Understanding Pans And Pandas: Two Intriguing Pediatric Disorders

PANS and PANDAS are neuropsychiatric conditions that affect the brain, causing a variety of symptoms, including mental health issues, behavioural changes, and difficulties with movement. PANS (Paediatric Acute-onset Neuropsychiatric Syndrome) is characterised by the sudden onset of obsessive-compulsive symptoms (OCD) or eating restrictions, along with acute behavioural deterioration. PANDAS (Paediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections) is a subset of PANS, specifically referring to cases triggered by a streptococcal infection. Both conditions can have severe impacts on children, causing extreme behavioural changes and requiring prompt medical attention and treatment.

PANS diagnostic criteria

PANS, or Pediatric Acute-onset Neuropsychiatric Syndrome, is characterised by the sudden onset of obsessive-compulsive disorder (OCD) or severely restricted food intake. To be diagnosed with PANS, a patient must meet the following criteria:

• An abrupt, acute, dramatic onset of obsessive-compulsive disorder or severely restricted food intake.
• Concurrent presence of additional neuropsychiatric symptoms with a similarly severe and acute onset from at least two of the following categories:
• Anxiety
• Emotional lability and/or depression
• Irritability, aggression, and/or severe oppositional behaviours
• Behavioural (developmental) regression
• Sudden deterioration in school performance
• Motor or sensory abnormalities
• Somatic signs and symptoms, including sleep disturbances, enuresis, or urinary frequency
• Symptoms are not better explained by a known neurological or medical disorder
• There is no age requirement

PANS is a clinical diagnosis, and it can be difficult to recognise as symptoms overlap with many other medical conditions. It is important to note that PANS is not limited to a strep infection trigger, unlike its subset PANDAS.

PANDAS diagnostic criteria

PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections) was first described in February 1998 by Dr Susan E. Swedo and her team at NIMH, who also provided guidelines for diagnosis. These criteria have since been updated and clarified.

To diagnose PANDAS, the patient must fulfil the following criteria:

• Presence of OCD and/or tics, particularly multiple, complex or unusual tics. The obsessions, compulsions and/or tics must be severe enough to meet the criteria for OCD or a tic disorder and interfere with the patient's ability to function.
• Age Requirement. Symptoms of the disorder first become evident between 3 years of age and puberty.
• Acute onset and episodic (relapsing-remitting) course. Clinical course is characterised by the abrupt, dramatic onset of OCD and/or tics, with comorbid neuropsychiatric symptoms. For some, symptoms may develop within a 24-48 hour period but may reach maximum intensity over a longer timeframe.
• Association with Group A Streptococcal (GAS) infection. Symptom exacerbations must be temporally related to GAS infections.
• Association with Neurological Abnormalities. During symptom exacerbations, patients will have abnormal results on neurological examination.

There are three severity levels for PANDAS: Mild, Moderate and Severe.

PANS and PANDAS symptoms

PANS and PANDAS are clinically defined disorders characterised by the sudden onset of severe obsessive-compulsive symptoms (OCD) or eating restrictions, along with acute behavioural deterioration.

PANS Symptoms

PANS symptoms may include:

• Anxiety
• Sensory amplification or motor abnormalities
• Behavioural regression
• Deterioration in school performance
• Mood disorder
• Urinary symptoms
• Sleep disturbances

PANDAS Symptoms

PANDAS symptoms are similar to those of PANS, but the two differ in their triggers. PANDAS is triggered by a Group A Streptococcus ("strep") infection, whereas PANS is triggered by other infections, such as the flu, chickenpox, mycoplasma, or Lyme disease.

PANDAS has five distinct criteria for diagnosis:

• Abrupt onset of OCD or dramatic, disabling tics
• An episodic symptom course, or relapsing-remitting course of severity
• Presence of neurologic and neuropsychiatric abnormalities
• Occurrence of a strep infection before symptom onset
• Young age at onset (average of 6-7 years)

PANS and PANDAS treatments

PANS and PANDAS are severe forms of obsessive-compulsive disorder (OCD) that appear suddenly in young children, accompanied by other confusing and distressing symptoms. PANDAS is a subset of PANS. While PANDAS is triggered by a strep infection, PANS may be caused by various other infectious agents, such as Lyme disease, influenza, Epstein-Barr virus, mycoplasma pneumonia, and more.

The treatment for PANS and PANDAS involves addressing the symptoms, the source, and the system.

Symptoms:

• Cognitive Behavioural Therapy (CBT): This is done by a trained mental health provider and includes exposure and response prevention to reduce ritual behaviours.
• Medication: Anti-depressants, also called anti-obsessional medication, such as selective serotonin reuptake inhibitors (SSRIs), are used to treat OCD and other forms of severe anxiety.

Source:

• Antibiotics: Antibiotics are used to treat and prevent strep or other bacterial infections. Antibiotics can dramatically improve many patients' neuropsychiatric symptoms.
• Antivirals: Antivirals are used if viral infections are persistent.
• Eradication of Carriage States: If a child has had or been exposed to an illness with prolonged coughing, testing for bacteria such as mycoplasma is considered.

System:

• Intravenous Immunoglobulin (IVIG) Treatments: IVIG is made from healthy blood plasma and is thought to balance the immune system. It is recommended for severe cases where antibiotics, CBT, and SSRIs were not successful in stopping symptom flare-ups.
• Plasmapheresis (Apheresis) or Plasma Exchange (PEX): This process removes harmful autoantibodies from the blood system.
• Steroids: Steroids can reduce inflammation in the child's brain.
• Tonsillectomy and Adenoidectomy: These procedures are considered when the chronic infection is recurring.

Other Treatments:

• Psychiatric Treatment: This includes medications and/or psychotherapy.
• Long-term Antibiotics: Children who have had a recurrence of PANDAS are often given antibiotics for an extended period to prevent recurring strep infections.
• Prophylaxis: Prophylaxis is recommended until patients are about 21 years old to prevent exposure to things like mono and strep.
• Family Support and Guidance: Doctors and mental health experts can give families guidance and support to help them through challenging times.

PANS and PANDAS causes

PANS (Pediatric Acute-onset Neuropsychiatric Syndrome) is a clinically defined disorder characterised by the sudden onset of obsessive-compulsive symptoms (OCD) or eating restrictions, along with acute behavioural deterioration in at least two designated domains. PANS is believed to be triggered by one or more pathogens, although the specific trigger is often unknown.

PANDAS (Paediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections) is a subset of PANS. PANDAS is triggered by a Group A Streptococcus ("strep") infection. The strep bacteria are ancient organisms that survive in the human body by hiding from the immune system for as long as possible. They do this by putting molecules on their cell walls so that they look almost identical to molecules found in the child's heart, joints, skin, and brain tissues. This process, called "molecular mimicry", allows the strep bacteria to evade detection for a long time. Eventually, the molecules on the strep bacteria are recognised as foreign to the body, and the child's immune system reacts by producing antibodies. Because of the molecular mimicry, the immune system reacts not only to the strep molecules but also to the human host molecules that were mimicked. These antibodies then attack the mimicked molecules in the child's own tissues, including the brain. This leads to the onset of OCD, tics, and other neuropsychiatric symptoms of PANDAS.

PANDAS has five distinct criteria for diagnosis:

• Abrupt onset of OCD or dramatic, disabling tics
• An episodic symptom course, or relapsing-remitting course of severity
• Presence of neurologic and neuropsychiatric abnormalities
• Occurrence of a strep infection before symptom onset
• Young age at onset (average of 6-7 years)

While the specific cause of PANDAS is known, the reason why some children develop PANDAS after a Group A strep infection while others do not is still unknown. Possible hypotheses include:

• Only certain strains of GAS trigger PANDAS symptoms
• Genetic vulnerability, including defects in clearing Group A strep and resolving inflammation after infection
• Location of the strep infection—strep infections can occur in the oropharynx, tonsils, and anus, and the bacteria may enter the brain through the nasal cavity

PANDAS is the only subtype of PANS that requires that symptoms be associated with a strep infection. PANS has been associated with a variety of other infections, including influenza, varicella, and mycoplasma pneumoniae. Lyme disease has also been proposed as a potential trigger.

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