Pandas syndrome rage

For most children with OCD, symptoms come on gradually. At first a child is often able to hide both his fears and the rituals he develops to calm them. Eventually the fears become too overwhelming, and the rituals too time consuming, to conceal from parents.

But there is another kind of OCD in which full-blown symptoms come on virtually overnight. This happens to some young children after they’ve had an infection, usually strep. It’s thought to be the result of the child’s immune system erroneously attacking brain cells. It may be accompanied by other symptoms, including restricted eating, anger, and depression.

“Parents, with great reluctance, talk about their child being possessed, either by a demon or by aliens,” says Dr. Susan Swedo, a pediatrician who has been studying the syndrome for decades.

What Are PANS and PANDAS?

PANS and PANDAS are conditions that a child can develop following an infection, usually strep. They are diagnosed if a child has sudden, severe OCD and other disturbing symptoms.  

PANS (pediatric acute-onset neuropsychiatric syndrome) is a more general term that does not specify the type of infection thought to trigger the symptoms. It’s called “acute onset” because the behavior changes come on suddenly, reaching full-scale intensity within 24 to 48 hours. It’s a syndrome because there are quite a few other symptoms that appear alongside the intense anxiety.

PANDAS (pediatric autoimmune neuropsychiatric disorder associated with streptococcal infections) is a more specific term used when the triggering infection is strep. PANDAS is a subgroup of PANS. Some 86 percent of acute onset OCD cases are linked to strep.

Children especially at risk are those who have what doctors call “occult” or hidden strep infections — that is, children who can be “carriers” of the infection but don’t get symptoms themselves, and hence don’t get treatment.

PANS cases have also been linked to other infections, including Lyme disease, mononucleosis, mycoplasma (walking pneumonia) and the flu (such as h2N1).

PANS and PANDAS are episodic disorders. Symptoms may disappear for extended periods then reappear, stimulated by a later exposure to strep of some other bacteria or virus. Symptoms may get increasingly severe with multiple recurrences.

Symptoms of PANS and PANDAS include:

• Acute-onset OCD
• Severe separation anxiety
• Handwriting suddenly becomes unrecognizable
• Drawings look like scribbles
• Has trouble eating
• Starts wetting the bed
• Can’t manage the math or reading he could do effortlessly the previous week
• Irritable
• Emotionally erratic
• Panic attacks
• Suicidal thoughts

Controversy Over Causes

While this syndrome has been recognized for decades, the link between the symptoms and an infection has been the subject of intense controversy, with some researchers and clinicians claiming insufficient evidence, and denying children treatment to fight the sometimes elusive infection.

But the medical community has coalesced around a clearer description of the symptoms, their causes, and how they should be treated. This should make it easier for parents to get care for severely affected children.

Dr. Swedo, who headed the pediatrics and developmental neuroscience branch of the National Institutes of Mental Health until she retired in 2019, first identified PANDAS in 1998, and has been in the vanguard of research and advocacy ever since. The larger category, PANS, was added in 2010 to account for cases that could not be linked definitively to strep infections.

There is now broad acceptance that PANDAS is, like rheumatic fever, a misdirected immune response to the strep bacteria, which mimics human heart or brain tissue. The immune system attacks the heart (in the case of rheumatic faver) or brain (in the case of PANDAS), causing this array of mental health symptoms.

What Does PANS Look Like in Children?

“What this is not is garden variety OCD, Tourette’s, ADHD, conduct disorder — anything else,” explains Dr. Swedo. “We’re talking about a six-year-old, seven-year-old, maybe as young as three or four, who undergoes an acute behavioral change, a very, very dramatic onset. ”

Acute onset OCD might take the form of a child suddenly asking for constant reassurance, or engaging in endless handwashing. Many children with PANS become obsessive about food, severely restricting what they will eat because of acute fears of contamination, choking or vomiting. This can develop into anorexia, and can result in extreme weight loss, necessitating hospitalization and the use of a feeding tube.

Separation anxiety can also appear suddenly, sometimes so overwhelming that a 10-year-old, or even a 14-year-old suddenly has to start sleeping with their mother. And panic attacks are common. “In those first few days, the child is in state of near-constant panic,” Dr. Swedo adds. “These changes are not subtle.”

Criteria for a PANS Diagnosis

All children with the disorder have acute onset of OCD symptoms or eating restrictions. But they also have a debilitating and baffling set of other neurological symptoms with similarly sudden onset. To be diagnosed with PANS they must have two of the following seven criteria:

• Separation anxiety, panic, other forms of anxiety
• Behavioral regression: Kids suddenly acting much younger than they should for their age, such as reverting to baby talk.
• Emotional lability: These children can be severely depressed, even suicidal
• Irritability, aggression and /or severely oppositional behaviors
• Deterioration in school performance: Sudden decline in math and reading competence, memory and concentration; increase in hyperactivity
• Motor or sensory abnormalities: Their handwriting and drawing deteriorates dramatically (also linked to regression), and they may be distressed by noise or light
• Somatic symptoms: These include sleep disturbances, betwetting and other changes in urinary frequency or intensity

If You Think Your Child Might Have PANS

Parents’ first stop when they experience such a dramatic behavior change is usually the pediatrician, or in extreme cases, the emergency room, Dr. Swedo notes. She recommends that you refrain from insisting to the doctor that your child has PANS, and bringing along articles on the disorder as backup, since medical professionals tend to be skeptical of a parental rush to diagnosis.

The best way to present this, she says, is: “I’m really worried about my child’s brain. There’s been an abrupt change in his behavior. This is what I’ve seen at home. It’s completely out of character.” The goal, as she puts it, is to partner with the doctor in terms of let’s figure it out together. PANS is one possible explanation for the sudden changes in behavior, but your physician will want to consider others as well.

In the past, pediatricians and other doctors have often dismissed families describing acute-onset OCD, Dr. Swedo said, assuming that parents must have missed earlier, less severe symptoms. But PANS and PANDAS should now be much more widely recognized.

It’s also good to check with your child’s teacher or school to see if strep has been going around. A child doesn’t have to have symptoms of strep to have an infection—some kids never get symptomatic.

What Should You Expect Your Doctor to Do?

If a child has urinary symptoms, Dr. Swedo recommends urinalysis and urine culture. She recommends a throat culture to look for strep, and an exam to see if an occult, or hidden, strep infection is observable.

If the rapid strep test is positive, she recommends a three-week course of antibiotics, to aggressively treat a possible sinus infection. Sinusitis is the trigger of symptoms for many kids, she says.

And Dr. Swedo also recommends that the pediatrician schedule a follow-up visit for three days later, to check in with the family. “The family stress is through the roof with this syndrome — by some measures higher than kids who’ve been diagnosed with leukemia,” she notes. “So a physician treating the medical causes should also make a referral, immediately, to a psychologist or psychiatrist—whatever is available in your area. ”

Many children respond within the first week of the antibiotic treatment. The longer the symptoms have been active before the child starts treatment, the longer the response may take. If the symptoms disappear, you finish the course of antibiotics and then practice what Dr. Swedo calls “watchful waiting.” Some kids, however, don’t respond to the antibiotic treatment alone. And many will have later episodes when symptoms recur, triggered by another infection.

Treatment for Subsequent Episodes

Treatment for recurrences of PANDAS involves targeting what Dr. Swedo calls the three S’s: “the symptoms, the source, and the system.”

• The treatment for the symptoms — OCD and other forms of severe anxiety — is cognitive behavioral therapy (specifically, exposure and response prevention) and anti-depressant (also called anti-obsessional) medication, a selective serotonin reuptake inhibitor or SSRI. These treatments have been shown to be effective in reducing the symptoms, but they do not target the source of the problem.

• The treatment for the source — the infection — is another course of antibiotics.

• The system refers to the immune system, which is attacking the brain and generating these neuropsychiatric symptoms. The first-line treatment for the misfiring immune system is with IVIG, or intravenous immunoglobulin. IVIG is made from healthy blood plasma and is thought to balance the immune system. IVIG is recommended only for severe cases in which repeated courses of antibiotics combined with CBT and SSRIs were not successful in stopping the flare-up of symptoms.

• If IVIG, in turn, is not successful, children are treated with plasmapheresis (the removal, filtering and return of blood plasma). A review of 40 patients treated with plasmapheresis, published last year, showed an average improvement of 65% at 6 months following treatment, and 78% at longer-term follow-up.

Dr. Swedo notes that these three targets for treatment — symptoms, source, and system — are exactly the same if you were treating a child with asthmatic childhood pneumonia. “You treat the source — this is the pneumonia part — with antibiotics. You treat the symptoms with cough syrup – you don’t ask the child to cough for the next month, until the pneumonia resolves. And you treat the immune system with the inhaled steroid, the Singulair, and all the rest.”

Long-term Antibiotics for PANDAS

Children who’s had a recurrence of PANDAS are often given antibiotics for an extended period as a form of prevention, or prophylaxis, for recurring strep infections. Strep is so common in the school population that it is now recommended that prophylaxis continue until patients are about 21, Dr Swedo says. “You want to get them through college, boot camp — places where they’re going to have high exposure to things like mono and strep.” Research shows that antibiotic prophylaxis can reduce significantly the number of episodes of PANDAS symptoms a child experiences.

Unfortunately, even the strep prophylaxis won’t prevent all later episodes of PANDAS symptoms because the immune system, once primed, continues to react dangerously to other kinds of infections as well, misidentifying the brain as foreign, and generating symptoms.

“But by preventing the strep,” Dr. Swedo notes, “you prevent over three quarters of those episodes, and you get them down to the point where then symptoms can be controlled with traditional anti-depressant medications and CBT.”

Other Resources

For more information on PANS and PANDAS, we recommend these resources:

PANDAS Physicians Network

This resource for physicians and medical professionals publishes consensus treatment protocols and guidelines for PANDAS. These guidelines were developed by the Diagnostic and Therapeutics subcommittee composed of PANDAS/PANS clinicians and researchers from medical institutions including Harvard, Yale, Stanford, Columbia, Georgetown and the National Institute of Mental Health. PPN also offers an archive of published studies on PANDAS/PANS.

National Institute of Mental Health 

The NIMH offers an extensive information sheet about PANDAS/PANS, including history, diagnostic criteria, treatment options, and a list of research papers.

The International OCD Foundation 

The IOCDF offers detailed information about PANDAS/PANS from diagnosis to treatment.

PANDAS Network

This non-profit organization founded by parents offers information and advocacy to improve the lives of children with PANDAS/PANS.

PANDAS syndrome. What is PANDAS Syndrome?

IMPORTANT
The information in this section should not be used for self-diagnosis or self-treatment. In case of pain or other exacerbation of the disease, only the attending physician should prescribe diagnostic tests. For diagnosis and proper treatment, you should contact your doctor.

PANDAS syndrome is an autoimmune neuropsychiatric pathology that develops in children due to streptococcal infection. The symptom complex of the disease is extremely diverse - from sudden anxiety and neurosis-like obsessive states to hyperkinesis and involuntary shouting out of words. The diagnosis is made on the basis of a psychiatric examination and laboratory confirmation of the presence of a streptococcal infection. Treatment includes antibiotics, intravenous immunoglobulin, psychotropic drugs. Sometimes the symptoms completely regress without therapy.

ICD-10

F06.8 Other specified mental disorders due to damage and dysfunction of the brain or physical disease

• Causes
• Pathogenesis
• Symptoms
• Complications
• Diagnostics
• Treatment of PANDAS syndrome
• Prognosis and prevention
• Prices for treatment

General

PANDAS is an English abbreviation in which P stands for childhood, A for autoimmune, N for neuropsychiatric, D for disorder, A for associated, S for streptococcal infection. The term was first used in 1998 by the American pediatrician S. Shwedo and his colleagues. According to some sources, the disease occurs in one child out of 200. The true prevalence of the condition is unknown. Prepubertal children (7 to 12 years of age) usually suffer. Boys are more often ill, the ratio with girls is 2.6-4.7:1.

PANDAS syndrome

Causes

It is believed that the occurrence of pathology is associated with a past infection caused by group A beta-hemolytic streptococcus (GABHS), for example, acute tonsillitis, scarlet fever, or chronic tonsillitis. Due to the peculiarities of the structure of the cell wall, the bacterium is able to trigger autoimmune reactions, namely, the production of autoantibodies that attack its own cells and body tissues, including brain cells.

In 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 GABHS, acute rheumatic fever.

Pathogenesis

The pathological reactions of the PANDAS syndrome are based on immune autoaggression. Along with other pathogenic properties (virulence, production of toxins), GABHS 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 GABHS, in addition to the pathogenic pathogen, attack the cells of the basal ganglia located in the caudate nucleus, globus pallidus, striatum, etc. These subcortical structures of the brain regulate motor (coordination of movements, skeletal muscle tone) and autonomic (control over respiratory rate and heartbeat, activity of the gastrointestinal tract) functions.

The basal ganglia are directly involved in the processes of higher nervous activity - memory, emotional reactions, speed of information processing. The defeat of these parts of the nervous system determines the main clinical picture of the PANDAS syndrome. According to most experts, the penetration of autoantibodies into the brain tissue is associated with a high permeability of the blood-brain barrier in children.

Symptoms

The disease occurs and proceeds acutely, which distinguishes it from most childhood neuropsychiatric disorders of other etiologies. The clinical picture varies considerably. The child becomes restless, irritable, emotionally unstable, his mood changes dramatically and often, concentration of attention is lost, memorization of new information and the ability to learn worsen.

The most typical obsessive-compulsive states are: repetitive actions (blinking, tilting the head to the side, shrugging), the desire to perform motor rituals (raising the leg high when descending stairs, repeatedly tying and untying shoelaces). Sleep is disturbed.

Characterized by unreasonable anxiety or fear. Often there is spontaneous uncontrolled shouting of words or repeated clattering, smacking, involuntary muscle twitches, tics, sweeping irregular movements of the limbs or head (hyperkinesis). Perhaps paroxysmal frequent sneezing, hiccups, sniffing for several hours.

A distinctive feature of PANDAS syndrome is the spontaneous disappearance of symptoms during sleep. The average duration of an attack is 10-12 weeks, after which recovery occurs. Sometimes there is a tendency to an undulating course, especially often this is observed in chronic tonsillitis, the exacerbation of which leads to a relapse of neuropsychic 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 impair the quality of life of the child. Due to the backlog in school, 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 to 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 age (7-12 years), the presence of signs of streptococcal infection (hyperemia of the pharynx, raids 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:

• Laboratory blood tests. 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 GABHS in the body, immunochromatographic express diagnostics (streptatest) and bacterial culture of a swab from the pharynx and tonsils are used.
• EEG. When conducting a 12-hour video-EEG monitoring, an electroencephalogram revealed increased convulsive activity, spike waves during a night's sleep.

Determination of specific antibodies to the basal ganglia in the blood significantly increases the reliability of the diagnosis, but this analysis is practically not used in clinical practice. Differential diagnosis is carried out with Sydenham's chorea, Tourette's syndrome, temporal epilepsy, etc. The following criteria for PANDAS syndrome are taken into account:

• presence of obsessive-compulsive disorders, tics;
• onset in prepubertal age;
• acute onset and paroxysmal course;
• association with GABHS infection;
• other neurological disorders.

Treatment of PANDAS syndrome

Depending on the severity of symptoms and mental status, patients may be treated as outpatients or inpatients. With severe behavioral disorders, round-the-clock monitoring is necessary. Some children experience spontaneous regression of disorders. Particular attention is paid to psychotherapy: individual conversations, training in special techniques for controlling tics and obsessive movements.

Sleep disturbances are corrected by non-drug methods - a strict regime of awakening and going to bed, the complete exclusion of the use of caffeine and caffeinated drinks (cola, "energy"), the distribution of activity during the day with the inclusion of physical exercises. The following drugs are used:

• Etiotropic therapy. Since the main cause of PANDAS syndrome is GABHS infection, for its eradication, all patients are prescribed antibacterial agents - phenoxymethylpenicillin, amoxicillin.
• Pathogenetic therapy. In order to suppress autoimmune damage to the basal ganglia, immunomodulatory drugs are used. In mild cases, NSAIDs are sufficient. With disadaptation, glucocorticosteroids or immunoglobulin are used. In extremely severe mental disorders, plasmapheresis or strong immunosuppressants (mycophenolate) are indicated.
• Symptomatic therapy. Antidepressants, SSRI inhibitors, are effective in relieving the symptoms of OCD. For increased anxiety, benzodiazepines or atypical antipsychotics are recommended.

Prognosis and prevention

PANDAS syndrome has a favorable prognosis. The life expectancy of patients does not differ from that in the general population. Lethal outcomes are casuistry. All clinical signs in the vast majority of cases respond well to treatment, in some children spontaneous recovery is possible without therapy.

To prevent exacerbation of chronic tonsillitis and subsequent neuropsychic attacks, antibiotic prophylaxis and courses of manual or hardware washing of tonsil lacunae are carried out. If, despite these measures, relapses continue, it is recommended to surgically remove the focus of persistent infection (palatine tonsils) - tonsillectomy.

You can share your medical history, what helped you in the treatment of PANDAS-syndrome.

Sources

1. PANDAS syndrome in cardiorheumatology / Evtushenko S.K., Perepechaenko Yu.M., Evtushenko L.F., Morozova T.M.// Bulletin of physiotherapy and balneology - 2005 - No. 4.
2. PANDAS-syndrome/ Swedo S. E., Klesling L.// J. Reumatologi - 1994 - No. 3.
3. Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS): an evolving concept/ Macerollo A., Martino D.// Tremor Other Hyperkinet Mov (NY) - 2013.
4. Revised Treatment Guidelines Released for Pediatric Acute Onset Neuropsychiatric Syndrome (PANS/PANDAS) – 2017.
5. can not be used for self-diagnosis and self-treatment. In case of pain or other exacerbation of the disease, only the attending physician should prescribe diagnostic tests. For diagnosis and proper treatment, you should contact your doctor.

   How to detect PANDAS syndrome | Directory of medical laboratory Optimum (Sochi, Adler)

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   More about the doctor

   PANDAS syndrome is a neuropsychic autoimmune pathology that develops in a child who has had a streptococcal infection - most often its causative agent is group A beta-hemolytic streptococcus.

   Diagnosis of PANDAS syndrome

   1. Collecting an anamnesis to clarify past infectious diseases and listen to the patient's complaints.
   2. Physical examination - to detect redness of the throat, swollen cervical lymph nodes, plaque on the tonsils.
   3. Hemograms - to detect inflammatory markers (increased erythrocyte sedimentation rate and leukocyte count).
   4. Biochemical blood test - to measure the level of ASLO (antistreptolysin-O), rheumatoid factor, C-reactive protein, anti-deoxyribonuclease.
   5. Bacteriological culture of scrapings from the pharynx - to identify and identify the causative agent of infection.
   6. Serological screening - to detect specific antibodies to gangliosides and double-stranded DNA.
   7. Universal rapid test - for the rapid detection of the presence of group A beta-hemolytic streptococcus.

   Causes of development

   The occurrence of PANDAS-syndrome is associated with scarlet fever, acute tonsillitis or chronic tonsillitis. A feature of the structure of the cell walls of their pathogen is the ability to "start" the production of autoimmune antibodies, which attack the cells of their own body. Predisposing factors - immunogenetic predisposition and the presence of other autoimmune pathologies.

   Pathogenesis

   Pathological reactions develop due to immune aggression - specific antibodies produced against beta-hemolytic streptococcus A act against cells of a large structure surrounding the limbic system of the brain and regulating movement coordination, skeletal muscle tone, respiratory rate and heart rate muscles, activity of the digestive tract, processes of higher nervous activity.

   Symptoms and first signs

   PANDAS-syndrome is characterized by an acute course – a sick child has:

   • restlessness;
   • emotional instability;
   • irritability;
   • impaired concentration;
   • loss of learning ability;
   • frequent blinking, sneezing, shrugging, smacking, head tilting to one side;
   • sleep disorder;
   • unreasonable fear;
   • hyperkinesis.

   A distinctive feature of the pathology is the spontaneous disappearance of clinical manifestations during sleep.

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