Did you rule out neurosyphilis?

Nitrini, Ricardo; Paiva, Anderson Rodrigues Brandão de; Takada, Leonel Tadao; Brucki, Sonia Maria Dozzi

doi:10.1590/S1980-57642010DN40400014

Abstract

Neurosyphilis, formerly a frequent cause of dementia, is now a rare condition in developed countries. However, syphilis remains common in many developing countries, where adequate diagnosis and treatment of early syphilis may be lacking, increasing the chances of neurosyphilis and prevalence of syphilitic dementia. Objectives: To present cases of syphilitic dementia seen in a cognitive and behavioral neurology unit in Brazil, emphasizing their first symptoms and the challenges they posed in diagnosis. Methods: At our unit of the Hospital das Clínicas of the University of São Paulo, all patients are submitted to blood treponemal tests. When the test is positive, a lumbar puncture is performed. We retrospectivelly reviewed all cases of neurosyphilis seen in our unit from January 1991 to November 2009. Results: Nine cases of neurosyphilis (0.77% of the 1160 cases in our files) were identified over the period. Patients with neurosyphilis were all men, had a mean age of 47.8 (±13.0) years (median of 43 years), and presented with various neuropsychiatric syndromes and elusive diagnoses. The median time from onset of symptoms to diagnosis was 24 months and only one patient made a full recovery after treatment. Conclusions: Neurosyphilis is not frequent but remains present, causing several types of neuropsychiatric syndromes. As it is very simple to rule out neurosyphilis by performing a blood treponemal test, this test should be performed in all patients with neuropsychiatric symptoms, particularly in regions of the world where syphilis is still a commonly occurring disease.

Key words:
neurosyphilis; syphilis; dementia; neuropsychiatry; paretic neurosyphilis

Resumo

Neurossífilis, anteriormente uma causa freqüente de demência, é atualmente rara nos países desenvolvidos. A sífilis é ainda uma doença comum em muitos países em desenvolvimento, onde o diagnóstico e tratamento da sífilis precoce podem não ser adequados, o que aumenta a possibilidade de ocorrência de neurossífilis e de demência. Objetivos: apresentar casos de demência sifilítica atendidos em uma unidade de neurologia cognitiva e do comportamento no Brasil, enfatizando os primeiros sintomas e os desafios que impuseram ao diagnóstico. Métodos: Em nossa unidade do Hospital das Clínicas da Universidade de São Paulo, todos os pacientes são submetidos a teste treponêmico no sangue. Quando o teste é positivo, é realizada punção lombar. Avaliamos retrospectivamente todos os casos de neurossífilis atendidos em nossa unidade de janeiro de 1991 a novembro de 2009. Resultados: Nove casos de neurossífilis (0,77% dos 1.160 casos de nossos arquivos) foram identificados neste período. Os pacientes com neurossífilis eram todos homens, com idade média de 47,8 (±13,0) anos (mediana de 43 anos), e apresentaram-se com vários tipos de síndromes neuropsiquiátricas, de difícil diagnóstico. O tempo médio entre o início dos sintomas e o diagnóstico foi de 24 meses e apenas um paciente teve recuperação completa após o tratamento. Conclusões: Neurossífilis não é frequente, mas ainda está presente causando vários tipos de síndromes neuropsiquiátricas. Como é muito simples excluir o diagnóstico de neurossífilis mediante teste treponêmico no sangue, este teste deve ser realizado em todo paciente com sintomas neuropsiquiátricos, particularmente nas regiões do mundo onde a sífilis é ainda uma doença comum.

Palavras-chave:
neurossífilis; sífilis; demência; neuropsiquiatria; paralisia geral progressiva

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References

Nitrini R. The cure of one of the most frequent types of dementia: a historical parallel. Alzheimer Dis Assoc Disord 2005;19:156-158.
Merritt HH, Adams RD, Solomon HC. Neurosyphilis. York, New York, Oxford University Press New; 1946.
Hahn RD, Webster B, Weickhardt G, et al. The results of treatment in 1,086 general paralytics the majority of whom were followed for more than five years. J Chronic Dis 1958;7: 209-227.
Miklossy J. Biology and neuropathology of dementia in syphilis and lyme disease. Handb Clin Neurol 2008;89:825-844.
Hook III EW. Central Nervous System Syphilis. In: WM Scheld, RJ Whitley, DT Durack (Eds.), Infections of the central nervous system. Raven Press, Ltd., New York, 1991;27: 639-655.
Knopman DS, DeKosky ST, Cummings JL, et al. Practice parameter: diagnosis of dementia (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2001; 56:1143-1153.
Lee JW, Wilck M, Venna N. Dementia due to neurosyphilis with persistently negative CSF VDRL. Neurology 2005;65: 1838.
Lee CH, Lin WC, Lu CH, Liu JW. Initially unrecognized dementia in a young man with neurosyphilis. Neurologist 2009; 15:95-97.
Güler E, Leyhe T. A late form of neurosyphilis manifesting with psychotic symptoms in old age and good response to ceftriaxone therapy. Int Psychogeriatr 2010;15:1-4.
World Health Organization. Global prevalence and incidence of selected curable sexually transmitted infections: overview and estimates. Geneva: World Health Organization; 2001.
Guimarães MD, Campos LN, Melo AP, et al. Prevalence of HIV, syphilis, hepatitis B and C among adults with mental illness: a multicenter study in Brazil. Rev Bras Psiquiatr 2009;31: 43-47.
Nitrini R, Caramelli P, Bottino CM, Damasceno BP, Brucki SM, Anghinah R. [Diagnosis of Alzheimer's disease in Brazil: diagnostic criteria and auxiliary tests: recommendations of the Scientific Department of Cognitive Neurology and Aging of the Brazilian Academy of Neurology ]. Arq Neuropsiquiatr 2005;63:713-719.
Storm-Mathisen A. Syphilis. In: PJ Vinken, GW Bruyn (Eds.), Infections of the Nervous System, part I. Handbook of Clinical Neurology , (1978) Vol. 33, pp. 337-394. Amsterdam: Elsevier.
Rocha F, Pacheco-Silva AC. A demência paralytica em São Paulo, Arq Brasil Neuriat Psiquiat 1924:6:1-22.
Kahn RH, Heffelfinger JD, Berman SM. Syphilis outbreaks among men who have sex with men: a public health trend of concern. Sex Transm Dis 2002;29:285-287.
Marra CM. Update on neurosyphilis. Curr Infect Dis Rep 2009;11:127-134.
Rossor MN, Fox NC, Mummery CJ, Schott JM, Warren JD. The diagnosis of young-onset dementia. Lancet Neurol 2010; 9:793-806.
Synnott IH. The great imitator. Med J Aust 1988;148:105.
Nitrini R. Clinical and therapeutic aspects of dementia in syphilis and lyme disease. Handb Clin Neurol 2008;89:819-823.
Lair L, Naidech AM. Modern neuropsychiatric presentation of neurosyphilis. Neurology 2004;63:1331-1334.
Nitrini R, Spina-França A. [High-dose intravenous penicillin therapy in neurosyphilis: study of 62 cases. II. Evaluation of cerebrospinal fluid]. Arq Neuropsiquiatr 1987;45:231-241.

Publication Dates

Publication in this collection
Oct-Dec 2010

History

Received
10 July 2010
Accepted
11 Sept 2010

This is an open-access article distributed under the terms of the Creative Commons Attribution License

[1] Nitrini R. The cure of one of the most frequent types of dementia: a historical parallel. Alzheimer Dis Assoc Disord 2005;19:156-158.

[2] Merritt HH, Adams RD, Solomon HC. Neurosyphilis. York, New York, Oxford University Press New; 1946.

[3] Hahn RD, Webster B, Weickhardt G, et al. The results of treatment in 1,086 general paralytics the majority of whom were followed for more than five years. J Chronic Dis 1958;7: 209-227.

[4] Miklossy J. Biology and neuropathology of dementia in syphilis and lyme disease. Handb Clin Neurol 2008;89:825-844.

[5] Hook III EW. Central Nervous System Syphilis. In: WM Scheld, RJ Whitley, DT Durack (Eds.), Infections of the central nervous system. Raven Press, Ltd., New York, 1991;27: 639-655.

[6] Knopman DS, DeKosky ST, Cummings JL, et al. Practice parameter: diagnosis of dementia (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2001; 56:1143-1153.

[7] Lee JW, Wilck M, Venna N. Dementia due to neurosyphilis with persistently negative CSF VDRL. Neurology 2005;65: 1838.

[8] Lee CH, Lin WC, Lu CH, Liu JW. Initially unrecognized dementia in a young man with neurosyphilis. Neurologist 2009; 15:95-97.

[9] Güler E, Leyhe T. A late form of neurosyphilis manifesting with psychotic symptoms in old age and good response to ceftriaxone therapy. Int Psychogeriatr 2010;15:1-4.

[10] World Health Organization. Global prevalence and incidence of selected curable sexually transmitted infections: overview and estimates. Geneva: World Health Organization; 2001.

[11] Guimarães MD, Campos LN, Melo AP, et al. Prevalence of HIV, syphilis, hepatitis B and C among adults with mental illness: a multicenter study in Brazil. Rev Bras Psiquiatr 2009;31: 43-47.

[12] Nitrini R, Caramelli P, Bottino CM, Damasceno BP, Brucki SM, Anghinah R. [Diagnosis of Alzheimer's disease in Brazil: diagnostic criteria and auxiliary tests: recommendations of the Scientific Department of Cognitive Neurology and Aging of the Brazilian Academy of Neurology ]. Arq Neuropsiquiatr 2005;63:713-719.

[13] Storm-Mathisen A. Syphilis. In: PJ Vinken, GW Bruyn (Eds.), Infections of the Nervous System, part I. Handbook of Clinical Neurology , (1978) Vol. 33, pp. 337-394. Amsterdam: Elsevier.

[14] Rocha F, Pacheco-Silva AC. A demência paralytica em São Paulo, Arq Brasil Neuriat Psiquiat 1924:6:1-22.

[15] Kahn RH, Heffelfinger JD, Berman SM. Syphilis outbreaks among men who have sex with men: a public health trend of concern. Sex Transm Dis 2002;29:285-287.

[16] Marra CM. Update on neurosyphilis. Curr Infect Dis Rep 2009;11:127-134.

[17] Rossor MN, Fox NC, Mummery CJ, Schott JM, Warren JD. The diagnosis of young-onset dementia. Lancet Neurol 2010; 9:793-806.

[18] Synnott IH. The great imitator. Med J Aust 1988;148:105.

[19] Nitrini R. Clinical and therapeutic aspects of dementia in syphilis and lyme disease. Handb Clin Neurol 2008;89:819-823.

[20] Lair L, Naidech AM. Modern neuropsychiatric presentation of neurosyphilis. Neurology 2004;63:1331-1334.

[21] Nitrini R, Spina-França A. [High-dose intravenous penicillin therapy in neurosyphilis: study of 62 cases. II. Evaluation of cerebrospinal fluid]. Arq Neuropsiquiatr 1987;45:231-241.

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