Lyme neuroborreliosis: known knowns, known unknowns.

Lyme borreliosis affects the nervous system in three principal ways-mononuclear cell meningitis, cranial neuropathies and radiculoneuropathies-the last a broad term encompassing painful radiculopathy, unifocal and multifocal peripheral nerve involvement. Diagnostic tools have been significantly refined-including improved peripheral blood and CSF serodiagnostics-and much has been learned about the interactions between the causative pathogen and the nervous system. Despite these advances in our understanding of this disease, a broad range of other disorders continue to be misattributed to nervous system Lyme borreliosis, supported by, at best, limited evidence. These misattributions often reflect limited understanding not only of Lyme neuroborreliosis but also of what constitutes nervous system disease generally. Fortunately, a large body of evidence now exists to clarify many of these issues, establishing a clear basis for diagnosing nervous system involvement in this infection and, based on well performed studies, clarifying which clinical disorders are associated with Lyme neuroborreliosis, which with non-neurologic Lyme borreliosis, and which with neither.

Nervous System Lyme Disease-Facts and Fallacies.

The central or peripheral nervous systems may be involved in up to 15% of patients with untreated infection with B burgdorferi sensu lato, characteristic involvement including meningitis, cranial neuritis, and radiculoneuritis. Diagnosis, based on a logical combination of clinical context and antibody-based testing, is usually straightforward, as is treatment. Misconceptions about what does and does not constitute neurologic disease, and about laboratory testing in this infection, have resulted in widespread anxiety that a broad range of other disorders may be attributable to nervous system Lyme disease. This article will review the reasons for these misunderstandings and the arguments against them.

An Unusual Cluster of Neuroinvasive Lyme Disease Cases Presenting With Bannwarth Syndrome in the Midwest United States.

Bannwarth syndrome (BWS), an infrequent manifestation of neuroinvasive Lyme disease (LD) characterized by radiculopathy, neuropathy, and lymphocytic pleocytosis, is more commonly documented in Europe than North America. Here, we describe a cluster of 5 neuroinvasive LD cases with BWS in the upper Midwest United States between July and August 2017.

Nervous System Lyme Disease.

Nervous system involvement occurs in 10% to 15% of patients infected with the tick-borne spirochetes Borrelia burgdorferi, B afzelii, and B garinii. Peripheral nervous system involvement is common. Central nervous system (CNS) involvement, most commonly presenting with lymphocytic meningitis, causes modest cerebrospinal fluid (CSF) pleocytosis. Parenchymal CNS infection is rare. If the CNS is invaded, however, measuring local production of anti-B burgdorferi antibodies in the CSF provides a useful marker of infection. Most cases of neuroborreliosis can be cured with oral doxycycline; parenteral regimens should be reserved for patients with particularly severe disease.

Nervous system Lyme disease.

Lymphocytic meningitis, cranial neuritis or radiculoneuritis occur in up to 15% of patients with untreated Borrelia burgdorferi infection. Presentations of multifocal PNS involvement can range from painful monoradiculitis to confluent mononeuropathy multiplex. Serologic testing is highly accurate after 4 to 6 weeks of infection. In CNS infection, production of anti-Bburgdorferi antibody is often demonstrable in CSF. Oral antimicrobials are microbiologically curative in virtually all patients, including acute European neuroborreliosis. Severe cases may require parenteral treatment. The fatigue and cognitive symptoms seen in some patients with extra-neurological disease are neither evidence of CNS infection nor specific to Lyme disease.

Nervous system Lyme disease.

Lyme disease, the multisystem infectious disease caused by the tick-borne spirochete Borrelia burgdorferi involves the nervous system in 10-15% of affected individuals. Manifestations include lymphocytic meningitis, cranial neuritis, radiculoneuritis, and mononeuropathy multiplex. Encephalopathy, identical to that seen in many systemic inflammatory diseases, can occur during active systemic infection. It is not specific to Lyme disease and only rarely is evidence of nervous system infection. Diagnosis of systemic disease is based on demonstration of specific antibodies in peripheral blood by means of two-tier testing with an ELISA and Western blot. Central nervous system infection often results in specific antibody production in the CSF, demonstrable by comparing spinal fluid to blood serologies. Treatment is straightforward and curative in most instances. Many patients can be treated effectively with oral antibiotics such as doxycycline; in severe CNS infection parenteral treatment with ceftriaxone or other similar agents is highly effective. Treatment should usually be for 2 to at most 4 weeks. Longer treatment adds no therapeutic benefit but does add substantial risk.