Immune Behavioral and NeuropsychiatricDisorders

Immune Behavioral and Neuropsychiatric Disorders: What are PANS, PANDAS, BGE and Autoimmune OCD?

PANS

PANS (Pediatric Acute-Onset Neuropsychiatric Syndrome) is viewed as an autoimmune/autoinflammatory condition that can be triggered by infections, metabolic disturbances, and other inflammatory reactions.

Infectious triggers include upper respiratory infections, influenza, sinus infections, mycoplasma pneumonia, tick infections (such as lyme borreliosis), and other sources. It is not always possible to identify the trigger.

At this time, PANS is a clinical diagnosis based on a child’s medical history, a physical examination and by ruling out other possible diagnosis that might better explain the constellation of symptoms.

PANS is characterized by the abrupt onset of OCD (obsessive compulsive disorder) and/or eating restrictions, with concurrent symptoms in at least 2 of 7 neuropsychiatric categories:

  • Anxiety
  • Emotional Lability and/or Depression
  • Irritability, Aggression, and/or Severe Oppositional Behaviors
  • Behavioral (Developmental) Regression
  • Sudden Deterioration in School Performance
  • Motor or Sensory Abnormalities
  • Somatic Signs and Symptoms, including Sleep Disturbances, Enuresis, or Urinary Frequency

PANDAS

PANDAS (Pediatric Autoimmune Neuropsychiatric Syndrome Associated with Streptococcus) is a subset of PANS. Symptoms follow an infection with group A beta-hemolytic streptococcus (GABHS), which can occur in many parts of the body including the throat, skin, intestines and perianal area.

In PANDAS, misdirected antibodies set off an inflammatory response that interferes with basal ganglia functions, producing behavioral, psychiatric, and neurologic symptoms including OCD, motor or vocal tics (or both) or both OCD and tics.

BGE

The basal ganglia are in an area of the brain associated with regulating functions such as motor movement, cognitive and emotional response, and procedural learning.  Post-infectious basal ganglia encephalitis (BGE) disorders are a subset of Autoimmune Encephalitis (AE) that occur when anti-basal ganglia autoantibodies (ABGA) are triggered by either bacterial, viral or fungal infections.

What PANS/PANDAS/BGE Share

After the initial onset, PANS/PANDAS/BGE symptoms follow a relapsing/remitting course. Initial triggers may differ from secondary triggers. During each recurrence, symptoms can worsen or disappear while new symptoms may manifest.

Symptoms can range from mild to severe. In mild cases, children might function well enough to continue to attend school. In severe cases, symptoms can become life-threatening due to extreme food restriction and/or suicidality.

According to a consortium of experts convened by the National Institute of Mental Health, appropriate treatment for these disorders is a triad that incorporates psychological support (CBT, ERP and/or psychotropic medication), antimicrobial treatment, and immunomodulation.

Seriousness of PANS/PANDAS/BGE

The diagnosis PANS/PANDAS/BGE is some times described as controversial because signs of inflammation and autoantibodies are absent in many children. This leads to an unwillingness by many clinicians to make a diagnosis, which prevents affected children from receiving effective treatments with antibiotics and immunomodulators, such as non-steroidal anti-inflammatory drugs (NSAIDs), intravenous immunoglobulin (IVIG), corticosteroids, and the B-cell inhibitor Rituximab.

Additionally, many children with PANS/PANDAS/BGE are diagnosed with a psychiatric illness and prescribed psychotropic medications rather than being evaluated and treated for an underlying infection and the possibility of a dysregulated immune response.

Unfortunately, parents are often seen as being intrusive or deriving psychological benefit from their children’s illness.  Additional suspicion is raised when more than one child in the family presents with symptoms, which is not uncommon.

Autoimmune OCD

Many experts, including Prof. Janet Cunningham of the EXPAND Scientific and Medical Advisory Board, believe it is time to consider the concept of an äutoimmune OCD subtype.  This conceptual review is the result of expert discussions and a PubMed search:

Immunological causes of obsessive-compulsive disorder: is it time for the concept of an “autoimmune OCD” subtype? 

In the conclusion, the authors state:

“There is increasing evidence for secondary immune-mediated forms of OCD. The DSM-5 and novel ICD-11 criteria include the category of secondary OCD, without, however, providing guidelines according to which such a diagnosis should be established. In the current paper, the authors have drafted a first proposal of clinical criteria for the definition of secondary autoimmune OCD. Future studies should investigate the prevalence (e.g., by analyzing the rate of neuronal antibodies in patients with OCD), diagnostic regimes (combination and comparison of different diagnostic methods), and optimal therapy of autoimmune OCD, including the development of clear treatment algorithms and clinical guidelines. Recognizing the autoimmune causes of OCD could inform additional therapeutic options for the affected patients to promote treatment response and reduce chronicity.”

The Impact of These Illnesses 

PANS/PANDAS/BGE/Autoimmune OCD impacts the well-being of the entire family.   According to a longitudinal research study conducted at the Stanford PANS Clinic, the median Caregiver Burden Inventory (CBI) during a 1st PANS flare was 37. This score is higher than the CBI for those caring for someone with Alzheimer’s and is equivalent to the CBI in Rett syndrome (https://www.ncbi.nlm.nih.gov/pubmed/3054949)

In addition to the caregiver burden, a family’s financial security becomes strained when a parent takes leave from their employment to stay home to care for their child. There are usually extraordinary out of pocket medical costs incurred too, some that may not be allowed as tax deductions. Furthermore, it is not unusual for more than one child in a family to be diagnosed with PANS/PANDAS. The extent of the increase in the CBI with more than one ill child has not yet been studied, but those families that find themselves in this situation can attest to the significantly increased impact.

For unaffected siblings, their life will be greatly impacted too.  However, it is important to remember that they are not alone. Many children must cope during their childhood with a serious illness in their family. In addition to the support of family and friends, the siblings school counselor and other professionals can be enlisted to help them feel safe, supported and even empowered.