Lyme disease

John J Halperin MD (

Dr. Halperin of Overlook Medical Center and Sidney Kimmel Medical College of Thomas Jefferson University has stock ownership in Merck, Abbott, Abbvie, and Johnson & Johnson.

)
John E Greenlee MD, editor. (

Dr. Greenlee of the University of Utah School of Medicine received an honorarium from Merck for authorship and compensation as an expert witness from Wheeler Trigg O'Donnell LLP.

)
Originally released May 8, 1995; last updated August 5, 2018; expires August 5, 2021

This article includes discussion of Lyme disease, erythema migrans disease, Garin-Bujadoux-Bannwarth syndrome, Lyme borreliosis, neuroborreliosis, Lyme disease associated meningitis, cranial neuritis, radiculitis, and facial nerve palsy. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Ten percent to 15% of patients with Lyme disease, infection with the tick-borne spirochete Borreliella burgdorferi, develop nervous system involvement. Controlled studies indicate that neuroborreliosis is generally curable with oral antibiotics, particularly doxycycline. Studies further indicate that persistent symptoms months after treatment are not only unrelated to nervous system infection but occur with the same frequency in treated patients and healthy controls. Diagnosis still rests on 2-tier serologic testing, with positive or equivocal ELISAs validated by Western blots; however, alternative paradigms, such as sequential recombinant ELISAs, show considerable promise. CSF CXCL13 concentration may provide additional support for the diagnosis of active CNS neuroborreliosis.

Key points

 

• Lyme disease, infection with the tick-borne spirochete Borreliella burgdorferi, affects the central or peripheral nervous system in up to 10% to 15% of patients.

 

• Clinical phenomena associated with neuroborreliosis typically include cranial neuropathy (most often the facial nerve), radiculopathy, and lymphocytic meningitis.

 

• Serodiagnosis after the first month of infection has high sensitivity and specificity.

 

• Treatment with 2- to 4-week courses of oral antibiotics is curative in most; parenteral treatment is recommended either if there is evidence of parenchymal brain or spinal cord involvement or if objectively demonstrable active disease persists after appropriate oral treatment.

 

• Persisting difficulties after treatment, often referred to as “post Lyme disease syndrome”, may occur but are not associated with nervous system infection and probably are no more common than in controls.

Historical note and terminology

The term "Lyme arthritis" was first introduced in 1977 and was subsequently broadened to "Lyme disease" when it was recognized that the disorder commonly involved multiple organ systems in addition to joints (Steere et al 1977). However, the syndrome was described much earlier in the 20th century. In 1910, Afzelius first reported the typical cutaneous lesion, erythema migrans (formerly known as erythema chronicum migrans). In 1922, Garin and Bujadoux described tick bite-associated meningoradiculitis, the most typical neurologic presentation in this disorder. Following a more detailed description of this syndrome by Bannwarth, the notion that bites of Ixodes ticks could lead to a syndrome of lymphocytic meningitis with painful radiculoneuritis became widely accepted by European clinicians. The first reports of Lyme arthritis described cases of what was thought to be juvenile rheumatoid arthritis among children in the region of Lyme, Connecticut (Steere et al 1977). Detailed epidemiologic studies led to the association of this disorder with bites of Ixodes ticks. In 1979, Reik and colleagues described a neurologic syndrome in American patients with Lyme disease virtually identical to that described by Garin and Bujadoux (Reik et al 1979). In 1983, the responsible spirochete, Borrelia burgdorferi, was identified as the causative agent in American patients with Lyme disease (Benach et al 1983; Steere et al 1983). Shortly thereafter, a closely related agent was identified in European patients (Asbrink et al 1984). Subsequent work has both broadened the scope of the neurologic disorders recognized as associated with this infection (known collectively as "neuroborreliosis") and refined the microbiological understanding of the responsible organisms. Advances in genomics have led to a change in taxonomy, differentiating the organisms responsible for Lyme disease and related disorders, now Borreliella, from the relapsing fever Borrelia (Barbour et al 2017). The broad group formerly termed B burgdorferi sensu lato is now termed Borreliella burgdorferi, with corresponding changes in the names for B afzelii and B garinii.

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