Abstract
Lyme disease, and the closely related disorders described in Europe, affects the nervous system in about 10–15% of infected individuals (Koedel U, Fingerle V, Pfister HW. Lyme neuroborreliosis-epidemiology, diagnosis and management. Nat Rev Neurol. 2015;11(8):446–56). Clinically it is manifest as a variably symptomatic lymphocytic meningitis, multifocal inflammation of peripheral nerves (common), and focal or multifocal inflammation of the CNS (very rare). In children it is important to recognize that meningitis can cause a pseudo-tumor-like disorder; in adults, painful radiculopathy is under-recognized. CNS infection is almost always accompanied by a CSF pleocytosis; this is not necessarily found if only the PNS is involved. Measurement of intrathecal antibody production is useful in patients with otherwise evident CNS inflammation. Antimicrobial therapy is highly effective in virtually all patients. The entity referred to as Lyme encephalopathy is not due to CNS infection or inflammation. The entity referred to as PTLDS may or may not exist as a distinct disorder, but is clearly not related to neuroborreliosis. If it is causally related to Lyme disease at all it occurs in only a very small subset of treated patients.
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References
Steere AC, Malawista SE, Hardin JA, Ruddy S, Askenase W, Andiman WA. Erythema chronicum migrans and Lyme arthritis. The enlarging clinical spectrum. Ann Intern Med. 1977;86(6):685–98.
Garin C, Bujadoux A. Paralysie par les tiques. J Med Lyon. 1922;71:765–7.
Benach JL, Bosler EM, Hanrahan JP, Coleman JL, Habicht GS, Bast TF, et al. Spirochetes isolated from the blood of two patients with Lyme disease. N Engl J Med. 1983;308:740–2.
Steere AC, Grodzicki RL, Kornblatt AN, Craft JE, Barbour AG, Burgdorfer W, et al. The spirochetal etiology of Lyme disease. N Engl J Med. 1983;308:733–40.
Asbrink E, Hederstedt B, Hovmark A. The spirochetal etiology of erythema chronicum migrans afzelius. Acta Derm Venereol. 1984;64(4):291–5.
Hollstrom E. Successful treatment of erythema migrans afzelius. Acta Derm Venereol. 1951;31(2):235–43.
Dressler F, Ackermann R, Steere AC. Antibody responses to the three genomic groups of borrelia burgdorferi in European Lyme borreliosis. J Infect Dis. 1994;169(2):313–8.
Lipsett SC, Branda JA, McAdam AJ, Vernacchio L, Gordon CD, Gordon CR, et al. Evaluation of the C6 Lyme enzyme immunoassay for the diagnosis of Lyme disease in children and adolescents. Clin Infect Dis. 2016;63(7):922–8.
Wormser GP, Tang AT, Schimmoeller NR, Bittker S, Cooper D, Visintainer P, et al. Utility of serodiagnostics designed for use in the United States for detection of Lyme borreliosis acquired in Europe and vice versa. Med Microbiol Immunol. 2014;203(1):65–71.
Wormser GP, Schriefer M, Aguero-Rosenfeld ME, Levin A, Steere AC, Nadelman RB, et al. Single-tier testing with the C6 peptide ELISA kit compared with two-tier testing for Lyme disease. Diagn Microbiol Infect Dis. 2013;75(1):9–15.
Smith RP, Schoen RT, Rahn DW, Sikand VK, Nowakowski J, Parenti DL, et al. Clinical characteristics and treatment outcome of early Lyme disease in patients with microbiologically confirmed erythema migrans. Ann Intern Med. 2002;136(6):421–8.
Pediatric_Lyme_Disease_Study_Group, Gerber MA, Shapiro ED, Burke GS, Parcells VJ, Bell GL. Lyme disease in children in southeastern Connecticut. N Engl J Med. 1996;335(17):1270–4.
Halperin JJ, Luft BJ, Volkman DJ, Dattwyler RJ. Lyme neuroborreliosis—peripheral nervous system manifestations. Brain. 1990;113:1207–21.
England JD, Bohm RP, Roberts ED, Philipp MT. Mononeuropathy multiplex in rhesus monkeys with chronic Lyme disease. Ann Neurol. 1997;41(3):375–84.
Ramesh G, Santana-Gould L, Inglis FM, England JD, Philipp MT. The Lyme disease spirochete borrelia burgdorferi induces inflammation and apoptosis in cells from dorsal root ganglia. J Neuroinflammation. 2013;10:88.
Halperin JJ, Luft BJ, Anand AK, Roque CT, Alvarez O, Volkman DJ, et al. Lyme neuroborreliosis: central nervous system manifestations. Neurology. 1989;39(6):753–9.
Logigian EL, Kaplan RF, Steere AC. Chronic neurologic manifestations of Lyme disease. N Engl J Med. 1990;323(21):1438–44.
Luo N, Johnson J, Shaw J, Feeny D, Coons S. Self-reported health status of the general adult U.S. population as assessed by the EQ-5D and Health Utilities Index. Med Care. 2005;43(11):1078–86.
Fallon BA, Keilp JG, Corbera KM, Petkova E, Britton CB, Dwyer E, et al. A randomized, placebo-controlled trial of repeated IV antibiotic therapy for Lyme encephalopathy. Neurology. 2008;70:992–1003.
Krupp LB, Hyman LG, Grimson R, Coyle PK, Melville P, Ahnn S, et al. Study and treatment of post Lyme disease (STOP-LD): a randomized double masked clinical trial. Neurology. 2003;60(12):1923–30.
Klempner MS, Hu LT, Evans J, Schmid CH, Johnson GM, Trevino RP, et al. Two controlled trials of antibiotic treatment in patients with persistent symptoms and a history of Lyme disease. N Engl J Med. 2001;345(2):85–92.
Berende A, ter Hofstede HJ, Vos FJ, van Middendorp H, Vogelaar ML, Tromp M, et al. Randomized trial of longer-term therapy for symptoms attributed to Lyme disease. N Engl J Med. 2016;374(13):1209–20.
Shadick NA, Phillips CB, Logigian EL, Steere AC, Kaplan RF, Berardi VP, et al. The long-term clinical outcomes of Lyme disease. A population-based retrospective cohort study. Ann Intern Med. 1994;121(8):560–7.
Wills AB, Spaulding AB, Adjemian J, Prevots DR, Turk SP, Williams C, et al. Long-term follow-up of patients with Lyme disease: longitudinal analysis of clinical and quality-of-life measures. Clin Infect Dis. 2016;62(12):1546–51.
Dersch R, Sarnes AA, Maul M, Hottenrott T, Baumgartner A, Rauer S, et al. Quality of life, fatigue, depression and cognitive impairment in Lyme neuroborreliosis. J Neurol. 2015;262(11):2572–7.
Dersch R, Sommer H, Rauer S, Meerpohl JJ. Prevalence and spectrum of residual symptoms in Lyme neuroborreliosis after pharmacological treatment: a systematic review. J Neurol. 2016;263(1):17–24.
Seltzer EG, Gerber MA, Cartter ML, Freudigman K, Shapiro ED. Long-term outcomes of persons with Lyme disease. JAMA. 2000;283(5):609–16.
Salazar JC, Gerber MA, Goff CW. Long-term outcome of Lyme disease in children given early treatment. J Pediatr. 1993;122(4):591–3.
Wormser GP, Weitzner E, McKenna D, Nadelman RB, Scavarda C, Molla I, et al. Long-term assessment of health-related quality of life in patients with culture-confirmed early Lyme disease. Clin Infect Dis. 2015;61(2):244–7.
Halperin JJ, Shapiro ED, Logigian EL, Belman AL, Dotevall L, Wormser GP, et al. Practice parameter: treatment of nervous system Lyme disease. Neurology. 2007;69(1):91–102.
Koedel U, Fingerle V, Pfister HW. Lyme neuroborreliosis-epidemiology, diagnosis and management. Nat Rev Neurol. 2015;11(8):446–56.
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Halperin, J.J. (2019). Nervous System Lyme Disease. In: Cho, T., Bhattacharyya, S., Helfgott, S. (eds) Neurorheumatology. Springer, Cham. https://doi.org/10.1007/978-3-030-16928-2_26
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