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Down syndrome and arterial ischemic stroke in childhood: a potential immunologic link with selective IgG4 subclass

Piero Pavone1, Raffaele Falsaperla1, Kasun De Silva2

  • 1Pediatric Acute and Emergency Operative Unit, Vittorio-Emanuele University Hospital, University of Catania, Via Plebiscito n. 628, 95125 Catania, Italy.

European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society
|March 12, 2014
PubMed

Insights

Children with Down syndrome (DS) experiencing infections are at risk for stroke, particularly if they have IgG4 subclass deficiency. This deficiency may explain the increased stroke occurrence in DS patients with infections.

Area of Science:

  • Neurology
  • Immunology
  • Pediatrics

Background:

  • Down syndrome (DS) is associated with an increased risk of various health complications.
  • Cerebral infarction (stroke) in children with DS, especially in the context of infections, warrants further investigation.
  • The role of immune dysregulation, specifically immunoglobulin deficiencies, in DS-related comorbidities is an area of active research.

Observation:

  • Four children with Down syndrome and cerebral infarction during infectious episodes were identified.
  • Infections included Mycoplasma pneumonia, Streptococcus oralis, and unspecified bibasilar pneumonia.
  • All affected children exhibited selective IgG4 subclass deficiency.

Findings:

  • A statistically significant difference in IgG4 subclass levels was observed in DS patients with stroke compared to DS patients with infections but no stroke, and healthy controls (P < 0.001).
  • This finding suggests a strong association between IgG4 subclass deficiency and the occurrence of stroke in children with Down syndrome.
  • The study highlights a potential link between para-infectious stroke and IgG4 subclass deficiency in this population.

Implications:

  • IgG4 subclass deficiency may be a contributing factor to the heightened incidence of stroke in children with Down syndrome during infectious illnesses.
  • Understanding this association could lead to improved diagnostic and therapeutic strategies for preventing stroke in vulnerable DS populations.
  • Further research is recommended to elucidate the precise mechanisms linking IgG4 subclass deficiency, infections, and cerebral infarction in Down syndrome.

Keywords:
ChildhoodDown syndromeInfectious diseaseStroke

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