Abstract
Neurosyphilis is a rare but life-threatening complication of syphilis that can develop even decades after the primary infection and can be unrecognized. Seizures and status epilepticus (SE) may represent the first manifestation in a previously undiagnosed syphilitic patient. We present an exemplification case of a new onset refractory status epilepticus caused by neurosyphilis and we reviewed the existing literature. We selected all studies reporting cases of SE in the context both of patients with a known diagnosis of syphilis and as the first manifestation of neurosyphilis. We identified 50 patients, mostly composed of immunocompetent, middle-aged males. Thirty-nine patients (83%) presented a new onset SE. A history of subtle and rapidly progressive mood and/or cognitive impairment suggesting a limbic encephalitis-like presentation was frequently observed. Focal frontal or temporal SE was reported in 26. Brain MRI frequently showed T2/FLAIR hyperintensities widely involving the medial temporal structures and the frontal lobes. This review should increase the clinician’s awareness of neurosyphilis as a possible etiology of a new onset SE of unknown etiology, especially in the context of a “limbic encephalitis”-like clinical presentation. Prompt recognition and treatment for neurosyphilis partially or completely reverse neurologic sequelae, changing the natural history of the disease.
Highlights
Published: 1 December 2021Syphilis, widely known as “the great imitator” for the multitude of its different and subtle clinical presentations, is an infectious venereal disease caused by the spirochete bacterium Treponema pallidum subspecies pallidum
We present a case of a neurosyphilis-related new onset refractory status epilepticus status epilepticus epilepticus in in neurosyphilis neurosyphilis (NORSE) [9]
In the work of Toudou-Daouda et al (2018) [43], five patients were described but just for one of them was it clearly stated that SE was related to neurosyphilis, just one case was considered for the present review
Summary
Widely known as “the great imitator” for the multitude of its different and subtle clinical presentations, is an infectious venereal disease caused by the spirochete bacterium Treponema pallidum subspecies pallidum. HIV infection with which syphilis has a synergistic link [1,2]. Syphilis can be acquired by person-to-person transmission (where organisms enter via skin micro-abrasion or mucous membranes) and through blood transfusion, solid organ transplant or vertically. The primary syphilis is generally characterized by the appearance of a solitary non-tender chancre generally affecting genital areas (but other mucosae could be affected as well) together with local lymphadenopathy. The hematogenous dissemination brings out the clinical protean manifestations of secondary syphilis (condyloma lata, hand and foot lesions, macular rash, diffuse lymphadenopathy, headache, myalgia, arthralgia, pharyngitis, hepatosplenomegaly, alopecia and malaise).
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