Pandas

PANDAS is the abbreviation for:

'Pediatric Autoimmune Neuropsychiatric Disorders Associated with Strepinfections'.
Literally translated, this means 'a neuro-psychiatric syndrome in children that is associated with an antibody reaction directed against body tissues due to an infection with the bacterium streptococci'. The infection can be scarlet fever, but also a harmless ear -, throat - or sinus infection.
Although there are psychiatric symptoms, a physical cause can be identified. The affected children functioned completely normally
before they got sick. The disease process is reversible with timely, adequate treatment.

After a seemingly harmless infection with GAS, Group A beta-hemolytic streptococci, PANDAS children suddenly develop psychiatric symptoms with a neurological origin in the brain.

• Group A streptococci can cause scarlet fever, laryngitis, erysipelas, impetigo, acute rheumatism, puerperal fever and blood poisoning (sepsis).
  

Antibodies cross the blood-brain barrier and cause a mild inflammatory response in an important switching station in the brain: basal ganglia.

In addition to tics, compulsion and violent tantrums, handwriting changes, frequent urination or suddenly being unkempt again are also included. The disease process is reversible with timely, adequate treatment.

PANDAS often affects children at the age when a streptococcal infection is most often seen in children and young people: on average ranging from 3 to 15 years, but earlier and later also occur. Early outbreaks of the disease last about 8 weeks, at a later age one episode, as it were, merges into another: the child remains stuck in a chronic, vague clinical picture.

Because multiple children in one family can develop PANDAS, it is suspected that a genetic factor plays a role. In addition, autoimmune diseases often run in the family of PANDAS children. Not every streptococcal infection causes PANDAS, it specifically concerns group A (GAS).

PANS

PANDAS is part of a larger disease complex that is called PANS.

PANS is the abbreviation for 'Pediatric acute-onset neuropsychiatric symptoms'.
PANS causes complaints that are comparable to the complaints of PANDAS, but without a link to streptococci.

Other bacterial and viral infections appear to play a role in PANS. Examples include: Epstein-Barr, H1N1 (bird flu virus), mycoplasma pneumoniae and Lyme.

Sometimes the name CANS is used, which stands for Childhood Acute Neuropsychiatric Symptoms.

Inflammatory response in basal ganglia

Antibodies produced by the body against group A streptococci ensure that the blood-brain barrier (BBB) becomes permeable. These antibodies travel further and mistakenly target neuroreceptors in the basal ganglia where a mild inflammatory reaction occurs.

These basic nuclei regulate the starting and stopping of all kinds of processes: movement, mood, cognition (for example concentration) and executive functions such as planning and organization.
Of the basal ganglia, both the tail nuclei on both sides (bilateral caudate nucleus) and the lens nuclei on both sides (bilateral nucleus lentiformis) can be affected.

In the image below by Henry Vandyke Carter [Public domain]
the tail and lens nuclei of the basal ganglia are drawn. They are part of the 'striated body' or corpus striatum. And that is part of the control loop for strengthening, braking and adjusting the movement activities.

Thalamus and frontal lobe

By examination with contrast agent via PET scans it has been shown in PANDAS children that the thalamus also may be affected. It is also sometimes mentioned that the frontal lobe may be involved. This mechanism may be linked to Sydenham's Chorea or St Vitus's Dance, the neurological manifestation of Acute Rheumatic Fever.

With this condition, children experience difficulty moving after experiencing a streptococcal infection. Mixed images of Chorea from Sydenham and PANDAS can also occur.

The symptoms develop very acutely. They can last for days or weeks. the severity and form can vary. There is a flare-up and an increase in complaints associated with infections. PANDAS probably develops into a chronic picture where with each new activation of the immune system, complaints are worsened and/or a new period of illness begins. In addition, stress, fatigue and hormonal changes also have an influence.

The possible complaints of PANDAS children are classified into behavioral, cognitive, motor and physical complaints, sometimes with an overlap.

Behavioral changes:

• obsessive compulsive behavioral problems (OCD) or obsessive-compulsive problems
• tics (sound tics and movement tics)
• compulsion to pull out one's own hair (trichotillomania)
• less affectionate behavior
• intense fears
• severe mood swings with
• irritable, irritable behavior
• rebellious behavior
• hyperactive, restless behavior
• refusing certain types of food (anorexia) or the opposite: excessive eating

Cognitive changes:

• concentration disorders
• easily distracted by sound and/or movement
• noticeably reduced arithmetic skills
• Motor changes (movements)
• rapid, undirected movements of fingers and toes; 'piano playing' (choreiform movements)
• strange movements of arms, legs and face like dance movements (chorea)
• strange position of the arm or leg (dystonia)
• changes in fine motor skills such as handwriting
• muscle twitches in the arm, leg or trunk (myoclonias)

Physical complaints:

• sleep problems (difficulty falling asleep and waking up early)
• urinary tract complaints and bedwetting
• joint complaints: painful, swollen and red joints
• muscle twitches in the arm, leg or trunk (myoclonias)
• refusing food or overeating

Treatment 

Since the 1990s, forms of therapy have been tested, especially in the United States, and PANDAS can be treated. For example, see this website. 

In most cases, the damage is largely reversible. With treatment, most children can lead a normal life again.

Of course it is much better to prevent children from developing a Pandas image by carefully monitoring for the combination of

1. Tics and/or compulsion
2. Handwriting changes and 
3. Urinary tract complaints without infection.
   

The treatment of PANDAS primarily consists of:

• Antimicrobial interventions to combat active infections.
• Resetting the immune system in severe cases through immunotherapy supplemented with very long-term prescribed antibiotics and other immunomodulatory and/or anti-inflammatory therapies.
  

To prevent secondary social-emotional and psychological damage, this can be supplemented with:
Treating the symptom with psychoactive medication (with the warning of starting in a very low dose, due to an increased risk of suicidality as a side effect), psychotherapy (particularly cognitive behavioral therapy) and other supportive interventions, especially those involving the immediate environment of the child is taught to deal with erratic behavior during relapses.

More information:

• Dutch website PANDASWEB
• Information on Wikipedia

A video about a child suffering PANDAS