PANDAS Disease – A Hypothesized Neurological Disorder

What is PANDAS?

Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections abbreviated as PANDAS defines a condition in children that is associated with streptococcal infections. It is diagnosed when the patient shows behavioral and physical symptoms followed by a streptococcal infection such as a sore throat, sinusitis, cellulitis, scarlet fever and others. [1]

This autoimmune condition is triggered by strep infections that later on affect the neurological functions of the individual. The immune system targets the healthy tissues instead of fighting the infection. It results in inflammation of the brain’s basal ganglia section. It induces a sudden inception of abnormal neurologic behaviors.

How common is PANDAS disease?

Most of the people don’t even know if it exists. PANDAS is a very rare condition. It is estimated that only one in 2000 children may be affected by it. [2] It is only possible to diagnose it by relating to the symptoms. Some researchers categorize PANDAS with other neuropsychiatric symptoms called CANS (childhood acute neuropsychiatric symptoms) or PANS (pediatric acute-onset neuropsychiatric syndrome).

Incidence of PANDAS in children image photo picture

Image 1: Incidence of PANDAS in children
Picture Source: www.careresourcegroup.com

The history of PANDAS emergence

PANDAS made headlines in the late 1990s when a research group identified a special type of children, which happened to develop a sudden onset of the obsessive-compulsive disorder or OCD symptoms. There is evidence of other behavioral changes too but OCD is the most prominent of all. [3]

The researchers concluded that there was something common in all these children. It was later found that an active infection with a beta-hemolytic Streptococcus was common in all. It is the same bacteria that cause strep throat. [4]

Other infectious agents, which cause auto-inflammatory reactions, were also identified. It includes influenza, varicella, and others. But the onset of PANDAS is only and only limited to streptococcal infections, as shown by study results.

PANDAS in children image photo picture

Photo 2: PANDAS in children
Picture Source: images1.houstonpress.com

What causes PANDAS disease?

Researchers agree that PANDAS is caused by an abnormal autoimmune response towards strep infection. Strep infection is one of the common infections to be related to a number of immune-related disorders. It includes such rheumatic fever, acute glomerulonephritis, and others. [5]

Strep particles mimic the body cells and successfully deceive the immune system. The immune system recognizes the alien particles and makes respective antibodies. During this process, many of the body cells are also attacked due to cross-reactivity. When these body cells are of the brain, it causes certain neurological disorders such as OCD to start hitting the individual. That’s how an individual develops the PANDAS.

What are possible complications of strep throat infection?

The possible complications of strep throat for future include;

  • Suffering from an acute rheumatic fever
  • Glomerulonephritis (a kidney infection)
  • Otitis media (an ear infection),
  • Toxic shock syndrome (a rare but fatal complication)
  • Peritonsillar abscess or retropharyngeal abscess (a fatal of throat)
  • PANDAS (Strep linked Pediatric Autoimmune Neuropsychiatric Disorder)

Model of pathogenesis for PANDAS image photo picture

Image 3: Model of pathogenesis for PANDAS
Picture Source: pediatrics.aappublications.org

Diagnosis of Pandas disease

Many of the doctors believe that PANDAS is emerged like typical effects of pediatric OCD or tics. But PANDAS has no as such diagnostic test either blood or neurological testing. They do not rely on any such thing, which confirms the condition to be this. [6]
The only way is to check it through standard diagnostic criteria. It is only a source to determine if a child is having PANDAS or not. The standard diagnostic criteria check the following. [7]

  • Emergence of obsessive-compulsive disorder
  • Presence of a tic disorder
  • Pediatric beginning of unusual changes
  • A gradual course of symptom severity
  • Any association with group A Beta-hemolytic streptococcal infections
  • A history of scarlet fever
  • Any neurological abnormality.
  • A sudden onset of symptoms and worsening of these symptoms
  • If the above-listed symptoms are present for more than 7 days in a row, next is to check the blood test for a possible streptococcal infection.
  • Sudden symptoms of PANDAS

An infected child may show any of the following symptoms while suffering from PANDAS.

Abrupt signs of obsessive-compulsive disorder liked PANDAS

  • Severely restricted food consumption
  • Stress and anxiety
  • Emotional instability
  • Irritability
  • Aggression
  • Behavioral changes
  • Mood swings
  • Low performance at school
  • Motor or sensory abnormalities
  • Insomnia
  • Frequent urge to urinate
  • ADHD symptoms
  • Social isolation
  • Joint pains

Behavioral change in children is a symptom of PANDAS image photo picture

Photo 4: Behavioral change in children is a symptom of PANDAS
Picture Source: i0.wp.com/blog.asha.org

Can adults develop PANDAS too?

PANDAS is a pediatric disorder and it is most likely to show its symptoms in early childhood till puberty. Most of the reactions stop by puberty. PANDAS do not hit the adults, only if they have already suffered from it in childhood. It means PANDAS can re-emerge in adults but it is very rare.

It is highly unlikely to experience post-strep neuropsychiatric symptoms for the first time in adult years. However, it is not fully studied for its occurrence. There are cases of immune-mediated OCD it adults but diagnosing it as PANDAS is not known at this time.

Who is at risk of PANDAS?

There are numerous risk factors that may cause the development of PANDAS. For example, a recurrent group- A streptococcal infections, having a family history of rheumatic fever, or the mother’s chance of having an autoimmune disease. [8] Surprisingly, PANDAS is much more common in boys and pre-pubertal children for unknown reasons.

How to treat ?

If the child starts to show the unusual symptoms, particularly related with PANDAS, they should be immediately taken to their pediatrician first. An initial examination will refer the case to a consultant psychiatrist or other mental health professional.

He will describe a treatment plan with medicines, cognitive behavioral therapy or others. In some cases, a pediatric neurologist or behavioral developmental specialist may also be involved in case management.

Cognitive behavioral therapy PANDAS Disease image photo picture

Image 5: Cognitive behavioral therapy
Picture Source: bodminsou.com

The first line of treatment for PANDAS is cognitive behavioral therapy or CBT along with medicines. The choice of treatment depends upon the severity of symptoms. Primary therapy is focused on controlling the symptoms, and some selective serotonin reuptake inhibitor (SSRI) medications are prescribed. [9]

The treatment for acute symptoms of PANDAS is to first treat the strep infection. Generally, antibiotics are the best choice for healing strep infection i.e. penicillin, amoxicillin etc. For severe symptoms, a combinational therapy is used. More research is required to find most suitable immune-modulating therapies.

Medicines are prescribed along with behavioral therapy for treating PANDAS image photo picture

Photo 6: Medicines are prescribed along with behavioral therapy for treating PANDAS
Picture Source: www.choice.com.au

What happens when PANDAS is not diagnosed?

There is a high possibility that many children suffering from PANDAS often go unrecognized or misdiagnosed. Some of the inexperienced doctors categorize it to be rebellious personality changes or parental negligence.

Typically, there aren’t many specialists of PANDAS despite so many cases documented. An incorrect diagnosis of a mental illness makes the child take unnecessary psychiatric medications.

An effective treatment for PANDAS should target the infection if present, and symptoms. It should also work to highlight the hidden immune system problems. Once the disease is correctly diagnosed, it has the high rate of successful treatment.

Future complications of PANDAS in children

There is a misconception that relates PANDAS to develop a number of health issues in at later years. PANDAS is not currently studied to cause any heart issues as seen in children with rheumatic fever. But an untreated or unrecognized PANDAS has a potential to increase the risk of having OCD and tic disorder even during adult years. [10]

What is the prognosis of PANDAS?

Most of the children diagnosed with PANDAS show a complete recovery. There are only a few who develop persistent neuropsychiatric symptoms or them experience-worsening symptoms after each strep infection.

It is not possible to estimate the complications and severity of effects for future. The purpose of treating PANDAS is to control the symptoms of OCD, tics, or other behaviors that definitely affect the daily life and school performance of the child.

MRI scans of a PANDAS patient, showing reduced inflammation after treatment image photo picture

Image 7: MRI scans of a PANDAS patient, showing reduced inflammation after treatment
Picture Source: www.nimh.nih.gov

Tips to prevent

There is evidence to suggest that prophylactic antibiotics help the children suffering from PANDAS to prevent strep infections. In that case, signs of PANDAS will not go worse. But there are chances for the daily antibiotics dose to cause undesirable effects such as diarrhea (mild side effect) and antibiotic resistance (major side effect). [11]

There is a dire need for research and studies to find the preventive methods for preventing the risk of PANDAS in children. The surgical option of tonsil removal and adenoids are not effective for PANDAS and are not required as a part of treatment.

References:

  1. http://childrenstreatmentcenter.com/pandas-disease-following-strep-throat-infection/
  2. https://www.medicinenet.com/pandas/article.htm
  3. https://www.nimh.nih.gov/health/publications/pandas/index.shtml
  4. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3783973/
  5. https://www.ncbi.nlm.nih.gov/books/NBK333433/
  6. http://www.pandasnetwork.org/understanding-pandaspans/what-is-pandas/
  7. https://www.pandasppn.org/ppn-pans-diagnostic-guidelines/
  8. http://www.pandasfoundation.org.uk/causes-risk-factors/
  9. https://www.pandasppn.org/ssris/
  10. http://www.pandasnetwork.org/understanding-pandaspans/long-term-prognosis/
  11. http://seattlemamadoc.seattlechildrens.org/antibiotic-use-may-increase-risk-for-ibd/

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