Deterioration of MRI findings related to Jarisch–Herxheimer reaction in a patient with neurosyphilis

Abstract

Syphilis is a systemic infection that can involve the central nervous system at any stage. In patients with syphilis, Jarisch-Herxheimer reaction (JHR) usually occurs within 24 h after antibiotic treatment [5]. Although status epilepticus (SE) due to JHR has been reported in some patients with neurosyphilis [2, 3], neuroradiological findings related to JHR have not been well described. Here we report the case of a patient with parenchymatous neurosyphilis who developed SE due to JHR, which was accompanied by deterioration of MRI findings in the acute phase of the reaction.A 48-year-old man presented with simple partial seizures 4 months before admission to our hospital. The seizures were well controlled by carbamazepine in the local hospital. He was admitted to our hospital with gradually worsening unsteady gait for a 2 weeks duration. On neurologic examination, homonymous hemianopia of the left lower quadrant, anisocoria, and truncal ataxia were observed.Neuropsychological examination showed impaired memory and attention; he was HIV negative. Cerebrospinal fluid (CSF) examination showed a mononuclear WBC count of 26/mm 3 with elevated total protein levels (70 mg/dL). There were no atypical cells in CSF. Rapid plasma reagin (RPR) and fluorescent treponemal antibody absorption (FTA-ABS) titers in the serum and the CSF were positive (serum RPR 1:16, serum FTA-ABS 1:1,280, CSF RPR 1:8, CSF FTA-ABS 1:1,024). MRI of the brain showed hyperintense lesions on T2-weighted images in the frontal, temporal, and occipitoparietal lobes; corpus callosum; and cingulate gyrus (Fig. 1a). The cerebral cortices in these lesions were partially enhanced on contrast-enhanced T1-weighted images (Fig. 1d, g). Consequently, a diagnosis of parenchymatous neurosyphilis was made, and the patient was started on a treatment with intravenous penicillin G at a dose of 24 million units daily for 2 weeks. However, the patient developed chills and fever 7 h after initiation of treatment, and these constitutional symptoms were followed by nuchal rigidity and fluctuations of consciousness. Subsequently, 16 h after treatment, the patient developed continuous seizures of the right upper extremity with severely impaired consciousness. The condition was diagnosed as developing complex partial SE. Repeat MRI study showed that pre-existing hyperintense lesions on T2-weighted images were enlarged in the frontal lobes (Fig. 1b). Furthermore, intense cortical enhancement in the frontal lobes, left occipitoparietal lobe, cingulate gyrus, and insula was demonstrated (Fig. 1e, h). Intravenous methylprednisolone (250 mg/day for a day) was administered on the day following commencement of the new set of symptoms. The seizures were well controlled with carbamazepine. Although visual field deficits persisted, unstable gait and cognitive dysfunction were significantly improved. Contrast enhancement in the cerebral cortices

Gummatous Neurosyphilis With Transient Worsening of Neurological Symptoms After Treatment Initiation

“…8 Deterioration of the MRI findings with JHR with subsequent improvement after continuation of antibiotic therapy may occur, as in this case. 9 Other conditions to consider when there is neurological deterioration in gummatous neurosyphilis despite antibiotic treatment are complications of the infection, such as seizures and cerebrovascular thrombosis with ischemia or antibioticrelated neurotoxicity. [9][10][11] In some instances, JHR can be life-threatening due to severe systemic inflammatory response and acute end-organ failure.…”

“…9 Other conditions to consider when there is neurological deterioration in gummatous neurosyphilis despite antibiotic treatment are complications of the infection, such as seizures and cerebrovascular thrombosis with ischemia or antibioticrelated neurotoxicity. [9][10][11] In some instances, JHR can be life-threatening due to severe systemic inflammatory response and acute end-organ failure. In neurosyphilis, it may result in the development of epilepsy or persistent neurological deficit.…”

“…8 Deterioration of the MRI findings with JHR with subsequent improvement after continuation of antibiotic therapy may occur, as in this case. 9…”

Epilepsy and syphilis: A systematic review and meta-analysis

“…In addition, five case reports described epileptic seizure as a symptom of the Jarisch-Herxheimer reaction. 21,32,46,52 One article contained a case report and investigation of the prevalence of epilepsy in patients with syphilis; another article contained case reports of two patients with syphilis and epilepsy and a patient who exhibited the Jarisch-Herxheimer reaction; two case reports described two patients each, and another case report described three patients. 10,16,36,37 Thus, we collected a total of 45 case reports describing patients with syphilis and epilepsy (excluding one case report that solely described electroencephalography findings 57 ).…”

Elevated CSF Cytokines in the Jarisch-Herxheimer Reaction of General Paresis

“…In another study, one patient with general paresis developed a JHR with a fever and multiple seizures following penicillin treatment. 13 The pathogenesis of JHR remains controversial. The literature has considered endotoxins, complement, histamine, tumor necrosis factor, release of toxic factors in dying spirochetes, and cytokines; however, to date, there has been no consensus regarding the cause.…”