PANDAS Syndrome

Definition

The term PANDAS is used as an acronym for Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections. PANDAS Syndrome is clinically diagnosed after a person develops a variety of physical and behavioral manifestations following a scarlet fever or strep throat infection.

The condition is considered rare and is estimated to appear in one out of 2,000 affected individuals although some signs in the diagnosis might be missed due to the typical symptoms related with the disease. PANDAS Syndrome usually occurs in children starting from age 3 to puberty. It is also possible for adults to develop the condition in rare instances although it has not been fully studied.

A number of researchers have also suggested including PANDAS in a group of other disorders that also have similar neuropsychiatric symptoms such as Pediatric Acute-onset Neuropsychiatric syndrome/PANS or Childhood Acute Neuropsychiatric Symptoms/CANS. A PAN is triggered by certain factors that create a wrong immune response resulting to an inflammation of the brain.

Symptoms of PANDAS Syndrome

Some Individuals with PANDAS Syndrome experience an unexpected appearance of a neuropsychiatric disorder such obsessive-compulsive disorder (OCD) or tics followed by Group A beta-hemolytic streptococcal infection (GABHS).

Symptoms of this condition can dramatically occur and the period of recovery is variable. Frequently, the symptoms could get worse during streptococcal infections and get better in between. Streptococcal infections are caused by a variety of streptococcus bacteria that depend in severity, ranging from mild infections of the throat to life-threatening infections of the organs or blood.

Symptoms of PANDAS Syndrome are often experienced by patients with one or more of the following indications in conjunction with OCD or tics:

• Joint pains
• Irresistible impulse to perform certain acts against one’s conscious will
• Obsessions
• ADHD symptoms such as uneasiness, hyperactivity, and lack of attention
• Mood swings like emotional lability, irritability, and sadness
• Separation anxiety in which the child has difficulty separating from certain people such as caregivers
• Sleep disruptions
• Motor tics such as shrugging of the shoulders and blinking of the eyes
• Vocal tics like shouting, humming, and clearing the throat
• Signs of detaching from parents or loved ones
• Changes in fine or motor movements
• Frequent urination during day-time and/or bed wetting during night-time
• Night-time fears

Causes

PANDAS Syndrome is the result of an autoimmune response to a strep infection. The strep bacteria or Streptococcus is linked with numerous of immune-related disorders such as acute glomerulonephritis, rheumatic fever, and scarlet fever. Streptococcus is an organism that lives in its human host and is known to hide in the immune system for a long period of time by mimicking the host cells.

This is referred to as “molecular mimicry” and permits the strep bacteria to avoid detection for a certain time. Nevertheless, the immune system will eventually recognize the strep bacteria as foreign cells and the immune system produces antibodies to attack the bacterium.

Unfortunately, the human host cells are also attacked by the antibodies due to the mimicked molecules. The cross-reactivity prompts an immune reaction that attacks the molecules that are being mimicked in the child’s own tissues. In some cases, the antibodies attack brain cells that lead to tics, OCD, and other symptoms of PANDAS syndrome.

Diagnosis

PANDAS syndrome is clinically confirmed base on the patient’s history and physical examination rather than other studies. The proposed criteria that are used to diagnose the condition are:

• Existence of OCD or a tic disorder, oppositional defiant behaviors, and symptoms of ADHD
• Abrupt onset between 3 years of age and puberty
• Neurological abnormalities such as motor hyperactivity, anxiety, wetting of the bed, temper tantrums, emotional lability, and changes in personality, and deterioration in shcool performance
• Episodic course of symptoms
• Temporal association with GABHS infections

If symptoms had been present for a week or more, blood tests might be performed to record a preceding streptococcal infection.

Treatment

Options of treating PANDAS Syndrome include:

Cognitive behavioral therapy (CBT)

It is a psychotherapy treatment done in a short-term basis that aims to solve current problems and change behavioral or thinking patterns of a patient

Antibiotics

These are best recommended for eradicating a strep infection associated with the symptoms. The physician might prescribe azithromycin, amoxicillin, or other antibiotics.

Medical therapy

This focuses on controlling the symptoms of OCD. Serotonin reuptake inhibitor (SSRI) medications are often used like Prozac and fluoxetine.

For symptoms that are severe and unmanageable, the treatments are intended to control the immune system response are used and found to be effective. These include:

Steroids

These are considered to be less effective although it could help in decreasing the symptoms during the treatment but they come back after stopping

Intravenous immunoglobulin

It is a therapy that can help in removing the “offending” antibodies

Plasmapheresis

It is a therapeutic method used through the bulk removal of plasma

References

1. http://www.nimh.nih.gov/health/publications/pandas/index.shtml
2. http://www.medicinenet.com/pandas/page3.htm#what_is_the_treatment_for_pandas
3. http://patient.info/doctor/pandas-paediatric-autoimmune-neuropsychiatric-disorder-associated-with-streptococcal-infection
4. Leckman JF, King RA, Gilbert DL, et al (2011). Streptococcal upper respiratory tract infections and exacerbations of tic and obsessive-compulsive symptoms: a prospective longitudinal study. J Am Acad Child Adolesc Psychiatry; 50:108.
5. Shulman ST (2009). Pediatric autoimmune neuropsychiatric disorders associated with streptococci (PANDAS): update. Curr Opin Pediatr; 21:127.
6. Pichichero ME (2009). The PANDAS syndrome. Adv Exp Med Biol; 634:205.