Hakim-Adams syndrome secondary to syphilis

Ricardo Krause Martinez de Souza, Alexandre Zatera, Fernanda Leal Suzano, Flávia de Oliveira Braga, Samanta Fabrício Blattes da Rocha, Ricardo Ramina, Pedro André Kowacs

Ambulatório de Desordens da Memória e do Comportamento, Instituto de Neurologia de Curitiba, Curitiba, Brazil.
Instituto de Psiquiatria do Paraná, Curitiba, Brazil.
Instituto de Psiquiatria do Paraná, Curitiba, Brazil;.
Servi?o de Neurologia e Neurocirurgia, Curitiba, Brazil.

DOI: 10.4236/crcm.2013.26101                

Abstract

Normal pressure hydrocephalus (NPH) secondary to syphilis is an atypical form of neurosyphilis with an unusual clinical presentation and uncommon neuroimaging findings. A case of Syndrome de Hakim-Adams occurring in a 79-year-old male was admitted for investigation of a dementia syndrome. The patient presented with clinical features and neuroimaging compatible with NPH. Syphilis serology was reactive and compatible with active disease. The patient was treated with crystalline penicillin (4 million units every 4 hours for 21 days) and was assessed again after three months, when his cognition and gait had improved and the urinary incontinence had resolved. According to this information, we describe a case of syphilis mimicking normal pressure hydrocephalus, making clear the importance of the investigation of sexually transmitted diseases in the differential diagnosis of dementia, even in patients without established risk supposedly.

Keywords

Neurosyphilis; Hakim-Adams Syndrome; Syphilis; Normal Pressure Hydrocephalus

Conflicts of Interest

The authors declare no conflicts of interest.

References

[1]	Marra, C.M. (2009) Update on neurosyphilis. Current Infectious Disease Reports, 11, 127-134. doi:10.1007/s11908-009-0019-1
[2]	De Souza, R.K., Vosgerau, R.P., Sato, H.K. and Kowacs, P.A. (2013) A male patient with delirium, seizures and (limbic) encephalitis with bilateral mesial temporal lobe involvement. Arquivos de Neuro-Psiquiatria, 71, 270. doi:10.1590/0004-282X20130018
[3]	Pedersen, K.K., Sorensen, P.S., Ankerhus, J. and Danielsen, U.T. (1979) Syphilitic normal pressure hydrocephalus. Acta Neurochirurgica (Wien), 48, 35-39. doi:10.1007/BF01406018
[4]	Fadil, H., Gonzalez-Toledo, E., Kelley, B.J. and Kelley, R.E. (2006) Neuroimaging findings in neurosyphilis. Journal of Neuroimaging: Official Journal of the American Society of Neuroimaging, 16, 286-289.
[5]	Relkin, N., Marmarou, A., Klinge, P., Bergsneider, M. and Black, P.M. (2005) Diagnosing idiopathic normalpressure hydrocephalus. Neurosurgery, 57, S4-S16.
[6]	Ishii, K., Kanda, T., Harada, A., Miyamoto, N., Kawaguchi, T., Shimada, K., et al. (2008) Clinical impact of the callosal angle in the diagnosis of idiopathic normal pressure hydrocephalus. European Radiology, 18, 2678-2683. doi:10.1007/s00330-008-1044-4
[7]	Hashimoto, M., Ishikawa, M., Mori, E. and Kuwana, N. (2010) Diagnosis of idiopathic normal pressure hydrocephalus is supported by MRI-based scheme: A prospective cohort study. Cerebrospinal Fluid Research, 7, 18.
[8]	Sahuquillo, J., Rubio, E., Codina, A., Molins, A., Guitart, J.M., Poca, M.A., et al. (1991) Reappraisal of the intracranial pressure and cerebrospinal fluid dynamics in patients with the so-called “normal pressure hydrocephalus” syndrome. Acta Neurochirurgica (Wien), 112, 50-61. doi:10.1007/BF01402454