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. Although much is said about “neurologic” symptoms that persist after microbiological cure of this infection, studies indicate that months after treatment these symptoms are not only unrelated to nervous system infection but occur with the same frequency in treated patients and healthy controls. Diagnosis rests on 2-tier serologic testing, with positive or equivocal ELISAs validated by either a Western blot or by a second, independent ELISA. CSF CXCL13 concentration may provide additional support for the diagnosis of active CNS neuroborreliosis.
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• Lyme disease, infection with the tick-borne spirochete Borrelia burgdorferi, affects the central or peripheral nervous system in up to 10% to 15% of patients.
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• Clinical phenomena associated with neuroborreliosis typically include one or more of the following: cranial neuropathy (most often the facial nerve), radiculopathy, and lymphocytic meningitis.
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• Serodiagnosis after the first month of infection has high sensitivity and specificity.
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• 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.
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• Persisting difficulties after treatment, often referred to as “post Lyme disease syndrome”, may occur but are not associated with nervous system infection and appear to be no more common than in controls.
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• Misinterpretation of testing and clinical observations results in many patients being treated unnecessarily for what is incorrectly thought to be Lyme disease, with considerable potential for side effects and patient expense.
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 (66). 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 (66). 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 (53). In 1983, the responsible spirochete, Borrelia burgdorferi, was identified as the causative agent in American patients with Lyme disease. Shortly thereafter, a closely related agent was identified in European patients. Subsequent work has both broadened the scope of the neurologic disorders recognized as associated with this infection (known collectively as "Lyme neuroborreliosis") and refined the microbiological understanding of the responsible organisms. Advances in genomics have led to a proposed change in taxonomy, differentiating the organisms responsible for Lyme disease and related disorders, with the recommended name Borreliella, from the relapsing fever Borrelia (02). 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.