Lemierre Syndrome with Meningo-Encephalitis, Severe Cerebral Artery Stenosis, and Focal Neurological Symptoms
a Service de Pédiatrie, Centre Hospitalier Intercommunal de Créteil, Créteil, France
A 5-year-old girl presented with otitis-derived meningo-encephalitis. The diagnosis was based on clinical symptoms: purulent otorrhea, meningeal syndrome, convulsions, alteration in consciousness, apnea, secondary enuresis, right cerebellar syndrome; no hemodynamic or respiratory failure, along with biological signs (blood: severe inflammatory syndrome; cerebrospinal fluid: neutrophilia, high protein level) and severe electroencephalographic abnormalities (diffuse bihemispheric slow delta waveforms [Figure 1; available at www.jpeds.com]). She was admitted to the Intensive Care Unit. Blood culture grew Fusobacterium necrophorum, suggesting Lemierre syndrome.
Rare but of increasing incidence, Lemierre syndrome is a head and neck–initiated septicemia caused by an anaerobic germ that induces septic thrombophlebitis of the facio-cervical veins complicated by septic metastasis, with an overall 10% mortality.1, 2 It typically involves a F necrophorum tonsillitis, septicemia, thrombosis of the internal jugular vein, and lung dissemination. The primary infection site usually is otitis media in young children.2
Neuroimaging revealed segmental right internal jugular vein thrombosis (not shown) as well as cavernous sinus thrombophlebitis (Figures 2 and 3; available at www.jpeds.com). An exceptionally severe stenosis with parietal thickening of the right anterior cerebral artery and the right internal carotid artery was demonstrated (Figures 2 and Figure 4, A). The cerebral and cerebellar parenchyma were normal. Metastatic sites included the spleen (disseminated nodules), left talus (scintigraphic hyperintensity), cerebrospinal fluid, brain, and possibly the lung (persistent cough). Hemostatic and immunological explorations were normal.
Figure 4. Carotid artery and anterior cerebral artery involvement in an 5-year-old girl with acute right otomastoiditis and neurologic symptoms. A, 3D TOF MRA coronal view. B, Follow-up coronal 3D TOF MRA image obtained 3 months later shows complete recovery of these arteries.
Neurological infection and inflammation are rare to exceptional features in Lemierre syndrome. Meningitis occurs in 1%.1, 3 Convulsions have been reported in 2 patients.4 Reported focal neurological signs following ischemia,4, 5 abscess,6, 7 or inflammation8 are hemiparesis,5, 6, 7 aphasia,5 and Horner syndrome.8 Arteritis can lead not only to embolism but also to complete occlusion.4, 5
In addition to antibiotic therapy, anticoagulation is debated but strongly recommended by the American College of Chest Physicians.9 Our patient initially received amoxicillin, metronidazole, and low-molecular-weight heparin. The electroencephalogram was normal after 2 weeks; she was discharged on amoxicillin and vitamin K antagonist after 1 month, when the daily 39°C fever, abundant ear discharge, and cough finally receded, and neurological examination and imaging normalized completely after 3 months only (Figure 4, B).
References available at www.jpeds.com
We thank Suzanne Verlhac, Anne Gauthier, Christine Barnerias, and Christophe Delacourt for their clinical and radiological insight.Figure 1.
Electroencephalogram: Diffuse bihemispheric slow delta wave forms, with frontal predominance.Figure 2.
Cavernous sinus thrombosis secondary to otomastoitidis. Axial T1 image after gadolinium administration demonstrated enlargement and pathological enhancement of the right cavernous sinus (arrow) with narrowing of the right internal carotid artery.Figure 3.
Axial CT view. Enlargement and heterogeneity of the right cavernous sinus.
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