest) and bacterial culture of a smear from the pharynx and tonsils are used.
- EEG. During a 12-hour video EEG monitoring, an electroencephalogram shows increased convulsive activity, spike waves during a night’s sleep.
Determination of specific antibodies to basal ganglia in the blood significantly increases the reliability of the diagnosis, however, this analysis is practically not used in clinical practice. Differential diagnosis is carried out with Sydenham’s chorea, Tourette’s syndrome, temporal lobe epilepsy, etc. The following criteria of PANDAS syndrome are taken into account:
- the presence of obsessive-compulsive disorders, tics;
- debut at prepubescent age;
- acute onset and paroxysmal course;
- connection with BHSA infection;
- other neurological disorders.
Treatment
Depending on the severity of symptoms and mental status, patients can be treated on an outpatient basis or in a hospital. With pronounced behavioral disorders, round-the-clock monitoring is necessary. Some children have spontaneous regression of disorders. Special attention is paid to psychotherapy: individual conversations, training in special techniques for controlling tics and obsessive movements.
Sleep disorders are corrected by non-drug methods – a strict regime of waking up and going to bed, the complete exclusion of the use of caffeine and caffeinated beverages (cola, “energy”), the distribution of activity during the day with the inclusion of physical exercises. The following medications are used:
- Etiotropic therapy. Since the main cause of PANDAS syndrome is BHSA infection, antibacterial agents are prescribed to all patients for its eradication – phenoxymethylpenicillin, amoxicillin.
- Pathogenetic therapy. Immunomodulatory drugs are used to suppress autoimmune damage to the basal ganglia. In mild cases, taking NSAIDs is sufficient. In case of maladaptation, glucocorticosteroids or immunoglobulin are used. In extremely severe mental disorders, plasmapheresis or strong immunosuppressants (mycophenolate) are indicated.
- Symptomatic therapy. To eliminate the symptoms of OCD, SSRI inhibitors are effective antidepressants. With increased anxiety, benzodiazepines or atypical antipsychotics are recommended.
Prognosis and prevention
Disease has a favorable prognosis. The life expectancy of patients does not differ from that in the general population. Deaths are casuistry. All clinical signs in the vast majority of cases respond well to treatment, some children may have spontaneous recovery without therapy.
To prevent exacerbation of chronic tonsillitis and subsequent neuropsychic attacks, antibiotic prophylaxis and courses of manual or hardware washing of tonsillar lacunae are carried out. If, despite these measures, relapses continue, surgical removal of the focus of persistent infection (palatine tonsils) – tonsillectomy is recommended.
Literature
- PANDAS-syndrom/ Swedo S.E., Klesling L.// J.Reumatologi — 1994 — №3.
- Pediatric аutoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS): an evolving concept/ Macerollo A., Martino D.// Tremor Other Hyperkinet Mov (NY) – 2013.
- Revised Treatment Guidelines Released for Pediatric Acute Onset Neuropsychiatric Syndrome (PANS/PANDAS) – 2017.