A case of neurosyphilis with a florid Jarisch-Herxheimer reaction

A 37 year old man with a 2 year history of progressive cognitive decline, unilateral tinnitus and deafness presented with complex partial seizures and a fever. On examination there was a sluggish right pupillary response but no other abnormal findings. Serum and CSF syphilis serology were both strongly positive. High dose intravenous penicillin therapy was complicated by a severe Jarisch-Herxheimer reaction (JHR) characterised by fever, obtundation, fluctuating upper motor neuron signs and complex visual and auditory hallucinations. These symptoms resolved over three days and the course of penicillin was completed. At discharge the patient's cognitive functioning was unchanged from the pretreatment state. He made gradual improvement over the following months but remains unable to live alone or work. Clinical, pathologic and radiologic findings of neurosyphilis are reviewed, as is the JHR, a self-limiting, systemic febrile response related to massive cytokine release that can occur in response to treatment of a number of bacterial infections. The similarities in pathophysiology of the JHR and the Septic Shock Syndrome are discussed, with particular reference to use of the JHR as a potential model for therapeutic agents in the treatment of septic shock.