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Lyme Neuroborreliosis

Lyme neuroborreliosis is a late manifestation of an infection by Borrelia spp., a tick-borne bacterial pathogen that is known for causing Lyme disease and a range of clinical syndromes. The ailment is primarily seen during the summer months and symptoms range from headaches and facial nerve palsy to severe sensory and motor deficits with altered consciousness. The diagnosis mandates a thorough clinical investigation, followed by microbiological studies.


Presentation

Lyme neuroborreliosis (LN) is one of the many forms of Borrelia spp. infection, a spirochetal bacterial pathogen that is transmitted to the human host after a tick bite, specifically from Ixodes species [1] [2] [3] [4]. Higher prevalence is encountered in the United States (where Borrelia burgdorferi is the main causative agent, with an incidence rate of 9.1 cases per 100 000 individuals), and in Europe, where B. garinii, B. afzelii and B. burgdorferi sensu stricto are identified as main pathogenic species [2] [4]. In order for LN to occur, patients first pass through the initial stages of borreliosis (commonly known as Lyme disease) - the appearance of erythema migrans (a sharply-defined circular or "target" lesion that develops in the proximity of the tick bite) within a period of 3-30 days after the bite, and constitutional complaints such as fever, headaches, joint pain, and fever [1] [3] [4]. LN appears when bacteria are disseminated into the peripheral and central nervous system (CNS) and findings are seen either during early stages of dissemination or after a delayed period of weeks to years as a late manifestation [3] [4]. LN is frequently described as a complication of Lyme disease in Europe than in the United States (35% vs. <10%, respectively) [3]. Headaches, cranial nerve palsies (mainly of the facial nerve, presenting as either unilateral or bilateral face droop), and papilledema are nonspecific features, which may prolong the time before a correct diagnosis is made, particularly in children where enteroviral meningitis is a common cause of such symptoms [3] [5]. A more prolonged clinical course and CNS abnormalities, however, are useful distinguishing characteristics [5]. The term Bannwarth's syndrome is used to describe the typical signs of LN (mainly seen in European patients) - lymphocytic meningitis, radiculitis accompanied by sharp pain lasting for weeks or months (with exacerbations during the night), and cranial neuritis [1] [3] [4] [6]. Apart from the involvement of the peripheral nervous system, Borrelia spp. can reach the CNS and cause significant damage to the brain and the spinal cord, presenting as a variable change in consciousness (from mild confusion to severe and life-threatening encephalitis) [1].

Fatigue
  • Patients and healthy controls were assessed for quality of life [Short Form (36) with subscores for physical and mental components (PCS, MCS)], fatigue (fatigue severity scale), depression (Beck depression inventory), verbal memory and learning and cognitive[ncbi.nlm.nih.gov]
  • KEYWORDS: Fatigue; Lyme disease; Lyme neuroborreliosis; Prevalence review; Residual symptoms; Systematic review[ncbi.nlm.nih.gov]
  • CONCLUSIONS: LNB can present as acute ocular motor disorders in conjunction with fatigue and other clinical manifestations.[ncbi.nlm.nih.gov]
  • We describe the case of a 55-year-old man with a brief history of fatigue and severe back pain, unresponsive to escalating doses of opiate analgesia. Blood tests and imaging studies were unremarkable and a functional diagnosis was considered.[ncbi.nlm.nih.gov]
  • The average Fatigue Severity Scale (FSS) score was 4.29. [8] Krupp defined severe fatigue 4.0 in her NIH sponsored clinical trial. [8] The 17 LBN subjects with residual symptoms also presented with a poor quality of life.[danielcameronmd.com]
Chills
  • The rash is often accompanied by flulike symptoms, such as headache , fatigue , chills, loss of appetite , fever , and aching joints or muscles.[britannica.com]
  • Lyme disease infection causes symptoms that may include a characteristic erythema migrans (EM) or "bulls-eye" rash that spreads from the site of the bite, fever, chills, headache, and fatigue.[labtestsonline.org]
  • Early signs and symptoms include fever , chills, headache , fatigue , muscle and joint pain , and swollen lymph nodes -- all common in the flu . In up to 80% of Lyme infections, a rash is one of the first symptoms, Aucott says.[webmd.com]
  • People infected with the seasonal flu virus feel miserable with fever, chills, muscle aches, coughing, congestion, headache and fatigue for a week or so.[niaid.nih.gov]
Weight Loss
  • Author information 1 Department of Internal Medicine, Leiden University Medical Center, Leiden, Netherlands. [email protected] Abstract A young man presented with recent-onset non-specific symptoms like headache, sleepiness and weight loss, interfering[ncbi.nlm.nih.gov]
  • Abstract A 66-year-old woman presented with severe shooting pains throughout her back and legs, followed by progressive deafness, weight loss and headache.[ncbi.nlm.nih.gov]
  • An 11 year old boy presented with long standing symptoms of severe weight loss and chronic headache, while the other patient had pre-existing mental and motor retardation and developed seizures and failure to thrive.[ncbi.nlm.nih.gov]
  • In the chronic stage, patients typically present with headache, malaise, significant weight loss, sensorineural hearing loss or a progressive spastic-ataxic gait disturbance. 4 Autonomic dysfunction is rarely seen in LNB. 4 If LNB is suspected, investigation[jnmjournal.org]
Nausea
  • Five of the patients presented with severe fatigue, malaise, nausea, headache and fever. Four had recognised a tick bite recently, and two developed erythema migrans.[ncbi.nlm.nih.gov]
  • Most of the predictive information was provided by the symptoms at 14 days: these included nausea, vomiting, memory and concentration disturbances, malaise, fatigue, headache, and arthralgia ( Supplementary Figure 2 ).[academic.oup.com]
  • None of those who received a diagnosis of possible or definite Lyme neuroborreliosis had symptoms of meningitis (headache with hypersensitivity to light and sound, and nausea or vomiting).[tidsskriftet.no]
  • There was no history of fevers, chills, rigors, nausea or vomiting. The patient and his wife had returned from Maine approximately 4 weeks ago where they had been vacationing. There was no recent or remote history of travel outside the country.[doi.org]
Failure to Thrive
  • An 11 year old boy presented with long standing symptoms of severe weight loss and chronic headache, while the other patient had pre-existing mental and motor retardation and developed seizures and failure to thrive.[ncbi.nlm.nih.gov]
Diplopia
  • A 22-year-old male presented with history of flu-like illness and headache, accompanied by vertical binocular diplopia, worse on downgaze and better in upgaze and right head tilt.[ncbi.nlm.nih.gov]
  • Other symptoms may include diplopia, hearing impairment, paralysis, altered sensation, difficulty in walking or cognitive impairment.[tidsskriftet.no]
  • She had experienced continuing headaches, lethargy and a self-limiting episode of diplopia that prompted her to see her general practitioner.[mja.com.au]
  • A 71-year-old woman with headache, malaise, fever, and diplopia. Initial coronal postcontrast T1 MR imaging ( A and B ) with enhancing bilateral third and fifth cranial nerves.[doi.org]
Photophobia
  • Clinical record A 58-year-old woman of European ancestry presented to a rural hospital in New South Wales in May 2014 with an 8-month history of worsening motor instability, confusion and bilateral occipital headaches associated with photophobia, lethargy[mja.com.au]
  • Photophobia As in various other infections and/or CNS disturbances (e.g. meningitis, migraine, psittacosis, typhus, Rocky Mountain Spotted Fever), photophobia may be a prominent feature. In our sample, 70% of respondents reported photophobia.[lymenet.org]
Hemianopsia
  • The patient is a 48-year old male with a progressive spastic paraparesis of months' duration who presented acute headache, confusion, severe left hemiparesis with sensory deficit and homonymous hemianopsia.[ncbi.nlm.nih.gov]
Headache
  • Abstract We present two patients with monosymptomatic headache resembling chronic tension-type headache as the first manifestation of Lyme neuroborreliosis.[ncbi.nlm.nih.gov]
  • In a multivariate analysis, both gender and having headache and neck stiffness were associated with a higher level of pleocytosis.[ncbi.nlm.nih.gov]
  • The ailment is primarily seen during the summer months and symptoms range from headaches and facial nerve palsy to severe sensory and motor deficits with altered consciousness.[symptoma.com]
  • Both patients had a prodromal stage involving headaches, and showed meningeal enhancement in addition to ischemic infarctions on brain magnetic resonance imaging and diffuse vasculitis on vascular imaging.[ncbi.nlm.nih.gov]
  • Abstract A 66-year-old woman presented with severe shooting pains throughout her back and legs, followed by progressive deafness, weight loss and headache.[ncbi.nlm.nih.gov]
Encephalopathy
  • , Lyme Disease — Encephalopathies, Lyme Disease — Lyme Disease Encephalopathies — Lyme Disease Encephalopathy — Lyme Disease Mononeuritis Multiplex — Mononeuritis Multiplex, Lyme Disease — Peripheral Nervous System Lyme Disease — Lyme Meningoradiculitis[mesh.kib.ki.se]
  • The well-documented neurologic spectrum includes lymphocytic meningitis, cranial neuropathy, and radiculoneuritis in the early disseminated stage; and peripheral neuropathy, chronic encephalomyelitis, and mild encephalopathy in the late persistent stage[ncbi.nlm.nih.gov]
  • The tertiary stage may mimic many conditions, including multiple sclerosis, polyneuropathy, viral encephalitis, brain tumor, vasculitis, encephalopathy, psychiatric illness, and myelopathy.[ncbi.nlm.nih.gov]
  • Similar but less marked changes were seen in the treated patients and in some of the patients with Lyme encephalopathy. No such abnormalities were seen in the serum of the patients.[ncbi.nlm.nih.gov]
  • Application of this method permits the identification of a rare B burgdorferi-associated multifocal encephalitis (brain infection) and its differentiation from a milder encephalopathy, or confusional state; the latter may not require CNS bacterial invasion[ncbi.nlm.nih.gov]
Radiculitis
  • Abstract Neurogenic pain with radiculitis is often the starting symptom in adult patients with tick-borne Lyme neuroborreliosis and in some cases the only clinical manifestation.[ncbi.nlm.nih.gov]
  • Figuring prominently in the European literature, but less emphasized in the United States, is painful radiculitis, radicular pain involving a limb or trunk dermatome.[ncbi.nlm.nih.gov]
  • Enhanced awareness of the clinical presentation of Lyme disease allows inclusion of LNB in the imaging differential diagnosis of facial neuritis, multiple enhancing cranial nerves, enhancing noncompressive radiculitis, and pediatric leptomeningitis with[ncbi.nlm.nih.gov]
  • Thirty patients had symptoms typical of the condition (such as radiculitis or peripheral facial nerve palsy). Six of these were diagnosed with definite Lyme neuroborreliosis, and one with possible Lyme neuroborreliosis.[ncbi.nlm.nih.gov]
  • The primary symptoms are usually painful radiculitis, facial palsy and lymphocytic meningitis. The aim of this study was to provide data on the seasonal variation, anamnesis, symptoms, laboratory data and course of the disease in adults ( 16 years).[ncbi.nlm.nih.gov]
Neck Stiffness
  • In a multivariate analysis, both gender and having headache and neck stiffness were associated with a higher level of pleocytosis.[ncbi.nlm.nih.gov]
  • Neck stiffness and fever were reported by 15.3% of patients. Most of these patients were younger than 50 years. Polyradiculoneuritis was frequently found in patients older than 50 years. Lymphopleocytosis was found in all patients.[ncbi.nlm.nih.gov]
  • stiffness during their acute illness (87% compared with 13%; odds ratio, 2.4 [CI, 1.0 to 5.5]; P 0.045); however, the performance of the two groups on neurocognitive tests did not significantly differ.[doi.org]
  • Persons with previous Lyme disease who had persistent symptoms after receiving treatment (n 67) were more likely than those who had completely recovered to have had fever, headache, photosensitivity, or neck stiffness during their acute illness (87% compared[ncbi.nlm.nih.gov]
  • No focal cranial nerve deficits were noted and there was no neck stiffness nor was any nuchal rigidity appreciable. Both Kernig's and Brudzinski's sign were negative.[doi.org]
Paresthesia
  • […] of chemotherapy (FCR: fludarabine, cyclophosphamide, rituximab and prednisone) the 63-year-old patient developed night sweat, arthralgia in elbows, wrists, proximal interphalangeal joints (PIPs) and strong neuropathic pain in both legs, followed by paresthesia[ncbi.nlm.nih.gov]
  • The most frequent symptoms/signs were radicular pain (100%), sleep disturbances (75.3%), erythema migrans (59.7%), headache (46.8%), fatigue (44.2%), malaise (39%), paresthesias (32.5%), peripheral facial palsy (PFP) (36.4%), meningeal signs (19.5%),[ncbi.nlm.nih.gov]
  • The symptoms of Lyme neuroborreliosis include: Cognitive decline Headaches Confusions Dizziness Vision problems Muscle tremors, seizures, and weakness Mood swings Meningitis : inflammation of the protective covering of the brain Paresthesia : tingling[study.com]
  • The symptoms/signs suggesting NS involvement were headache, vertigo, disturbances of sleep, memory or concentration disorders, radicular pain, paresthesias, neck stiffness, and peripheral facial palsy.[cid.oxfordjournals.org]

Workup

The diagnosis of LN is a difficult one to make without a proper clinical and microbiological investigation. The physician should first conduct a detailed patient interview during which the incidence of tick bites and typical skin lesions must be assessed, whereas a comprehensive travel history (given the fact that visiting endemic areas is a risk factor) may provide important clues as well [1] [4]. Tick-borne disease must be considered in all patients with undisclosed cutaneous and constitutional symptoms in the summer months, as the vast majority of such events occur from May to September [1] [2]. After a detailed physical examination (particularly focused on neurological evaluation), laboratory studies are the cornerstone for confirming the condition. Current recommendations advocate initial serological testing (through enzyme immunoassays or enzyme-linked immunosorbent assays - EIA and ELISA, respectively) of cerebrospinal fluid (CSF) or blood for immunoglobulin (Ig) G and IgM antibodies, followed by more sensitive methods such as Western blot [1] [4] [6] [7] [8]. CSF examination yields lymphocytic pleocytosis with normal biochemical composition [4], and the detection of neutrophilic predominance can firmly exclude Lyme disease as the etiology of neurologic infections [5]. Polymerase chain reaction (PCR) testing is a novel technique that allows identification of pathogenic DNA and its implementation is recommended whenever possible [4] [7]. Imaging studies of the endocranium and the CNS, magnetic resonance imaging (MRI) being the main candidate, reveal nonspecific findings and thus are of limited benefit for discriminating between etiologies of such symptoms [1] [9].

Brucella Abortus
  • Giambartolomei , Brucella abortus Induces the Secretion of Proinflammatory Mediators from Glial Cells Leading to Astrocyte Apoptosis , The American Journal of Pathology , 10.2353/ajpath.2010.090503 , 176 , 3 , (1323-1338) , (2010) . Tereance A.[doi.org]
Diffuse Vasculitis
  • Both patients had a prodromal stage involving headaches, and showed meningeal enhancement in addition to ischemic infarctions on brain magnetic resonance imaging and diffuse vasculitis on vascular imaging.[ncbi.nlm.nih.gov]

Treatment

  • The CSF mononuclear cell count was used as a surrogate marker of treatment outcome.[ncbi.nlm.nih.gov]
  • Residual symptoms following treatment were found in 28.1% of patients, and risk of residual symptoms was significantly associated with delay from symptom debut to initiation of treatment.[ncbi.nlm.nih.gov]
  • Treatment was given orally in 77 (30%) patients, intravenously in 110 (44%), both orally and intravenously in 65 (26%), and unknown in one. Treatment practices differed between the health regions (p 0.002).[ncbi.nlm.nih.gov]
  • A model based on pretreatment data and the findings at the end of 14-day antibiotic treatment accurately predicted which patients would have an unfavorable outcome 6 or 12 months after treatment.[ncbi.nlm.nih.gov]
  • However, due to considerable imprecision, relevant differences between treatments cannot be excluded. No evidence suggesting benefits of extended antibiotic treatments could be identified. Further well-designed trials are needed.[ncbi.nlm.nih.gov]

Prognosis

  • Epidemiologic data, tick bite histories, duration of symptoms, clinical findings, radiologic findings, treatment durations and prognosis were investigated. RESULTS: Totally 7 patients had been treated with the diagnosis of Lyme neuroborreliosis.[ncbi.nlm.nih.gov]
  • Abstract The prognosis and impact of residual symptoms on quality of life in patients with Lyme neuroborreliosis (LNB) is subject to debate.[ncbi.nlm.nih.gov]
  • Prognosis is excellent and the time to recovery may be influenced when treated appropriately.[thieme-connect.com]
  • The book also features a chapter on prognosis, offering an evidence-based review of outcome studies, as well as practical advice to physicians to help them manage the challenging clinical scenario of chronic symptoms attributed to Borrelia infection.[books.google.com]
  • When EM is promptly treated with appropriate antimicrobial agents, the prognosis is excellent. Persons in endemic areas should take measures to prevent tick bites. Copyright 2015 Elsevier Inc. All rights reserved.[ncbi.nlm.nih.gov]

Etiology

  • Abstract Borrelia burgdorferi, the etiologic agent of Lyme borreliosis, was isolated from the CSF of a patient with elevated serum IgG antibody titers against B burgdorferi and a history of multiple tick bites.[ncbi.nlm.nih.gov]
  • CSF examination yields lymphocytic pleocytosis with normal biochemical composition, and the detection of neutrophilic predominance can firmly exclude Lyme disease as the etiology of neurologic infections.[symptoma.com]
  • Sixty-six samples from children with neuroinfections other than borrelial etiology were used as controls.[ncbi.nlm.nih.gov]
  • An etiological diagnosis of the dementia was made at the end of the follow-up of 5.0 2.9 years.[prohealth.com]
  • Etiology Caused by tick-borne spirochete Borrelia burgdorferi sensu lato . Its passage through the Blood-Brain-Barrier results in Central Nervous System (CNS) infection, causing widespread neurological symptoms 4.[slideshare.net]

Epidemiology

  • This review highlights differences in the epidemiology, clinical manifestations, diagnosis, and management of Lyme disease in the United States, Europe, and Asia, with an emphasis on neurologic manifestations and neuroimaging.[ncbi.nlm.nih.gov]
  • KEYWORDS: Borrelia burgdorferi; Epidemiology; Lyme disease; Neurology; Public health[ncbi.nlm.nih.gov]
  • Therfore, based on the epidemiology of LNB in children from Tyrol, the aim of our study was to point out the necessity of a clear definition of pediatric LNB to avoid underdiagnosis and overtreatment.[ncbi.nlm.nih.gov]
  • Most aspects of the epidemiology, clinical manifestation and treatment of Lyme neuroborreliosis are well known and accepted; only the management of so-called chronic Lyme disease is surrounded by considerable controversy.[ncbi.nlm.nih.gov]
  • The facts about Lyme disease, from epidemiology to treatment to control Lyme borreliosis, popularly known as Lyme disease, remains the most commonly reported tick-transmitted infection in North America and Europe.[books.google.com]
Sex distribution
Age distribution

Pathophysiology

  • An observed decrease in gelsolin concentration in the blood of TBE and LNB patients supports previous findings indicating the involvement of gelsolin in the pathophysiology of an inflammatory response.[ncbi.nlm.nih.gov]
  • Cases such as these can help explain the pathophysiology of mental illness. Research in LAD provokes an avalanche of additional questions.[ncbi.nlm.nih.gov]
  • CIP is associated with poor quality of life and high morbidity. 3 Pathophysiological aspects of the condition remain obscure and treatment options are limited and often unsatisfactory. 9 Consequently, it is crucial that all physicians and gastroenterologists[jnmjournal.org]
  • A similar pathophysiology occurs in LAD. METHOD: A retrospective chart review and epidemiological calculations were performed.[ncbi.nlm.nih.gov]
  • A similar pathophysiology occurs in LAD. Method: A retrospective chart review and epidemiological calculations were performed.[doi.org]

Prevention

  • Prompt diagnosis and treatment appear to facilitate symptomatic relief and prevent persistent neurologic deficits.[ncbi.nlm.nih.gov]
  • According to the Centers for Disease Control and Prevention, it is the most commonly reported vector-borne illness and the fifth most common disease in the National Notifiable Diseases Surveillance System.[ncbi.nlm.nih.gov]
  • The final chapter is a guide to prevention strategies, including the management of tick bites.[books.google.com]
  • Department of Microbiology and Immunology, Kunming Medical University , Kunming, China . 4 4 Institute for Tropical Medicine, Kunming Medical University , Kunming, China . 5 5 Yunnan Province Integrative Innovation Center for Public Health, Disease Prevention[ncbi.nlm.nih.gov]
  • Studies on the effects of biodiversity loss and ecosystem changes on LB emergence may identify new paradigms for the prevention and control of LB and other tick-borne diseases. Full text loading...[eurosurveillance.org]

References

Article

  1. Hildenbrand P, Craven DE, Jones R, Nemeskal P. Lyme neuroborreliosis: manifestations of a rapidly emerging zoonosis. AJNR Am J Neuroradiol. 2009;30(6):1079-1087.
  2. Centers for Disease Control and Prevention (CDC). Lyme disease-United States, 2003-2005. MMWR Morb Mortal Wkly Rep. 2007;56(23):573-576.
  3. Sinha A, Dietzman T, Ross D, Sulieman S, Fieldston E. Lyme neuroborreliosis: a diagnostic headache. Hosp Pediatr. 2014;4(6):400-404
  4. Subedi S, Dickeson DJ, Branley JM. First report of Lyme neuroborreliosis in a returned Australian traveller. Med J Aust. 2015 Jul 6;203(1):39-40.
  5. Shah SS, Zaoutis TE, Turnquist J, Hodinka RL, Coffin SE. Early differentiation of Lyme from enteroviral meningitis. Pediatr Infect Dis J. 2005;24(6):542-545.
  6. Strle F, Ružić-Sabljić E, Cimperman, J, et al. Comparison of findings for patients with Borrelia garinii and Borrelia afzelii isolated from cerebrospinal fluid. Clin Infect Dis. 2006;43:704-710.
  7. Aguero-Rosenfeld ME, Wang G, Schwartz I, et al. Diagnosis of Lyme borreliosis. Clin Microbiol Rev 2005;18:484–509.
  8. Centers for Disease Control and Prevention. Recommendations for test performance and interpretation from the Second National Conference on Serologic Diagnosis of Lyme Disease. MMWR Morb Mortal Wkly Rep 1995;44:590-591.
  9. Morgen K, Martin R, Stone RD, et al. FLAIR and magnetization transfer imaging of patients with post-treatment Lyme disease syndrome. Neurology. 2001;57:1980–1985.

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Last updated: 2018-06-22 01:56