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Generalized Convulsive Status Epilepticus Secondary to Jarisch-Herxheimer Reaction in Neurosyphilis: A Case Report and Literature Review

Introduction: Jarisch-Herxheimer reaction (JHR) is defined as a transient immunologic reaction, classically seen in patients during syphilis treatment. JHR is a rare cause of status epilepticus (SE) in neurosyphilis. We describe a case of generalized convulsive SE (GCSE) probably secondary to JHR in a patient with neurosyphilis. Case Report: A 23-year-old man presented with progressive behavioral changes with 4 months of onset and pupillary alteration with 9 months of onset. He had experienced blurred vision and had noticed mydriasis in the left eye. Brain magnetic resonance imaging was normal; however, sexually transmitted infections were not investigated. After 1 month, the blurred vision disappeared, but mydriasis remained. Three months passed, and he reported progressive behavioral changes. Laboratory tests were within the normal limits, except by positive blood venereal disease research laboratory. Later, the cerebrospinal fluid analysis was requested, which revealed a positive venereal disease research laboratory. Electroencephalogram and brain magnetic resonance imaging were normal. Treatment was started with crystalline penicillin G. A GCSE began 12 hours after the first dose of penicillin. He did not develop classic signs and symptoms of the JHR. An EEG showed periodic lateralized epileptiform discharges. Conclusions: To the author’s knowledge, there are 3 cases in the literature of nonconvulsive SE with JHR in neurosyphilis, and the present report was the first to describe the occurrence of GCSE secondary to JHR in neurosyphilis. Another important finding, in this case, was the isolated unilateral mydriatic tonic pupil, rarely reported as an early isolated symptom of neurosyphilis. In this way, it is essential that patients presenting with isolated unilateral tonic pupil receive an adequate investigation for syphilis.