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Lyme Disease and Neurology Issues

Sample Articles on Seizures, ALS, Neuron Damage, Headaches, and Many Other Brain Pathologies

1. Eur J Paediatr Neurol. 2010 May;14(3):274-7. Epub 2009 Sep 12.

Uncommon manifestations of neuroborreliosis in children.

Baumann M, Birnbacher R, Koch J, Strobl R, Rostásy K.

Department of Pediatrics, Division of Pediatric Neurology and Inherited Metabolic Disorders, Medical University of Innsbruck, Anichstrasse 35, A-6020 Innsbruck, Austria. matthias.baumann@uki.at

Lyme borreliosis is a tick-borne spirochetal infection which affects the skin, joints, heart and nervous system. Children with a neuroborreliosis usually present with a facial nerve palsy or aseptic meningitis, but the spectrum also includes other rare manifestations. We report four unusual cases of childhood neuroborreliosis and show that seizures with regional leptomeningeal enhancement, acute transverse myelitis, meningoradiculitis with pain and paraesthesia and cranial nerve palsies other than facial nerve palsy can be the leading symptoms of children with neuroborreliosis. All children had serological evidence of an acute infection with Borrelia burgdorferi, a pleocytosis in the cerebrospinal fluid and a complete response to antibiotic treatment. An intrathecal synthesis of IgG antibodies was detected in three children. Thus, diagnostic work up in children with unusual neurological symptoms should include cerebrospinal fluid studies with determination of the white blood cell count and calculation of the antibody index against B. burgdorferi. Copyright 2009 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

PMID: 19748808 [PubMed - in process]

2. Eur J Neurol. 2009 May;16(5):639-42. Epub 2009 Mar 20.

Tumefactive demyelinating disease treated with decompressive craniectomy.

Nilsson P, Larsson EM, Kahlon B, Nordström CH, Norrving B.

Department of Neurology, Clinical Sciences Lund, Lund University, Sweden. petra.c.nilsson@skane.se

Comment in: Eur J Neurol. 2009 May;16(5):e102.

BACKGROUND: Tumefactive demyelinating disease (TDD) is a rare primary demyelinating disease with diagnostic and therapeutic challenges. METHODS AND RESULTS: We report a 50-year old woman with TDD successfully treated with decompressive craniectomy and corticosteroids. The patient presented with seizures, subacute progressive hemispheric syndrome, and a tumourlike abnormality on MRI. Demyelinating disease was initially considered unlikely. Due to a rapidly evolving herniation syndrome hemicraniectomy was performed. Outcome was favourable with only very mild neurological deficits 6 weeks later. CONCLUSION: TDD should be considered as a differential diagnosis in tumour-like presentations, and appears to have distinctive neuroimaging features. In the advent of treatement failure from high dose corticosteroids and plasmapheresis and development of severe mass effect, decompressive hemicraniectomy is an important treatment option.

PMID: 19309337 [PubMed - indexed for MEDLINE]

3. Przegl Lek. 2007;64 Suppl 3:38-40.

CNS Lyme disease manifestation in children.

Kaciński M, Zajac A, Skowronek-Bała B, Kroczka S, Gergont A, Kubik A.

Department of Pediatric Neurology, Jagiellonian University, Krakow, Poland. neupedkr@cm-uj.krakow.pl

BACKGROUND: Neurological symptoms develop in 10-20% of children with borreliosis. AIM OF THE STUDY: It was a presentation of clinical manifestation of neuroborreliosis in children. MATERIAL AND METHODS: Children with neuroborreliosis and other neurological diseases were admitted to the University Hospital during 2005-2006 without any selection. Of these 9 patients, there were seven males and two females, ranging in age between 3-17 years. Neurological diagnostic was performed using ELISA Biomedica kit and western blot bands. A 2-6 week sequential treatment with either i.v. ceftazidime or amoxicillin and oral doxycycline or amoxicillin was provided. Children were monitored regularly during the next 4-24 months. RESULTS: The 9 children with borreliosis constitute 0.53% of the pediatric neurology department's patients. The clinical manifestation of LD were usual and unusual from patient to patient. They included three cases of facial nerve paralysis (with bilateral paralysis in one case). In two cases, they included transverse myelitis and in a single case, hemiparesis, meningitis and acute ataxia. Typically, other patients with early stage borreliosis first manifest focal seizures, raising the suspicion that borreliosis could be responsible for triggering seizures. The antibiotic treatment was successful in 7 patients and only partially effective in 2 children with facial nerve paralysis. CONCLUSIONS: The most common symptom of neuroborreliosis in children is motor dysfunction. Acute ataxia may be a clinical presentation of neuroborreliosis. It is probable that borreliosis_triggers seizures in children with EEG abnormalities.

PMID: 18431910 [PubMed - indexed for MEDLINE]

4. Praxis (Bern 1994). 2007 May 16;96(20):815-7.

[Lymphadenopathy and absences]

[Article in German]

Staub E, Strozzi S, Aebi C.

Medizinische Poliklinik, Universitätskinderklinik, Inselspital Bern.

A 6-year-old boy presented with deterioration of general well-being during several weeks, headache and swelling of lymph nodes in the neck. In addition, the parents reported brief episodes resembling typical absence seizures. Serological tests and the examination of cerebrospinal fluid revealed neuroborreliosis. At the same time, electroencephalography showed characteristic patterns of absence epilepsy. The boy's condition improved rapidly during a 2-week course of intravenous ceftriaxone and after initiation of antiepileptic therapy. To our knowledge, absence epilepsy has not previously been reported in association with neuroborreliosis. We consider the two conditions to be coincidental.

PMID: 17566418 [PubMed - indexed for MEDLINE]

5. Int J STD AIDS. 2005 Dec;16(12):832-4.

Neurosyphilis in a young adult: very early tertiary syphilis?

Sabbatani S, Manfredi R, Chiodo F.

Department of Clinical and Experimental Medicine, Division of Infectious Diseases, University of Bologna Alma Mater Studiorum, S. Orsola Hospital, Via Massarenti II, 1-40138 Bologna, Italy.

A rare episode of early neurosyphilis occurred in a 34-year-old, otherwise healthy, woman. Based on an isolated positive Borrelia burgdorferi serology (later interpreted as a cross-reaction), early ceftriaxone was initiated, in the suspect of Lyme borreliosis. Even after the diagnosis was corrected into that of a neurosyphilis, ceftriaxone administration was continued, until it achieved complete clinical and microbiological success after 24 days of treatment in a day-hospital setting, and three-weekly penicillin administrations. When considering the differential diagnosis, a luetic aetiology should not be underestimated when facing young patients with signs-symptoms of a meningoencephalitis. Our case report was characterized by an extremely low patient's age, compared with the occurrence of tertiary neurosyphilis, more than three years after the last sexual contacts. The diagnosis was confirmed by highly positive treponemal and non-treponemal serum and cerebrospinal fluid serology, and several suggestive clinical manifestations: seizures, altered mentation, cognitive impairment, lip drop, and anisochoria. These concomitant findings, together with a neuroradiological report indicating a diffuse meningoencephalitis, allowed us to confirm the diagnosis of neurosyphilis, together with a demonstrated cross-reaction of B. burgdorferi serology. Although ceftriaxone benefits from its once-daily administration (and can be easily delivered on outpatient basis), it is not the firstline treatment of neurosyphilis. However, our experience demonstrated a favourable and rapid response to ceftriaxone, in the absence of toxicity and disease sequelae.

PMID: 16336771 [PubMed - indexed for MEDLINE]

6. Arch Dis Child. 2000 Jul;83(1):67-71.

Primarily chronic and cerebrovascular course of Lyme neuroborreliosis: case reports and literature review.

Wilke M, Eiffert H, Christen HJ, Hanefeld F.

Kinderklinik der Georg-August- Universitat, Abteilung Kinderheilkunde, Schwerpunkt Neuropadiatrie, Robert-Koch-Strabetae 40, 37075 Gottingen, Germany.

As part of an ongoing study aiming to define the clinical spectrum of neuroborreliosis in childhood, we have identified four patients with unusual clinical manifestations. Two patients suffered from a primarily chronic form of neuroborreliosis and displayed only non-specific symptoms. 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. Two further children who presented with acute hemiparesis as a result of cerebral ischaemic infarction had a cerebrovascular course of neuroborreliosis. One was a 15 year old girl; the other, a 5 year old boy, is to our knowledge the youngest patient described with this course of illness. Following adequate antibiotic treatment, all patients showed substantial improvement of their respective symptoms. Laboratory and magnetic resonance imaging findings as well as clinical course are discussed and the relevant literature is reviewed.

PMCID: PMC1718399 PMID: 10869004 [PubMed - indexed for MEDLINE]

7. Eur J Emerg Med. 1999 Dec;6(4):397-401.

Frightening dreams and spells: a case of ventricular asystole from Lyme disease.

Weissman K, Jagminas L, Shapiro MJ.

Brown University School of Medicine, Rhode Island Hospital, Department of Emergency Medicine, Providence 02903, USA.

We present a case of a 20-year-old woman who presented with a febrile illness, frightening dreams and repeated short episodes of apparent seizure activity. Third degree heart block and ventricular asystole were noted on the monitor when the patient experienced a spell during conscious sedation for a lumbar puncture. The combination of heart block and a predominantly lymphocytic cerebrospinal fluid led to the diagnosis of Lyme disease. Lyme titres were strongly positive and subsequently confirmed by Western Blot analysis. Cardiac aetiologies and specifically heart block associated with Lyme disease should be considered in patients from endemic areas presenting with fever and unexplained spells or seizure-like activity.

PMID: 10646930 [PubMed - indexed for MEDLINE]

8. J Emerg Med. 1999 Jul-Aug;17(4):661-4.

Lyme carditis: complete AV dissociation with episodic asystole presenting as syncope in the emergency department.

Rosenfeld ME, Beckerman B, Ward MF, Sama A.

Department of Emergency Medicine, North Shore University Hospital, Manhasset, New York 11030, USA.

We report a case of Lyme carditis in an otherwise-healthy young male who presented to the Emergency Department (ED) with syncope and a possible seizure. This patient, without documented history of Lyme disease, acutely developed third-degree atrioventricular (AV) block with episodic asystole, which required placement of a transvenous pacemaker in the ED and resolved only after the patient had been placed on ceftriaxone. We discuss the significance of Lyme carditis and its increasing prevalence, and review the current literature. We also recommend appropriate screening modalities for patients with known Lyme disease, or an atypical profile for cardiac abnormalities.

PMID: 10431957 [PubMed - indexed for MEDLINE]

9. N Engl J Med. 1998 May 21;338(21):1549-50.

Case 1-1998: a boy with a seizure.

Awad A.

Comment on: N Engl J Med. 1998 Jan 8;338(2):112-9.

PMID: 9599118 [PubMed - indexed for MEDLINE]

10. Am J Emerg Med. 1998 May;16(3):265-9.

Lyme carditis: a rare presentation in an unexpected setting.

Robinson TT, Herman L, Birrer RB, Wallis KJ, Sama A.

Department of Emergency Medicine, Catholic Medical Center of Brooklyn and Queens, Jamaica, NY 11432, USA.

A case is reported of a 27-year-old man who presented to an inner city trauma center after he had experienced several seizure-like episodes. He was diagnosed with Lyme carditis and required 6 weeks of treatment with intravenous ceftriaxone for complete resolution of his symptoms. The case is discussed along with a review of the literature.

PMID: 9596429 [PubMed - indexed for MEDLINE]

11. Pediatr Infect Dis J. 1998 Mar;17(3):189-96.

Neurocognitive abnormalities in children after classic manifestations of Lyme disease.

Bloom BJ, Wyckoff PM, Meissner HC, Steere AC.

Division of Rheumatology/Immunology, Tufts University School of Medicine, and the Floating Hospital for Children, Boston, MA, USA.

BACKGROUND: In adults a subtle encephalopathy characterized primarily by memory impairment, irritability and somnolence may occur months to years after classic manifestations of Lyme disease. However, only limited information is available about whether there is an equivalent disorder in children. METHODS: Case series of five children seen in a Lyme disease clinic in a university referral center for evaluation of neurocognitive symptoms that developed near the onset of infection or months after classic manifestations of Lyme disease. The diagnosis was based on clinical symptoms, serologic reactivity to Borrelia burgdorferi and intrathecal antibody production to the spirochete. Evaluation included detailed neuropsychologic testing. After evaluation the children were treated with intravenous ceftriaxone for 2 or 4 weeks. Follow-up was done in the clinic and a final assessment was made by telephone 2 to 7 years after treatment. RESULTS: Along with or months after erythema migrans, cranial neuropathy or Lyme arthritis, the five children developed behavioral changes, forgetfulness, declining school performance, headache or fatigue and in two cases a partial complex seizure disorder. All five patients had IgG antibody responses to B. burgdorferi in serum as well as intrathecal IgG antibody production to the spirochete. Two patients had CSF pleocytoses and three did not. Despite normal intellectual functioning the five children had mild to moderate deficits in auditory or visual sequential processing. After ceftriaxone therapy, the four children in whom follow-up information was available experienced gradual improvement in symptoms. CONCLUSIONS: Children may develop neurocognitive symptoms along with or after classic manifestations of Lyme disease. This may represent an infectious or postinfectious encephalopathy related to B. burgdorferi infection.

PMID: 9535244 [PubMed - indexed for MEDLINE]

12. Rev Neurol. 1997 Jan;25(137):131-5.

[A 58-year-old woman with disorientation, incoherence and seizures]

[Article in Spanish]

Morera J, Díaz-Marín C.

Seción de Neurología, Hospital Universitario de San Juan, Alicante, España.

PMID: 9091197 [PubMed - indexed for MEDLINE]

13. Brain. 1996 Dec;119 ( Pt 6):2143-54.

Inflammatory brain changes in Lyme borreliosis. A report on three patients and review of literature.

Oksi J, Kalimo H, Marttila RJ, Marjamäki M, Sonninen P, Nikoskelainen J, Viljanen MK.

Department of Internal Medicine, Turku University Central Hospital, Finland.

Despite a rapid increase in the number of patients with Lyme neuroborreliosis (LNB), its neuropathological aspects are poorly understood. The objective of this study was evaluation of neuropathological, microbiological, and magnetic resonance imaging (MRI) findings in three patients with the Borrelia burgdorferi infection and neurological disease from whom brain tissue specimens were available. Perivascular or vasculitic lymphocytic inflammation was detected in all specimens. Large areas of demyelination in periventricular white matter were detected histologically and by MRI in one patient. The disease had a fatal outcome in this patient. Brain MRI suggested malignancies in two patients before histopathological studies were carried out. One of these two patients was a child with sudden hemiparesis. Another was a 40-year-old man presenting with epileptic seizures and MRI-detected multifocal lesions, which disappeared after repeated courses of antibiotics. We conclude that cerebral lymphocytic vasculitis and multifocal encephalitis may be associated with B. burgdorferi infection. The presence of B. burgdorferi DNA in tissue samples from areas with inflammatory changes indicates that direct invasion of B. burgdorferi may be the pathogenetic mechanism for focal encephalitis in LNB.

PMID: 9010017 [PubMed - indexed for MEDLINE]

14. Dtsch Med Wochenschr. 1995 May 5;120(18):636-40.

[Agranulocytosis caused by infectious-toxic bone marrow damage after Borrelia infection]

[Article in German]

Kamp T, Zink C, Beer C, Kaboth W, Nerl C.

I. Medizinische Abteilung, Städtisches Krankenhaus München-Schwabing.

A 19-year-old girl developed a fever of up to 40 degrees C and, during an episode of high fever, generalized seizures. Physical examination on admission was unremarkable, except for several small lymph nodes. Differential blood count showed a leukopenia (1700/microliters) with 14% stab and 7% segmented neutrophils. After initial clinical improvement she again became feverish and the differential count now showed agranulocytosis with a total white cell count of 1400/microliters. Because of the time of year and the geographic location borreliosis was now considered in the differential diagnosis. The antibody titre against Borrelia was raised to 1:64 (IgM) and 1:256 (IgG). Her condition and the differential blood count rapidly improved on intravenous antibiotic treatment with cefotiam (2 g two times daily) and gentamicin (120 mg two times daily), as well as filgrastim (granulocyte-colony stimulating factor) subcutaneously. Antibiotic treatment was continued after 6 days with oral ampicillin (1 g three times daily) for 3 weeks. Follow-up examination six weeks later found the patient to be symptom-free.

PMID: 7750430 [PubMed - indexed for MEDLINE]

15. Pediatr Neurol. 1994 Oct;11(3):258-62.

Tonic "seizures" in a patient with brainstem demyelination: MRI study of brain and spinal cord.

Libenson MH, Stafstrom CE, Rosman NP.

Department of Pediatrics, Floating Hospital for Children, Boston, Massachusetts 02111.

Tonic seizures are a poorly understood manifestation of demyelinating disease, first reported in 4 patients with multiple sclerosis. We describe a patient with tonic extension of the left limbs caused by a right-sided brainstem lesion as the first manifestation of demyelinating disease. A 19-year-old man was referred with a 4-month history of spontaneous attacks of mild paresthesias of the left arm and leg, followed by 15-45 s of rigid extension of the left limbs, occurring up to 25 times per day. Two months after onset, an MRI scan revealed areas of T2 abnormality in the lateral right cerebral peduncle and deep frontal white matter. The EEG was normal, including during hyperventilation which induced a typical episode. All attacks were successfully suppressed by carbamazepine, phenytoin, and valproate monotherapy. Serologic testing for toxoplasmosis, cytomegalovirus, Epstein-Barr virus, Lyme disease, and HIV was negative. Cerebrospinal fluid oligoclonal bands were absent but cerebrospinal fluid immunoglobulin G was mildly elevated (4.2 mg/dl). Over the next 30 months, serial MRIs revealed a normal spinal cord and persistence of the midbrain lesion, with resolution of some of the white matter lesions but reappearance of others. At 46 months, the midbrain lesion resolved on MRI, and the spasms no longer occurred spontaneously, nor could they be elicited by hyperventilation. While two previous reports have shown internal capsule lesions to underlie the tonic spasms in demyelinating disease, this is the first report in which a brainstem lesion has been causative.

PMID: 7880343 [PubMed - indexed for MEDLINE]

16. Microbiol Immunol. 1993;37(4):325-9.

Neurologic abnormalities in two dogs suspected Lyme disease.

Azuma Y, Kawamura K, Isogai H, Isogai E.

Monami Animal Clinic, Hokkaido, Japan.

A 2-year-old mongrel dog developed neurological signs following tick bite. These included astasia, persistent tonic convulsions and hyper-reflexia. Both serum IgG and IgM antibody titers against Borrelia burgdorferi were positive in enzyme-linked immunosorbent assay (ELISA). The neurological signs subsided after high-dose penicillin and streptomycin treatment. A strain of spirochetes (P427a) was isolated from the midgut of Ixodes persulcatus feeding on the dog. Morphological characteristic, immunological property and protein profile revealed that the isolate was B. burgdorferi. Similarly, a 2-year-old Labrador retriever dog developed neurological signs after tick bite and showed a positive IgG antibody titer against B. burgdorferi. Antibiotic treatment was effective also in this case. These findings suggest that neurological symptoms shown in both dogs were caused by infection with B. burgdorferi.

PMID: 8350773 [PubMed - indexed for MEDLINE]

17. Rev Neurol (Paris). 1993;149(8-9):489-91.

[Epilepsy disclosing neuroborreliosis]

[Article in French]

Mourin S, Bonnier C, Bigaignon G, Lyon G.

Service de Neurologie Pédiatrique, Cliniques Universitaires Saint-Luc, Bruxelles.

After two seizures, a 13 year-old boy experienced headache, fatigue and loss of appetite over a period of 3 weeks. There was a bilateral papilledema with normal visual acuity. CT and MRI disclosed two ischemic foci, that were interpreted as evidence of vasculitis. High serum levels of IgG and IgM antibodies specific to Borrelia burgdorferi, were present. The patient had attended an outdoor scout camp in a area, in south-east Belgium, known to be endemic for tick-born borreliosis. The clinical symptoms, the levels of the specific antibodies and the radiologic abnormalities responded dramatically to treatment. We believe that seizures in this case were related to cerebral vasculitis. This case confirms the extreme diversity of the neurological manifestations of Borreliosis.

PMID: 8009148 [PubMed - indexed for MEDLINE]

18. Neurologia. 1992 Feb;7(2):50-4.

[Positive anti-Borrelia antibodies in patients with clinical manifestations compatible with neuroborreliosis]

[Article in Spanish]

Izquierdo G, Aguilar J, Barranquero A, Navarro G, Borobio MV, Angulo S, Domínguez I, Quesada MA.

Servicio de Neurología, Hospital Universitario Virgen de la Macarena, Sevilla.

The clinical features of 19 patients with neurological manifestations unexplained by another disease and positive serology for Borrelia burgdorferi were studied. ECM was present in only 11% of the cases and 32% referred tick bite. The characteristic features for suspicion of NB according to our series was the presence of polyneuritis in 84% of the cases specially in the form of multiple mononeuritis and involvement of the facial nerve (79%) leading to even greater suspicion with the association of V pair involvement. Seizures, sleep disorders, and higher mental dysfunction may be found in association with other more characteristic neurological features. The typical triad of NB (aseptic meningitis, facial paralysis and polyradiculoneuritis) was found in 21% of the patients and in the absence of another disease to justify the same neuroborreliosis (NB) seemed evident. In all the cases components of this triad were found. Headache, arthralgia, fever and, less frequently, arthritis are other symptoms often past with the presence of anti-BB antibodies. Patients with the shortest evolution most frequently presented antecedents of facial paralysis, sensory alterations and Romberg's sign than patients of longer evolution. CSF demonstrated the presence of pleocytosis in 24% of the cases and in only one patient a slight increase in the intrathecal activity of IgG was observed which may be of use in differential diagnosis with MS. MR showed alterations in 61% of the patients and, while not specific, the lesions present subcortical predominance.

PMID: 1610600 [PubMed - indexed for MEDLINE]

19. Ann Emerg Med. 1990 May;19(5):572-6.

The enlarging clinical spectrum of Lyme disease: Lyme cerebral vasculitis, a new disease entity.

Brogan GX, Homan CS, Viccellio P.

School of Medicine, SUNY/Stony Brook 11794-7400.

Comment in: Ann Emerg Med. 1990 Nov;19(11):1353-4.

The case of a patient with cerebral vasculitis with a right thalamic infarct associated with cerebral spinal fluid Lyme disease is presented. This entity has not been described in the United States, and only one similar case in the world literature could be found. The patient presented with a progressive headache and subsequent development of grand mal seizure activity. Lyme disease has been associated with cranial nerve palsies, peripheral and cranial radiculopathies, aseptic meningitis, encephalitic symptoms, chorea, and demyelinating polyneuropathy presenting like Guillain-Barré syndrome. These syndromes can occur separately or in combination. Stroke and strokelike syndromes have been attributed to Lyme disease. The literature concerning the neurologic manifestations of Lyme disease is reviewed.

PMID: 2331105 [PubMed - indexed for MEDLINE]

20. MMWR Morb Mortal Wkly Rep. 1989 Oct 6;38(39):678-80.

Seizures temporally associated with use of DEET insect repellent--New York and Connecticut.

Centers for Disease Control (CDC).

PMID: 2506420 [PubMed - indexed for MEDLINE]

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