From a Sore Throat to a Cerebellar Abscess and Septic Pulmonary Emboli; an Atypical Presentation of a “Forgotten Disease”

Case Presentation:

A 21‐year‐old female with a history of asthma presented to the emergency department for evaluation of left ear pain, sore throat and productive cough for the past three days. She was found to be febrile and tachycardic, which resolved after receiving antipyretics. Rapid Strep screen was negative and patient was discharged home with presumed viral pharyngitis.

She returned to the emergency department five days later with complaints of headache, neck pain and back pain. Family reported symptoms of confusion, drowsiness and slurred speech over the past two days. On exam patient was somnolent but arousable, ill appearing with dry mucous membranes and in mild respiratory distress. She had left‐sided facial and neck swelling without erythema that was remarkably tender to palpation. A complete neurological exam was difficult due to altered level of consciousness, however no obvious focal deficits were noted. She was hypotensive, tachycardic, afebrile and saturating well on room air. Initial labs revealed leukocytosis, profound thrombocytopenia and elevated creatinine. CT head did not show any intracranial abnormality. CT chest showed multiple bilateral cavitary and non‐cavitary parenchymal lesions; this increased the index of suspicion for Lemierre’s syndrome with septic emboli to the lungs. This prompted imaging of the neck, which showed multiple small retropharyngeal abscesses and thrombophlebitis of left facial vein.

Blood cultures returned positive for Fusobacterium necrophorum. Despite appropriate antibiotic coverage, her respiratory status declined. Repeat imaging of the brain and neck revealed a cerebellar mass and thrombus of the left internal jugular vein. The left internal jugular vein was ligated to prevent further organ damage from septic emboli. Patient had a three‐week hospital course, complicated by empyema requiring VATS.

Discussion:

Lemierre’s syndrome was first coined in 1936, describing a condition that started as tonsillitis and progressed to thrombophlebitis of the internal jugular vein. It is usually caused by the gram negative anaerobe, Fusobacterium necrophorum. Diagnosis is supported by imaging studies that show thrombus in the internal jugular vein. This case of Lemierre’s initially lacked this classic radiographic finding, but instead showed thrombophlebitis of the facial vein.

As antibiotics gained popularity, Lemierre’s syndrome became known as “the forgotten disease”. Fusobacterium necrophorum is generally sensitive to penicillin and amoxicillin, however due to increasing concerns regarding overuse of antibiotics, physicians prefer conservative measures with symptomatic treatment for what is likely a viral illness. As antibiotic stewardship has become customary, there appears to be a resurgence of this syndrome in the literature.

Conclusions:

Unfortunately, the initial clinical presentation of Lemierre’s is vague and resembles viral pharyngitis. A high index of suspicion is needed when making this diagnosis, as it progresses rapidly and is associated with high mortality. It is important to recognize that there may be variations in typical radiographic findings.