1338 - Elsberg Syndrome (ES), a Rare Cause of Cauda Equina Syndrome

Case Diagnosis: Acute lumbosacral radiculitis with myelitis (Elsberg Syndrome)

Case Description: A 47-year-old immunocompetent male presented with a vesicular rash in the left L4 dermatome, left lower limb pain, and severe spasms involving the left quadriceps muscles after receiving an adjuvanted recombinant zoster vaccine (Shingrix). During the hospital course, the patient developed decreased sensation below the left T8 level, urinary retention, and weakness and spasticity involving the left hip flexors, knee extensors, knee flexors, and ankle dorsiflexors. Laboratory evaluation was notable for VZV DNA on skin biopsy and lymphocytic pleocytosis. Magnetic resonance imaging (MRI) revealed abnormal enhancement of several left-sided cauda equina nerve roots with adjacent myelitis, including the left L4 nerve root extending along the left femoral nerve. Treatment with intravenous acyclovir was initiated. Neuropathic pain and spastic dystonia were treated with gabapentin, baclofen, diazepam, and botulinum toxin injections to the left quadriceps muscles with significant improvement.

Discussions: ES is a rare infectious syndrome characterized by lumbosacral radiculitis with or without myelitis. HSV-2 and VZV reactivation are the most common causes. There are no definitive cases of VZV reactivation after the Shingrix vaccine. ES typically presents with acute lower limb weakness and numbness, saddle anesthesia, constipation, and urinary retention. ES presenting as spastic dystonia wasn’t identified in the literature. Cauda equina enhancement on MRI and/or EMG evidence of radiculopathy is required for the diagnosis. Lymphocytic pleocytosis and positive virologic studies support the diagnosis. Myelitis occurs in the distal spinal cord and signal abnormality extending rostral to T9 warrants evaluation of alternative causes. Antivirals and steroids shorten the duration of symptoms, but patients usually have permanent deficits. Oral antispasmodics and botulinum toxin can improve pain related to spasticity and improve function.

Conclusions: We will review the clinical evaluation and symptomatic treatment of ES.