Differential Diagnosis of Lemierre's Syndrome in a Patient with Acute Paresis of the Abducens and Oculomotor Nerves
A 22-year-old woman reported to our emergency department with recent swallowing difficulties and throat pain. The clinical investigation revealed a local inflammation of the tonsils on the left side. The patient was otherwise healthy. A rapid test for group A streptococci was negative and the patient was discharged with a diagnosis of acute tonsillitis and symptomatic therapy with ibubrufen.\nSix days later the patient presented at our ophthalmology outpatient department with acute paresis of the abducens nerve and partial paresis of the right oculomotor nerve ( and ). Signs of paresis of the occulomotor nerve included general mydriasis of the right eye with impairment in function of the musculus levator and general impairment in motion of the right eye. Paresis of the right abducens nerve was seen when the patient was looking to the right side. The prism cover test showed +40 pdpt when looking to the right, a primary position at +20 pdpt when looking to the left +10 pdpt. One week later, the patient also developed a discrete paralysis of the abducens nerve on the left eye. In addition, there was an onset of exophthalmos on the right side (18-95-15), which was documented by the Hertel Exophthalmometer. The fundus and retina showed no pathological signs. Also, the neurological investigation revealed general photophobia, incomplete trismus and discrete signs of meningism together with fever. Hematology and clinical chemistry results were compatible with sepsis indicating substantial inflammation with leucocytosis of 16,000/µL (15,000/µL neutrophils, 45% left shift) and C-reactive protein of 271 g/L, activated coagulation with profound thrombocytopenia (21,000/µL), and an international normalized ratio of 1.27.\nTo clarify these inconclusive clinical symptoms, we performed a magnetic resonance imaging (MRI) investigation of the head, which revealed marked thromboses at various locations including the cavernous sinus on both sides (), more pronounced on the right side, and a thrombosis of the superior ophthalmic vein in the right eye (). Moreover, we found a small starting abscess in the right temporal lobe, which grew rapidly (). Due to the numerous symptoms, and the patient's difficulty in opening her mouth, we performed a computed tomography of the neck, which revealed an abscess in the left tonsil with a maximum diameter of 6 mm (). In the right internal jugular vein, we found a partially occluding thrombus. Despite the absence of pulmonary symptoms, we performed an additional computed tomography of the lung, which revealed multiple septic emboli and starting abscesses ().\nGiven the presence of the peritonsillar abscess and the various types of thromboses in intracranial and extracranial veins, we established a diagnosis of Lemierre's syndrome and started high-dose intravenous antibiotic therapy with amoxicillin/clavulanic acid and clindamycin after taking two pairs of blood cultures. In addition, anticoagulation therapy with 20,000 units of heparin was started because of the presence of numerous venous thrombi. The next day, both anaerobic cultures were positive for Gram-negative pleomorphic bacilli, which were subsequently identified as Fusobacterium necrophorum using 16s-RNA sequencing. With minimal inhibitory concentrations of 0.094 to 0.004 mg/L, the isolate was highly susceptible to penicillin, amoxicillin, ceftriaxon and metronidazole. Operative drainage of the left tonsillary abscess was delayed by 5 days due to thrombocytopenia. Although the patient's general clinical condition seemed to improve gradually, she developed a progressive brain abscess in the right temporal lobe (). As a result, she was switched to ceftriaxon and metronidazole for better penetration of the blood-brain barrier. Nevertheless, the patient developed a mycotic aneurysm in the right internal carotid artery (), directly adjacent to the previously thrombosed cavernous sinus. Due to the risk of aneurysm rupture with major bleeding, the patient was transferred from our hospital to a neurosurgical intensive care unit of a large university clinic. Despite intensive anti-coagulation therapy with heparin, the thromboses in the IJV, the cavernous sinus and the ophthalmic vein as well as the cranial nerve palsy persisted during the first two weeks. Three months after that observation period and an intensive antibiotic therapy, the patient was discharged from the hospital in a very good condition. There was only a discrete residual sign of an abducens nerve paralysis on both sides with emphasis on the right side. The oculomotor nerve paralysis on the right side had completely disappeared without any residual paralysis. The patient had no other complications in this follow-up.