A Clinical Diagnosis: False-negative CSF PCR in HSV-2-associated Elsberg Syndrome
Ameya Kale1, Melisha Budhathoki2, Alia Tewari2, Pooja Jethwani2, Priya Narwal2
1Neurology, UConn Health, 2UConn Health
Objective:
N/A
Background:
Elsberg syndrome (ES) is a rare cause of acute-subacute lumbosacral viral radiculitis mimicking cauda equina syndrome (CES). It can be caused by HSV-2 after genitourinary infection. We describe a case of ES preceded by vaginal HSV-2 infection with negative CSF PCR and a good functional outcome.
Design/Methods:

Case report

Results:
A 45-year-old woman presented with 1 week of headache, dysuria, low back pain, urinary retention, constipation, vaginal and rectal pain. Two days prior she was found to have multiple tender ulcerative vulvovaginal lesions with PCR swab positive for HSV-2. Valacyclovir was initiated but she developed persistent urinary retention prompting foley catheterization. On examination she had intact strength, reflexes and sensation to all modalities in all extremities. MRI of the neuraxis showed no significant enhancement, demyelination or mass lesion. Lumbar puncture on day 10 revealed opening pressure of 24 cm H2O, 37 nucleated cells (95% lymphocytes), 18 RBCs, protein 113 mg/dL, glucose 100 mg/dL, 1 paired oligoclonal band. ESR, CSF albumin, albumin index, IgG synthesis rate was elevated. CSF HSV-2 PCR was negative but HSV1/2 IgG titer was elevated. HIV, VDRL, tick panel, ANA, Anti-Ro/La antibodies were negative. Treatment with IV acyclovir followed by valacyclovir to complete 21 days and IV methylprednisolone 1 gram daily for 5 days was started with improvement in urinary retention and constipation. At 1 month follow up, she had minimal residual urinary hesitancy.
Conclusions:
Most cases of ES are self limited, though delayed treatment can lead to paraplegia. Imaging may show enhancement of lumbosacral nerve roots or conus, but can be negative. CSF reveals lymphocytic pleocytosis and elevated protein. CSF PCR though gold standard can be falsely negative. In these cases history of antecedent or prior herpes viral infection is supportive of diagnosis. Acyclovir is recommended given a favorable risk-benefit profile, and most patients also receive IV corticosteroids.
10.1212/WNL.0000000000216491
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