Elsberg syndrome

Last revised by Rohit Sharma on 13 Jun 2023

Elsberg syndrome is an established but rarely recognized cause of cauda equina syndrome and lower thoracic myelitis, presumed to be associated with an infectious etiology. 

Elsberg syndrome is likely responsible for 10% of combined cauda equina syndrome and myelitis 1.

The onset of symptoms is typically with acute cauda equina syndrome 1. The most common clinical feature described is urinary retention, followed by "saddle" paresthesia, constipation, and lower limb weakness 1. Reflex assessments have been variably described among affected patients, with some patients having hyperreflexia whilst others having hypo- or areflexia 1.

It thought that Elsberg syndrome most often occurs with reactivation or primary herpes simplex virus 2 (HSV-2) infection 1 but has also been reported in the setting of varicella zoster virus 1,3.

Radiologic findings are not entirely specific but may help in the differential diagnosis.

Spinal cord lesions are commonly multiple and discontinuous, usually not expansile, and centrally or ventrally positioned in the cord 1. Cord lesions are not necessarily located in the most caudal portion of the conus 1.

Smooth and continuous nerve root enhancement is typical 1. It can be diffuse or limited to ventral or dorsal roots 1

Nerve root enhancement is usually contemporaneous with spinal cord involvement, although either nerve root enhancement or spinal cord parenchymal signal abnormality can predominate 1. In some cases, root enhancement can occur at a later point of the illness 1.

Elsberg syndrome is usually self-limited but commonly leaves some degree of permanent neurological deficit 1. There is no evidence that any specific treatment improves the long-term neurological recovery 4.

Detection of viral infection is useful, but sensitivity appears to be low 1. Treatment with acyclovir is appropriate even in the absence of demonstration of viral infection given the favorable risk-benefit profile of this drug, and may reduce the duration of symptoms 1,4. Concurrent treatment with corticosteroids is also suggested to reduce the duration of symptoms 4.

Despite treatment, it is thought that up to 30% of patients may have recurrence within one year 4.

The condition was first described by Charles Elsberg (1871-1948), American neurosurgeon, in 1931 5.

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