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Brief resolved unexplained event (BRUE) is a common pediatric problem that presents to ambulatory and emergency settings. Infants presenting with a BRUE can be separated into low- and high-risk groups per recent guidelines. Most low-risk infants who present with a BRUE can be discharged home with anticipatory guidance and education provided to the caregivers; however, high-risk infants should undergo further testing and observation to determine the cause of their event. Congenital neurosyphilis can be a rare cause of a BRUE. Therefore, high-risk infants with a BRUE should have evaluations focused on potential diagnosis supported by the patient's history and physical examination. [ Pediatr Ann. 2017;46(2):e61–e64.]

Summary – Late congenital syphilis is a rare disease and its neurological manifestation is exceptional. We report a case that was revealed by juvenile dementia paralytica, combined with basal ganglia calcifications after a computed tomography.Case report – A twelve-year old patient showed a diminished school performance, then gradually over a period of six months suffered from dementia with general paresis and was bed ridden. Serologies of treponematosis in the blood stream and cerebrospinal fluid confirmed neurosyphilis in its meningoencephalitis form. An intact hymen and the family background suggested congenital origin. Computed tomography of the brain indicated diffuse cortical and subcortical atrophy with secondary hydrocephalus, and several fairly symmetrical bilateral calcifications at the level of the basal ganglia calcifications (caudate nuclei, lenticular nuclei and thalamus). After a 24-month course of penicilline, the evolution was not very positive, although the patient did recover deglutition and the ability to maintain a seated position.Comments – This case report underlines the importance of studying late congenital neurosyphilis and of considering its occurrence in cases of child dementia with or without brain calcifications. Children belonging to a population at risk with behavioural problems or a diminished school performance should undergo serologies of treponematosis., Introduction – La syphilis congénitale tardive est une affection rare, son expression neurologique est exceptionnelle. Nous en rapportons une observation révélée par une démence paralytique juvénile, associée à des calcifications des noyaux gris centraux à la tomodensitométrie.Observation – Une patiente de douze ans présentait une diminution des résultats scolaires, puis progressivement en six mois une démence avec paralysie générale et grabatisation. Les sérologies des tréponématoses dans le sang et le liquide céphalorachidien confirmaient la neurosyphilis dans sa forme méningo-encéphalitique. La présence d’un hymen intact et l’histoire familiale faisaient évoquer une origine congénitale. La tomodensitométrie cérébrale montrait une atrophie cortico-sous corticale diffuse avec hydrocéphalie secondaire, et de multiples calcifications bilatérales, assez symétriques, au niveau des noyaux gris centraux (noyaux caudés, lenticulaires et thalamus). L’évolution avec un recul de 24 mois après pénicilline était peu favorable, avec cependant récupération de la déglutition et de la station assise.Commentaires – Cette observation souligne l’intérêt de connaître la neurosyphilis congénitale tardive, et de l’évoquer devant une démence de l’enfant avec ou sans calcifications cérébrales. Les enfants appartenant à une population « à risque » et qui présentent des troubles du comportement ou une diminution des résultats scolaires doivent pouvoir bénéficier de sérologies des tréponématoses.

The clinical symptoms, serological results and computed tomographic findings of two Zimbabwean children, one with the early meningeal and one with the late parenchymatous form of congenital neurosyphilis are reported. The paediatrician's suspicion of the existence of this condition should remain high as long as syphilis continues to flourish worldwide.

Juvenile neurosyphilis is rare and may be arrested or modified in the early stage by antibiotic treatment for intercurrent infections. Inadequate treatment may weaken the signs and symptoms of neurosyphilis so much that a high index of clinical suspicion is required for early and correct diagnosis. It should be remembered, however, that benzathine penicillin does not constitute adequate treatment for neurosyphilis.

É apresentado um caso de neurolues congênita (meningovascular e parenquimatosa) associada a hidrocefalia do tipo bloqueado. A natureza luética da afecção foi confirmada por exames paraclínicos e histopatológico. O registro do caso se justifica pelo fato da não existência na literatura compulsada de outro semelhante comprovado histopatològicamente. A hidrocefalia ocorreu em conseqüência do bloqueio do sistema ventricular pelo processo inflamatório de natureza crônica.

An investigation was undertaken to determine the presence and incidence of neurosyphilis in cases of congenital syphilis. Twenty infants, diagnosed as having congenital syphilis on the basis of a positive serum fluorescent-treponemal-antibody-absorbed immunoglobulin M (FTA-IgM) test, were studied. Eleven had clinical disease, while 3 had non-specific illnesses, but x-rays very suggestive of syphilis. Six were asymptomatic newborns, born of mothers known to have positive VDRL tests. There was no clinical evidence of neurosyphilis. The cerebrospinal fluid (CSF) was definitely abnormal in 2 babies, most likely abnormal in a third, and very doubtfully positive in 2 premature babies. The incidence of neurosyphilis in this series is much lower than that reported in the past. The problems of diagnosing neurosyphilis, and reasons for the low incidence here are discussed.

Pupils in congenital neurosyphilis differ from the classic Argyll-Robertson pupil in acquired cases, and often tend to be large and unreactive. Constriction to pilocarpine in the reported patient would suggest that peripheral parasympathetic damage is responsible for such pupillary findings. Congenital neurosyphilis should be included among the causes of “tonic” pupil.

Excerpt The prevalence of all forms of congenital syphilis has declined remarkably in the penicillin era, and only three reports of congenital neurosyphilis have been found in the English literatur...