Intracranial involvement and neurologic manifestations in Lemierre syndrome: analysis of 712 patients.

Objective Lemierre syndrome is a rare septic thrombophlebitis following a head-neck infection in the adolescence and young adulthood. Intracranial involvement in these patient 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, the pattern of intracranial involvement and neurological symptoms in Lemierre syndrome has never been studied systematically.

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

Results Among 712 patients, 187 (26.3 %) had intracranial involvement at any time during their clinical course. These included 146 (78.1 %) with cerebral veins thrombosis, most often in the lateral sinus (N = 93), the jugular bulb (N = 76) or the cavernous sinus (N = 51); 81 (43.3 %) with intracranial suppurations; and 22 (11.8 %) with arterial involvement. At least two forms of involvement were found in 57 patients (27%; Figure). The most common neurological symptoms at presentation were focal neurologic signs (57/187, 30 %) followed by headache (47/187, 25 %) and decreased level of consciousness (37/187, 19 %). Compared with patients without intracranial involvement, those with it had a similar sex distribution, but were slightly younger, more likely to report a previous otomastoiditis as primary infection and less likely to have consulted a general practitioner before the presentation that led to diagnosis (Table). Among patients with a complete clinical follow-up, those with intracranial involvement were more likely to die (8.0 % vs 1.5 %) and, among survivors, to have sequelae at discharge (8.6 % vs 1.7 %).

Conclusion Systematic neurologic assessment at presentation and in-hospital neurologic monitoring may be indicated in patients with Lemierre syndrome. As different intracranial involvements frequently co-occur, the demonstration of any of them should prompt the search of others.

Abb 1. Patterns of intracranial involvement in 141 patients with Lemierre syndrome.

Publication History

Article published online:
18 June 2021