Demencia rápidamente progresiva por neurosífilis (parálisis general). Un caso tratable de demencia / Rapidly progressive dementia due to neurosyphilis (general paralysis). A treatable case of dementia

Resumen Las demencias rápidamente progresivas son cuadros de deterioro en más de un dominio cognitivo con compromiso funcional que progresan en menos de 1 a 2 años; y la neurosífilis es una de las etiologías. La sífilis es una infección bacteriana crónica que causa una serie de cuadros clínicos muy variables durante los primeros 2 a 3 años, seguido de una etapa latente prolongada que puede evolucionar a una etapa de infección terciaria. Luego de un período de años, o incluso décadas, un tercio de las personas con sífilis latente no tratada tendrá manifestaciones clínicas de sífilis terciaria como neurosífilis. Presentamos el caso de un varón de 41 años de edad que consultó por cuadro de postración, precedido por alteraciones cognitivas conductuales progresivas de 18 meses de evolución. Se constató cuadro demencial asociado a parkinsonismo farmacológico secundario a risperidona, por lo que se suspendió dicho tratamiento. Las neuroimágenes mostraron una atrofia cerebral grave; VDRL reactiva en suero y líquido cefalorraquídeo (LCR); además de un leve aumento de proteínas en LCR. Se realizó el diagnóstico de neurosífilis tardía iniciando tratamiento con penicilina G cristalina 1 400 000 UI cada 4 h por 14 días con excelente respuesta. Nuestro caso nos permite reflexionar sobre la importancia de solicitar estudios diagnósticos de sífilis en pacientes jóvenes que presentan cuadro de demencia rápidamente progresiva, ya que esta enfermedad tiene un tratamiento que puede revertir parcial o totalmente los síntomas.

Abstract Rapidly progressive dementias are conditions of impairment in more than one cognitive domain with functional compromise that progress in less than 1 to 2 years; and neurosyphilis is one of the etiologies. Syphilis is a chronic bacterial infection that causes a series of highly variable clinical conditions during the first 2 to 3 years, followed by a prolonged latent stage that can progress to a tertiary infection stage. After a period of years, or even decades, a third of people with untreated latent syphilis will have clinical manifestations of tertiary syphilis such as neurosyphilis. We present the case of a 41-year-old man who consulted for prostration symptoms, preceded by progressive behavioral cognitive alterations of 18 months of evolution. A dementia picture was found associated with pharmacological parkinsonism secondary to risperidone, so this treatment was suspended. Neuroimaging showed severe cerebral atrophy; serum and cerebrospinal fluid (CSF) reactive VDRL, in addition to a slight increase in CSF proteins. The diagnosis of late neurosyphilis was made and treated with crystalline penicillin G 1 400 000 IU every 4 h for 14 days with an excellent response. Our case allows us to reflect on the importance of requesting diagnostic studies of syphilis in young patients who present a rapidly evolving dementia, since this disease has a treatment that can partially or totally reverse the symptoms.

Manifestaciones psiquiátricas de la neurosífilis: reporte de un caso de parálisis / Psychiatric manifestations of neurosyphilis: A case report of progressive general paralysis

A pesar de que la neurosífilis es poco frecuente en la actualidad, esta entidad no ha desaparecido. Las manifestaciones psiquiátricas de la parálisis general progresiva se pueden presentar a través de una gran variedad de síndromes tales como psicosis, manía y demencia. Se presenta el caso de un varón de 56 años de edad con inicio insidioso de delirios de daño y de grandeza, alucinaciones auditivas y alteraciones conductuales tales como reacciones impulsivas y agresividad. Los análisis serológicos y de líquido cefalorraquídeo (LCR) revelaron la presencia de Treponema pallidum. El paciente recibió tratamiento antibiótico, así como psicofarmacológico, con mejoría notable de los síntomas psicóticos, maniacos y conductuales. Este caso enfatiza la importancia de descartar neurosífilis en presencia de manifestaciones psiquiátricas pleomórficas y evolución atípica, así como deterioro de funciones mentales superiores.

Although neurosyphilis is infrequently seen nowadays, it cannot be said that it has disappeared. The psychiatric manifestations of syphilis-related progressive general paralysis can adopt a wide variety of presentations, including psychosis, mania and dementia. The case of a 56-year-old male patient is presented; it has an insidious onset of delusions of damage and grandiosity, auditory hallucinations and behavioral changes such as increased impulsivity and aggressiveness. Serological and cerebrospinal fluid laboratory analyses revealed the presence of Treponema pallidum. The patient received antibiotic and psychopharmacological treatment, showing significant improvement of all his symptoms. This case emphasizes the importance of ruling out neurosyphilis in the presence of pleomorphic or atypical psychiatric manifestations of atypical clinical course as well as a decline of superior mental functions.

Effect of Donepezil on Dysgnosia in Patients with General Paralysis of the Insane / 中山大学学报(医学科学版)

[Objective]To investigate the effect of Donepezil on dysgnosia in patients with mild-moderate general paralysis of the insane(GPI).[Methods]Forty-seven patients with GPI were assigned into study group(n=24)or control group(n=23). All these patients were treated with a large dose of penicillin for 2 weeks and then with Benzathine for 3 weeks. And the Donepezil admin-istered to patients in study group.The mini-mental state examination(MMSE)and the clinical dementia rating(CDR)were scored as the measurement of efficiency.[Result]The scores of the MMSE and CDR were not statistically different between the two groups at baseline and 4 weeks after treatment,and both were increased 12 weeks after treatment(P<0.05),especially in study group as com-pared with those in control group(P < 0.05).[Conclusion]Donepezil as an adjunctive therapy may be effective for dysgnosia in patients with mild-moderate GPI. Donepezil is also safe and well tolerated.

Neurossífilis: uma breve revisão / Neurosyphilis: a brief review

A neurossífilis (NS) designa todas as formas de comprometimento do sistema nervoso central (SNC) causadas pela bactéria Treponema pallidum. Em pacientes imunocompetentes, ocorre principalmente no estádio terciário da sífilis (embora suceda em outros estádios), acometendo apenas 10 porcento dos pacientes com infecção primária não tratada. Apesar de um decréscimo significativo na incidência de neurossífilis nas últimas três décadas, a invasão do sistema nervoso humano ainda ocorre e a apresentação clínica não segue a evolução tradicional da era pré-antibiótica. Observa-se, atualmente, que a NS pode apresentar quadros clínicos muito similares aos de outras enfermidades do sistema nervoso, podendo ser confundida, mesmo após anos de acompanhamento, com doenças neurológicas ou psiquiátricas. Neste trabalho, fizemos uma revisão histórica, conceitual, epidemiológica, clínica e diagnóstica com o objetivo de ampliar o conhecimento sobre esta doença e melhorar a sua compreensão...

Neurosyphilis (NS) designates all the adverse effects on the central nervous system (CNS) caused by Treponema pallidum. It occurs at the third stage of syphilis in about 10 percent of patients with untreated primary infection. Despite a significant decrease in the incidence of neurosyphilis during the last three decades, the invasion of the human nervous system still takes place and the clinical presentation does not follow the traditional evolution of the pre-antibiotic era. At present, it is observed that NS may present clinical features very similar to those of other illnesses of the nervous system, and can be mistaken, even after years of follow-up, for neurological or psychiatric illnesses. In this work, we conducted an historical, epidemiological, conceptual, clinical and diagnostic review, with the purpose of expanding the knowledge and improving the understanding of this disease...

Manifestaciones neuropsiquiátricas de la neurolúes: presentación de un caso de parálisis general neurosifilítica / Neuropsychiatric manifestations of neurosyphilis: a case of general paresis

Introducción: La parálisis general se debe a la lesión tardía y degenerativa de la sustancia gris cerebral por el Treponema pallidum, caracterizada por demencia, signos oculares, neurológicos y humorales. Es frecuente su comienzo con manifestaciones delictuales. Caso clínico: Describimos el caso de un paciente de 61 años, masculino, sin antecedentes. Ingresa para comprobar imputabilidad (asesinó a su primo); atento, vigil, desorientado, disártrico, musita, pensamiento detallista y concreto. No sabe causa de acusación ni argumenta en su defensa; sin productividad psicótica. Signos vitales estables, exámenes de laboratorio normales. Se ingresa con diagnóstico de deterioro psicoorgánico. Se maneja con clorpromazina 12,5 mg en la mañana, tarde y 50 mg noche. Evoluciona con temblor, rigidez, enlentecimiento, alucinaciones visuales, auditivas y soliloquios. Por sospecha de impregnación se suspende la clorpromazina, se solicitan exámenes y se evaluó por neurólogo. Inatento, apráxico, marcha con aumento de base de sustentación, adiadococinesia, dismetría, Romberg (+) y pupilas de Argyll Robertson. VDRL de 1/512 sérico y 1/8 en el LCR. Se diagnostica parálisis general neurosifilítica. Se maneja con penicilina G acuosa 6 millones UI c/ 6 h IV por 14 días y olanzapina 20 mg c/noche con respuesta parcial. Conclusión: La parálisis general es poco frecuente pero debe ser sospechada en pacientes de edad media con demencia y signos característicos neuro-oculares.

Introduction: General Paresis is caused by a late and degenerative injury by Treponema pallidum to cerebral grey matter, characterized by dementia, as well as ocular, neurological, and mood changes. It generally first manifests in criminal activity. Clinical case: We describe the case of a 61-year-old male patient, with no past medical history. Admitted to prove imputability (he assasinated his cousin). Attentive, alert, disoriented. Dysarthria and murmured speech. Highly detailed and concrete thinking. He does not know the cause of the accusation, and he does not argue in his defense neither. No psychotic symptoms. Stable vital signs and normal laboratory exams. Admitted with a diagnosis of psycho-organic deterioration. Controlled with clorpromazine 12.5 mg BID and 50 mg/night. Evolution with tremor, rigidity, slowing. Visual and auditory hallucinations and soliloquy. On suspicion of neuroleptic impregnation we discontinue clorpromazina. Laboratory tests and neurologist evaluationare requested. Inattentive, apraxia, adiadochokinesia, dysmetria, Romberg (+) and Argyll Robertson pupils. Serum VDRL 1/512 and CSF1/8. Diagnose general paresis (neurospyhilisis). Treatment with aqueous penicillin G 6 million Ul every 6 hours IV for 14 days and olanzapine 20 mg every night with partial response. Conclusion: General paresis is infrequent but should be suspected in middle-aged patients with dementia and characteristic neuro-ocular signs.

Bayle e a descrição da aracnoidite crônica na paralisia geral: sobre as origens da psiquiatria biológica na França / Bayle y la descripción de la aracnoiditis crónica en la parálisis general: sobre los orígenes de la psiquiatría biológica en Francia / Bayle et la description de l'aracnites chronique dans la paralysie générale: sur les origines de la psychiatrie biologique en France / Bayle and the description of chronic arachnoiditis in general paralysis: on the origins of biological psychiatry in France

A tese de medicina defendida em 1822, em Paris, por Antoine-Laurent Bayle, intitulada “Pesquisas sobre as doenças mentais”, constitui um momento de virada na história das concepções biológicas em psicopatologia. Apoiado sobre um método anátomo-clínico rigoroso. Bayle apresenta seis observações clínicas de pacientes com uma história crônica e progressiva de comportamento exaltado, idéias de grandeza, de poder e de ambição que se transformam no final de algum tempo em delírios maníacos com agitação psicomotora. Simultaneamente, vai-se instalando um quadro de paralisia progressiva de vários grupos musculares, chegando até a incapacidade física extrema. Na fase terminal, os pacientes apresentam uma condição tipicamente demencial e de profundo comprometimento corporal, que os conduz à morte. Ao exame cadavérico, Bayle constatou a presença sistemática de uma inflamação crônica das meninges cerebrais (aracnoidite crônica) à qual ele atribui o fundamento biológico dos sintomas observados. Dessa forma. Bayle realiza uma descrição clínica rigorosa de uma entidade psicopatológica típica, de evolução crônica e progressiva e demonstra sua relação com uma lesão cerebral específica e objetivamente demonstrável. Tal descoberta, inicialmente recebida com muitas reservas, constituiria posteriormente uma espécie de paradigma para a pesquisa e para o projeto teórico e terapêutico para a psiquiatria biológica que começava a se organizar na França ao longo do século XIX.

Antoine Laurent Bayle’s medical thesis, entitled “Studies on mental diseases” and defended in Paris in 1822, represented an important advance in the history of biological conceptions in psychopathology. Based on a rigorous anatomic and clinical method, Bayle presented clinical observations on six patients with chronic and progressive histories of exalted behavior and ideas of grandeur: power and ambition, a condition that eventually evolved to maniac delusion with psychomotor agitation. Simultaneously, a situation of progressive paralysis of several muscle groups set in, leading to extreme physical incapacity. In the final stage the patients showed a typically demential condition with serious physical impairment which led to death. Post-mortem examinations showed the presence, is all cases, of chronic inflammation of the cerebral meninges (chronic arachnoidits), which Bayle held to be the biological basis of the symptoms noted. Bayle thus provided a rigorous clinical description of typical psychopathological entity with chronic and progressive evolution, and showed its relationship to specific and objectively demonstrable cerebral damage. This discovery, first received with considerable reservation, later served as a type of paradigm for research and for the theoretical and therapeutic project for biological psychiatry that began in France during the 19th century.

La tesis de medicina defendida en 1822 en Paris, por Antoine-Laurent Bayle, titulada “Investigación sobre las enfermedades mentales”, constituye un momento de viraje en la historia de las concepciones biológicas en psicopatología. Apoyado sobre un método anatómico-clínico riguroso. Bayle presenta seis observaciones clínicas de pacientes con una historia crónica y progresiva de comportamiento exaltado, ideas de grandeza, de poder y de ambición que se transformaron al final de algún tempo en delirios maníacos con agitación psicomotora. Simultáneamente, se va instalando un cuadro de parálisis progresiva de varios grupos musculares, llegando hasta la incapacidad física extrema. En la fase terminal, los pacientes presentan una condición típicamente demencial e de profundo comprometimiento corporal, que los conduce a muerte. Al examen cadavérico, Bayle constato la presencia sistemática de una inflamación crónica de las meninges cerebrales (aracnoidits crónica) a la cual él atribuyó el fundamento biológico de los síntomas observados. De esta forma, Bayle realiza una descripción clínica rigurosa de una entidad psicopatológica típica, de evolución crónica y progresiva y demuestra su relación con una lesión cerebral específica y objetivamente demostrable. Tal descubierta, inicialmente recibida con muchas reservas, constituiría posteriormente una especie de paradigma para la investigación y para el proyecto teórico e terapéutico para la psiquiatría biológica que comenzaba a organizarse en Francia a lo largo del siglo XIX.

La thèse de médecine soutenue en 1822 à Paris par Antoine-Laurent Bayle, intitulée "Recherches sur les maladies mentales" constitue un tournant dans l'histoire des conceptions biologiques en psychopathologie. Fondé sur une méthode anatomoclinique rigoureuse, Bayle y présente six observations cliniques de patients ayant une histoire chronique et progressive de comportement exalté, idées de grandeur, de puissance et d'ambition qui se transforment au bout d'un certain temps en délires maniaques accompagnés d'agitation psychomotrice. Simultanément, un tableau de paralysie progressive de plusieurs groupes musculaires se met en place, allant jusqu'à l'incapacité physique extrême. Dans la phase terminale, les patients présentent une condition typiquement démentielle et de profond affaiblissement corporel, finissant par en mourir. À l'examen cadavérique, Bayle a constaté la présence systématique d'une inflammation chronique des méninges cérébrales (arachnites chronique) à laquelle il attribue le fondement biologique des symptômes observés. Bayle nous fournit ainsi une description clinique rigoureuse d'une entité psychopathologique typique à l'évolution chronique et progressive et démontre son rapport à une lésion cérébrale spécifique et objectivement démonstrable. Telle découverte, reçue avec beaucoup de réserves au départ, constituera ensuite une sorte de paradigme pour la recherche et pour le projet théorique et thérapeutique de la psychiatrie biologique qui commençait à s'organiser en France au cours du XIX siècle.

General paralysis of insane: two case reports / 临床神经病学杂志

Objective To explore the clinical manifestation,laboratory tests,imaging and treatment of general paralysis of insane(GPI).Methods The clinical data of 2 patients with GPI analyzed retrospectively.Results 2 sufferers were both male,and mainly presented with memory decline and mental disorder.One case presented with visceral crisis.Both rapid plasma reagin test and treponema gelatin agglutination test in serum and CSF were positive.Brain MRI scanning showed ventriculomegaly.After antisyphilitic treatments,2 patients obviously improved.Conclusion GPI are often seen in middle-aged males and its misdiagnosis rate is high.The diagnosis of GPI should be considered in young patients with dementia without clear reason.Early diagnosis and active treatments are important for the prognosis of this disease.