Vol.2, No.6, 377-379 (2013) Case Reports in Clinical Medicine
Hakim-Adams syndrome secondary to syphilis
Ricardo Krause Martinez de Souza1, Alexandre Zatera2, Fernanda Leal Suzano2*,
Flávia de Oliveira Braga2, Samanta Fabrício Blattes da Rocha2, Ricardo Ramina3,
Pedro André Kowacs2
1Ambulatório de Desordens da Memória e do Comportamento, Instituto de Neurologia de Curitiba, Curitiba, Brazil
2Instituto de Psiquiatria do Paraná, Curitiba, Brazil; *Corresponding Author: fernandasuzano@uol.com.br
3Serviço de Neurologia e Neurocirurgia, Curitiba, Brazil
Received 8 May 2013; revised 12 June 2013; accepted 8 July 2013
Copyright © 2013 Ricardo Krause Martinez de Souza et al. This is an open access article distributed under the Creative Commons
Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is
properly cited.
Normal pressure hydrocephalus (NPH) second-
ary to syphilis is an atypical form of neuro-
syphilis with an unusual clinical presentation
and uncommon neuroimaging findings. A case
of Syndrome de Hakim- Adams occurr ing 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 p enicillin (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 inconti-
nence had resolved. According to this infor-
mation, we describe a case of syphilis mimick-
ing normal pressure hydrocephalus, making clear
the importance of the investigation of sexually
transmitted diseases in the differential diagno-
sis of dementia, even in patients without estab-
lished risk supposedly.
Keywords: Neurosyphilis; Hakim-Adam s Syndrome;
Syphilis; Normal Pressure Hydrocephalus
Syphilis is a sexually transmitted disease caused by
the spirochete Treponema pallidum (T. pallidum), which
causes neurosyphilis when it invades the nervous system.
The initial neurological manifestations of neurosyphilis
are asymptomatic meningitis, acute syphilitic meningitis
and meningovascular syphilis. In the late stages of the
disease, there may be involvement of the spinal cord and
cerebral cortex, causing tabesdorsalis or general paresis,
which is also known as paralytic dementia [1]. Other
presentations of neurosyphilis have been described, such
as limbic encephalitis [2] and normal pressure hydro-
cephalus [3], which are referred to as atypical forms of
The aim of this paper is to describe a case of atypical
neurosyphilis mimicking NPH in which treatment with
crystalline penicillin resulted in a considerable improve-
ment in symptoms.
A 79-year-old male was admitted to the Curitiba In-
stitute of Neurology for investigation of a dementia syn-
drome. The patient presented with symptoms marked by
gait apraxia, dementia and urinary incontinence that had
been worsening progressively over 1.5 years. On admis-
sion he was taking donepezil 5 mg. In the previous six
months his symptoms had worsened, leading him to seek
a second opinion. Laboratory tests were carried out and
the patient had a brain MRI scan, lumbar puncture and
neuropsychological assessment.
HIV serology was nonreactive and vitamin B12, folic
acid and TSH levels were normal. Syphilis serology was
reactive and compatible with active disease (second line
of the Table 1).
Cerebrospinal fluid (CSF) analysis revealed an in-
crease in protein concentration (72 mg/dL) any cellular
alterations. Opening pressure was 12 cm H2O, and VDRL
(venereal disease research laboratory) and FTA-ABS
(fluorescent treponemal antibody-absorption) tests were
The MRI revealed ventricular dilation (Evans’ index
of 0.31) out of proportion to the degree of atrophy (Fig-
ure 1). Neuropsychological assessment showed cogni-
tive alterations associated with frontal-subcortical dys-
function, confirming the diagnosis of dementia.
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R. K. M. de Souza et al. / Case Reports in Clinical Medicine 2 (2013) 377-379
Table 1. Serological profile of syphilis (total antibodies).
Active syphilis + +
Treatedorlatentsyphilis + +
Recent primary syphilis or
false positive +
CMIA/EIA: chemiluminescent microparticle immunoassay/syphilis enzyme
immunoassay; RPR: rapid plasma reagin; TPHA/TP-PA: Treponema palli-
dum hemagglutination assay/Tre ponema pallidum particle agglutination assay.
Figure 1. Brain MRI scan. (A) Axial FLAIR (Fluid At-
tenuated Inversion Recovery) sequence showing dilation
of the ventricular system out of proportion to the degree
of cerebral atrophy, and rounding of the frontal and oc-
cipital horns (arrows); (B) Coronal T2-weighted sequence
showing a callosal angle of 90˚, dilation of the temporal
horns (arrows) and tight high-convexity and medial sur-
face subarachnoid spaces (dotted arrows).
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. A
NPH scale [8] was used to measure the clinical improve-
When syphilis is suspected, both treponemal (FTA-
ABS, TP-PA and EIA) and non-treponemal (VDRL and
RPR) tests should be carried out [1]. When the VDRL
test in CSF is reactive, a diagnosis of neurosyphilis can
be made. However, a nonreactive VDRL test does not
exclude a diagnosis of neurosyphilis. FTA-ABS in CSF
is sensitive but is not specific. However, if it is not reac-
tive, a diagnosis of neurosyphilis can usually be excluded
[1]. Our patient’s serum was reactive in CMIA and RPR
tests, indicating active syphilis (Table 1); his CSF find-
ings included elevated protein levels and a nonreactive
FTA-ABS test. There have been reports of rare cases in
which serologic tests for syphilis in CSF have been non-
reactive [1].
NPH secondary to syphilis is an atypical form of neu-
rosyphilis with an unusual clinical presentation [3] and
uncommon neuroimaging findings [4]. Pedersen et al.,
1979, described a case of NPH secondary to syphilis that
improved significantly with antibiotics [3]. A diagnosis
of NPH is made when its triad of signs and symptoms
(gait apraxia, dementia and urinary incontinence) is pre-
sent together with an opening CSF pressure of less than
18 cm H2O and ventricular dilation that cannot be attrib-
uted exclusively to cerebral atrophy. The neuroimaging
characteristics observed in our case, which included a
callosal angle of approximately 90˚ [6], tight high-con-
vexity and medial surface subarachnoid spaces [7] and
rounding of the frontal and temporal horns, support a
diagnosis of NPH. The patient’s NPH score [8] was the
parameter used to classify the response to treatment. The
NPH scale assesses three domains (gait, cognition and
sphincter control), with scores of between 1 and 5 for
each item. The total score can vary from a minimum of 3
points (patient completely incapacitated) to a maximum
of 15 (total independence). The result for our patient was
9/15 before treatment and 13/15 after treatment. There
was an improvement of 1 point in gait and cognition and
2 points in sphincter control. The overall improvement of
4 points was considered significant.
The patient presented with the classic Hakim-Adams
triad, with neuroimaging compatible with NPH. The
finding of normal opening pressure excluded the possi-
bility of hypertensive hydrocephalus. In view of the risks
associated with ventriculoperitoneal shunt surgery in an
elderly patient and the presence of active syphilis in the
blood, we decided to administer empirical treatment for
Copyright © 2013 SciRes. OPEN ACCESS
R. K. M. de Souza et al. / Case Reports in Clinical Medicine 2 (2013) 377-379
Copyright © 2013 SciRes.
neurosyphilis using penicillin. The improvement in the
NPH score [8] observed after administration of the anti-
biotic corroborated the diagnosis of atypical neurosyphi-
Our case illustrates an atypical form of neurosyphilis
(Hakim-Adams syndrome), which is considered in the
literature to be a rare yet an important manifestation of
this disease as it involves potentially reversible dementia.
The absence of positive serology for syphilis in the CSF
should not exclude a diagnosis of neurosyphilis in cases
in which the clinical findings are suggestive of this in-
fection. Physicians should bear in mind that tests can
yield false-negative results and should trust their clinical
impressions when these are relevant to the patient’s
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