Lemierre syndrome and unexpected death in childhood

John D Gilbert1, Morgyn S Warner, Roger W Byard

  • 1Forensic Science SA, Adelaide, Australia.

Insights

Lemierre syndrome, a rare necrotizing head infection, is re-emerging. This case highlights rapid deterioration and death in a child due to Fusobacterium necrophorum.

Area of Science:

  • Infectious Diseases
  • Pediatric Pathology
  • Microbiology

Background:

  • Lemierre syndrome, caused by Fusobacterium necrophorum, is a rare but severe infection historically termed the 'forgotten disease'.
  • Recent literature suggests an increasing incidence of Lemierre syndrome, challenging its 'forgotten' status.
  • The syndrome typically involves necrotizing infections of the head and neck.

Observation:

  • A case report details a 10-year-old boy with a five-day history of ear infection.
  • Symptoms included fever, drowsiness, and ipsilateral neck swelling, rapidly progressing to cardiac arrest.
  • Autopsy revealed right otitis media with contiguous spread of infection.

Findings:

  • Infection extended into subcutaneous tissues, the extradural space, and involved septic thrombophlebitis of the sigmoid sinus.
  • No evidence of meningitis was found.
  • The autopsy confirmed necrotizing infection originating from otitis media.

Implications:

  • This case underscores the potential for rapid, fatal deterioration in children with seemingly common infections.
  • It highlights the importance of recognizing and managing Lemierre syndrome, even in the antibiotic era.
  • An autopsy-guided approach to suspected childhood sepsis is presented as a critical diagnostic tool.

Related Concept Videos

Inborn Errors of Metabolism01:20

Inborn Errors of Metabolism

Phenylketonuria (PKU) is a protein metabolism disorder characterized by high blood levels of the amino acid phenylalanine. This results from a mutation in the gene responsible for phenylalanine hydroxylase, an enzyme that converts phenylalanine into tyrosine. When this enzyme is deficient, phenylalanine builds up in the blood, leading to symptoms such as vomiting, rashes, seizures, growth deficiency, and severe mental retardation. An early diagnosis and a diet restricting phenylalanine intake...
Lethal Alleles02:41

Lethal Alleles

Agouti: A Lethal Allele
Lucien Cuénot discovered lethal alleles in 1905 while studying the inheritance of coat color in mice. The agouti gene is responsible for the color of the coat in mice. This gene codes for an agouti-signaling protein, which is responsible for melanin distribution in mammals. The wild-type allele gives rise to gray-brown coat color in mice, while the mutant allele gives rise to yellow coat color. In addition to coat color, the agouti gene is associated with the yellow...
Lysosomal Hydrolases01:22

Lysosomal Hydrolases

Lysosomes are the site for the degradation of macromolecules and biological polymers released during membrane trafficking events such as secretory, endocytic, autophagic, and phagocytic pathways. The membrane-enclosed area of the lysosome, called the lumen, contains hydrolytic enzymes active in an acidic environment. These acid hydrolases are functional at a pH between 4.5 and 5 and are involved in cellular processes such as cell signaling, energy metabolism, restoration of the plasma membrane,...
Esophageal Achalasia01:27

Esophageal Achalasia

Esophageal achalasia is a chronic neurogenic disorder characterized by impaired relaxation of the lower esophageal sphincter (LES) and absent or ineffective peristalsis in the distal esophagus. This leads to a functional obstruction without a physical blockage, despite significant disruption of esophageal motility.EtiologyAchalasia is caused by degeneration of the myenteric (Auerbach's) plexus, specifically the loss of inhibitory ganglion cells that produce vasoactive intestinal peptide (VIP)...
Rocky Mountain Spotted Fever01:26

Rocky Mountain Spotted Fever

Rocky Mountain Spotted Fever (RMSF) is a severe tick-borne illness caused by Rickettsia rickettsii, a Gram-negative, coccobacillary bacterium. This pathogen is an obligate intracellular parasite, requiring a host cell for replication. Transmission occurs through the bite of an infected tick. In the United States, the most important vectors are Dermacentor variabilis (American dog tick) and Dermacentor andersoni (Rocky Mountain wood tick), though other tick species may also serve as vectors.