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Paediatric Acute-Onset Neurologic Syndrome (PANS) (aka PANDAS): Information for Primary Care

Summary: Paediatric acute neurologic symptoms (PANS) is uncommon, however family physicians should consider PANS when seeing the patient with sudden onset OCD, eating disorder or tic symptoms that appears correlated to a streptococcal infection. As referral to neurologists and psychiatrists may take time, family physicians can intervene quickly by investigating and treating any potential strep infections. Any residual OCD or tic symptoms can be managed as one would manage them from any other cause.
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Case

“It was like he was fine one day, and then he woke up the next day, and now he has to wash his hands all the time… Not only that, but he’s regressed at home and school, and he can’t do anything on his own anymore. He’s clingy and anxious all the time… Its just not our child, like he’s been possessed… Its been a week like this now...”

What is Paediatric acute-onset neurologic syndrome (PANS)?

PANS describes a small group of children/youth that develop a neurologic syndrome with symptoms of Obsessive Compulsive Disorder (OCD) or tic disorders such as Tourette Syndrome, which occurs after a streptococcal infection.

 

PANS was previously known under the term Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS).

PANS Criteria

  1. Abrupt, dramatic onset of obsessive-compulsive disorder or severely restricted food intake
  2. Concurrent presence of additional neuropsychiatric symptoms, with similarly severe and acute onset, from at least two of the following seven categories
  • Anxiety
  • Emotional lability and/or depression
  • Irritability, aggression and/or severely oppositional behavoiurs
  • Behavioural (developmental) regression
  • Deterioration in school performance
  • Sensory or motor abnormalities
  • Somatic signs and symptoms, including sleep disturbances, enuresis or urinary frequency
  1. Symptoms are not better explained by a known neurologic or medical disorder, such as Sydenham chorea, systemic lupus erythematosus, Tourette disorder or others.

Epidemiology

By definition, PANS occurs in children and adolescents and affects a small subset of patients with OCD, but exact prevalence is unknown.

Pathophysiology

Exactly what happens in PANS is not well understand, however, the following steps are felt to occur:

  • Individual develops a strep infection

  • The individual’s body produces antibodies against the strep bacteria

  • The antibodies end up reacting with the person’s own brain, specifically areas implicated in OCD, hence leading OCD and other neuropsychiatric symptoms

History

Classic history is the previously well child who presents with sudden onset of various symptoms:

  • Primary symptoms such as:
    • Obsessive compulsive disorder (ODC) and/or
    • Tic disorder
  • Secondary symptoms include, but are not limited to the following:
    • ADHD symptoms (problems with being inattentive, hyperactive, distractible, fidgety)
    • Separation anxiety, i.e. the child has problems separating from parents
    • Mood changes such as emotional lability, e.g. crying or laughing out loud, which is a marked change from previous
    • Regression
    • Temper tantrums; altered speech, including “baby talk” and selective mutism; limited insight;
    • Auditory and visual hallucinations may occur

Medical history

  • Infectious diseases
    • Any symptoms of a pharyngitis?
      • Classic presentation is pharyngitis  --> then OCD symptoms
      • However, due to severity of pharyngitis, other presentations are possible (Cooperstock, 2017):
        • OCD without any complaints of pharyngitis 
        • OCD symptoms first (i.e. as early as 1-3 days before the onset of group A streptococcal pharyngeal (GAS) symptoms) then pharyngitis 
  • Autoimmune disease
  • Inflammatory disease
  • Immunodeficiency

Family history

  • Movement disorders
  • OCD, hoarding
  • Other conditions such as mood / anxiety disorders
  • Systemic conditions such as rheumatic disorders

Investigations

If you suspect PANS, then order the following as soon as possible:

  • Throat swab
  • ASOT
  • Document when the symptoms started, and when the samples were taken (ideally as close together as possible)
  • Other tests 
    • Complete blood cell count with manual differential

    • Erythrocyte sedimentation rate and

    • C-reactive protein

    • Comprehensive metabolic panel

    • Urinalysis (to assess hydration and to rule out inflammation for children with urinary complaints)

    • Clean-catch urine culture for those with pyuria

​ 6-8 weeks later

  • Repeat ASOT

Diagnosis

Presumptive diagnosis can be made with
 

1) Throat swab (+) plus
 

2) ASOT (+) whereby:

High ASOT associated with OCD or tic-like symptoms

Low, falling ASOT associated with the convalescent phase
 

Note: Combination of Throat swab (+) plus ASOT (+) is required. Having only throat swab (+) or ASOT (+) alone is insufficient to make the diagnosis.

Differential diagnosis

Consider the following differential diagnosis for an individual with PANS:

  • Obsessive compulsive disorder (OCD)
  • Anorexia nervosa
  • Avoidant/restrictive food intake disorder
  • Tourette’s syndrome
  • Transient tic disorder
  • Bipolar disorder
  • Sydenham chorea
  • Autoimmune encephalitis
  • Systemic autoimmune disease
  • Wilson’s disease

Management in Primary Care

For OCD symptoms felt due to PANS (i.e. abrupt onset of neuropsychiatric symptoms and evidence of recent GAS infection, e.g. positive throat swab):

  • Antibiotic course for 7-10 days
    • Even if the episode of GAS was already treated, it is recommended to treat again due to failure rates for penicillin and amoxicillin therapy
    • Antistreptococcal therapy is associated with prompt resolution of PANS symptoms
  • Consider referral to neurology or mental health clinic
     

For OCD symptoms that persist:

  • When a child has symptoms of OCD with PANS, the symptoms of OCD are treated the same way that one would treat a child with OCD symptoms unrelated to PANS
  • First line for mild to moderate OCD
    • Non-medication strategies such as cognitive behavioural therapy (CBT).
    • Consider referral to a mental health professional that provides CBT (e.g. psychologist, mental health clinic, etc.)
    • If non-medication strategies are ineffective, then consider a trial of medication (WFSBP Treatment Guidelines for Anxiety, Obsessive-Compulsive and Post-Traumatic Stress Disorders).
  • First-line for OCD
    • Fluvoxamine (Luvox)
    • Fluoxetine (Prozac)
    • Sertraline (Zoloft)
    • Citalopram (Celexa)
    • Second-line for OCD
    • Clomipramine

Experimental treatments include intravenous immunoglobulin (IVIG) however there is insufficient evidence to recommend IVIG in most individuals, nor is it even available in most centres.

Patient Information 

PANDAS: Frequently Asked Questions about Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections
https://www.nimh.nih.gov/health/publications/pandas/index.shtml 

References

Kellner M: Drug treatment of obsessive-compulsive disorder, Dialogues Clin Neurosci. Jun 2010; 12(2):187-197.

 

Bandelow B, Zohar J, Hollander E, Kasper S, Möller HJ, WFSBP Task Force on Treatment Guidelines for Anxiety, Obsessive-Compulsive and Post-Traumatic Stress Disoders, Zohar J, Hollander E, Kasper S, Möller HJ, Bandelow B, Allgulander C, Ayuso-Gutierrez J, Baldwin DS, Buenvicius R, Cassano G, Fineberg N, Gabriels L, Hindmarch I, Kaiya H, Klein DF, Lader M, Lecrubier Y, Lépine JP, Liebowitz MR, Lopez-Ibor JJ, Marazziti D, Miguel EC, Oh KS, Preter M, Rupprecht R, Sato M, Starcevic V, Stein DJ, van Ameringen M, Vega J. World J Biol Psychiatry. 2008; 9(4):248-312.

 

Cooperstock M et al.: Clinical Management of Pediatric Acute-Onset Neuropsychiatric Syndrome: Part III—Treatment and Prevention of Infections, J. Child and Adolescent Psychopharmacology, 27(7): Sep 1, 2017. 

http://online.liebertpub.com/doi/full/10.1089/cap.2016.0151

About this Document

Written by members of the eMentalHealth.ca/PrimaryCare team which includes members of the Department of Psychiatry and Family Medicine at the University of Ottawa. Special acknowledgements to Dr. Asif Doja, neurologist. Reviewed by members of the Family Medicine Program at the University of Ottawa, including Dr's Farad Motamedi; Mireille St-Jean; Eric Wooltorton.

Disclaimer

Information in this pamphlet is offered ‘as is' and is meant only to provide general information that supplements, but does not replace the information from a health professional. Always contact a qualified health professional for further information in your specific situation.

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Date Posted: Oct 22, 2014
Date of Last Revision: Mar 26, 2018

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