Objective:

To evaluate the frequency of Central Nervous System (CNS) Actinomyces infections initially diagnosed as Tolosa-Hunt Syndrome (THS), and to discuss potential changes to diagnostic criteria to reduce poor outcomes.  

Background:

A 43-year-old man was diagnosed with Tolosa-Hunt Syndrome on initial presentation. Infectious work up at that time was negative. He returned to emergency department with altered mental status weeks later, and was found to have leptomeningeal enhancement, vasculitis, and acute multifocal strokes. He continued to decompensate on broad-spectrum antimicrobials. Patient was ultimately found to have CNS Actinomycosis after Karius next generation sequencing identified Actinomyces. This diagnosis was confirmed with pathology evaluation of biopsied material. Unfortunately, due to the extent of the disease, he was compassionately extubated and passed away.  

Design/Methods:

MEDLINE, EMBASE, the Cochrane Library, and Scopus were searched on November 12, 2023 for articles concerning Tolosa Hunt Syndrome that mentioned an infection. 

Results:

A total of 593 articles were reviewed and eight cases of CNS Actinomyces which were initially diagnosed as Tolosa-Hunt were included in final analysis. The average time to presentation was 5.1 weeks. 62.5% had dental history prior to symptom onset. CSF cultures were obtained in 75% of cases, all of which were negative for Actinomyces. 88% were ultimately diagnosed as CNS Actinomyces by pathology evaluation. 62.5% of cases saw initial symptom improvement upon starting steroids. 25% of cases were fatal.  

Conclusions:

With the low reported prevalence of CNS Actinomyces, the inadequacy of current testing for Actinomyces, and the significant overlap of symptoms with THS, it is essential that clinicians are aware of CNS Actinomyces as a potential infectious etiology in order to provide early and potentially curative treatment. The current definition of THS, as published in the ICHD-3, may encourage harmful practices, and should be revised. Further research into improving cultural sensitivities and next generation sequencing for Actinomyces is necessary.