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A pain in the neck could mean Lemierre's syndrome

Lemierre's syndrome (infected clot in the jugular vein of the neck) develops most often after a strep throat infection has created an abscess near the tonsils, where bacteria migrate to the nearby jugular vein. There they cause an infected clot (thrombosis) that can travel to the lungs or heart, which can be fatal.

In the era before antibiotics, Lemierre's syndrome was common, but the incidence declined in the 1960s and 1970s, as use of antibiotics for pharyngitis (sore throat) became increasingly common. In the past decade, incidence has again increased, and some have attributed this change to the increasingly judicious use of antibiotics for pharyngitis.

A recent article details the clinical steps taken that eventually diagnosed Lemierre's syndrome in a 16-year-old girl who visited her doctor because of a 2-day history of sore throat, fatigue, fever, headache, and vomiting. She had no rhinorrhea or cough. She had a mildly tender anterior neck, but no swollen lymph nodes. Following a strep-negative throat culture, viral pharyngitis was diagnosed and treated accordingly. Four days later, the patient returned to the doctor with worsening sore throat, difficulty swallowing, dizziness while standing, and a headache and neck pain that became increasingly severe. Her appearance and vital signs were alarming, with rapid heartbeat and low blood pressure.

The doctor began aggressive rehydration for suspected deep-tissue infection and, given the possibility of meningitis, performed a lumbar puncture, as well as a computerized tomography scan of her neck, chest, abdomen, and pelvis to evaluate the possibility of lymphoma. There was asymmetric thickening of the right pharyngeal soft tissues, and the right internal jugular vein was occluded to the level of the thoracic inlet. The rest of the vasculature was normal, and there was no lymph node enlargement. Blood cultures showed bacterial infection. These findings were consistent with a diagnosis of septic thrombophlebitis of the internal jugular vein, or Lemierre's syndrome. Following surgery on the jugular vein, the patient recovered with supportive care.

In conclusion, the authors note that the clinicians caring for the patient in this case would have been well served to remember her chief symptom: a pain in the neck. This authors of this article were supported in part by a patient safety grant from the Agency for Healthcare Research and Quality (HS11540).

See "A pain in the neck," by Sandra J. Bliss, M.D., Scott A. Flanders, M.D., and Sanjay Saint, M.D., M.P.H., in the March 4, 2004,  New England Journal of Medicine 350, pp. 1037-1042.

Editor's Note: Another article on clinical problem-solving underscores how a more thorough review of a man's medical history, including earlier exposure to asbestos while in the Navy, could have sped up the eventual diagnosis of malignant peritoneal mesothelioma. For more details, see: Cornia, P.B., Lipsky, B.A., Dhaliwal, G., and Saint, S., "Red snapper or crab?"(AHRQ grant HS11540) in the April 1, 2004, New England Journal of Medicine 350, pp. 1443-1448.

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