Sífilis: formas de presentación poco frecuentes / Syphilis: uncommon presentation forms

Resumen Existen formas de presentación poco frecuentes de sífilis, dentro de las cuales se incluyen la neurosífilis, otosífilis y sífilis ocular. La neurosífilis es la infección del sistema nervioso central por Treponema pallidum. Las manifestaciones clínicas de neurosífilis son variadas e incluyen formas tempranas, tardías y atípicas. Además, la sífilis puede comprometer prácticamente cualquier estructura ocular, en cualquier etapa de la enfermedad, como así también la otosífilis. El diagnóstico de estas en tidades suele ser dificultoso. Sin embargo, resulta impor tante considerarlas como diagnósticos diferenciales, ya que la mayoría de estas manifestaciones son reversibles con tratamiento antibiótico adecuado. Se presenta una serie de casos de pacientes con diagnóstico de neurosí filis, otosífilis y sífilis ocular, que cursaron internación en un hospital de tercer nivel: meningitis sifilítica con compromiso de pares craneales y convulsiones (caso 1), sífilis ocular (caso 2), paresis general (caso 3) y tabes dorsalis (caso 4). La mitad de los pacientes presentó hipoacusia neurosensorial bilateral. El 50% presentó VDRL reactiva en líquido cefalorraquídeo. Todos fueron tratados con penicilina G sódica y en el 50% se optó por el uso de ceftriaxona como modalidad para finalizar el tratamiento en internación domiciliaria. Respecto a la evolución de los pacientes, uno de ellos falleció como consecuencia del cuadro de neurosífilis (caso 1), otro se perdió en el seguimiento (caso 4) mientras que, de los dos restantes, el caso 3 presentó recaída de su enferme dad a los 6 meses del tratamiento y el caso 2 resolvió ad integrum su sintomatología.

Abstract Uncommon forms of syphilis exist, among which neurosyphilis, otosyphilis, and ocular syphilis are included. Neurosyphilis is the infection of the central nervous system caused by Treponema pallidum. The clinical manifestations of neurosyphilis are diverse and include early, late, and atypical forms. Syphilis can affect virtually any ocular structure and can oc cur at any stage of the disease, as well as otosyphilis. The diagnosis of these conditions is often challeng ing. However, it is important to consider them as a differential diagnosis, as most of these clinical mani festations are reversible with appropriate antibiotic treatment. A case series study of patients diagnosed with neurosyphilis, otosyphilis, and ocular syphilis, who were admitted to a tertiary-level hospital, is here presented: syphilitic meningitis with cranial nerve in volvement, and seizures (case 1), ocular syphilis (case 2), general paresis (case 3), and tabes dorsalis (case 4). Half of the patients presented bilateral sensori neural hearing loss; and also half of the patients had reactive VDRL in cerebrospinal fluid. All were treated with aqueous penicillin G, and in two of these cases, ceftriaxone was chosen to complete ambulatory treat ment. One patient had an unfavorable outcome and died (case 1); another was lost in follow-up (case 4); one completely resolved his symptoms (case 2); and another one experienced symptom relapse six months after treatment (case 3).

Tabes dorsal y meningitis sifilítica en paciente con virus de inmunodeficiencia humana / Tabes dorsalis and syphilitic meningitis in a patient with human immunodeficiency virus

RESUMEN La neurosífilis es una infección causada por la bacteria Treponema pallidum, subespécie pallidum (T. pallidum). Se puede presentar en cualquier momento del curso de la sífilis, e ingresa al organismo por la primoinfección derivada de chancro primario sifilítico. La mayoría de los pacientes genera una repuesta inmunológica efectiva que evita el desarrollo de las complicaciones de la infección en el SNC, sin embargo, algunos no eliminan eficazmente tal invasión, por lo cual desarrollan neurosífilis asintomática o sintomática. La enfermedad, en cuanto a su evolución, se divide en etapas tempranas y tardías. Las primeras etapas incluyen meningitis asintomática, meningitis sintomática, goma sífilitica y sífilis meningovascular, mientras que las etapas tardías incluyen demencia paralítica y tabes dorsal. Dado que a la fecha no se cuenta con una prueba altamente específica y sensible, el diagnóstico se basa en la sospecha clínica, estudios serológicos y presencia de anormalidades del LCR. La importancia de su diagnóstico consiste en evitar complicaciones y secuelas potencialmente graves de la evolución de la enfermedad sin tratamiento. El manejo de la neurosífilis se circunscribe a recibir la terapia con penicilina. El seguimiento incluye el seguimiento de las pruebas serológicas y del LCR en pacientes específicos. Los pacientes con coinfección con VIH pueden tener un desarrollo más temprano de las características neurológicas que las personas sin la infección, así como alta probabilidad de una respuesta incompleta al tratamiento. Se presenta el caso de un hombre en la quinta década de la vida con infección por el virus de inmunodeficiencia humana (VIH) sin terapia antirretroviral, con cuadro subagudo de fiebre, compromiso de las funciones mentales superiores, pupila de Argyll Robertson, mioclonías y marcha atáxica. Las pruebas treponémicas desarrolladas en la hospitalización fueron positivas, se obtuvo un LCR anormal por la presencia de hiperproteinorraquia, así como anticuerpos antitreponemapallidum en 6,56 positivos en LCR. Se consideró el diagnóstico de tabes dorsal, por lo que se inició un tratamiento con penicilina cristalina intravenosa, 24 millones de unidades internacionales (UI)/día, durante 14 días, con evolución clínica favorable. Este artículo revisa la definición etiológica, la patogénesis, las manifestaciones clínicas, el diagnóstico y el tratamiento de la neurosífilis, con especial atención a la presencia de la neurosífilis con la coinfección con VIH y su relevancia para los clínicos en el ámbito de la neurología.

SUMMARY Neurosyphilis is an infection caused by the bacterium Treponema pallidum subspecies pallidum (T. pallidum). It can occur at any time during the course of syphilis, and enters the body through the cousin-infection derived from primary chancre syphilitic, most patients generate an effective immune response that prevents the development of complications of infection in the CNS, however, some patients do not effectively eliminate the invasion to the CNS, thus developing asymptomatic or symptomatic neurosyphilis. It has been divided into early and late stages. The early stages include asymptomatic meningitis, symptomatic meningitis, gum syphilis, and meningovascular syphilis, while the late stages include paralytic dementia and tabes dorsalis. Since, to date, there is no highly specific and sensitive test, the diagnosis is based on clinical suspicion, serological studies, and the presence of CSF abnormalities. The importance in its diagnosis derives in avoiding the complications and potentially serious sequelae of the evolution of the disease without treatment. The management of neurosyphilis is limited to receiving penicillin therapy. Follow-up includes follow-up of serological and CSF tests in specific patients. Patients with co-infection with HIV may have an earlier development of neurological characteristics than people without the infection, as well as a high probability of an incomplete response to treatment. We present the case of a man in his fifth decade of life with human immunodeficiency virus (HIV) infection without antiretroviral therapy, with subacute fever, compromise of higher mental functions, Argyll Robertson pupil, myoclonus, and ataxic march. The treponemal tests developed in the hospitalization were positive, an abnormal CSF was obtained due to the presence of hyperprotein spinal cord, as well as anti-treponema pallidum antibodies in 6.56 positive in CSF, the diagnosis of tabes dorsalis was considered, for which treatment with penicillin was started intravenous crystalline 24 million international units (IU) day, for 14 days, with favorable clinical evolution. This article reviews the definition of aetiology, pathogenesis, clinical manifestations, diagnosis and treatment of neurosyphilis, with special attention to the presence of neurosyphilis with co-infection with HIV and its relevance to clinicians in the field of neurology.

Republication: Two premature neonates of congenital syphilis with severe clinical manifestations

Congenital syphilis (CS) is a public health burden in both developing and developed countries. We report two cases of CS in premature neonates with severe clinical manifestations; Patient 1 (gestational age 31 weeks, birth weight 1423 g) had disseminated idiopathic coagulation (DIC) while Patient 2 (gestational age 34 weeks and 6 days, birth weight 2299 g) had refractory syphilitic meningitis. Their mothers were single and had neither received antenatal care nor undergone syphilis screening. Both neonates were delivered via an emergency cesarean section and had birth asphyxia and transient tachypnea of newborn. Physical examination revealed massive hepatosplenomegaly. Laboratory testing of maternal and neonatal blood showed increased rapid plasma reagin (RPR) titer and positive <i>Treponema pallidum</i> hemagglutination assay. Diagnosis of CS was further supported by a positive IgM fluorescent treponemal antibody absorption test and large amounts of <i>T. pallidum</i> spirochetes detected in the placenta. Each neonate was initially treated with ampicillin and cefotaxime for early bacterial sepsis/meningitis that coexisted with CS. Patient 1 received fresh frozen plasma and antithrombin III to treat DIC. Patient 2 experienced a relapse of CS during initial antibiotic treatment, necessitating parenteral penicillin G. Treatment was effective in both neonates, as shown by reductions in RPR. Monitoring of growth and neurological development through to age 4 showed no evidence of apparent delay or complications. Without adequate antenatal care and maternal screening tests for infection, CS is difficult for non-specialists to diagnose at birth, because the clinical manifestations are similar to those of neonatal sepsis and meningitis. Ampicillin was insufficient for treating CS and penicillin G was necessary.

Republication: Two Premature Neonates of Congenital Syphilis with Severe Clinical Manifestations

Congenital syphilis (CS) is a public health burden in both developing and developed countries. We report two cases of CS in premature neonates with severe clinical manifestations; Patient 1 (gestational age 31 weeks, birth weight 1423 g) had disseminated idiopathic coagulation (DIC) while Patient 2 (gestational age 34 weeks and 6 days, birth weight 2299 g) had refractory syphilitic meningitis. Their mothers were single and had neither received antenatal care nor undergone syphilis screening. Both neonates were delivered via an emergency cesarean section and had birth asphyxia and transient tachypnea of newborn. Physical examination revealed massive hepatosplenomegaly. Laboratory testing of maternal and neonatal blood showed increased rapid plasma reagin (RPR) titer and positive <i>Treponema pallidum</i> hemagglutination assay. Diagnosis of CS was further supported by a positive IgM fluorescent treponemal antibody absorption test and large amounts of <i>T. pallidum</i> spirochetes detected in the placenta. Each neonate was initially treated with ampicillin and cefotaxime for early bacterial sepsis/meningitis that coexisted with CS. Patient 1 received fresh frozen plasma and antithrombin III to treat DIC. Patient 2 experienced a relapse of CS during initial antibiotic treatment, necessitating parenteral penicillin G. Treatment was effective in both neonates, as shown by reductions in RPR. Monitoring of growth and neurological development through to age 4 showed no evidence of apparent delay or complications. Without adequate antenatal care and maternal screening tests for infection, CS is difficult for non-specialists to diagnose at birth, because the clinical manifestations are similar to those of neonatal sepsis and meningitis. Ampicillin was insufficient for treating CS and penicillin G was necessary.

Meningitis sifilítica aguda / Acute syphilitic meningitis

Se trata de un paciente de 28 años de sexo femenino, que presentó un cuadro clínico rápidamente progresivo de cambios en el comportamiento y deterioro del estado de conciencia. Ingresó con puntaje de Glasgow de 8/15 y requirió intubación traqueal. Tenía respuesta plantar extensora bilateral, pupila izquierda midriática de 5 mm, sin respuesta a la luz, ptosis palpebral del mismo lado y nistagmo con componente rápido hacia la izquierda. El contenido de proteínas del líquido cefalorraquídeo fue de 0,86 g/l con pleocitosis moderada de predominio linfocitario. Tres semanas antes del ingreso, presentó parto vaginal eutócico; no tuvo control prenatal durante el embarazo. En el recién nacido se demostró serología reactiva para sífilis y FTA-ABS positivo. La paciente también fue hallada reactiva para serología de sífilis en el líquido cefalorraquídeo, con diagnóstico de meningitis sifilítica aguda. La prueba ELISA para VIH fue negativa.

A 28 year-old woman presented with a rapidly progressive clinical picture of behavioral changes and altered level of consciousness. She was admitted with Glasgow Coma Score 8/15, 5 mm dilated non-reactive left pupil, right ptosis and nystagmus with left lateral gaze. Protein content at cerebrospinal fluid was 0,86 g/L with pleocytosis. Three weeks earlier, the patient underwent vaginal delivery of a pregnancy without prenatal care, and the newborn was found to be reactive to syphilis serology and positive on FTA-ABS test. The patient was found positive as well for syphilis on serologic tests for syphilis, corresponding yhe picture to neurosyphilis.HIV testing was negative in the patient.