Pediatric Acute-onset Neuropsychiatric Syndrome

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As a Pediatric Acute-onset Neuropsychiatric Syndrome ( PANS ) is a rapid-onset systemic neurological disease with physical and psychiatric symptoms referred. The first manifestation is always in childhood or adolescence. A chronically progressive , sometimes fluctuating course with a lifelong illness is possible.

Characteristics

PANS is characterized by a strong beginning in children and adolescents. The neurological syndrome presents itself with thought disorders and behavioral abnormalities (especially from the compulsive spectrum ) as well as motor symptoms and / or tics as key symptoms , accompanied by various neurological abnormalities. The time of the onset of PANS is assigned to childhood and early adolescence, but if it becomes chronic there is a possibility of lifelong persistence . In the field of adult medicine, the Post-Inflammatory Brain Syndrome (PIBS) also describes a structurally analogous syndrome.

backgrounds

discovery

PANS was defined and researched at the world's largest research institute for mental illness, the US National Institute of Mental Health (NIMH), a sub-agency of the US Department of Health, as part of PANDAS research. The criteria for PANS are a further development of those of PANDAS, a streptococcus-specific form of PANS that has been known since the 1990s.

causes

PANS is initially a purely clinical diagnosis, the exact pathophysiological causes of which have not yet been fully elucidated. In some cases it appears to be the result of an infection with bacteria or viruses (e.g. streptococci, mycoplasma, borrelia) or other pathogens. Certain metabolic disorders and genetic changes are also suspected to trigger PANS. Changes in inflammation markers ( e.g. neopterin and TNF-α ) and metabolic products (e.g. kynurenine ) are also described. Research into the causes and definition of PANS syndrome is ongoing.

It is assumed that immunological processes act on structures of the brain , especially in the area of ​​the basal ganglia . Research cites chorea minor as a prototypical pathogenic mechanism , with the difference that in PANS the psychiatric symptoms dominate. As a result, there are behavioral changes and motor disorders as well as thought disorders and obsessive-compulsive symptoms 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 PANS / PANDAS syndrome acquired in childhood. Early, consistent treatment is therefore advocated by some researchers in order to avoid chronification. Pathomechanically analogous processes can also be found in other infectious or post-infectious neuropsychiatric syndromes such as anti-NMDA receptor encephalitis , systemic lupus (SLE) or post-inflammatory brain syndrome (PIBS).

Further research

Research into PANS is still ongoing, particularly with regard to etiology , therapy and prognosis . The question of whether tic and obsessive-compulsive disorder occurring for the first time in adulthood can be triggered by reactions to the body's own antibodies or to what extent dyfunctions of the immune system are involved in the development of tic disorders is still being researched. Corresponding first indications of this can be found in immunological studies.

Terminology (PANS vs. PANDAS)

Before the definition of PANS came the discovery of PANDAS in the 1990s , which is now considered to be a sub-category of PANS caused by streptococci. PANS and PANDAS therefore share very similar diagnostic criteria, but PANS does not necessarily have to be caused by a streptococcal infection. The reason for the introduction of the PANS category was that there was increasing evidence from research that a more decisive factor than a certain pathogen could be the presence of certain immunological factors in the patient's brain. PANS therefore subsumes infectious triggers under the name PITANDS on the one hand, and non-infectious reasons on the other, such as B. Metabolic disorders in question. PANDAS is a subgroup of PITANDS. It is assumed that the PANS syndrome may also be caused by mycoplasmas and other pathogens. Various pathogens and genuinely immunological phenomena as possible causes of PANS are in the scientific discussion. The question of a possible genetic vulnerability that can pave the way for the outbreak of the PANS syndrome is still the subject of scientific research. In Europe, the same pathogenic mechanism is also being researched under the name dopamine (post-streptococcal) related autoimmune encephalitis . Due to earlier controversies in scientific circles, the PANS syndrome can also be found in research under the name CANS (Childhood Acute Neuropsychiatric Symptoms). This term, which is little known outside of research and introduced by a non-governmental psychiatric working group, is not in use in public.

Diagnosis and course

The main symptoms (necessarily with a fulminant to foudroyant beginning) correspond to the picture of a dysfunction of the basal ganglia . A spontaneous, rapid onset in childhood and adolescence, often in close proximity to a previous infection, as well as an intermittent course is essential for the diagnosis.

The following diagnostic criteria currently apply (as of 2017):

Sudden onset of at least two of the following symptoms:

Further neurological and / or neuropsychiatric symptoms , of which at least two must be present, lead to the diagnosis "PANS":

  • Movement and balance disorders
  • Sensory hypersensitivity
  • anxiety
  • Emotional lability and / or depression
  • Irritability, aggression and / or behavior inappropriate (rebellious) for the situation
  • Backward development in behavioral development / maturity (in children and adolescents)
  • Deterioration in school, study or work performance
  • Enuresis
  • Disintegration of the handwriting
  • REM sleep disorders
  • Baseless outbursts of anger
  • Development of cognitive deficits
  • Sensory and / or motor abnormalities of various kinds (including deterioration in fine motor skills)
  • Sleep disorders (possibly also reversal of the day / night rhythm) as well as disorders of REM sleep
  • Somatic symptoms such as a. Pain
  • Other neurological-psychiatric abnormalities such as separation fears, cognitive deficits, memory problems, etc. Ä.

Additional criterion:

treatment

Since the PANS syndrome and its subgroups (PITANDS / PANDAS) are still the subject of research, there were no generally recognized guidelines for treatment for a long time. In July 2017, a three-part treatment guideline was published in a special edition of the Journal of Child and Adolescent Psychopharmacology for the first time by consensus of all noteworthy PANS researchers in the USA . The guideline includes:

The National Institute of Mental Health described immune-based treatment options and antibiotic therapies years ago. If a bacterial trigger is proven, prophylactic antibiotics can be effective in preventing further attacks. This research, especially driven by Tanya Murphy at the University of South Florida , has not yet been completed, but antibiotic prophylaxis is already of importance in practice, especially in PANDAS cases. Study results are available specifically for the PANDAS subgroup. Early treatment, symptomatic as well as causal, is also recommended by the new guidelines in order to avoid chronification, as has been described for a long time.

Situation in Germany

The European guideline for Tourette syndrome and other tic disorders ( European clinical guidelines for Tourette syndrome and other tic disorders ) has taken PANS-PANDAS into account since 2011. The German Society for Child and Adolescent Psychiatry names PANS in its treatment guidelines for OCD (2007) 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. The S3 guideline on obsessive-compulsive disorder in adults mentions the PANS subgroup PANDAS as a possible, rare reason for symptoms from the obsessive-compulsive spectrum, but without addressing any therapeutic consequences.

State of research

At the beginning of 2017, the facts listed above were reviewed in a joint review by the Pandas Network (Menlo Park, California), the University at Buffalo (Buffalo, New York) and the Departments of Pediatrics and Psychiatry of the University of South Florida (St. Petersburg, Florida ), approved.

Web links

Individual evidence

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