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Neurosyphilis is an infection of the central nervous system caused by Treponema pallidum and may be symptomatic or asymptomatic in children with congenital syphilis. This study aims to describe the cortical activation pattern of a four-month-old infant with neurosyphilis using functional near-infrared spectroscopy (fNIRS). Born at term weighing 3,475 kg, she presented a Venereal Disease Research Laboratory (VDRL) test of 1:32 and changes in the cerebrospinal fluid test. She underwent treatment with crystalline penicillin for 10 days before discharge from the hospital. In the audiological evaluation, she presented normal tympanometry, otoacoustic emissions evoked by transient stimulus, brainstem auditory evoked potential with click stimulus at 80 and 30 dB nHL bilaterally. The Bayley III Scale was applied to assess language, cognition and motor development, showing delays in expressive language and broad motor skills. In the fNIRS acquisition, data were collected through 20 channels divided between the cerebral hemispheres. The /ba/ and /da/ stimuli were presented at 40 dB HL with the Psychopy software through a headphone. Data analysis used the MNE and MNE-NIRS toolboxes in the Spyder environment. The average by channel, ROI, and condition was exported for analysis. A similar theta coefficient was observed between the conditions and channels evaluated in both cerebral hemispheres, with a greater amplitude of oxyhemoglobin (HbO) being observed in the anterior position when compared to the posterior region of the temporal lobe. Therefore, this case report highlights the need to monitor the child development of babies with neurosyphilis.

Background: The attentional network test (ANT) is widely used to evaluate the performance of three attentional networks: alerting, orienting and executive attention networks. This study aimed to investigate the characteristics of attention functions in HIV-negative patients with early forms of neurosyphilis (NS) and their correlation with abnormalities in brain magnetic resonance imaging (MRI)., Methods: Thirty patients with early forms of NS, 31 patients with syphilis but without NS (Non-NS) and 35 healthy controls were recruited from an HIV-negative cohort between September 2020 and November 2022. The participants were evaluated with the ANT and the Mini-Mental State Examination (MMSE). Brain MRI was performed in NS and Non-NS patients., Results: No significant differences were observed in the MMSE scores among the three groups. However, patients with early forms of NS showed poorer performance in orienting and alerting functions than Non-NS group (F = 6.952, P = 0.011 and F = 8.794, P = 0.004, respectively); No significant difference was observed in executive function between the two groups (F = 0.001, P = 0.980). Multivariate analysis of variance using the Bonferroni post hoc test indicated that patients with NS exhibited less efficient orienting function (P = 0.023), and alerting function (P = 0.003) but not executive function (P = 0.99), compared to Non-NS patients. Additionally, a significant difference was found in orienting function between patients with NS and healthy controls (P < 0.001) compared to healthy controls. MRI scans revealed that the NS group had a higher prevalence of abnormalities in the frontal lobes and/or the temporoparietal junction compared to the Non-NS group (24/25 vs. 13/19, P = 0.032)., Conclusions: The orienting and alerting functions but not executive function were significantly less efficient in early forms of NS group than in the Non-NS group (P < 0.01). This indicates deficits in selective attention in patients with early forms of NS. Brain MRI scans revealed abnormalities in the frontal and/or parietal lobes, as well as the temporoparietal junction, suggesting potential neuropathological correlates of these attentional deficits., (© 2024. The Author(s).)

Evidence from epidemiological, clinical, and biological research resulted in the immune hypothesis: the hypothesis that immune system dysfunction is involved in the pathophysiology of schizophrenia spectrum disorders (SSD). The promising implication of this hypothesis is the potential to use existing immunomodulatory treatment for innovative interventions for SSD. Here, we provide a selective historical review of important discoveries that have shaped our understanding of immune dysfunction in SSD. We first explain the basic principles of immune dysfunction, after which we travel more than a century back in time. Starting our journey with neurosyphilis-associated psychosis in the nineteenth century, we continue by evaluating the role of infections and autoimmunity in SSD and findings from assessment of immune function using new techniques, such as cytokine levels, microglia density, neuroimaging, and gene expression. Drawing from these findings, we discuss anti-inflammatory interventions for SSD, and we conclude with a look into the future.

Abstract: This study aimed to compare between the clinical and laboratory characteristics of neurosyphilis and those of syphilis in human immunodeficiency virus (HIV) positive and explore the risk factors associated with the occurrence of neurosyphilis in the HIV infected.In-patients diagnosed with HIV and syphilis co-infection who underwent a lumbar puncture and completed cerebrospinal fluid (CSF) examination were divided into neurosyphilis group and syphilis group. The demographic characteristics, symptoms and signs, and laboratory tests of the 2 groups were comparatively analyzed. Logistic regression analysis was used to explore the risk factors associated with the occurrence of neurosyphilis.Among 81 patients, 33 patients were assigned to the neurosyphilis group, and 48 to the syphilis group. There were no significant differences in the age, gender, marital status, acquired immunodeficiency syndrome course, opportunistic infections, serum HIV viral load, and history of syphilis treatment. The difference in HIV transmission route between the 2 groups was statistically significant (P = .010), and the patients from the neurosyphilis group were mainly infected via heterosexual contact. The proportion of serum toludine red unheated serum test (TRUST) titer ≥1:16 in the neurosyphilis group were 78.8%, which was significantly higher compared to the syphilis group (48.9%). The level of CSF white blood cell count, CSF protein, and CSF HIV viral load in the neurosyphilis group were significantly higher than those of the syphilis group. The proportion of patients with neurological symptoms and signs in the neurosyphilis group was significantly higher compared to the syphilis group (P < .001). Multivariate logistic regression analysis showed that heterosexual contact transmission route, not received antiretroviral therapy, lower CD4 cell count and higher serum TRUST titer, untreated with syphilis, and neurological symptoms and signs were risk factors associated with the occurrence of neurosyphilis.The serum TRUST titer, CSF white blood cell count, CSF protein level, CSF HIV viral load, and the percentage of neurological symptoms and signs in the neurosyphilis group were higher. Heterosexual transmission route, not received antiretroviral therapy, and untreated with syphilis prompted the possibility of neurosyphilis occurrence., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Individuals with previous syphilis may experience cognitive impairment. The goal of this study was to determine if those at high risk for laboratory-defined neurosyphilis are cognitively impaired, and whether treatment based on cerebrospinal fluid (CSF) findings results in better outcomes., Methods: Participants had a new syphilis diagnosis, serum RPR titer ≥ 1:32 or peripheral blood CD4+ T cells ≤ 350/ul (in persons living with HIV) and did not endorse neurological symptoms. They underwent computerized cognitive assessment with the CogState. Thirty-two were randomized to either undergo lumbar puncture (LP) or to not undergo LP and 14 underwent LP; 64 were not randomized and 48 opted to undergo LP., Results: Demographics, cognitive complaints and cognitive impairment did not differ between randomized and nonrandomized participants. Two-thirds were cognitively impaired, and impairment was not more common in those with cognitive complaints. The adjusted odds of increased severity of impairment were 3.8 times greater in those with CSF pleocytosis compared to those without. Time to cognitive normalization, improvement or decline did not differ between those who did not undergo LP and those who underwent LP and whose treatment was based on CSF analysis. Taking into account pre-treatment cognitive impairment, the risk of cognitive decline was lower in those with CSF pleocytosis treated for neurosyphilis compared to those without CSF pleocytosis not treated for neurosyphilis, (HR 0.24 (95% CI 0.07-0.88], p = 0.03)., Conclusion: In individuals at high risk for laboratory-defined neurosyphilis, cognitive complaints are not a good indicator of cognitive impairment. Severity of cognitive impairment was greater in those with CSF pleocytosis. Identification and treatment of those with neurosyphilis may mitigate subsequent cognitive decline., Competing Interests: The authors have declared that no competing interests exist.

Competing Interests: Competing interests: None declared.

Objective To investigate the diagnostic value of electroencephalogram (EEG) complexity in patients with neurosyphilis by comparing the changes of electroencephalogram Lempel-Ziv complexity (EEG-LZC) before and after anti-syphilis treatment. Methods The EEG complexity of neurosyphilis patients diagnosed in our hospital from July in 2015 to June in 2017 was analyzed and compared with other diagnostic results such as serology examination and cerebrospinal fluid examination. Results A total of 27 patients were diagnosed, including 19 males and 8 females, of which 6 were mesenchymal(cerebrospinal membrane and meningeal vascular), 16 were parenchymal(paralytic dementia, spinal cord tuberculosis and optic neuropathy), and 5 were asymptomatic. After intensive anti-syphilis therapy, the LZC increased significantly in all patients while the trend and degree of change were consistent with other diagnostic results. Conclusion The LZC can be used as one of the diagnostic indexes meanwhile the trend and degree of its change can be used as the reference index of curative effect to neurosyphilis.

Neurosyphilis is a rare disease that until the 2000s was almost eradicated due to population awareness of HIV and efficient treatment. Since then, the prevalence of the entity is rising due to risk-associated behaviour such as unprotected intercourse. Neurosyphilis is still a difficult entity to diagnose especially when combined with acute HIV infection which can influence the usual clinical course of disease. In rare occasions, both acute HIV and early syphilis infection can present as mono or multiple cranial nerve palsies. This case demonstrates a rare manifestation of misdiagnosed early syphilis infection combined with acute HIV infection in a 34-year-old man with prior history of unprotected sex with men., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Neurosyphilis (NS) has different clinical manifestations and can appear during any stage of syphilis. We aimed to identify the factors affecting poor outcome in NS patients. Patients with positive cerebrospinal fluid Venereal Disease Research Laboratory test, and positive serological serum treponemal or nontreponemal tests were classified as definite NS. The data of 141 patients with definite NS were submitted from 22 referral centers. Asymptomatic NS, syphilitic meningitis, meningovascular syphilis, tabes dorsalis, general paresis, and taboparesis were detected in 22 (15.6%), 67 (47.5%), 13 (9.2%), 10 (7%), 13 (9.2%), and 16 patients (11.3%), respectively. The number of HIV-positive patients was 43 (30.4%). The most common symptoms were headache (n = 55, 39%), fatigue (n = 52, 36.8%), and altered consciousness (50, 35.4%). Tabetic symptoms were detected in 28 (19.8%), paretic symptoms in 32 (22.6%), and vascular symptoms in 39 patients (27.6%). Eye involvement was detected in 19 of 80 patients (23.7%) who underwent eye examination and ear involvement was detected in eight of 25 patients (32%) who underwent ear examination. Crystallized penicillin was used in 109 (77.3%), procaine penicillin in seven (4.9%), ceftriaxone in 31 (21.9%), and doxycycline in five patients (3.5%). According to multivariate regression analysis, while headache was a protective factor in NS patients, double vision was significantly associated to poor outcome. We concluded that double vision indicated unfavorable outcome among NS patients. A high clinical suspicion is needed for the diagnosis NS. As determined in our study, the presence of headache in syphilitic patients can help in early diagnosis of central nervous system disease.

Objective: To explore the clinical manifestations and imaging features of neurosyphilis and to discuss the obstacles in the diagnosis and treatment of neurosyphilis., Methods: We present this case study involving three cases of definite neurosyphilis, focusing on their clinical data., Results: Case 1 is a patient with numb and weak left lower limb. Case 2 showed slow reaction and dementia behaviors including worse memory and the decrease of calculation and orientation ability in this patient. Case 3 is a peripheral incomplete left oculomotor nerve palsy patient. Magnetic resonance imaging findings of three patients are different. And single photon emission computed tomography showed the regional cerebral blood flow was all hypoperfused. There were some difficulties in diagnosing and treating the patients in these three cases., Conclusion: The clinical manifestations and imaging findings of neurosyphilis are diverse. Clinicians should pay attention to neurosyphilis. After clear diagnosis, patients would receive norm treatment in time.

We report a 33-year-old Japanese man who suffered from repetitive generalized tonic-clonic seizures which were medically intractable. Neurosyphilis was serologically diagnosed in blood and cerebrospinal fluid, and penicillin G (PcG) was consequently effective. The EEG during PcG pre-treatment showed frequent right occipital spikes and right frontocentral slow waves, which disappeared after treatment. During pre-treatment, positron emission tomography with 18-fluorodeoxyglucose and Tc-99m ethyl cysteinate dimer single-photon emission computed tomography revealed occipital hypermetabolism and hyperperfusion ("hot" area) and fronto-temporo-parietal hypometabolism and hypoperfusion ("cool" area) over the right hemisphere. The spike sources of magnetoencephalography during pre-treatment were localized to "hot" areas, and the slow activities were distributed to the fronto-temporo-parietal region, corresponding to "cool" areas. The inflammatory seizure focus and reversible dysfunctional zone associated with neurosyphilis were clearly delineated using these techniques.

The authors present a case of neurosyphilis associated with predominant psychiatric symptoms. The elderly man was admitted because of confused behavior, maniform state, lack of critical judgement and grandiose delusions. On admission, right central facial nerve paresis, hand tremor and parkinsonism were also found. Acute brain imaging and routine laboratory tests failed to identify a firm etiology of the confusional state. The psychiatric treatment resulted in complete recovery from delirium. Afterwards, maniform psychosis dominated the clinical picture for which antipsychotics were administered. Later, rapid cognitive deterioration and progression of motor symptoms were observed. MRI revealed cortical and hippocampal atrophy and white matter hyperintensities. Lumbar puncture found pleocytosis and elevated cerebrospinal fluid protein levels. Neurosyphilis had been confirmed by serologic tests. The cognitive symptoms improved and the psychiatric symptoms remitted under penicillin treatment. Four years after diagnosis, there is a gradual progression in the cognitive decline. Two additional hospitalizations were necessary due to the relapses of psychiatric symptoms. Orv Hetil. 2018; 159(6): 234-238.

Background: Involvement of the central nervous system in patients with syphilis (neurosyphilis) may result in several neuropsychiatric symptoms. Rarely, patients with neurosyphillis may develop movement disorders with different phenomenology. Subtle orofacial dyskinesias have been reported in patients with neurosyphilis, known as the candy sign., Case Report: We describe a patient with neurosyphilis who presented with severe orofacial involuntary movements., Discussion: Our patient had orofacial movements at presentation and severity of the movements was much higher than the candy sign that has been reported in patients with neurosyphilis. This report contributes towards the ever-expanding clinical spectrum of neurosyphilis., Competing Interests: Funding: None. Conflicts of interest: The authors report no conflict of interest. Ethics Statement: All patients that appear on video have provided written informed consent; authorization for the videotaping and for publication of the videotape was provided.

Background: Neurosyphilis is the tertiary stage of Treponema pallidum infection that involves the central nervous system, which occurs within days or weeks after an initial syphilis infection, especially in immunocompromised patients. The diagnosis of neurosyphilis is quite challenging as it is uncommon and often presents with obscure symptoms since any organ system may be involved., Case Presentation: We describe a case of a 40-year-old African man who is human immunodeficiency virus positive with early neurosyphilis who presented with a stiff neck, headache, confusion, restlessness, and a left-sided chest pain; he did not respond to an empiric treatment of ceftriaxone and fluconazole for meningitis, and tramadol for headache. Ten days after admission, he developed generalized tonic-clonic convulsions; on examination he had ipsilateral facial nerve palsy and an oral ulcer, and responded well to benzathine penicillin treatment., Conclusions: Laboratory diagnosis of neurosyphilis is challenging because to date there is no single laboratory test which is considered sensitive enough for diagnosis of the disease, especially in resource-limited settings. Clinical judgment is still an important part of diagnosis; and neurosyphilis should be considered a diagnostic differential in patients with Human Immunodeficiency Virus presenting with central nervous system involvement and in other high-risk patients.

Background: Syphilis remains a significant public health problem. We conducted a prospective study to define more precisely the clinical and biological characteristics of patients with neurosyphilis (NS), and we assessed the diagnostic value of nested polymerase chain reaction (PCR) testing for Treponema pallidum in cerebrospinal fluid (CSF) samples., Methods: From 2001 to 2013, we included 40 patients (90% men; 45% infected with human immunodeficiency virus) with NS, defined as syphilis with neurological and/or ophthalmological symptoms and CSF abnormalities., Results: Thirty patients (75%) had early, 5 (12.5%) had late, and 5 had meningovascular NS. Twenty-four patients (80%) with early NS had ophthalmological symptoms, 14 (47%) had neurological symptoms, and 8 (26%) had both. All patients with meningovascular NS had only neurological symptoms. All patients with late NS had neurological symptoms, and 2 (40%) also had ocular symptoms. Ophthalmological symptoms were present in 65% of all patients with NS, and neurological symptoms in 60%. Seventeen patients (42.5%) had CSF white blood cell counts >20/μL (mean, 57/μL), and 27 (67.5%) had high CSF protein levels (>0.5 g/L; mean value, 1 g/L). CSF PCR results were positive in 42%, and CSF VDRL results in 30%. The nested PCR assay had an overall sensitivity of 42.5%, a specificity of 97%, a positive predictive value of 77%, and a negative predictive value of 86%., Conclusions: Early NS is the most frequent presentation, with an overrepresentation of polymorphous ophthalmological symptoms. PCR is highly specific and of potential value when used with other biological parameters., (© The Author 2016. Published by Oxford University Press for the Infectious Diseases Society of America. All rights reserved. For permissions, e-mail journals.permissions@oup.com.)

Background: Neurosyphilis is defined as any involvement of the central nervous system by the bacterium Treponema pallidum. Movement disorders as manifestations of syphilis have been reported quite rarely., Case Presentation: We report a case of a 42-year-old Russian man living in Estonia with rapidly progressive dementia and movement disorders manifesting as myoclonus, cerebellar ataxia and parkinsonism. The mini mental state examination score was 12/30. After excluding different neurodegenerative causes, further diagnostic testing was consistent with neurosyphilis. Treatment with penicillin was started and 6 months later his mini mental state examination score was 25/30 and he had no myoclonus, parkinsonism or cerebellar dysfunction., Conclusion: Since syphilis is easily diagnosed and treatable, it should be considered and tested in patients with cognitive impairment and movement disorders.

Background: Neurosyphilis is caused by the invasion of Treponema pallidum into the central nervous system. General paresis (GP) is a type of neurosyphilis. The main manifestation of general paresis is dementia; however, this is different from the other types of dementia, which can be cured by adequate doses of penicillin in the early stage. Neurosyphilis is the "great imitator" because it can mimic many types of medical disorders. In addition, the manifestations of neurosyphilis are not typical. Psychiatric disorders as a cause of general paresis have become more common due to the use of antibiotics. Patients with a psychiatric manifestation are often misdiagnosed. The purpose of this study was to explore the differences in the clinical and neuropsychological characteristics of general paresis between patients misdiagnosed as having a primary psychiatric disease and patients diagnosed correctly upon seeing a doctor. The results may assist clinicians in the early identification of neurosyphilis with a mental disorder., Method: The demographic and clinical manifestations, laboratory tests, and neuroimaging and neuropsychological characteristics were analysed in 55 general paresis patients with psychiatric disorders, including 29 patients misdiagnosed as primary psychiatric disease and 26 patients diagnosed as having general paresis after being seen once by a doctor., Result: All of the patients had positive assay results for cerebral spinal fluid (CSF) Treponema pallidum hemagglutination (TPHA). Only 43.3 % of misdiagnosed patients and 30.8 % of general paresis patients had positive results for the CSF rapid plasma reagin (RPR) test; 96.4 % patients had abnormal neuroimaging. Mood disturbances were the most common psychiatric disorder in the general paresis patients, especially agitation, between the two groups (patients with general paresis who were misdiagnosed as having primary psychiatric disease and patients who had never been misdiagnosed) (p = 0.011)., Conclusion: Our findings reinforce the importance of performing serologic testing for syphilis. This should be a part of the evaluation of patients with psychiatric disorders, especially patients with cognitive impairment. When the syphilis serology is positive, the patient should be examined thoroughly for neurosyphilis by lumbar puncture. Brain imaging could also aid the physician in discriminating these patients from those with a functional mental disorder.

This case describes an emergency department (ED) presentation of ocular syphilis in a human immunodeficiency virus (HIV) infected patient. This is an unusual presentation of syphilis and one that emergency physicians should be aware of. The prevalence of syphilis has reached epidemic proportions since 2001 with occurrences primarily among men who have sex with men (MSM). This is a case of a 24-year-old male who presented to our ED with bilateral painless vision loss. The patient's history and ED workup were notable for MSM, positive rapid plasmin reagin (RPR) and HIV tests and fundus exam consistent with ocular syphilis, specifically uveitis. Ocular manifestations of syphilis can present at any stage of syphilis. The 2010 Centers for Disease Control and Prevention guidelines now recommend that ocular syphilis be treated as neurosyphilis regardless of the lumbar puncture results. There is a paucity of emergency medicine literature on ocular syphilis. For emergency physicians it is important to be aware of iritis, uveitis, or chorioretinitis as ocular manifestations of neurosyphilis especially in this high-risk population and to obtain RPR and HIV tests in the ED to facilitate early diagnosis, and treatment and to prevent irreversible vision loss.

Periodic lateralized epileptiform discharges (PLEDs) are an electroencephalographic pattern recorded in the setting of a variety of brain abnormalities. It is best recognized for its association with acute viral encephalitis, stroke, tumor, or latestatus epilepticus. However, there are other conditions that have been recognized as the underlying pathology for PLEDs such as alcohol withdrawal, Creutzfeldt-Jacob disease, anoxic brain injury, and hemiplegic migraine. However, there are only rare case reports of PLEDs in patients with neurosyphilis. Here, we report 2 patients presenting with encephalopathy and seizures with PLEDs, ipsilateral or contralateral to their main brain magnetic resonance imaging abnormalities. Further workup revealed neurosyphilis in both patients, one in association with human immunodeficiency virus (HIV) infection. Given the increasing incidence of neurosyphilis with or without HIV infection, these cases suggest neurosyphilis as a consideration in the differential for patients presenting with PLEDs., (© EEG and Clinical Neuroscience Society (ECNS) 2014.)

Unlabelled: Penicillin encephalopathy is a rare, potentially reversible phenomenon of drug-induced neurotoxicity., Case: A 65-year-old female with a history of HIV was admitted with a three-day history of worsening headache, confusion, and lethargy. On examination she was awake but confused. Cerebrospinal fluid (CSF) and serum venereal disease research laboratory (VDRL) test returned positive and the patient was started on intravenous penicillin G with probenecid. On the second day of therapy, she developed myoclonic jerking, consistent with penicillin neurotoxicity. Repeat labs also showed new onset renal failure. Penicillin and probenecid therapy were stopped with a resolution of symptoms. Subsequently, therapy without probenecid was reinstituted uneventfully., Discussion: Herein, we describe a female who developed penicillin neurotoxicity after initiation of intravenous penicillin therapy with probenecid for neurosyphilis. It is important that penicillin-induced toxicity be considered if characteristic myoclonic movements accompany encephalopathy. The presence of coexistent renal compromise should heighten the vigilance of clinicians.

Purpose of Review: This article reviews the etiology, clinical manifestations, diagnosis, and treatment of neurosyphilis, with a focus on issues of particular relevance to neurologists., Recent Findings: The number of cases of infectious syphilis in the United States has steadily increased since 2000. The highest rates are among men who have sex with men, and approximately half of these individuals are infected with human immunodeficiency virus (HIV). Neurosyphilis is a serious complication of syphilis that can develop at any time in the course of syphilis. Two neuroimaging patterns should alert the neurologist to a diagnosis of neurosyphilis: cerebral gummas, which are dural-based lesions that can mimic meningiomas, and medial temporal lobe abnormalities that can mimic herpes encephalitis. Penicillin G is the recommended treatment for neurosyphilis, but ceftriaxone may be an acceptable alternative., Summary: The diagnosis of neurosyphilis can be challenging. A sound understanding of the clinical manifestations and the strengths and limitations of diagnostic tests are essential tools for the neurologist.

Background: No gold standard currently exists for the diagnosis of general paresis (GP), thus often resulting in unnecessarily delayed therapeutic decision., Methods: A retrospective chart review was performed for 85 inpatients with GP in Zhongshan Hospital, Medical College of Xiamen University, and the characteristics of their clinical profiles, serum and cerebrospinal fluid (CSF) examinations, neuroimaging examination, and electroencephalogram (EEG) data were analyzed., Results: Among the 85 GP patients, the clinical symptoms that were frequently observed upon admission included a variety of psychiatric-behavioral symptoms and varying degrees of cognitive impairment. All of the patients had positive serum Treponema pallidum particle agglutination (TPPA) assays, 96.47% of the patients had positive CSF TPPA assays, and 41.18% of the patients had both CSF pleocytosis and elevated CSF protein levels. Focal atrophy in one cerebral region or in multiple regions was evident in neuroimages. The EEG data primarily showed slightly abnormal EEG activity., Conclusion: These results demonstrate the complexity of the clinical characteristics of GP and highlight the importance of early diagnosis., (Copyright © 2014 Elsevier B.V. All rights reserved.)

A 60-year-old man presented with progressive dementia and generalized convulsions. An initial MRI revealed a widespread high-intensity area with a mass effect in the right frontal and temporal lobes on T2-weighted images. Findings on digital subtraction angiography were normal. Serum and CSF tests showed high titers of antibodies to Treponema pallidum, which helped to distinguish neurosyphilis from glioma. He was initially treated with penicillin injection; however, it caused liver dysfunction and penicillin was switched to erythromycin. Even after antibiotic therapy for 2 months, his dementia did not improve. He underwent brain MRI four times during the treatment course, and they showed steady progression of brain atrophy in the right hemisphere. Taking these findings together, we diagnosed Lissauer form of general paresis. To the best of our knowledge, this is the first case of Lissauer form of paretic neurosyphilis, in which the progression of brain atrophy was clearly demonstrated on MRI.

The present paper reports a clinical case of local syphilitic meningoencephalitis known as Argyll-Robertson syndrome and manifested in the form of acute sensorineural loss of hearing. The patient was a 46 year old resident of the city of Irkutsk. He experienced the sharp impairment of hearing involving both ears with the accompanying feeling of dizziness, disturbed orientation of the locomotorbehaviour, the lurching gate, subfebrility, the loss of the ability to speak and write. The diagnosis was based on the presence of anisocoria, the absence of consensual pupillary reaction to the light, weak atropine action on the pupils, well expressed positive results of the VRDL (blood) and RW (liquor) tests, an enhanced signal from the left temporal region in the T-2 regime during MRI, positive hearing dynamics within 12 hours after the onset of anti-inflammatory, antibacterial and infusion therapy. The specific feature of the clinical case being considered is differential diagnostics was performed with the purpose of distinguishing it from otogenic brain abscess associated with chronic left-hand mesotympanitis.

We report one Chinese patient with neurosyphilis exhibiting periodic lateralized epileptiform discharges (PLEDs) in the electroencephalogram (EEG). The patient (male, 59 years old) manifested with progressive cognitive decline and abnormal behavior. After several days, he gradually lost contact with others, and fell into a coma. EEG revealed periodic abnormal waves, predominantly located in the right anterior frontal region. The serum and cerebrospinal fluid Venereal Disease Research Laboratory test and Treponema pallidum hemagglutination assay were positive. Magnetic resonance imaging (MRI) of brain showed focal atrophy in the right frontal and temporal region. Diffusion-weighted MRI showed "lace sign" in cortex, which could be seen as an early and special feature in Creutzfeldt-Jakob disease (CJD). To differentiate "PLEDs of neurosyphilis on EEG" from "periodic synchronous discharges (PSD) of CJD on EEG," we treated this patient with diazepam 20 mg intravenously. After 10 minutes, periodic abnormal waves on EEG disappeared, with improved mental status, which confirmed the diagnosis of PLEDs of neurosyphilis. Then, after the treatment with penicillin, the patient improved and returned to work.

Objective: This report describes neurosyphilis presenting as vocal fold paresis and velopharyngeal incompetence. This represents the first reported case of neurosyphilis presenting as a vagal neuropathy since 1963., Study Design: Case report., Methods: Review of medical records and literature search., Results: The patient presented with voice changes and nasopharyngeal reflux of liquids. Examination showed a left vocal fold paresis and moderate velopharyngeal incompetence. Laryngeal electromyography revealed evidence of denervation, and neurologic evaluation showed bilateral Adie's pupils and a positive CSF VDRL. A remote history of syphilis treated with one intramuscular injection was obtained. The patient was treated with 6 weeks of penicillin and regained normal vocal function with minimal residual intermittent hypernasality., Conclusions: Given the recent increase in the incidence of syphilis in the United States, this case serves as a reminder of the importance of including syphilis in the differential diagnosis of cranial neuropathies and the increasing likelihood of syphilis presenting to otolaryngology and speech pathology practices. Syphilis, a disease whose incidence had declined progressively for 60 years in the United States, is now resurgent and must be considered in the evaluation of patients presenting with unexplained cranial neuropathies., (Copyright © 2014 The Voice Foundation. All rights reserved.)

Background: Neurosyphilis is known as "the great imitator" due to its wide range of clinical symptoms and abnormalities upon magnetic resonance imaging (MRI). Typical findings of both neurosyphilis and viral encephalitis include unilateral or bilateral MR hyperintensities in mesiotemporal lobes upon T2-weighted imaging or fluid attenuation inversion recovery (FLAIR) imaging. Accordingly, patients with neurosyphilis are frequently misdiagnosed with viral encephalitis, which prevents them from receiving appropriate treatment and often results in greater neurologic damage., Methods: Clinical characteristics and MRI changes of 6 neurosyphilis patients admitted to our hospital between March 2012 and November 2012 were retrospectively reviewed., Results: All 6 cases were tested positive for assays measuring Treponema pallidum hemagglutination (TPHA), rapid plasma reagin (RPR), and antibodies against syphilis in the serum and cerebrospinal fluid (CSF). Likewise, all patients were negative for antibodies against viral pathogens. T2-weighted or FLAIR MRI of the brains in all cases revealed either unilateral or bilateral hyperintensities in the mesiotemporal lobes, including the hippocampi. Electroencephalography showed relevant, localized slow or spiked waves. Patient prognoses were good in the 4 cases that received early anti-syphilis treatment, but the 2 cases that received delayed treatment due to misdiagnoses did not see substantial symptomatic improvements., Conclusions: Neurosyphilis should be considered when there is mesiotemporal involvement upon MRI. Early treatment for syphilis is critical for positive outcomes., (© 2013. Published by Elsevier B.V. All rights reserved.)

A 55-year-old man with no relevant history was analyzed for weight loss, night sweats, and left upper quadrant pain. An abdominal ultrasound and CT scan were performed, revealing a lobulated rim-enhancing mass in the left adrenal gland. Further analysis by an F-FDG PET/CT scan demonstrated high uptake in the periphery of the adrenal lesion with central photopenia. Because a primary malignancy was suspected, an adrenalectomy was performed. Histopathology, however, revealed a necrotizing granulomatous infection. Serum was tested positive for syphilis and Treponema pallidum infection. Results of additional HIV tests were negative. The adrenal tumor proved to be an expression of gummatous syphilis.

Objective: General paresis of the insane is a late and severe form of neurosyphilis characterized by nonspecific neuropsychiatric symptoms. There are a limited number of case reports of mood disorders presenting in neurosyphilis, with depressive illness being the most common., Methods: We performed a literature review of case reports of secondary bipolar disorder induced by syphilitic infection., Results: Herein reported is a case of a 53-year-old woman who initially presented with symptoms of mania and depression, mimicking bipolar disorder, but was subsequently diagnosed with general paresis of the insane., Conclusion: The present case report emphasizes that if a substantial delay occurs in syphilis diagnosis and management, the patient may have a very poor prognosis., (© 2012 John Wiley and Sons A/S.)

Neurosyphilis is an infectious disease that has reappeared over the past two decades. It is caused by Treponema pallidum subspecies pallidum that can affect the central nervous system (CNS) during any stage of the disease. Besides early CNS involvement predominantly presenting with symptoms of meningitis, a parenchymal affection of the brain leading to severe neuropsychiatric symptoms particularly emerges at later stages, but is rarely seen nowadays due to early antibiotic treatment. Together with the clinical findings, a characteristic combination of serological and cerebrospinal fluid (CSF) abnormalities leads to the diagnosis of neurosyphilis and is required to assess its activity. However, particularly at later stages of disease and after antibiotic treatment, serological and CSF abnormalities may become ambiguous and, therefore, difficult to interpret. This can be accompanied by persisting or fluctuating neuropsychological deficits. To this day, no well-controlled clinical data exists concerning the treatment of late-stage neurosyphilis, neither on type, optimal dosage, duration, and long-term efficacy of antibiotic therapy. Therefore, treatment and follow-up of late-stage neurosyphilis are challenging tasks. Here, we present three cases of neurosyphilis with severe neuropsychiatric symptoms in non-immunocompromised patients and a review of the recent literature.

We studied 1387 patients with different forms of syphilis for neurological deficiency. Thirty patients had vascular neurosyphilis. Two cases are described in detail. A 40-year-old man had recurring episodes of acute disturbance of the cerebral blood flow and progressive cognitive disorders. A 32-year-old patient presented with progressive dementia, hallucinatory symptoms and minor multifocal neurological changes. The diagnosis of neurosyphilis was verified by CSF-test results. The diagnosis of cerebral vasculitis was proved by the data of magnetic resonance imaging (MRI), magnetic resonance (MR) angiography, ophthalmological methods, and duplex scanning of brachiocephalic arteries, transcranial duplex scanning. Diagnostic criteria of syphilitic cerebral vasculitis were defined and presented.

Background/aims: We investigated the clinical and cognitive features of patients with mild general paresis of the insane (GPI) in comparison to Alzheimer's disease (AD) patients, mild frontotemporal dementia (FTD) patients and normal elderly individuals., Methods: Twelve patients with mild GPI, 24 patients with mild AD, 11 patients with mild FTD and 36 healthy subjects participated in the current study. A comprehensive neuropsychological battery was used to assess memory, language, attention, executive function, visuospatial ability, etc. In addition, cranial MRI were also obtained from the 3 patient groups., Results: Mild GPI showed a similar pattern of cognitive impairments in memory, language and executive function to mild AD, but a mixed pattern of dissimilarities to mild FTD. Mild GPI patients had more complaints and positive signs in the nervous system than mild AD patients, and cranial MRI results showed that mild GPI patients usually had atrophy of the medial temporal lobe., Conclusion: There is an overall decline in cognitive functions of mild GPI patients, which is comparable to that in AD patients, suggesting that there is a wide range of structural changes in the brains of mild GPI patients as well. However, further studies are needed to verify whether these structural changes are similar to those in AD., (Copyright © 2011 S. Karger AG, Basel.)

The psychiatric presentation of advanced neurosyphilis is well documented, yet there is no set protocol to treat these occurrences. This report indexes a 61-year-old African-American male with no known psychiatric history but with a pertinent medical history of neurosyphilis (non-human immunodeficiency virus). He developed an acute episode of disinhibition involving violence and poor executive judgment. The authors' propose that this patient's symptoms of disinhibition may have resulted from syphilitic invasion of frontal-subcortical structures, resulting in either top-down or bottom-up dysregulation and deterioration of social behavior. His episode resolved with administration of monotherapy carbamazepine., (Copyright 2010 Elsevier Inc. All rights reserved.)

Only two spirochetal infections are known to cause nervous system infection and damage: neurosyphilis and neuroborreliosis (nervous system Lyme disease). Diagnosis of both generally relies on indirect tools, primarily assessment of the host immune response to the organism. Reliance on these indirect measures poses some challenges, particularly as they are imperfect measures of treatment response. Despite this, both infections are known to be readily curable with straightforward antimicrobial regimens. The challenge is that, untreated, both infections can cause progressive nervous system damage. Although this can be microbiologically cured, the threat of permanent resultant neurologic damage, often severe in neurosyphilis and usually less so in neuroborreliosis, leads to considerable concern and emphasizes the need for prevention or early and accurate diagnosis and treatment.

Background: The chemokine CXCL13 is known to dictate homing and motility of B cells in lymphoid tissue and has been implicated in the formation of ectopic lymphoid tissue in chronic inflammation. Whether it influences B cell trafficking during acute infection, is largely unclear. In previous studies, we showed that (I) CXCL13 levels are markedly increased in the B cell-rich cerebrospinal fluid (CSF) of patients with acute Lyme neuroborreliosis (LNB), and (II) CXCL13 is released by monocytes upon recognition of borrelial outer surface proteins by Toll-like receptor 2. Here, we assessed the role of CXCL13--in comparison to other chemokines--in the recruitment of B cells to the CSF of patients with acute LNB., Methods: Measurement of chemokines was done by ELISA. B cells were isolated from whole blood using magnetic cell separation (MACS). For migration experiments, a modified Boyden chamber assay was used and the migrated B cells were further analysed by FACS. The migration was inhibited either by preincubation of the CSF samples with neutralizing antibodies, heating to 60 degrees C, removal of proteins >3 kDa, or by pre-treatment of the B cells with pertussis toxin. The principal statistical tests used were one-way analysis of variance and Bonferroni test (chemokine measurements) as well as paired Student's t-test (migration experiments)., Results: Measurements of chemokine levels revealed an increase in three of the four known major B cell chemoattractants CXCL13, CCL19 and CXCL12 in LNB CSF. The CXCL13 CSF:serum ratio, as a measure of the chemotactic gradient, was substantially higher than that of CCL19 and CXCL12. Moreover, the chemotactic activity of LNB CSF was reduced up to 56% after preincubation with a neutralizing CXCL13 antibody, while combined preincubation with antibodies against CXCL13, CCL19, and CXCL12 did not lead to further reduction. Since treatment with pertussis toxin, heating to 60 degrees C, and removal of proteins >3 kDa abrogated the chemotactic activity, further not yet identified chemokines seem to be involved in B cell recruitment to LNB CSF., Conclusion: Combined, our study suggests a key role of CXCL13 in B cell migration to sites of infection as shown here for the CSF of LNB patients.

Objective: To describe the clinical findings in 4 patients with ocular syphilis and to provide a review of the recent rise in syphilis cases in the United States, along with a brief description of current diagnosis and treatment guidelines., Design: Retrospective, observational case series., Participants: Four consecutive patients who presented to our facility during a 6-month interval in 2005 with ocular syphilis., Methods: Review of clinical, laboratory, photographic, and angiographic records of patients with neurosyphilis who underwent intravenous penicillin G treatment., Main Outcome Measures: Resolution of signs and symptoms of ocular syphilis, including changes in visual acuity., Results: Four patients (3 male: mean age, 46 [range, 39-60 years]; 1 female: age 46 years) demonstrated cerebrospinal fluid findings consistent with neurosyphilis, as evidenced by increased leukocyte counts (>5 white blood cells/mm3), and positive Venereal Disease Research Laboratory or serum fluorescent treponemal antibody absorbed tests. All 4 patients presented with a variety of clinical findings that led to a delay in diagnosis and treatment. Two patients presented with discrete placoid lesions consistent with acute syphilitic posterior placoid chorioretinitis, a manifestation typically observed among the immunocompromised., Conclusions: Despite a decade of steady decline, syphilis has reemerged in the United States with outbreaks throughout the country in the past few years. Ocular findings, including posterior placoid chorioretinitis, are important diagnostic features in the early treatment of tertiary syphilis and neurosyphilis. Ophthalmologists have the opportunity to play a key role in the early diagnosis and management of this potentially fatal disease.

The widespread use of antibiotics in recent years has caused a significant reduction in the incidence of neurosyphilis and changes in its clinical features. We present a case that initially presented as persistent headache and untreatable psychosis. Neurosyphilis was diagnosed during the clinical evaluation. Blood serum analyses for syphilis were positive for rapid plasma reagin titres, the Venereal Disease Research Laboratories test and fluorescent treponemal antibody absorption. A lumbar puncture was performed and cerebrospinal fluid analysis resulted in the diagnosis of neurosyphilis. The patient completed a 2-week course of treatment with aqueous crystalline penicillin G and his symptoms subsequently improved. We suggest that neurosyphilis should always be included in the differential diagnosis of untreatable psychosis.