PANDAS

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Classification according to ICD-11
8E4A.0 Paraneoplastic or autoimmune disorders of the central nervous system, brain or spinal cord
ICD-11 ( WHO version 2019)

The acronym PANDAS stands for P ediatric A utoimmune N europsychiatric D isorders A ssociated with S treptococcal Infections . It is considered a subcategory of Pediatric Acute-onset Neuropsychiatric Syndrome (PANS). It describes a neuropsychiatric syndrome that is now widely recognized and has already been successfully reproduced in animal experiments , in which neuropsychiatric symptoms suddenly set in in childhood and adolescence after infections with β-hemolytic streptococci of group A , which , if left untreated, can take a long-term, chronic course.

Causes and course

It is assumed that antibodies which are formed in streptococcal infections of the throat and pharynx or the middle ear ( scarlet fever , otitis media ) and are directed against the cell surface of the bacteria cross-react with special structures of the brain - especially the basal ganglia that are responsible for the Coordination of movements are responsible. The assumed pathogenic mechanism essentially corresponds to that of chorea minor , with the difference that in PANDAS the psychiatric symptoms dominate. Imaging studies indicate changes in parts of the brain ( striatum ) in PANDAS patients. As a result, there are behavioral changes and motor disorders as well as an obsessive-compulsive disorder with a sudden, often fulminant onset, which, if left untreated, can become chronic and persist for life. Some of the cases of obsessive-compulsive disorder and tic disorder in adults may also be the result of a PANDAS syndrome acquired in childhood. Early, consistent treatment is therefore advocated by some researchers in order to avoid chronification.

PANDAS vs. PANS

The state National Institute of Mental Health of the USA has subsumed PANDAS as a subcategory of PANS Pediatric Acute-onset Neuropsychiatric Syndrome since 2012 . The clinical diagnosis PANS was declared a generic term based on the current state of knowledge. PANS is an immunologically caused neurological syndrome with neuropsychiatric and systemic symptoms. Current research suggests that the presence of basal ganglia antibodies in the patient's brain may be the result of a previous infection. On the one hand, PANS summarizes infectious triggers under the name PITANDS, on the other hand non-infectious causes such as metabolic disorders come into question. PANDAS is a subgroup of PITANDS. A possible cause of PANS by mycoplasma and other pathogens is also assumed and currently being researched.

Symptoms

Diagnostic criteria for PANDAS according to the National Institute of Mental Health (NIMH) are:

  1. Abrupt, dramatic onset of symptoms (behavioral and thought disorders ) from the compulsive spectrum and / or tics or refusal to eat.
  2. Simultaneous presence of additional neuropsychiatric symptoms of similar severity and also sudden onset. There must be at least two of the following categories:
    1. anxiety
    2. Emotional lability and / or depression
    3. Irritability, aggression and / or behavior inappropriate to the situation
    4. Backward development in behavioral development / maturity (in children and adolescents)
    5. Deterioration in school or work performance
    6. Sensory and / or motor abnormalities (including deterioration in handwriting or dysgraphia )
    7. Somatic symptoms such as B. Difficulty sleeping, enuresis (bed-wetting) and / or a change in the frequency of urination
    8. Other neurological-psychiatric abnormalities such as separation fears, cognitive deficits, memory problems, etc. Ä.
  3. The symptoms cannot be better explained by other diseases, e.g. B. by chorea minor (Sydenham chorea), lupus erythematosus , Tourette's syndrome and others.

state of research

As a syndrome that is still being researched, PANDAS is not yet listed in the ICD-10 or DSM-5 as an independent disease, but it was discussed in the context of the development of the DSM-5 and described as a real existing entity. The European guideline for Tourette's syndrome and other tic disorders ( European clinical guidelines for Tourette syndrome and other tic disorders ) PANDAS included since 2011. The German Society for Child and Adolescent Psychiatry mentions in its treatment guidelines for obsessive-compulsive disorder (as of 2007) the PANDAS- syndrome and the need to determine the antistreptolysin - titer in children and adolescents with newly broken-compulsive disorder, who have undergone recently an infection allegedly caused by streptococci. PANDAS is also taken into account in the new German S3 guideline on obsessive-compulsive disorder in adults.

In 2011, the International OCD Foundation publicly demanded for the first time that a streptococcal antibody test should generally be carried out on children with suddenly onset tic or obsessive-compulsive disease.

The research also increasingly focuses on the question of whether tic and obsessive-compulsive disorders in adulthood can also be triggered by the mechanism described above. Corresponding first indications of this are given by a work of the "Dipartimento di Scienze Psichiatiche e Medicina Psicologica" of the Sapienza University in Rome as well as further studies. In Germany, too, PANDAS is now the subject of studies and scientific work as a research concept in the field of tic and obsessive-compulsive diseases.

At the end of 2011, the NIMH initiated a long-term study to clarify whether intravenous immunoglobulin therapy can relieve symptoms caused by PANDAS. This study should deliver the first reliable results in 2016 and, if successful, would enable a causal therapy that does not yet exist. The study was completed in 2017.

Overall, PANDAS is still in intensive research regarding its status as an independent clinical picture as well as its consequences.

Discovery and History

A connection between rheumatic fever , Sydenham's chorea and obsessive-compulsive symptoms was already known in research in the 1970s and -80s. PANDAS was systematically researched and named as a symptom complex for the first time in 1998 by S. E. Swedo and co-authors. The authors put forward the hypothesis that this is an incomplete form of chorea minor , as occurs in rheumatic fever . The US NIMH, the International OCD Foundation and the German Society for Child and Adolescent Psychiatry are already taking PANDAS seriously. The last major criticism of the PANDAS concept came in 2004 from Kaplan and Kurlan. They criticized the fact that, on the one hand, the diagnostic criteria were formulated too vaguely and, on the other hand, insufficiently secured by prospective studies. The question of practical clinical diagnostic and therapeutic consequences therefore arises in particular. They do not completely reject the concept of PANDAS, but come to the conclusion that the PANDAS hypothesis needs more thorough research. Practical diagnostic or therapeutic measures should only be derived after further controlled prospective studies are available. Leonard and Swedo have used this criticism as an occasion for further work, which is intended to refute the objections of Kurlan and Kaplan.

Roger Kurlan and Harvey S. Singer, Donald L. Gilbert, David S. Wolf and Jonathan W. Mink published another article in the journal Pediatrics in December 2011 , in which the concept of antibody-induced psychiatric and / or neurological disorders is recognized becomes. However, Kurlan, together with the other authors, now calls for the PANDAS, which is limited to streptococcal antibodies, to be subsumed under a condition called “Childhood Acute Neuropsychiatric Symptoms” (CANS). The background is that the current state of research has shown that other antibodies and autoimmune processes can also cause PANDAS-like symptoms. This discussion resulted in the "agreement" on a white paper on PANS (Pediatric Acute-onset Neuropsychiatric Syndrome) at the beginning of 2012 . Like PANDAS, PANS describes the phenomenon of rapidly onset obsessive-compulsive disorder and tic disorders in childhood, but assumes that several infectious and non-infectious triggers are possible causes. PANDAS as a syndrome caused by streptococci would then only be a subcategory of PANS. The NIMH now recognizes this redefinition.

A documentary film "My kid is not crazy" about the long-term observation of affected children and their families has been available about PANS since 2016. The author Tim Sorel also lets medical professionals have their say. The film toured the United States with great success.

The PANDAS Network initiative for "PANDAS PANS Awareness Day" is successful in many American states.

treatment

Since PANDAS is still being researched, treatment is usually symptomatic. Studies have shown that immune-based and antibiotic therapies are promising causative treatments. If streptococcal infection is proven, antibiotics may be effective. The research on this has not yet been completed, however, and antibiotic prophylaxis has not yet found its way into everyday clinical practice. To date, only study results on the PANDAS subgroup are available. Early treatment, symptomatic as well as causal, seems advisable in order to avoid chronification. A pan-European series of studies ( EMTICS ) is to investigate which influencing factors (e.g. genetics, infections and autoimmune factors) play a role in the development of tic disorders by 2017. The German Society for Neurology therefore recommends that, until the results of the Europe-wide study are available, if PANDAS is suspected, long-term antibiotics or immunosuppressive therapy should be avoided.

Web links

Individual evidence

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  3. ↑ a b nimh.nih.gov
  4. ↑ a b wsj.com
  5. ↑ acnp.org ( Memento of the original from October 5, 2013 in the Internet Archive ) Info: The archive link was inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice. (PDF; 159 kB) p. 1595 ff. @1@ 2Template: Webachiv / IABot / www.acnp.org
  6. ↑ L. Brimberg, I. Benhar et al. a .: Behavioral, pharmacological, and immunological abnormalities after streptococcal exposure: a novel rat model of Sydenham chorea and related neuropsychiatric disorders. In: Neuropsychopharmacology Volume 37, Number 9, August 2012, pp. 2076-2087, doi: 10.1038 / npp.2012.56 , PMID 22534626 , PMC 3398718 (free full text).
  7. ↑ D. Zhang: Anti-streptococcus IgM antibodies induce repetitive stereotyped movements: cell activation and co-localization with Fcα / μ receptors in the striatum and motor cortex. In: Brain Behav Immun . 2012 May, 26 (4), pp. 521-533, PMID 22285613 .
  8. ↑ K. Yaddanapudi, M. Hornig, R. Serge u. a .: Passive transfer of streptococcus-induced antibodies reproduces behavioral disturbances in a mouse model of pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection. In: Mol Psychiatry. 2010 Jul, 15 (7), pp. 712-726, doi: 10.1038 / mp.2009.77 . Epub 2009 Aug 11.
  9. ^ Germana Moretto, Pasquini Massimo a. a .: What every psychiatrist should know about PANDAS: a review . Department of Psychiatric Sciences and Psychological Medicine, “Sapienza” University of Rome. In: Clinical Practice and Epidemiology in Mental Health. 2008.
  10. ↑ Y. Hachiya, R. Miyata, N. Tanuma, et al. a .: Autoimmune neurological disorders associated with group-A beta-hemolytic streptococcal infection. In: Brain Dev. 2012 Nov 8. doi: 10.1016 / j.braindev.2012.10.003 , PMID 23142103 .
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  16. ^ B. Coffey, N. Wieland: Tics, anxiety, and possible PANDAS in an adolescent. In: J Child Adolesc Psychopharmacol . 2007 Aug, 17 (4), pp. 533-538.
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  24. ↑ Susan E. Swedo, James F. Leckman, Noel R. Rose: From Research Subgroup to Clinical Syndrome. Modifying the PANDAS Criteria to define PANS . pandasnetwork.org ( Memento of the original dated December 8, 2013 in the Internet Archive ) Info: The archive link was inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice. (PDF; 1.0 MB) @1@ 2Template: Webachiv / IABot / pandasnetwork.org
  25. ↑ H. Rhee, DJ Cameron: Lyme disease and pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS): an overview. In: Int J Gen Med. 2012, 5, pp. 163-174. doi: 10.2147 / IJGM.S24212 , PMID 22393303 . Epub 2012 Feb 22.
  26. ↑ Hanna Rhee, Daniel J Cameron: Lyme disease and pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS): an overview. In: International Journal of General Medicine. 2012.
  27. ↑ a b Esther Entin: Pediatric Acute Onset Neuropsychiatric Syndrome, PANS . 2012.
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  29. ^ Harvard University
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  32. ↑ TK Murphy, EA Storch, AB Lewin u. a .: Clinical factors associated with pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections. In: J Pediatr . 2012 Feb, 160 (2), pp. 314-319. doi: 10.1016 / j.jpeds.2011.07.012 . Epub 2011 Aug 25.
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  35. ↑ Guidelines for Obsessive Compulsive Disorder . ( Memento of the original from October 15, 2014 in the Internet Archive ) Info: The archive link was inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice. (PDF; 568 kB) awmf.org @1@ 2Template: Webachiv / IABot / www.awmf.org
  36. ↑ German Society for Psychiatry and Psychotherapy, Psychosomatics and Neurology: S3 guideline for obsessive-compulsive disorder (AWMF register number 038/017), May 2013.
  37. ↑ iv-ts.de ( Memento of the original from September 24, 2011 in the Internet Archive ) Info: The archive link was inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice. @1@ 2Template: Webachiv / IABot / www.iv-ts.de
  38. ↑ Sagnik Bhattacharyya, Sumant Khanna, Rita Christopher u. a .: Anti-Brain Autoantibodies and Altered Excitatory Neurotransmitters in Obsessive – Compulsive Disorder . In: Neuropsychopharmacology. 2009, pp. 2489-2496.
  39. ↑ E. Hollander, S. Kim, A. Braun u. a .: Cross-cutting issues and future directions for the OCD spectrum. In: Psychiatry Res. 2009 Nov 30, 170 (1), pp. 3-6, doi: 10.1016 / j.psychres.2008.07.015 , PMID 19811839 . Epub 2009 Oct 6.
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  41. ↑ clinicaltrials.gov
  42. ↑ Clinical Management of Pediatric Acute-onset Neuropsychiatric Syndrome (PANS): Part II - Use of Immunomodulatory Therapies . In: C PANS / PANDAS Consortium, PANS Clinic, Stanford University. (Ed.): J Child Adolesc Psychopharmacol, planned 2017, currently in Peer Rv. 2017.
  43. ^ Margo Thienemann, Tanya Murphy, James Leckman, Richard Shaw, Kyle Williams: Clinical Management of Pediatric Acute-Onset Neuropsychiatric Syndrome: Part I - Psychiatric and Behavioral Interventions . In: Journal of Child and Adolescent Psychopharmacology . tape 27 , no. 7 , September 1, 2017, ISSN  1044-5463 , p. 566-573 , doi : 10.1089 / cap.2016.0145 , PMID 28722481 , PMC 5610394 (free full text).
  44. ↑ Jennifer Frankovich, Susan Swedo, Tanya Murphy, Russell C. Dale, Dritan Agalliu: Clinical Management of Pediatric Acute-Onset Neuropsychiatric Syndrome: Part II - Use of Immunomodulatory Therapies . In: Journal of Child and Adolescent Psychopharmacology . tape 27 , no. 7 , July 19, 2017, ISSN  1044-5463 , p. 574-593 , doi : 10.1089 / cap.2016.0148 ( liebertpub.com [accessed October 3, 2017]).
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  46. ^ Michael B. First: DSM 5 - Obsessive Compulsive Spectrum Disorders Conference, 2006 . American Psychiatric Association, DSM-5 Development. dsm5.org ( January 8, 2015 memento in the Internet Archive ) Retrieved September 21, 2012.
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  53. ↑ pandasnetwork.org ( Memento of the original dated December 8, 2013 in the Internet Archive ) Info: The archive link was inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice. (PDF; 1.0 MB) @1@ 2Template: Webachiv / IABot / pandasnetwork.org
  54. ↑ Home | My Kid Is Not Crazy . In: My Kid is Not Crazy . ( mykidisnotcrazy.com [accessed October 3, 2017]).
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  56. ↑ intramural.nimh.nih.gov (PDF; 94 kB)
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  61. ↑ Demian Obregon, Ellisa Carla Parker-Athill, Jun Tan, Tanya Murphy: Psychotropic effects of antimicrobials and immune modulation by psychotropics: implications for neuroimmune disorders. In: Neuropsychiatry. (London), 2012 August, 2 (4), pp. 331-343. doi: 10.2217 / npy.12.41 .
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  64. ↑ Lisa A Snider, Lorraine Lougee, Marcia Slattery, Paul Grant, Susan Swedo: Antibiotic Prophylaxis with Azithromycin or Penicillin for Childhood-Onset Neuropsychiatric Disorders. In: Biol Psychiatry. 2005, 57, pp. 788-792.
  65. ↑ M. Murphy, M. Pichichero: Prospective identification and treatment of children with pediatric autoimmune neuropsychiatric disorder associated with group A streptococcal infection (PANDAS). In: Arch Pediatr Adolesc Med. 156, 2002, pp. 356-361.
  66. ↑ Lisa A. Snider, Lorraine Lougee, Marcia Slattery and others. a .: Antibiotic Prophylaxis with Azithromycin or Penicillin for Childhood-Onset Neuropsychiatric Disorders . (PDF; 97 kB) In: Biol Psychiatry. 2005, 57, pp. 788-792.
  67. ↑ the EMTICS study site
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