DOCSLIB.ORG

Pseudotumor Cerebri in Lyme Disease: a Case Report and Literature Review

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Pseudotumor Cerebri in Lyme Disease: a Case Report and Literature Review PSEUDOTUMOR CEREBRI [PTC] IDIOPATHIC INTRACRANIAL HYPERTENSION [IIH] BENIGN INTRACRANIAL HYPERTENSION [BIH] Jacqueline M.S. Winterkorn, PhD, MD Case: A 29 year old woman c/o headaches that have been diagnosed as migraine but have been worsening lately and even awaken her from sleep. The HA radiates down into her neck. She also describes “seeing stars” when she bends down or stands up suddenly. The morning of the exam, while riding in the car, she noticed side by side double vision. She is obese, having recently reached 300 lbs on her 5’6” frame. Her hands get so swollen that she can’t wear her wedding ring. PSEUDOTUMOR CEREBRI [PTC] 1896 Quinke, 1904 Nonne Idiopathic Intracranial Hypertension [IIH] MODIFIED DANDY CRITERIA • Signs and symptoms of increased ICP ° Papilledema, HA, TVO, diplopia, tinnitus • Normal neuro exam-No localizing findings • Increased ICP >250 mm H20 • Normal CSF • Normal neuroimaging Pseudotumor Cerebri [PTC] • Returning to PTC terminology • BIH: VF loss - Not visually benign • IIH: Increasingly finding basis for elevated ICP - no longer idiopathic EPIDEMIOLOGY OF PTC • Incidence 1/100,000 • 90% obese • 90% women • age at diagnosis 30 • In obese women of childbearing age, incidence 19/100,000 MAJOR SYMPTOMS Result from increased ICP • Headache….…………………….>95% • Visual Symptoms .……………30-70% ° Transient Visual Obscurations..70% ° Eye Pain ………………………..50% ° Horizontal Diplopia………...10-20% ° Loss of vision less than 20/200…25% • Intracranial Noises ……………….60% OTHER COMMON SYMPTOMS • Stiff Neck • Dizziness • Nausea • Ataxia • Back and leg pain • Paresthesias RARE SYMPTOMS Think of another diagnosis • Hearing Loss • Vertical ocular motor complaints [CN IV] • Facial Palsy [CN VII] • Torticollis CLINICAL SIGNS of PTC PAPILLEDEMA • Stages ° 1. Early: dilation of RGC axons ° 2. Acute decompensation:heme, CW, elevation, venous engorgement ° 3. Chronic: Grossly swollen ° 4. Atrophic: Pallor, pseudodrusen • May be asymmetric • Swelling resolves with Optic Atrophy Early papilledema Bucket Brigade Metaphor • Optic nerve transport like bucket brigade • High ICP/IOP blocks fast transport • buckets collect at feet of ischemic members • The untransmitted material builds up as papilledema Stereo pair Papilledema after removal of torcular meningioma Papilledema after Left Jugular ligation Chronic Papilledema ASYMMETRIC PAPILLEDEMA • Usually symmetric • Occasionally unilateral • Subarachnoid meshwork within optic canal acts as incomplete barrier to transmission of CSF pressure • Decreased elasticity of lamina cribrosa • Optic atrophy obviates disc swelling • If asymmetry, expect RAPD CLINICAL SIGNS OF PTC VISUAL LOSS • Vision is normal in early papilledema • OPTIC NEUROPATHY develops late ° Visual Acuity ° Color ° Visual Fields ° Pupils PTC and Glaucoma • Similar VF defects • Similar progression • Similar early sparing of central vision • Similar pressure on lamina cribrosa VISUAL FIELDS with INCREASED ICP • Early: Blindspot enlargement • VF defects ° inferior nasal loss ° arcuate scotomas ° central scotomas • Late: generalized VF constriction • Blindness Nasal step Altitudinal Loss What Visual Fields are Not Seen in PTC • Localizing Patterns ° homonymous hemianopsias ° bitemporal defects ° cecocentral defects • Look for alternate diagnosis Follow HVF 24-2 No role for VEP VEP latency • Delayed P100 reported • Remains normal until serious VF loss • Not a way to follow optic nerve function Most at Risk for Visual Loss? • Recent weight gain • Black males • Anemic Normal NEUROIMAGING IN PTC • CT normal ° normal [or small] ventricles ° empty sella ° enlarged optic nerves in 36% • MRI preferable • Common findings in “normal” MRI: ° empty sella ° flattening of posterior orbit ° dilated optic nerve sheath Brodsky CLINICAL SIGNS OF PTC OCULAR MOTOR DISTURBANCE • DIPLOPIA ° VIth Cranial Nerve ° Divergence Insufficiency ° IVth Cranial Nerve ° Skew deviation NEUROIMAGING for CAUSES of Elevated ICP Look for • mass • venous sinus stenosis, thrombosis • dural AVM Techniques • MRI/MRA • MRV • CT arteriography • Cerebral angiogram CAUSES of ELEVATED ICP • Idiopathic Intracranial Hypertension →Intracranial Mass Lesion • Hydrocephalus • Venous Sinus obstruction • Intracranial Hemorrhage • Meningitis • Sleep apnea • Craniosynostosis • Vitamin A toxicity Cerebral Veins and Sinuses 24 yo man with sudden onset HA, diplopia and tinnitus • HA started 2 days after surgical removal of brachial arch cyst • Involved ligation of right int jugular vein Gadolinium-enhanced axial MRI MRI-Venogram [TR30 TE9] demonstrates mastoiditis [arrow] coronal view, shows absence of flow in right transverse sinus, c/w venous thrombosis Sleep Apnea • Abnormal airway during sleep. Multiple sites of obstruction: • Elongated enlarged soft palate • Retruding jaw pushes an enlarged tongue • Short neck • Obese 4/5/00 4 yo girl evaluated for craniosynostosis/brachycephaly 4/5/00 CAUSES OF HIGH ICP • Vitamin A preparations ° Isotretinoin [Accutane] ° Vitamin A >100,000 units/day –dry desquamated skin –hair loss –bone pain –hepatosplenomegaly • Mechanism of action - blocks CSF reabsorption LUMBAR PUNCTURE IN PTC OP, cell count, glucose, protein, VDRL • LP required for diagnosis • Increased Opening Pressure >250 mm H2O • Normal Protein ° when decreased probably dilutional • No cells ° misdiagnosis of aseptic meningitis Error/Misrepresentation in Lyme literature • “Pseudotumor cerebri and Lyme disease: a new association…” • CSF showed ° lymphocytosis/pleocytosis ° elevated protein • Lyme meningitis/not Pseudotumor cerebri e.g. Kan, Sood, Maytal Pediatr Neurology 1998 18:439 Pseudotumor cerebri in Lyme disease: a case report and literature review. Pediatr Neurol 1998 May;18(5):439-41 Kan L, Sood SK, Maytal J. Division of Pediatric Neurology, Schneider Children's Hospital, Long Island Jewish Medical Center, Long Island Campus for the Albert Einstein College of Medicine, New Hyde Park, New York 11040, USA. Pseudotumor cerebri is an unusual presentation of Lyme disease. The case of an 8-year- old girl with pseudotumor cerebri secondary to acute neuroborreliosis is reported. She presented with acute onset of headache, papilledema, sixth nerve palsy, increased intracranial pressure, and cerebrospinal fluid pleocytosis. Serum and cerebrospinal fluid Lyme antibodies were positive. Twelve reported cases that mostly presented with systemic findings and signs of Lyme disease before development of pseudotumor cerebri were reviewed. We conclude that acute neuroborreliosis can present with pseudotumor cerebri as an initial manifestation. It is important to include Lyme disease in the differential diagnosis of pseudotumor cerebri in an area endemic for Lyme disease. CASE: A 35 year old African American woman complained of visual loss in both eyes, but much worse in her left eye, where she could not count fingers for the past 2 weeks. She has suffered from headache for 4-6 months, treated as “sinusitis.” She has had occasional tinnitus and double vision. Sarcoid-pachymeningitis DIAGNOSIS • Summary ° Intracranial Hypertension ° Visual Loss from elev ICP ° Pachymeningitis • DX: Meningeal Sarcoidosis ° Pathophysiol: Decreased absorption at arachnoid granulations • Beware of papilledema “decreasing” because disc is developing atrophy. CONDITIONS MIMICKING PTC • Intracranial lesions - real tumor, abscess • Dural venous sinus outflow abnormality • Meningeal Sarcoidosis • Carcinomatous meningitis • Infections eg. Viral encephalitis • Hydrocephalus • GBS and spinal cord tumors with increased protein in CSF • Pseudopapilledema and headache Don’t be misled by migraines in a patient with pseudopapilledema [ONHDrusen] CAUSES OF PTC Obstructed intracranial venous drainage • Decreased absorption at arachnoid granulations ° scarring from meningitis or heme • Obstruction to venous drainage ° venous sinus thrombosis ° AVM →radical neck dissection ° middle ear or mastoid infection [Gradenigo] • Hypercoagulable state • Increased intraabdominal pressure- fat distribution Radical Neck dissection: jugular ligation CAUSES OF PTC Obstructed intracranial venous drainage • Decreased flow through arachnoid granulations ° scarring from meningitis or heme • Obstruction to venous drainage ° AVM ° venous sinus thrombosis ° radical neck dissection →middle ear or mastoid infection [Gradenigo] • hypercoagulable state • increased intraabdominal pressure - fat Transverse sinus Mastoiditis obstruction CAUSES OF PTC Obstructed intracranial venous drainage • Decreased flow through arachnoid granulations ° scarring from meningitis or heme • Obstruction to venous drainage ° bilateral radical neck dissection ° venous sinus thrombosis ° middle ear or mastoid infection [Gradenigo] ° hypercoagulable state →increased intraabdominal pressure Sugarman suggests bariatric surgery -- stomach stapling -- to enforce weight loss CAUSES OF PTC Endocrine/Metabolic Dysfunction • Obesity • Hypoparathyroidism • Adrenal insufficiency • Steroid withdrawal • Estrogen metabolism MEDICATIONS ASSOCIATED WITH PTC • Amiodarone • Antibiotics ° Nalidixic acid ° Penicillin ° Tetracycline, Minocycline, Doxycycline • Danazol • Lithium • Indomethacin • Steroid withdrawal UNASSOCIATED CONDITIONS same frequency as general population • Menarche • Irregular menses • Oral contraceptives • Pregnancy • Hyperthyroidism • Corticosteroid intake • Many drugs MEDICAL MANAGEMENT OF PTC • Follow Vision ° VA, quantitative visual fields, RAPD ° fundus photos • Encourage Weight Loss [10%] • Decrease CSF production/Diuretics • Treat Headache • Treat depression ° Support Group 1-800-926-1230
Load more

Recommended publications
  • Common and Uncommon Neurological Manifestations of Neuroborreliosis
    Schwenkenbecher et al. BMC Infectious Diseases (2017) 17:90 DOI 10.1186/s12879-016-2112-z RESEARCHARTICLE Open Access Common and uncommon neurological manifestations of neuroborreliosis leading to hospitalization Philipp Schwenkenbecher1†, Refik Pul1†, Ulrich Wurster1, Josef Conzen2, Kaweh Pars1, Hans Hartmann3, Kurt-Wolfram Sühs1, Ludwig Sedlacek4, Martin Stangel1, Corinna Trebst1† and Thomas Skripuletz1*† Abstract Background: Neuroborreliosis represents a relevant infectious disease and can cause a variety of neurological manifestations. Different stages and syndromes are described and atypical symptoms can result in diagnostic delay or misdiagnosis. The aim of this retrospective study was to define the pivotal neurological deficits in patients with neuroborreliosis that were the reason for admission in a hospital. Methods: We retrospectively evaluated data of patients with neuroborreliosis. Only patients who fulfilled the diagnostic criteria of an intrathecal antibody production against Borrelia burgdorferi were included in the study. Results: Sixty-eight patients were identified with neuroborreliosis. Cranial nerve palsy was the most frequent deficit (50%) which caused admission to a hospital followed by painful radiculitis (25%), encephalitis (12%), myelitis (7%), and meningitis/headache (6%). In patients with a combination of deficits, back pain was the first symptom, followed by headache, and finally by cranial nerve palsy. Indeed, signs of meningitis were often found in patients with neuroborreliosis, but usually did not cause admission to a hospital. Unusual cases included patients with sudden onset paresis that were initially misdiagnosed as stroke and one patient with acute delirium. Cerebrospinal fluid (CSF) analysis revealed typical changes including elevated CSF cell count in all but one patient, a blood-CSF barrier dysfunction (87%), CSF oligoclonal bands (90%), and quantitative intrathecal synthesis of immunoglobulins (IgM in 74%, IgG in 47%, and IgA in 32% patients).
    [Show full text]
  • Overdiagnosis and Overtreatment of Lyme Neuroborreliosis Are Preventable
    Postgrad Med J 1999;75:650–656 © The Fellowship of Postgraduate Medicine, 1999 Postgrad Med J: first published as 10.1136/pgmj.75.889.650 on 1 November 1999. Downloaded from Overdiagnosis and overtreatment of Lyme neuroborreliosis are preventable Avinash Prasad, Douglas Sankar Summary Lyme disease has become a leading vector-borne infectious disease all over the The problems of diagnosis and world, with 10–40% of patients eventually developing Lyme neuroborreliosis.1 treatment of Lyme neuroborrelio- This clinical entity is another great mimicker, like tuberculosis and syphilis, as its sis can be minimised by strictly clinical manifestations are protean. The high prevalence and mimicker status of following the clinical diagnostic Lyme neuroborreliosis increases the physician’s responsibility for precise criteria, and understanding the diagnosis and treatment. pitfalls of laboratory tests. The In spite of advances in the diagnostic armamentarium, the diagnosis of Lyme diagnosis is based solely on objec- disease and Lyme neuroborreliosis remains problematic. In one study only a tive clinical findings, with sero- third of patients referred to a Lyme disease clinic were found to have Lyme dis- logic test results used only to ease, either active or by history.2 Diseases such as depression and cancer may be confirm the diagnosis. It must be overlooked, if guidelines for diagnosis and treatment of Lyme neuroborreliosis underscored that serologic test- are not followed carefully. In this paper, the importance of strictly following the ing, when ordered without regard criteria for clinical diagnosis is emphasised, and the pitfalls of the diagnostic for clinical presentation (ie, used methods are discussed.
    [Show full text]
  • Can Lyme Disease Cause Dementia? - 08-23-2020 by Dr
    Can Lyme disease cause dementia? - 08-23-2020 by Dr. Daniel Cameron - Daniel Cameron, MD, MPH - https://danielcameronmd.com Can Lyme disease cause dementia? Sunday, August 23, 2020 https://danielcameronmd.com/can-lyme-disease-cause-dementia/ In a retrospective study, entitled “Secondary dementia due to Lyme neuroborreliosis,” Kristoferitsch and colleagues describe several case reports of patients diagnosed with dementia-like syndromes due to Lyme neuroborreliosis or Lyme disease that help address the question - can lyme disease cause dementia.2 Rapid improvement with antibiotic treatment The authors' case report featuring a 76-year-old woman demonstrates how Lyme disease can cause dementia-like symptoms. The patient developed progressive cognitive decline, loss of weight, nausea, gait disturbance and tremor over a 12-month period. She was referred to a neurology clinic for evaluation. Three months earlier, the woman had been diagnosed with tension headaches and a depressive disorder. Medications, however, did not improve her symptoms. Further testing revealed bilateral white matter lesions and an old lacunar lesion located at the left striatum. Extensive neurocognitive testing found “a severe decline of attention, memory and executive functions corresponding to subcortical dementia,” the authors write. “LNB [Lyme neuroborreliosis] was diagnosed when further CSF [cerebral spinal fluid] examinations disclosed a highly elevated Bb-specific-AI indicating local intrathecal Bb-specific antibody synthesis,” Kristoferitsch writes. After a 3-week course of treatment with ceftriaxone, the woman “recovered rapidly,” the authors write. “In a telephone call in February 2018 at the age of 82 years, the patient reported no gait problems or cognitive impairment and had just returned from a trip to Cuba,” the authors write.
    [Show full text]
  • Neuroborreliosis with Unusual Presentation: a Case Report
    Open Access Case Report DOI: 10.7759/cureus.5758 Neuroborreliosis with Unusual Presentation: A Case Report Salman Khan 1 , Gurjaspreet K. Bhattal 2 , Nikhil H. Shah 3 , Jorge Lascano 2 , Apurwa Karki 4 1. Internal Medicine, Guthrie Clinic/Robert Packer Hospital, Sayre, USA 2. Internal Medicine, University of Florida, Gainesville, USA 3. Cardiology, University of Florida, Gainesville, USA 4. Internal Medicine - Critical Care, Guthrie Clinic/Robert Packer Hospital, Sayre, USA Corresponding author: Salman Khan, [email protected] Abstract Lyme disease is the most common vector-borne disease in the northern hemisphere. Neurological complications usually manifest in patients who do not receive treatment for Lyme disease. Neurological involvement may be early or late, depending on the duration of the symptoms. Early neuroborreliosis presents with symptoms such as headache and meningism; late neuroborreliosis can present with signs and symptoms of encephalopathy and stroke-like symptoms. The diagnosis is based on clinical manifestations and lumbar puncture finding. Treatment consists of intravenous antibiotics for a period of three to four weeks. Patients who receive early treatment usually have an excellent prognosis, with very few patients developing post-treatment Lyme disease syndrome. Here, we report an unusual case of Lyme disease with extremely high cerebrospinal fluid protein level and devastating neurological sequelae. The diagnosis of neuroborreliosis is based on neurological symptoms and lumbar puncture findings. Categories: Infectious Disease Keywords: neuroborreliosis, csf, lyme disease Introduction Lyme disease is a tick-borne illness caused by Borrelia burgdorferi, and it is the most common vector-borne disease in the northern hemisphere, characterized by the involvement of various organ systems [1].
    [Show full text]
  • Acute Inflammatory Myelopathies
    UCSF UC San Francisco Previously Published Works Title Acute inflammatory myelopathies. Permalink https://escholarship.org/uc/item/3wk5v9h9 Journal Handbook of clinical neurology, 122 ISSN 0072-9752 Author Cree, Bruce AC Publication Date 2014 DOI 10.1016/b978-0-444-52001-2.00027-3 Peer reviewed eScholarship.org Powered by the California Digital Library University of California Handbook of Clinical Neurology, Vol. 122 (3rd series) Multiple Sclerosis and Related Disorders D.S. Goodin, Editor Copyright © 2014 Bruce Cree. Published by Elsevier B.V. All rights reserved Chapter 28 Acute inflammatory myelopathies BRUCE A.C. CREE* Department of Neurology, University of California, San Francisco, USA INTRODUCTION injury caused by the acute inflammation and the likeli- hood of recurrence differs depending on the etiology. Spinal cord inflammation can present with symptoms sim- Additional important diagnostic and prognostic features ilar to those of compressive myelopathies: bilateral weak- include whether the myelitis is partial or transverse, ness and sensory changes below the spinal cord level of febrile illness, the number of vertebral spinal cord injury, often accompanied by bowel and bladder impair- segments involved on MRI at the time of acute attack, ment and sparing cranial nerve and cerebral function. the rapidity from symptom onset to maximum deficit, Because of the widespread availability of magnetic reso- and the severity of involvement. nance imaging (MRI) and computed tomography (CT) imaging, compressive etiologies can be rapidly excluded, METHODOLOGIC CONSIDERATIONS leading to the consideration of non-compressive etiologies for myelopathy. The differential diagnosis of non- Large observational cohort studies or randomized con- compressive myelopathy is broad and includes infectious, trolled trials concerning myelitis have never been under- parainfectious, toxic, nutritional, vascular, and systemic taken.
    [Show full text]
  • Acute Transverse Myelitis in Lyme Neuroborreliosis
    View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by RERO DOC Digital Library Infection (2010) 38:413–416 DOI 10.1007/s15010-010-0028-x CASE REPORT Acute transverse myelitis in Lyme neuroborreliosis S. Bigi • C. Aebi • C. Nauer • S. Bigler • M. Steinlin Received: 14 January 2010 / Accepted: 3 May 2010 / Published online: 27 May 2010 Ó Urban & Vogel 2010 Abstract strong clinical suspicion of Lyme neuroborreliosis, appro- Introduction Acute transverse myelitis (ATM) is a rare priate treatment should be started and the CSF/blood index disorder (1–8 new cases per million of population per repeated to confirm the diagnosis. year), with 20% of all cases occurring in patients younger than 18 years of age. Diagnosis requires clinical symptoms Keywords Lyme Á Neuroborreliosis Á Transverse myelitis and evidence of inflammation within the spinal cord (cerebrospinal fluid and/or magnetic resonance imaging). ATM due to neuroborreliosis typically presents with Introduction impressive clinical manifestations. Case presentation Here we present a case of Lyme Acute transverse myelitis (ATM) is an inflammatory neuroborreliosis-associated ATM with severe MRI and myelopathy with an incidence of 1–8 cases per million of CSF findings, but surprisingly few clinical manifestations population per year. Of all affected patients, 20% are and late conversion of the immunoglobulin G CSF/blood younger than 18 years of age [1]. Diagnosis is based on index of Borrelia burgdorferi sensu lato. clinical symptoms, cerebral spinal fluid (CSF) findings and Conclusion Clinical symptoms and signs of neuroborre- spinal neuroimaging results. In addition to the inflamma- lial ATM may be minimal, even in cases with severe tory myelopathies, demyelination, infection (e.g.
    [Show full text]
  • Neuropsychiatric Lyme Borreliosis: an Overview with a Focus on a Specialty Psychiatrist's Clinical Practice
    healthcare Review Neuropsychiatric Lyme Borreliosis: An Overview with a Focus on a Specialty Psychiatrist’s Clinical Practice Robert C. Bransfield ID Department of Psychiatry, Rutgers-Robert Wood Johnson Medical School, Piscataway, NJ 08854, USA; bransfi[email protected]; Tel.: +1-732-741-3263; Fax.: +1-732-741-5308 Received: 10 July 2018; Accepted: 23 August 2018; Published: 25 August 2018 Abstract: There is increasing evidence and recognition that Lyme borreliosis (LB) causes mental symptoms. This article draws from databases, search engines and clinical experience to review current information on LB. LB causes immune and metabolic effects that result in a gradually developing spectrum of neuropsychiatric symptoms, usually presenting with significant comorbidity which may include developmental disorders, autism spectrum disorders, schizoaffective disorders, bipolar disorder, depression, anxiety disorders (panic disorder, social anxiety disorder, generalized anxiety disorder, posttraumatic stress disorder, intrusive symptoms), eating disorders, decreased libido, sleep disorders, addiction, opioid addiction, cognitive impairments, dementia, seizure disorders, suicide, violence, anhedonia, depersonalization, dissociative episodes, derealization and other impairments. Screening assessment followed by a thorough history, comprehensive psychiatric clinical exam, review of systems, mental status exam, neurological exam and physical exam relevant to the patient’s complaints and findings with clinical judgment, pattern recognition and knowledgeable
    [Show full text]
  • Neuroborreliosis in Childhood Clinical, Immunological and Diagnostic Aspects
    Linköping University Medical Dissertations No. 1048 Neuroborreliosis in Childhood Clinical, Immunological and Diagnostic Aspects Barbro Hedin Skogman Divisions of Pediatrics and Infectious Diseases Department of Clinical and Experimental Medicine Faculty of Health Sciences Linköping University Sweden Linköping 2008 © Barbro Hedin Skogman 2008 Cover design: drawing by Otto Skogman, tick photo by Sjukhusfotograferna, University Hospital and final design with help from Dennis Netzell, LiU-Tryck, Linköping. Paper I and II have been reprinted with permission by Elsevier Ltd and Oxford University press Printed in Sweden by LiU-Tryck, Linköping, 2008 ISBN: 978-91-7393-961-4 ISSN 0345-0082 To Stefan ”Ticks and other weird bugs…”, by Otto, Bobo and Balder 1 ABSTRACT Lyme Borreliosisis is a multi-organ infectious disease caused by the spirochete Borrelia burgdorferi. The spirochete is transmitted to humans by tick bites. Neuroborreliosis (NB) is a disseminated form of the disease, in which the spirochetes invade the nervous system. In children, subacute meningitis and facial nerve palsy are typical clinical manifestations of NB. The aim of this thesis was to study clinical, immunological and laboratory characteristics in children being evaluated for NB in a Lyme endemic area of Sweden, in order to identify factors of importance for prognosis and clinical recovery. A total of 250 patients and 220 controls were included during 1998-2005, with a prospective and a retrospective part. Less than half (41%) of children with signs and symptoms indicative of NB got the diagnosis confirmed by detection of Borrelia specific flagella antibodies in CSF (clinical routine method). Surprisingly few patients were diagnosed as having other infectious or neurologic diseases and consequently, many patients ended up with an uncertain diagnosis.
    [Show full text]
  • ALS Or ALS Mimic by Neuroborreliosis—A Case Report
    Received: 1 August 2019 | Revised: 10 October 2019 | Accepted: 21 October 2019 DOI: 10.1002/ccr3.2569 CASE REPORT ALS or ALS mimic by neuroborreliosis—A case report Isabelle Wirsching | Nora Ort | Nurcan Üçeyler Department of Neurology, University of Würzburg, Würzburg, Germany Abstract Comprehensive investigation in motor neuron disease is vital not to miss a treat- Correspondence able differential diagnosis. Neuroborreliosis should be considered during an ALS Nurcan Üçeyler, Department of Neurology, University of Würzburg, Josef-Schneider- work-up. However, false-positive CSF results do occur, and thus, results should be Str. 11, Würzburg, Germany. interpreted carefully in context of all clinical test results. Email: [email protected] KEYWORDS Present address ALS mimic, El Escorial, motor neuron disease, muscle atrophy, neuroborreliosis Isabelle Wirsching, Charité Department of Neurology, University of Berlin, Berlin, Germany Funding information This publication was funded by intramural funds and the funding program Open Access Publishing of the University of Würzburg, Germany. N.Ü. was funded by the German Research Foundation (DFG; UE-171/5-1). 1 | INTRODUCTION 2 | CASE REPORT We report a 63-year-old man, who developed bilateral atrophic 2.1 | Patient history arm paresis with preserved reflexes. Criteria for probable, lab- oratory-supported ALS (El Escorial) were fulfilled. However, The 63-year-old man was admitted for second opinion cerebrospinal spinal fluid analysis revealed neuroborreliosis. with the suspected diagnosis of a motor neuron disease. We give a detailed description of our patient's case and review He was seen at three visits (July, August, and December literature on neuroborreliosis-associated ALS mimics. 2018). The patient had been healthy until February 2018, The pathophysiology of ALS is incompletely understood, but then developed progressive muscle weakness starting and despite reports on promising first candidates,1,2 objective at his left proximal arm.
    [Show full text]
  • Reversible Dementia Due to Lyme Neuroborreliosis
    Open Access Austin Internal Medicine Case Report Reversible Dementia due to Lyme Neuroborreliosis Bielawski MM2 and Myers JP1* 1Department of Medicine, Division of Infectious Disease, Abstract Summa Health System, Ohio, USA Introduction: Neuroborreliosis accounts for fewer than 10% of patients with 2Resident of Internal Medicine, Summa Health System/ Lyme disease. We present a patient with impaired memory and cognition, right Northeast Ohio Medical University Program, Ohio, USA leg weakness and left hip pain who was suspected of having Lyme disease *Corresponding author: Myers JP, Internal Medicine because of atypical axillary erythema migrans. Residency Summa Akron City Hospital, Ohio, USA Case Description: A 76 year old man with non-critical aortic stenosis, Received: January 18, 2018; Accepted: February 23, asthma, and prostatic hypertrophy presented to his internist’s office in June, 2018; Published: March 08, 2018 2016 with a one-month history of frequent falls, forgetfulness, right lower extremity weakness, and left hip pain. During a recent insurance physical, he was told that these symptoms might be due to dementia and that he should promptly be evaluated by his primary care physician. His physician found the patient to have significant cognitive problems, a right axillary targetoid skin rash and small (~0.5cm diameter) blotchy satellite macular lesions on his trunk and extremities. Detailed history revealed frequent visits of the patient to a 100-acre land plot that he owns in western Pennsy vania where he had frequent exposure to ticks. His most recent visit was 10 days prior to the visit to his primary care physician. The patient was examined and immediately admitted to the hospital for evaluation and treatment.
    [Show full text]
  • Lyme Neuroborreliosis in Children
    brain sciences Review Lyme Neuroborreliosis in Children Sylwia Kozak 1 , Konrad Kaminiów 1 , Katarzyna Kozak 1 and Justyna Paprocka 2,* 1 Students’ Scientific Society, Department of Pediatric Neurology, Faculty of Medical Sciences in Katowice, Medical University of Silesia, 40-752 Katowice, Poland; [email protected] (S.K.); [email protected] (K.K.); [email protected] (K.K.) 2 Department of Pediatric Neurology, Faculty of Medical Sciences in Katowice, Medical University of Silesia, 40-752 Katowice, Poland * Correspondence: [email protected] Abstract: Lyme neuroborreliosis (LNB) is an infectious disease, developing after a tick bite and the dissemination of Borrelia burgdorferi sensu lato spirochetes reach the nervous system. The infection occurs in children and adults but with different clinical courses. Adults complain of radicular pain and paresis, while among the pediatric population, the most common manifestations of LNB are facial nerve palsy and/or subacute meningitis. Moreover, atypical symptoms, such as fatigue, loss of appetite, or mood changes, may also occur. The awareness of the various clinical features existence presented by children with LNB suspicion remains to be of the greatest importance to diagnose and manage the disease. Keywords: lyme borreliosis; neuroborreliosis; children; meningitis; facial nerve palsy; neurological manifestations; treatment; diagnosis Citation: Kozak, S.; Kaminiów, K.; Kozak, K.; Paprocka, J. Lyme 1. Introduction Neuroborreliosis in Children. Brain Lyme borreliosis (LB) is a tick-borne disease caused by spirochetes of the Borrelia Sci. 2021, 11, 758. https://doi.org/ burgdorferi sensu lato complex, especially by the following genospecies: B. burgdorferi sensu 10.3390/brainsci11060758 stricto, B. afzelii, and B. garinii [1].
    [Show full text]
  • Cerebral Vasculitis and Intracranial Multiple Aneurysms in a Child with Lyme Neuroborreliosis
    CASE REPORT Kortela et al., JMM Case Reports 2017;4 DOI 10.1099/jmmcr.0.005090 Cerebral vasculitis and intracranial multiple aneurysms in a child with Lyme neuroborreliosis Elisa Kortela,1,* Jukka Hytönen,2 Jussi Numminen,3 Margit Overmyer,4 Harri Saxen5 and Jarmo Oksi6 Abstract Introduction. Lyme borreliosis is a multisystem tick-borne disease caused by Borrelia burgdorferi. Neurological manifestations are reported in up to 15 % of adult patients with Lyme disease, while the frequency among children is higher. The most common manifestations are painful radiculopathy, facial nerve paresis and lymphocytic meningitis. Epileptic seizures and cerebral vasculitis with stroke or aneurysms are very rare complications. Case presentation. We describe a paediatric patient with sensorineural auditory dysfunction, headache, fatigue and epileptic seizures as sequelae of meningoencephalitis/Lyme neuroborreliosis (LNB) caused by B. burgdorferi. Brain magnetic resonance imaging revealed widespread enhancement of the leptomeninges, cranial nerves and artery walls compatible with vasculitis and disturbances in cerebrospinal fluid (CSF) circulation. The patient was treated with ceftriaxone for 2 weeks. Two years later, the patient had an ischemic stroke. Brain magnetic resonance angiography revealed multiple aneurysms, which were not present previously. The largest aneurysm was operated rapidly. The patient was treated with another course of intravenous ceftriaxone for 4 weeks and pulse therapy with corticosteroids. He recovered well. Conclusion. This unique case demonstrates complications of LNB that can result in serious morbidity or even mortality. Lumbar puncture and analysis should be considered for paediatric patients with epileptic seizures or cerebrovascular events living in a Lyme borreliosis endemic area. INTRODUCTION production [positive cerebrospinal fluid (CSF)/serum anti- Lyme borreliosis is a multisystem tick-borne disease caused body index].
    [Show full text]