Background: Lemierre syndrome is a rare septic thrombophlebitis following a head-neck infection in children, adolescents, and young adults. Intracranial involvement in these patients is frequent, a major prognostic factor for early complications when observed at presentation, and among the main causes of death and long-term sequelae. However, with available information limited to case reports and small series, intracranial involvement in this condition has never been explored systematically.

Aims: To systematically describe the patterns of intracranial involvement and neurological symptoms in Lemierre syndrome.

Methods: We analyzed 712 patients described between 2000 and 2017 and identified through systematic search of grey and published literature. We distinguished three types of intracranial involvement: cerebral venous sinus thrombosis, intracranial suppuration, and arterial stroke or carotid damage. In these patients, we characterized neurological manifestations, clinical and demographic characteristics, and rate of death or clinical sequelae.

Results: Among 712 patients, 187 (26.3%) had intracranial involvement, including 146 (78.1%) with cerebral vein thrombosis, most often in the lateral sinus (N = 93), jugular bulb (N=76) or cavernous sinus (N=51; Figure 1); 81 (43.3%) with intracranial suppurations; and 22 (11.8%) with arterial involvement. At least two forms of involvement were found in 51 patients (27%; Figure 2). Neurological symptoms were most commonly focal neurologic signs (57/187, 30%) followed by headache (47/187, 25%) and decreased consciousness (37/187, 19%). Compared with patients without intracranial involvement, these patients had similar sex distribution, but were slightly younger, more likely to have otomastoiditis as primary infection, and less likely to have previously consulted a general practitioner. Patients with intracranial involvement were more likely to die (8.0% vs. 1.5%) and to have sequelae at discharge (8.6% vs. 1.7%).

Thrombosis location in 146 patients with Lemierre syndrome and cerebral venous sinus thrombosis.
Proportional area plot of the patterns of intracranial involvement in 187 of 712 patients with Lemierre syndrome.

Conclusions: Systematic neurologic assessment at presentation and in-hospital neurologic monitoring should be considered in Lemierre syndrome. Pending confirmation from ongoing research, any pattern of intracranial involvement should prompt the search of others.

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ISTH Congress Abstracts - https://abstracts.isth.org/abstract/intracranial-involvement-and-neurologic-manifestations-in-lemierre-syndrome-analysis-of-712-patients/