Neuropediatrics 2023; 54(S 01): S1-S32
DOI: 10.1055/s-0043-1777152
Neuroimmunologie

The Swiss Pediatric Inflammatory Brain Disease Cohort Study: First Insights into Epidemiology

Authors

  • L. Hulliger

    1   Institute of Social and Preventive Medicine, University of Bern, Bern, Schweiz

  • F. Bauder

    2   Department of Child Neurology, Kantonsspital Winterthur, Winterthur, Schweiz

  • A. Capone Mori

    3   Department of Child Neurology, Clinic for Children and Adolescents, Kantonsspital Aarau, Aarau, Schweiz

  • P. Dill

    4   Department of Child Neurology, University Children's Hospital Basel, Basel, Schweiz

  • S. Garcia-Tarodo

    5   Department of Child Neurology, University Hospitals of Geneva (HUG), Geneva, Schweiz

  • B. Goeggel-Simonetti

    6   Department of Child Neurology, Paediatric Institute of Southern Switzerland, Ospedale San Giovanni, Bellinzona, Schweiz

  • A. Hackenberg

    7   Department of Child Neurology, University Children's Hospital of Zurich, Zurich, Schweiz

  • J. Kalser

    8   Department of Child Neurology, University Children's Hospital Lausanne (CHUV), Lausanne, Schweiz

  • C. Kuehni

    1   Institute of Social and Preventive Medicine, University of Bern, Bern, Schweiz

  • O. Maier

    9   Department of Child Neurology, Children's Hospital of Eastern Switzerland, St. Gallen, Schweiz

  • G. Oesch Nemeth

    10   Department of Child Neurology, University Children's Hospital Bern, Bern, Schweiz

  • R. Schmid

    2   Department of Child Neurology, Kantonsspital Winterthur, Winterthur, Schweiz

  • S. Strozzi

    11   Division of Neuropaediatrics and Neuropsychology, Department of Child and Adolescent Medicine, Kantonsspital Graubünden, Chur, Schweiz

  • S. Bigi

    12   Division of Pediatric Neurology, Department of Pediatrics, Children's Hospital Lucerne, Lucerne, Schweiz
Further Information
 

Background: Pediatric-onset inflammatory brain diseases (P-IBDs) such as pediatric multiple sclerosis (POMS) are rare conditions affecting children during key periods of brain development. We present the first epidemiological data from our recently established national P-IBD registry.

Methods: The registry includes all patients with a P-IBD living or treated in Switzerland. Data are available from 2015 onward, and prospective data collection started in 2019. Patients with infectious/metabolic etiologies or Guillain-Barré syndrome are excluded. Continuous variables are summarized as median (IQR); categorical variables are given as proportions (%). The incidence rate of P-IBDs was calculated using the population data from the Swiss Federal Office of Statistics.

Results: So far, 225 patients (139 females, 62%) with a minimal and 113 patients (69 females, 61%) with a full dataset have been enrolled. POMS (108, 48%), acute disseminated encephalomyelitis (30, 13%), and optic neuritis (23, 10%) are the most frequent diagnoses. The incidence rate of P-IBD and POMS is 1.35/100,000 and 0.51/100,000 children, respectively. Age at symptom onset and diagnosis is 11.4 years (IQR: 7.4 years) and 11.9 years (IQR: 6.8 years), respectively. The most frequent initial symptoms were visual deficits (12%), nausea/vomiting (11%), and headaches (11%). Among the 40 POMS patients with a full dataset, visual deficits (19%), fatigue (9%), and hemisyndrome (9%) were most frequently reported and 37 (93%) received immunotherapy 62 days (IQR: 51 days) after diagnosis. Fingolimod and interferon β were used equally often as initial immunotherapy (11, 28% for both substances).

Conclusion: Demyelinating diseases and particularly POMS are the most frequent P-IBD in Switzerland with incidence rates comparable to the international literature. The registry allows for a structured and systematic analysis of Swiss P-IBD patients and provides a platform for future collaborative studies.



Publication History

Article published online:
13 November 2023

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