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March 2023; 10 (2) Clinical/Scientific NoteOpen Access

Elsberg Syndrome Secondary to Cytomegalovirus Infection in an Immunocompetent Patient

A Case Report

View ORCID ProfileLucie Lefeuvre, Manuel Schibler, Patrice H. Lalive
First published December 23, 2022, DOI: https://doi.org/10.1212/NXI.0000000000200079
Lucie Lefeuvre
From the Department of Neurosciences (L.L., P.H.L.), Division of Neurology, Geneva University Hospital, Geneva and Faculty of Medicine, Switzerland; Laboratory of Virology (M.S.), Laboratory Medicine Division, Diagnostic Department, Geneva University Hospitals, Switzerland; Division of Infectious Disease (M.S.), Department of Medicine, Geneva University Hospitals, Switzerland; and Diagnostic Department (P.H.L.), Division of Laboratory Medicine, Geneva University Hospital, Switzerland.
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Manuel Schibler
From the Department of Neurosciences (L.L., P.H.L.), Division of Neurology, Geneva University Hospital, Geneva and Faculty of Medicine, Switzerland; Laboratory of Virology (M.S.), Laboratory Medicine Division, Diagnostic Department, Geneva University Hospitals, Switzerland; Division of Infectious Disease (M.S.), Department of Medicine, Geneva University Hospitals, Switzerland; and Diagnostic Department (P.H.L.), Division of Laboratory Medicine, Geneva University Hospital, Switzerland.
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Patrice H. Lalive
From the Department of Neurosciences (L.L., P.H.L.), Division of Neurology, Geneva University Hospital, Geneva and Faculty of Medicine, Switzerland; Laboratory of Virology (M.S.), Laboratory Medicine Division, Diagnostic Department, Geneva University Hospitals, Switzerland; Division of Infectious Disease (M.S.), Department of Medicine, Geneva University Hospitals, Switzerland; and Diagnostic Department (P.H.L.), Division of Laboratory Medicine, Geneva University Hospital, Switzerland.
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Citation
Elsberg Syndrome Secondary to Cytomegalovirus Infection in an Immunocompetent Patient
A Case Report
Lucie Lefeuvre, Manuel Schibler, Patrice H. Lalive
Neurol Neuroimmunol Neuroinflamm Mar 2023, 10 (2) e200079; DOI: 10.1212/NXI.0000000000200079

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Abstract

Objectives Infectious lumbosacral radiculitis and myelitis, a clinical entity called Elsberg syndrome, is classically linked to HSV-2 and VZV. Here, we report a case of an Elsberg syndrome caused by primary cytomegalovirus (CMV) infection in an immunocompetent patient.

Methods Here is a case report at an academic medical center. Cerebral and spinal cord MRI, electroneuromyography, and serum and CSF analysis were performed.

Results We investigated a 31-year-old healthy woman presenting with acute paresthesia of both feet ascending to the pelvic region, urinary retention, and constipation. Neurologic examination revealed symmetrical hyperesthesia of both inferior limbs up to the pelvic region, with patellar and Achilles hyporeflexia. Although MRI was normal, a dysfunction of the S1 left nerve root was observed on electroneurography. CSF analysis was inflammatory. Blood CMV PCR was positive, and anti-CMV IgG/IgM values indicated seroconversion. Taken together, these results strongly suggested an Elsberg syndrome caused by CMV primary infection. After a course of ganciclovir, a marked improvement of the symptoms was observed.

Discussion This case highlights that CMV primary infection can be a cause of Elsberg syndrome in immunocompetent patients. CMV testing should be discussed in these patients to initiate adequate antiviral therapy.

Glossary

CMV=
cytomegalovirus;
CSF=
cerebrospinal fluid;
PMN cells=
polymorphonuclear cells

A 31-year-old woman, in good health, complained of headache and chills. Then, over a week, she progressively developed neuralgic pain on the latero-inferior part of both thighs, ascending diffused tactile hyperesthesia from both feet to the pelvic region, urinary retention, and constipation. She had no weakness. Patellar and Achilles reflexes were hypovivid. Cutaneo-anal reflex and Babinski sign were negative. A chilled water bladder filling test did not show abnormal bladder contraction.

Brain and spinal cord MRI were normal. A dysfunction of sensitive fibers of S1 left root was observed on electroneurography, consistent with an acute cauda equina polyradiculopathy.

CSF analysis showed a marked elevation of leucocytes (87 M/l) with 98% monolymphocytes and 1% PMN, and elevation of proteins (0.66 g/L), with a positive CSF/serum albumin ratio. A multiplex PCR panel performed in the CSF (BIOFIRE FILMARRAY Meningitis-Encephalitis [ME] Panel), testing 14 different germs (including viruses, bacteria, and yeast), was negative. However, serial cytomegalovirus (CMV) serology values were consistent with a recent primary infection. Indeed, on admission day, the CMV IgM antibody level was strongly positive at 19.3 U/mL (N < 1), whereas the CMV IgG antibody level was slightly positive (at 1.9 U/mL [N < 1]). Four weeks after admission, the IgM index decreased at 5.2 and the IgG index increased at 3.8 (Table). In addition, CMV serology was negative in a blood sample performed a year before. CMV DNA was detected at a low level 3 days after admission. No other active infectious disease was detected in the serum, including HIV, A/B/C/E hepatitis, HSV1/2, EBV, VZV, and Borrelia burgdorferi. There was no finding suggesting an immune-mediated or a toxico-metabolic disease.

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Biological Data of the Patient

Once CMV primary infection was diagnosed, the IV acyclovir treatment (10 mg/kg/tid) initiated empirically was replaced by IV ganciclovir (5 mg/kg/bid) for 2 days, followed by valganciclovir (900 mg/bid) for 9 days. Neuralgic pain and hyperesthesia disappeared within 2 days after ganciclovir initiation. Urinary retention and constipation rapidly improved but remained problematic during 6 weeks after symptoms onset. Four weeks after admission, a second CSF analysis showed a decreased leucocyte count (15 M/l) and a normal protein level (Table).

Discussion

Elsberg syndrome is defined as an acute or subacute bilateral lumbosacral radiculitis, often accompanied by lower spinal cord myelitis, and is typically associated with HSV-2 and VZV infections.1

In this patient, clinical and paraclinical examinations confirmed a cauda equina polyradiculopathy. CMV primary infection was demonstrated by specific CMV seroconversion. Since the neurologic deficit was restricted to the peripheral nervous system, the negative PCR for CMV in the CSF was expected, similarly to what is observed in other subacute inflammatory peripheral nerve diseases, such as Guillain-Barré syndrome. In CMV polyradiculopathies, positive CSF PCR for CMV was found mostly in immunosuppressed patients with AIDS.2,3,4

The patient fulfilled Mayo Clinic diagnostic criteria for Elsberg syndrome.1 Differential diagnosis may include MOG-IgG–related disease when radiculitis is associated with myelitis, which was not the case for this patient. We therefore concluded that the Elsberg syndrome was linked to primary CMV infection in this patient. Of note, neurologic complications of primary CMV infection in immunocompetent adults are rare. From 1950 to 2007, 290 cases were registered.5 CMV infection was mostly diagnosed based on immunoglobulin class-switching from anti-CMV IgM to IgG antibodies during the course of illness. Gastrointestinal tract (mainly colitis) was the first site affected. Nervous system was the second site with 56 cases reported. Neurologic manifestations included myelitis, meningitis, meningoencephalitis, myeloradiculopathy, cranial nerve palsy, and polyneuropathy.5 The pathogenesis of peripheral neuropathy after CMV infection remains unclear, in particular whether it is due to a direct toxic effect of the viral infection or due to autoimmunity triggered by molecular mimicry.3

To the contrary, CMV can cause various severe manifestations in immunocompromised patients through either primary CMV infection or reactivation of latent CMV infection.5 Indeed, in immunocompromised patients, encephalitis, myeloradiculopathy, polyradiculopathy, and polyneuropathy secondary to CMV infection are classically reported.6 Notably, a rapidly progressive polyradiculopathy, predominantly involving nerve roots, is well-described in association with CMV infection in AIDS or in transplanted patients.2,7

In immunocompromised patients, the prognostic of CMV polyradiculopathy without treatment is very poor with a 100% mortality rate. Under appropriate antiviral therapy, the symptom resolution most often takes weeks and may be partial.4 In our case, the recovery was observed after a shorter course of treatment and may be explained by the fact that the patient was immunocompetent.4

A prompt diagnosis of CMV infection in immunocompetent patients with peripheral neurologic manifestations may improve their outcome through initiating an early and appropriate antiviral therapy.

Study Funding

The authors report no targeted funding.

Disclosure

The authors report no relevant disclosures.

Appendix Authors

Table

Footnotes

  • Go to Neurology.org/NN for full disclosures. Funding information is provided at the end of the article.

  • Submitted and externally peer reviewed. The handling editor was Editor Josep O. Dalmau, MD, PhD, FAAN.

  • Received September 17, 2022.
  • Accepted in final form April 11, 2022.
  • Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

References

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    . Elsberg syndrome: a rarely recognized cause of cauda equina syndrome and lower thoracic myelitis. Neurol Neuroimmunol Neuroinflamm. 2017;4(4):e355. doi: 10.1212/NXI.0000000000000355
    OpenUrlAbstract/FREE Full Text
  2. 2.↵
    1. Rubin DI
    . Acute and chronic polyradiculopathies. Continuum (Minneap Minn). 2011;17(4):831–854. doi: 10.1212/01.CON.0000403798.87740.cc
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    1. Thongpooswan S,
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    3. Alfishawy M, et al.
    Polyradiculopathy and gastroparesis due to cytomegalovirus infection in AIDS: a case report and review of literature. Am J Case Rep. 2015;16:801–804. doi: 10.12659/ajcr.894512
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  4. 4.↵
    1. Kim YS,
    2. Hollander H
    . Polyradiculopathy due to cytomegalovirus: report of two cases in which improvement occurred after prolonged therapy and review of the literature. Clin Infect Dis. 1993;17(1):32–37. doi: 10.1093/clinids/17.1.32
    OpenUrlCrossRefPubMed
  5. 5.↵
    1. Rafailidis PI,
    2. Mourtzoukou EG,
    3. Varbobitis IC,
    4. Falagas ME
    . Severe cytomegalovirus infection in apparently immunocompetent patients: a systematic review. Virol J. 2008;5:47. doi: 10.1186/1743-422X-5-47
    OpenUrlCrossRefPubMed
  6. 6.↵
    1. Kleinschmidt-DeMasters BK,
    2. Gilden DH
    . The expanding spectrum of herpesvirus infections of the nervous system. Brain Pathol. 2001;11(4):440–451. doi: 10.1111/j.1750-3639.2001.tb00413.x
    OpenUrlCrossRefPubMed
  7. 7.↵
    1. Papasotiriou M,
    2. Papachristou E,
    3. Marangos M, et al.
    Cytomegalovirus polyradiculopathy of late onset in a young renal transplant recipient. Clin Nephrol. 2013;80(1):75–78. doi: 10.5414/cn107479
    OpenUrlCrossRef

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