EFNS guidelines on the diagnosis and management of European Lyme neuroborreliosis

TLDR

Lyme neuroborreliosis is a nervous system infection caused by Borrelia burgdorferi sensu lato that can persist as post‑Lyme disease syndrome when symptoms last beyond six months. The guideline aims to provide evidence‑based recommendations for diagnosing and treating Lyme neuroborreliosis. It defines diagnostic criteria (symptoms, CSF pleocytosis, intrathecal antibodies) and recommends a 14‑day antibiotic regimen—oral doxycycline or IV ceftriaxone for peripheral disease, IV ceftriaxone for CNS disease, and a 3‑week course for late LNB—while noting age restrictions for children. PCR and CSF culture are useful only when symptoms are under six weeks; otherwise they are not recommended, and no diagnostic tests such as CXCL13 or antigen detection are supported; doxycycline and IV ceftriaxone are equally effective for peripheral disease, while antibiotic therapy does not benefit post‑Lyme disease syndrome.

Abstract

Lyme neuroborreliosis (LNB) is a nervous system infection caused by Borrelia burgdorferi sensu lato (Bb).To present evidence-based recommendations for diagnosis and treatment.Data were analysed according to levels of evidence as suggested by EFNS.The following three criteria should be fulfilled for definite LNB, and two of them for possible LNB: (i) neurological symptoms; (ii) cerebrospinal fluid (CSF) pleocytosis; (iii) Bb-specific antibodies produced intrathecally. PCR and CSF culture may be corroborative if symptom duration is <6 weeks, when Bb antibodies may be absent. PCR is otherwise not recommended. There is also not enough evidence to recommend the following tests for diagnostic purposes: microscope-based assays, chemokine CXCL13, antigen detection, immune complexes, lymphocyte transformation test, cyst formation, lymphocyte markers. Adult patients with definite or possible acute LNB (symptom duration <6 months) should be offered a single 14-day course of antibiotic treatment. Oral doxycycline (200 mg daily) and intravenous (IV) ceftriaxone (2 g daily) are equally effective in patients with symptoms confined to the peripheral nervous system, including meningitis (level A). Patients with CNS manifestations should be treated with IV ceftriaxone (2 g daily) for 14 days and late LNB (symptom duration >6 months) for 3 weeks (good practice points). Children should be treated as adults, except that doxycycline is contraindicated under 8 years of age (nine in some countries). If symptoms persist for more than 6 months after standard treatment, the condition is often termed post-Lyme disease syndrome (PLDS). Antibiotic therapy has no impact on PLDS (level A).