Patient Presentation
A 10-year-old male came to clinic with personality changes.
About three weeks ago, he had a sore throat, fever and then after a few days began acting differently.
Initially he made some odd comments that the family noticed and was moodier.
His teacher noticed him asking to wash his hands frequently. She inquired and he said that he didn’t want to eat or drink at school.
When the teacher persisted, he said that the food and water was poisoned.
The teacher contacted his mother. When his mother questioned him, he said that he didn’t know where the food or water came from or who had cooked it at school.
His mother asked him about how he felt about the food and water at home and he said that he wasn’t worried much but that he still had to be careful.
The mother also noticed that he seemed to be washing his hands more at home too.
His mother stated that he seemed to be preoccupied with making sure his homework was done and was in his bag to go to school the next day.
His teacher reported that the quality of his work was unchanged; but he seemed to be having problems concentrating and was working harder and longer to produce the same work.
In the office, he stated that he just felt better after washing his hands and that he wanted to make sure his homework was done.
He denied any other obsessive or compulsive behaviors or thoughts.
Mother and patient said he was functioning otherwise well.
The past medical history showed a boy who was described as “sensitive” but not a worrier. He had some viral-induced bronchospasm as a preschooler.
The family history was negative for any neurological or psychiatric illnesses except for an aunt with post-partum depression and a paternal cousin with attention deficit disorder.
Older family members had coronary artery disease or strokes.
The review of systems was negative including fevers, weight changes, sweating, and chills.
He also denied any visual or auditory hallucinations, tics, orchoreiform movements. He was having problems sleeping.
The pertinent physical exam showed a healthy appearing boy with growth parameters around 25%. He was afebrile with normal pulse and blood pressure.
He easily answered questions with a normal speech rate and content.
He was clean and dressed appropriately.
Skin examination showed some general dry skin on his upper arms, but no rashes and no nailbed abnormalities.
HEENT examination was normal except for minor clear rhinorrhea. His pharynx had no erythema, exudates or ulcers.
Heart examination was normal with no murmur and good pulses.
Neurological examination was normal.
He was Tanner stage 1.
A short mental status examination revealed him to be oriented to time, place, person and situation.
The laboratory evaluation included a rapid strep test and throat culture that were negative. Complete blood count and thyroid function tests were normal.
Anti-streptolysin O titers came back the following day at 494 IU/ml (normal = 0-240 IU/ml).
A diagnosis of possible PANDAS was made. The mother and physician decided to treat with a 10-day course of cefadroxil. Additionally he was referred for psychological counseling.
At the 3 week follow-up, the patient’s clinical course had improved. He had no concerns about his homework, was washing his hands an appropriate amount and didn’t feel compelled to do so.
He still had some worries about the food and water at school but was willing to eat at school if the items were brought from home.
His mother and he were happy with the progress and were going to continue with the cognitive behavioral therapy, with another follow up appointment was scheduled at 6 weeks.
Discussion
PANDAS is an acronym which stands for pediatric autoimmune neuropsychiatric disease associated with streptococcal disease.
It was first described in 1998. The exact pathophysiology is not known but various evidence supports an autoimmune mediated mechanism.
The proposed mechanism is that streptococcal infection produces antibodies that are capable of cross-reacting with specific areas of the brain such as the basal ganglia and then produces behavioral and neuropsychiatric symptoms.
Current treatment is controversial because of lack of research studies. Antibiotic treatment for evidence of current streptococcal infection is usually given. Prophylactic antibiotic treatment is not recommended.
Neuropsychiatric drugs are used for treatment of disabling tics and/or OCD in addition to cognitive behavioral therapy. Immunomodulation therapy such as plasmapheresis is recommended to be reserved for patients participating in research protocols.
Learning Point
Diagnostic criteria for PANDAS is:
- Age – 3 years to puberty
- Tics or obsessive-compulsive disorder (OCD) – based on DSM-IV criteria
- Episodic or up and down course of symptom severity – sudden onset of symptoms with slow gradual resolution over weeks or months, episodes may recur after another streptococcal infection
- Streptococcal infection – evidence of temporally related infection. Such evidence could include a positive throat culture, ASO titers peaking at 3-6 weeks, or anti-DNAse peaking after 6-8 weeks.
Concurrent associated neuropsychiatric symptoms – especially hyperactivity, choreiform movements or tics. Other problems can include: learning problems, depression, anxiety, mood swings, sleep problems, fine or gross motor impairment such as writing problems.
This patient met most of the criteria for PANDAS: he was the proper age, had OCD, had sudden onset of symptoms with slow resolution, had temporal evidence of streptococcal infection and some neuropsychiatric symptoms. However, he did not have hyperactivity, choreiform movements or tics specifically.
Questions for Further Discussion
1. What medical evaluation should be done for patients with acute psychiatric symptoms?
2. What other psychiatric diseases may be associated with an immunological etiology?
3. Is PANDAS associated with development of other psychiatric diseases in the long term?
4. What is the relationship of PANDAS to Sydenham’s chorea?
Related Cases
- Disease
- Symptom/Presentation
- Age
To Learn More
To view pediatric review articles on this topic from the past year check PubMed.
Information prescriptions for patients can be found at MedlinePlus for these topics: Streptococcal Infections, Obsessive Compulsive Disorder and Movement Disorders
To view current news articles on this topic check Google News.
To view images related to this topic check Google Images.
Kurlan R, Kaplan EL,
The Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infection (PANDAS) Etiology for Tics and Obsessive-Compulsive Symptoms: Hypothesis or Entity? Practical Considerations for the Clinician
Pediatrics. 2004;113;883-886.
Murphy TK, Sajid MW, Goodman WK.
Immunology of obsessive-compulsive disorder.
Psychiatr Clin North Am. 2006 Jun;29(2):445-69.
de Oliveira SK.
PANDAS: a new disease?
J Pediatr. 2007 May-Jun;83(3):201-8.
da Rocha FF, Correa H, Teixeira AL.
Obsessive-compulsive disorder and immunology: A review.
Prog Neuropsychopharmacol Biol Psychiatry. 2008 .
ACGME Competencies Highlighted by Case
1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
2. Essential and accurate information about the patients’ is gathered.
3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
4. Patient management plans are developed and carried out.
5. Patients and their families are counseled and educated.
7. All medical and invasive procedures considered essential for the area of practice are competently performed.
8. Health care services aimed at preventing health problems or maintaining health are provided.
9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.
10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.
13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.
17. A therapeutic and ethically sound relationship with patients is created and sustained.
25. Quality patient care and assisting patients in dealing with system complexities is advocated.
Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa Children’s Hospital
Date
May 27, 2008