Article type: Case Report
Article
title: Generalized convulsive status epilepticus secondary to jarisch-herxheimer reaction in neurosyphilis: a case report and literature review
Journal: The Neurologist
Year: 2019
Authors:
Jamir Pitton Rissardo, Ana LetÃcia Fornari Caprara, Juliana Oliveira Freitas
Silveira
E-mail: jamirrissardo@gmail.com
ABSTRACT
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. 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. 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.
Key-words: Neurosyphilis, jarisch-herxheimer reaction, status epilepticus, unilateral tonic pupil, adie’s tonic pupil
Full text available at:
DOI
10.1097/NRL.0000000000000219
Citation
Rissardo
JP, Caprara AL, Silveira JO. Generalized convulsive status epilepticus secondary to jarisch-herxheimer reaction in neurosyphilis: a case report and literature review. Neurologist 2019;24:29-32.
Table 1. Case reports of patients with neurosyphilis who probably developed status epilepticus secondary to Jarisch-Herxheimer reaction.
Table 2. Case reports of patients with unilateral mydriatic tonic pupil as an early isolated symptom of neurosyphilis