A RELATIVELY NEW diagnosis, pediatric autoimmune neuropsychiatric disorder associated with streptococcal infections (PANDAS) describes the abrupt development or worsening of neuropsychiatric abnormalities, particularly obsessive-compulsive disorder (OCD) or tic disorders, following a group A streptococcal (GAS) infection in children.1-3 PANDAS was first described in a 1998 article by Swedo and colleagues that proposed working diagnostic criteria and called for more research into this rare but potentially serious disorder.4 Since 1998, however, the quality of research into PANDAS has been limited by small study populations, low quality, and/or conflicting results. As a result, families affected by this disorder are often left in distress and without definitive answers about their child's condition. To increase awareness, this article focuses on what is currently known about PANDAS and the implications for nursing.
The exact pathophysiology of PANDAS is unknown, although many theories have been proposed. One widely accepted hypothesis is that PANDAS is an autoimmune response triggered by GAS pharyngitis (strep throat) and possibly skin infection, resulting in characteristic neuropsychiatric signs and symptoms such as OCD and tic disorders.3 Neuropsychiatric signs and symptoms are varied and unpredictable and may include irritability, hyperactivity, personality changes, separation anxiety, changes in handwriting, aggression, emotional outbursts, and self-injury behaviors1,5-7 PANDAS is considered to be a subset of pediatric acute-onset neuropsychiatric syndrome (PANS), a similar disorder that does not require a known trigger.8
Diagnosing PANDAS is challenging due to the wide variety of neuropsychiatric signs and symptoms displayed by affected children. To diagnose this disorder, the clinician must first rule out differential diagnoses such as Sydenham chorea, a movement disorder triggered by rheumatic fever. Sydenham chorea has clinical features similar to those of PANDAS but requires a different treatment regimen.1,3,9
PANDAS is a clinical diagnosis based on these five criteria:3,10
- OCD and/or tic disorder (Tourette syndrome, chronic motor or vocal tic disorder). Tics may be multiple, unusual, or complex.
- onset between age 3 years and puberty.
- abrupt onset and episodic course of symptoms.
- history of GAS infection before onset of symptoms. Clinicians should be aware that children with PANDAS may have had a GAS infection without complaining of a sore throat or other symptoms of pharyngeal infection.10 Confirmation of GAS infection requires a positive throat or skin culture or rapid antigen detection test for GAS, or increased antistreptococcal antibody titers.3
- neurologic abnormalities, such as motoric hyperactivity and choreiform movements (irregular jerky movements in distal muscles). Choreiform movements may become apparent when the child stands with eyes closed and arms outstretched, with the fingers slightly separated.10
Because treatment for PANDAS can be prolonged and complex, it requires a multidisciplinary approach. Antibiotic therapy is prescribed when a positive skin or throat culture or rapid antigen detection test indicates an acute GAS infection. Research supports the effectiveness of antibiotic therapy to eliminate the underlying infection and reduce or even eradicate PANDAS symptoms.11,12 However, in some studies the use of antibiotics was ineffective, so evidence for the appropriate use of antibiotics is inconclusive.11,13
Based on the theory that PANDAS is an autoimmune disorder, the clinician may prescribe an immune-modulating therapy such as glucocorticosteroids, plasmapheresis, or I.V. immune globulin. Although the evidence supporting these therapies is also inconclusive, plasmapheresis has shown promise in the reduction of symptom severity.1,6,11,13,14
OCD and tic disorders may be managed with a combination of medication and cognitive-behavioral therapy. For example, a selective serotonin reuptake inhibitor such as fluvoxamine or sertraline may be prescribed to help manage OCD and anxiety.15 Cognitive-behavioral therapy, which teaches foundational coping skills, is an evidence-based treatment for OCD, but little research has been done regarding its value as the main treatment for PANDAS.13 Referral to a mental health provider, neurologist, or other specialist may be indicated based on the nature and severity of the child's symptoms.
Monitor for exacerbations
After treatment, signs and symptoms of PANDAS can recur. Although OCD and tic issues fade with time in some children, they can persist into adulthood in others. Symptom exacerbations may be increasingly severe and continue for months.16
Clinicians and parents must monitor the child closely for any subsequent GAS infections, which can incite a symptom flare. Clinicians should be aware that an occult GAS infection such as a sinus infection, although rare, may linger longer and require a longer course of antibiotics.2
Tell parents to notify the healthcare provider of symptom exacerbations immediately so that prompt, appropriate treatment can be initiated; for example, with another course of antibiotics if indicated to treat a recurring or new GAS infection.12 Monitoring the child's vitamin D levels is recommended, as studies have shown an association between low levels and an increase in infections.12 Research also supports treatment with nonsteroidal anti-inflammatory drugs to reduce the duration of symptom flares.13
Nurses must educate patients and parents to monitor for any signs and symptoms of recurring infection, specifically GAS infection, in the patient or in any of the patient's close contacts. Close surveillance with prompt treatment is recommended to prevent exacerbations and prevent GAS transmission.12 Instruct patients and/or parents to finish the entire course of antibiotic therapy as prescribed, and to replace the child's toothbrush to prevent reinfection.1 Also teach them about any other prescribed medications, including potential adverse reactions.
Because PANDAS is a relatively new phenomenon and is underresearched, most nurses are unaware of the disorder and the impact it has on children and their families. This lack of nursing knowledge is especially problematic during this time when more research is needed. With the science not yet established, the art of nursing is especially important to help families who are struggling to find answers about their child's condition.
Since the identification of PANDAS in 1998, these authors have identified only three articles written from a nursing standpoint, and only one of these involved nursing research to establish evidence-based knowledge about this disorder.6 Nurses should be encouraged to become involved in research to address the many unanswered questions about PANDAS.
Ease the burden on children and families
Nurses cannot ignore the human aspect of PANDAS when children and families are left fearful, frustrated, and without answers. Parents of children affected by this diagnosis have expressed feelings of not being heard or believed by the medical community. When they eventually learned about PANDAS, they were frustrated with the inconsistent information they received.6
Nurses must listen to families' concerns and provide them with exceptional nursing care to begin the healing process. This includes providing resources for education and support. Refer them to www.pandasnetwork.org for information about the latest research and additional resources such as ways to contact support groups. See For more information... for additional resources.
For more information...
Child Mind Institute. PANDAS and PANS: About acute-onset OCDhttps://childmind.org/article/pandas-and-pans-about-acute-onset-ocd
International OCD Foundation. Sudden and Severe Onset OCD (PANS/PANDAS)https://iocdf.org/pandas
National Institute of Mental Health. PANDAS—Questions and Answerswww.nimh.nih.gov/health/publications/pandas/index.shtml
PANDAS Physicians Networkwww.pandasppn.org
3. Pichichero ME. PANDAS
: pediatric autoimmune neuropsychiatric disorder associated with group A streptococci. UpToDate. 2019. http://www.uptodate.com
4. Swedo SE, Leonard HL, Garvey M, et al Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections: clinical description of the first 50 cases. Am J Psychiatry
5. Murphy TK, Storch EA, Lewin AB, Edge PJ, Goodman WK. Clinical factors associated with pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections. J Pediatr
6. McClelland M, Crombez MM, Crombez C, et al Implications for advanced practice nurses when pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS
) is suspected: a qualitative study. J Pediatr Health Care
7. Orlovska S, Vestergaard CH, Bech BH, Nordentoft M, Vestergaard M, Benros ME. Association of streptococcal throat infection with mental disorders: testing key aspects of the PANDAS
hypothesis in a nationwide study. JAMA Psychiatry
11. Farhood Z, Ong AA, Discolo CM. PANDAS
: a systematic review of treatment options. Int J Pediatr Otorhinolaryngol
12. Cooperstock MS, Swedo SE, Pasternack MS, Murphy TKfor the PANS
Consortium. Clinical management of pediatric acute-onset neuropsychiatric syndrome
: part III—treatment and prevention of infections. J Child Adolesc Psychopharmacol
13. Sigra S, Hesselmark E, Bejerot S. Treatment of PANDAS
: a systematic review. Neurosci Biobehav Rev
14. Chiarello F, Spitoni S, Hollander E, Matucci Cerinic M, Pallanti S. An expert opinion on PANDAS
: highlights and controversies. Int J Psychiatry Clin Pract