Elsberg syndrome

Last revised by Henry Knipe on 7 Oct 2021

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

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

The onset of symptoms is typically acute, with urinary retention leading to suspected cauda equina involvement 1.

It is most often reactivation or primary herpes simplex virus 2 infection 1 but has been reported in the setting of varicella zoster virus 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. Cord lesions are not necessarily located in the most caudal portion of the conus 1.

Smooth and continuous nerve root enhancement is typical. 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. 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 neurologic deficit 1.

Detection of viral infection is useful, but sensitivity appears to be low.  Treatment with acyclovir is appropriate even in the absence of demonstration of viral infection given the favorable risk-benefit profile of this drug 1.

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