Lemierre syndrome leading to an ischaemic stroke and malignant cerebral oedema
- 1 Neurosurgery, University of Puerto Rico Medical Sciences Campus, San Juan, Puerto Rico, Puerto Rico
- 2 Department of Radiological Sciences and Diagnostic Radiology, Neuroradiology, University of Puerto Rico Medical Sciences Campus, San Juan, Puerto Rico, Puerto Rico
- Correspondence to Dr Orlando De Jesus; drodejesus@aol.com
Abstract
Lemierre syndrome is an often misdiagnosed disease caused by an anaerobic bacterial infection that produces thrombophlebitis of the internal jugular vein and septic metastasis to distal organs, especially the lungs. Carotid stenosis or thrombosis is a rare complication. We present a patient with Lemierre syndrome who developed malignant cerebral oedema secondary to an ischaemic stroke which required a decompressive craniectomy.
Background
Lemierre syndrome (LS) is a septic thrombophlebitis of the internal jugular vein (IJV) caused by an anaerobic bacterial infection of the head/neck area.1 Lemierre described these findings and reported them to be caused by Bacillus funduliformis, now known as Fusobacterium necrophorum.2 This syndrome is considered the ‘forgotten disease’ due to its rarity, with an incidence of nearly 3 cases per 1 million people.3 We present a rare case of LS with thrombosis of the IJV and infectious vasculitis of the internal carotid artery (ICA), producing multiple ischaemic cerebral strokes. The patient developed malignant cerebral oedema, which required an emergent decompressive craniectomy.
Case presentation
A 36-year-old male patient with poor oral hygiene had progressive left-sided tooth pain for the past 2 weeks due to an untreated dental cavity. The patient presented to our emergency department with low-grade fever, chills, unilateral neck pain and nasal sinus obstruction. He had no neurological deficits on examination. He had no significant medical history. The patient was admitted to the hospital for further workup and treatment.
Investigations
Blood analysis showed an increased white cell count of 33 x 10ˆ9/L, C reactive protein of 25 mg/dL and positive blood cultures with a Gram-negative rod-shaped bacillus. Broad-spectrum antibiotics (piperacillin 4.5 g every 6 hours, tazobactam 4.5 g every 6 hours and meropenem 2 g every 8 hours) were immediately started. A head CT angiography demonstrated pansinusitis with dehiscence of the anterior cranial fossa floor and dehiscence of the left carotid canal with direct communication to the underlying sphenoid sinus, causing a severely irregular and stenotic ICA (figure 1). Neck CT angiography showed a thrombosed left IJV (figure 2). With this finding, the patient was started on enoxaparin 1 mg/kg daily. An MRI of the brain showed ischaemic infarcts in the left thalamus, basal ganglia and internal capsule, most likely due to the development of vasospasm and ICA infectious arteritis (figure 3).
(A) Axial head CT angiogram shows pansinusitis (red arrows) and left carotid canal dehiscence, causing direct communication of the canal with the opacified left sphenoid sinus and severe irregular left internal carotid artery stenosis (yellow arrow). (B) Coronal oblique head CT angiogram reconstruction shows dehiscence of the left planum sphenoidale (yellow arrow), exposing the anterior cranial fossa.
(A) Axial neck CT angiogram shows the left internal jugular vein with central non-enhancement and only a thin rim of peripheral enhancement (yellow target cross), most consistent with thrombosis. (B) Head CT angiogram maximum intensity projection shows multifocal stenosis (multiple yellow arrows) involving the left internal carotid artery, anterior cerebral artery and middle cerebral artery as well as, to a lesser degree, the left vertebral artery and right internal carotid artery.
(A) Coronal T1-weighted contrast-enhanced brain MRI shows linear enhancement at the left Sylvian fissure, along the M1 segment of the left middle cerebral artery (yellow arrows), suggestive of leptomeningitis and arteritis. Axial diffusion (B) and apparent diffusion coefficient map (C) brain MRI sequences demonstrate restricted diffusion at the left basal ganglia, left thalamus, and posterior limb of the left internal capsule (yellow arrow).
Treatment
Three days after hospitalisation, the patient underwent functional endoscopic sinus surgery for surgical drainage of the sphenoid sinus with positive intraoperative cultures for F. necrophorum. Initial blood culture results were also positive for F. necrophorum. Contrast echocardiography revealed no signs of thrombosis, vegetations, nor a patent foramen ovale. The following day, the patient developed a right 3/5 haemiparesis but was alert and oriented. Follow-up MRI showed ischaemic infarct progression and progressive ventriculitis with imaging findings at the suprasellar cistern suggestive of purulent debris (figure 4). The patient began complaining of shortness of breath, and a chest CT scan with contrast showed multiple peripheral parenchymal lesions suggestive of septic emboli (figure 5).
(A) Brain MRI axial fluid-attenuated inversion-recovery (FLAIR) sequence shows lack of expected cerebrospinal fluid signal suppression at the suprasellar cistern (yellow circle) (B) Corresponding restricted diffusion in the apparent diffusion coefficient map (yellow circle); suggesting the presence of purulent debris in the subarachnoid spaces.
Axial chest CT scan in the lung window shows multiple peripheral parenchymal nodularities (yellow arrows), some with cavitations (red arrow), a pattern that can be seen in the setting of septic emboli.
On the seventh posthospitalisation day, the patient became tachycardic, tachypneic, aphasic, lethargic and localising only to painful stimuli. He was hypoxic and required emergent intubation due to respiratory failure. An emergent head CT scan showed a new proximal ICA artery ischaemic infarct with malignant cerebral oedema and associated midline shift (figure 6A). An emergent decompressive craniectomy was performed (figure 6B).
(A) Axial head CT scan showing extensive left haemispheric oedema (black arrowhead) corresponding to the left anterior cerebral artery, middle cerebral artery and posterior cerebral artery vascular territories; most consistent with known ischaemic infarct, causing marked left to right midline shift and ventricular compression. (B) Axial head CT scan after left decompressive craniectomy (yellow arrows) with the resolution of midline shift.
Outcome and follow-up
After the decompressive craniectomy, he had a slowly clinical improvement. He underwent a tracheostomy and gastrostomy and was discharged to an inpatient rehabilitation centre after 18 days in the hospital, where he completed 42 days of antibiotic therapy for the infectious process.
At the 3 months follow-up, the patient remained with unimproved right haemiparesis and motor aphasia. He followed commands adequately and performed activities of daily living with minimal assistance. Six months after hospitalisation, a custom peek allograft cranioplasty was implanted at the craniectomy defect.
Discussion
LS mainly affects healthy young adults after a peritonsillar abscess that spreads to the lateral pharyngeal space and causes thrombophlebitis of the IJV, leading to septicaemia and producing septic emboli, particularly to the lungs and joints.3 4 The mortality rate of LS ranges from 4% to 18%, and the diagnosis is established through clinical symptoms, radiological findings and positive microbiological cultures.3–7 Involvement of the ICA is extremely rare, and its pathogenesis is most likely secondary to infectious vasculitis.8 The vasculitis causes an atypical circumferential arterial wall thickening, inflammation, endothelial dysfunction, vasospasm and stenosis, leading to cerebral infarcts.9 10 A rapid diagnosis and treatment are essential to prevent severe complications and death.11 12 Traditionally, the treatment of choice has been penicillin, although clindamycin, metronidazole or ampicillin–sulbactam for 6 weeks are alternatives for resistant strains.11 The role of anticoagulation is controversial; the risks and benefits should be weighed carefully before treatment since direct outcome measures have not been reported, given the rarity of the disease.13
LS may be complicated with stenosis of the ICA.8–10 14 15 Thrombosis of the ICA in LS is a very rare complication, with a few cases reported in the literature.16–20 Most cases of LS involving cerebrovascular strokes occur in the paediatric population.21 22 Goyal et al described the only case of F. necrophorum pharyngitis complicated with a massive stroke causing malignant cerebral oedema and death; however, there was no evidence of IJV thrombosis.16 They reported the case as a variant of LS. Valerio et al recently published a systematic review and found that patients with LS had a significant risk for thromboembolic complications and death, with an estimated 4% mortality in the antibiotic era.23 This mortality rate is dramatically lesser than 90% in the preantibiotic era. Our case reminds us of the possible complications secondary to LS, including thrombosis of the IJV, thromboembolic events, cerebral infarcts and malignant cerebral oedema, which may ultimately require an emergent decompressive craniectomy for its treatment.
Learning points
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Lemierre syndrome can develop ischaemic cerebral infarcts and malignant cerebral oedema.
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Early diagnosis and treatment of cerebral strokes and cerebral oedema can prevent significant morbidity.
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If a large internal carotid artery stroke with malignant cerebral oedema occurs, decompressive craniectomy is recommended.
Ethics statements
Footnotes
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Contributors All the authors contributed equally to the manuscript.Concept and design: CCC, ODJ, EJL and EAP. Drafting the manuscript: CCC, ODJ, EJL and EAP. Revising the manuscript: CCC, ODJ, EJL and EAP. Approval of the final manuscript: CCC, ODJ, EJL and EAP.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Competing interests None declared.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.
References
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