When infection mimics cauda equina syndrome: a cautionary tale

A 63-year-old man presented to the emergency department with low back pain, perineal and genital numbness, together with bilateral lower limb paraesthesia and urinary retention. He was admitted under the orthopaedic service for investigation of suspected cauda equina syndrome. Magnetic resonance imaging of his spine did not reveal any evidence of cauda equina compression. Magnetic resonance imaging of his brain demonstrated nonspecific multiple hyperintensities in the right frontotemporal and left temporo-occipital regions. Computed tomography of his chest, abdomen, and pelvis did not identify any evidence of malignancy. Cerebrospinal fluid from a lumbar puncture showed a high leucocyte count (predominantly lymphocytes). Viral cerebrospinal fluid polymerase chain reaction was positive for varicella zoster virus. A diagnosis of varicella zoster virus myeloradiculitis (Elsberg syndrome) was established and the patient was treated with intravenous aciclovir. Unfortunately, the patient succumbed to a devastating intracerebral haemorrhage during his inpatient stay, probably due to vasculopathy from the underlying varicella zoster virus infection. This case describes a rare infectious mimic of cauda equina syndrome. Elsberg syndrome is an infectious syndrome characterised by bilateral lumbosacral myeloradiculitis, with varicella zoster virus being a well-recognised aetiological agent. We discuss the relevant literature in detail and identify the key, cautionary lessons learned from this case.

Elsberg Syndrome

Context: Elsberg Syndrome (ES) is a rare syndrome associated with acute or subacute infections, often accompanied by myelitis confined to the lower spinal cord, in addition to paresthesia, weakness in the lower limbs, urinary retention, constipation, among others. It is a self-limiting disease, manifested through primary HSV type 2 infection, especially in immunocompetent patients. Case report: Male patient J.F.C., 58 years old, reports a sudden paresthesia in the lower limbs without other associated clinical conditions for approximately 2 months that progressed to paresis, cramps and loss of sphincter control. Admitted to the General Hospital of Palmas, Tocantins on 02/18/2020 with suspicion of Peripheral Polyneuropathy that was discarded after electroneuromyography. Imaging exams without relevant findings. After a week of hospitalization, multiple lesions appeared in the region of the lower third of the dorsum and buttocks, compatible with lesions by HSV (herpes simplex virus) and the diagnosis of ES was concluded. At the moment, he was undergoing treatment with acyclovir and awaiting the evolution of the condition. Conclusion: ES is an often unrecognized cause of lumbosacral radiculitis, which is why it is important to establish as a diagnosis differential. The analysis of CSF, PCR and MRI confirm the diagnosis in cases of HSV-2 lumbosacral radiculomyelitis. CSF usually reveals a lymphocytic pleocytosis and a slight elevation of proteins.

Elsberg Syndrome in the Setting of Asymptomatic SARS-CoV-2 infection: Case Report

Background: Elsberg syndrome is a rare cause of lumbosacral radiculitis with concomitant thoracic or lumbosacral myelitis that can be seen following an acute or reactivated viral infection. COVID-19 disease, caused by the SARS-CoV-2 virus, has quickly spread to a global pandemic since its first discovery in the winter of 2019. During this time, there has been an increasing number of case reports describing SARS-CoV-2 associated neuroinflammatory disease.Case Presentation: A 68-year-old man presented in June 2020 with a fall due to progressive lower extremity weakness and numbness, occurring shortly after the initial coronavirus surge in New York City. He developed ascending numbness to the level of the lower abdomen over the preceding month. He subsequently experienced low back pain, and gastrointestinal and genitourinary dysfunction. An extensive laboratory and radiologic evaluation ensued which was notable for elevated SARS-CoV-2 IgG antibodies despite an absence of preceding COVID-19 symptoms. Initial electrodiagnostic testing was notable for absent late responses in the lower extremity nerve conductions with normal distal sensorimotor conductions, and incomplete muscle activation with otherwise normal motor unit morphology and recruitment on electromyography. Repeat testing two weeks later revealed similar nerve conductions, but also the interval development of active neurogenic changes and reduced motor unit recruitment in the L3-L4 myotomes. This was suggestive of a lower thoracic and lumbosacral myelopathy and lumbosacral polyradiculopathy without peripheral neuropathy. A diagnosis of Elsberg syndrome was made and treatment with intravenous methylprednisolone yielded mild clinical improvement and the electrodiagnostic re-emergence of the lower extremity late responses.Conclusions: We report here, to our knowledge, the first case of suspected COVID-19 associated Elsberg syndrome, which may help to shed light on ways in which to approach diagnostic and treatment options in COVID-19 patients presenting with uncommon neurological and autonomic manifestations.