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Neurosyphilis: a Case Report Case Report NEUROLOGY North Clin Istanbul 2015;2(1):66-68 doi: 10.14744/nci.2015.96268 Neurosyphilis: a case report Tugce Toptan, Betul Ozdilek, Gulay Kenangil, Mustafa Ulker, Fusun Mayda Domac Department of Neurology, Erenkoy Training and Research Hospital for Neurologic and Psychiatric Disorders, Istanbul, Turkey ABSTRACT Syphilis is a multisystem chronic infection caused by treponema pallidum. It can cause psychiatric disorders includ- ing depression, mania, psychosis, personality changes, delirium and dementia. With the introduction of penicillin into practice, the number of cases with syphilis decreased and its incidence increased with AIDS and HIV sero- positivity. In this article, we present a case of neurosyphilis that manifested itself with neuropsychiatric symptoms. Key words: Dementia; general paresis; neurosyphilis; psychiatric manifestations. yphilis is a multisystem chronic infection caused This inflammatory disease progresses slowly as Sby treponema pallidum support [1, 2]. Al- neurosyphilis or gummatous syphilis [5, 6]. though there is a decrease in the number of cases of Neurosyphilis is classified as early and late syph- syphilis with the introduction of penicillin into use, ilis. Cerebrospinal fluid (CSF), meninges and vas- it is still an important cause of the sexually trans- cular structures are involved in the early stages of mitted diseases in developing countries because of neurosyphilis, while in the late stage; cerebral tis- the increase of incidence of AIDS and HIV sero- sue and spinal cord parenchyma are affected [7, 8]. positivity in the world [3, 4]. Therefore, neurosyphilis can manifest with many Syphilis progresses into four stages if untreated different symptoms. as primary, secondary, latent and tertiary stages. Pri- In this article, we present a case of neurosyphilis mary syphilis is characterized by a typical painless with progressive cognitive changes and intractable syphilitic ulcer called chancre seen at the inocula- behavioral and psychiatric problems whose primary tion region after an incubation period lasting for and secondary phases were not detected. 2-3 weeks. Secondary syphilis appears weeks or months later in nearly 25% of untreated patients CASE REPORT and lymphadenopathy, gastrointestinal abnormali- ties and central nervous system alterations are seen. A 40-year-old male patient was admitted to our At the end of the latent period, tertiary syphilis hospital with amnesia, nervousness, personality develops in 25% of the untreated patients. Tertiary changes, hostile attitudes, aggressive behaviors, hal- syphilis is seen 1-30 years after primary infection. lucinations and illusions with a history of one year. Received: September 04, 2014 Accepted: October 21, 2014 Online: April 24, 2015 Correspondence: Dr. Betul OzDIlEK. Erenkoy Ruh ve Sinir Hastaliklari Egitim ve Arastirma Hastanesi, Noroloji Klinigi, Istanbul, Turkey. Tel: +90 216 - 302 59 59/417 e-mail: [email protected] © Copyright 2015 by Istanbul Northern Anatolian Association of Public Hospitals - Available online at www.kuzeyklinikleri.com Toptan et al., Neurosyphilis 67 His symptoms started 3 years ago with complaints 36 mg/dL, Pandy test positivity and leukocyte 3/ of unwillingness to move and loss of appetite. He mm3. In serologic examination of CSF, fluorescent was consulted by a psychiatrist and put on anti- treponemal antibody absorption (FTA- ABS) and psychotic drugs with no benefit. In the following VDRL was positive at 1/2 and TPHA at 1/10240 year, urinary and fecal incontinence and speech dilutions. The patient was diagnosed as neurosyph- disorders began. His medical and family history ilis and after consultation with the department of were not remarkable. On his physical examination, infectious diseases, intravenous crystalized penicil- necrotic unhealed and deeply seated wounds were line G (24 million units/day) was administered for detected on his left knee and big toe of his right 21 days. He was consulted with the department of foot. On neurological examination, his cooperation psychiatry for the behavioral disorders and agita- was poor and he was disoriented to place and time. tion. He was diagnosed as organic brain syndrome He had dysarthria and his comprehension abil- and offered risperidone (3 mg/d), haloperidol (10 ity was restricted to single commands. On cranial mg/d) and carbamazepine (800 mg/d) therapy. nerve examination, right and left pupil size were 2 Control CSF tests performed 6 months later were and 3 mm, respectively with bilateral decrease in reported as TPHA positivity at 1/64 dilution, light reflexes. The patient had bilateral bradykine- VDRL positivity and RPR 27.54 s/co. During his sia. His Romberg test was negative. Blood analy- follow-up, we did not observe any improvement in ses including complete blood count, hepatic, renal, his psychiatric symptoms, cognitive functions, uri- thyroid function tests, fasting blood glucose, elec- nary and fecal incontinence. trolytes, erythrocyte sedimentation rate, C-reactive protein were within normal limits. Markers of DISCUSSION vasculitis were negative. Among serum serological tests, Treponema Pallidum hemagglutination assay Neurosyphilis has been a rarely seen clinical entity (TPHA) was positive at 1/2560 dilution. Vene- within the last decade. It can be encountered in al- real Diseases Research Laboratories (VDRL) and most all psychiatric disorders including dementia, Rapid Plasma Reagin (RPR) tests were also posi- personality changes, mania, depression, psychosis tive. Cranial magnetic resonance (MR) images were and delirium. evaluated as global cerebral and cerebellar atrophy In our patient, psychiatric manifestations started and sequela of trauma in subcortical white matter with introversion followed by gradually worsening of the right parietal lobe without contrast enhance- cognitive decline, psychotic symptoms, dysarthria, ment (Figure 1). Lumbar puncture was performed. urinary and fecal incontinence. His medical history Cerebrospinal fluid (CSF) analysis revealed the did not reveal any clinical and dermatological com- following results. Glucose was 67 mg/dL, protein plaints and signs peculiar to primary and secondary A B C Figure 1. Flair-weighted axial section (A), T2-weighted sagittal section (B) and T1-weighted coronal section (C) of cranial MR images show diffuse cerebral, cerebellar atrophy and ventricular enlargement secondary to atrophy. 68 North Clin Istanbul – NCI stages of syphilis. Differential diagnosis of the pa- Indeed, irreversible neuronal damage is seen in gen- tient involved treatable causes of cognitive dysfunc- eral paresis, while the others reflect types of CNS tion and psychiatric disorders, primary degenerative inflammation [11]. dementia and vascular disease. Creutzfeldt-Jakob As the case presented here is in the advanced disease was ruled out because of negative radiologi- stage of neurosyphilis, no improvement was ob- cal and electroencephalographic findings. Primary served in cognitive functions, psychotic manifesta- dementia was discarded because of the patient age tions and symptoms of dysarthria, urinary and fecal and development of cognitive decline within a short incontinence despite penicillin treatment. Nowa- time (1-2 years) and severe course. MRI was nega- days, it should be remembered that syphilis in early tive for cerebrovascular disease, space-occupying stages can be overlooked, left untreated and can lead mass lesion and vascular dementia. There was no to irreversible manifestations, especially in develop- sign for substance abuse in his medical history. Nor- ing countries. mal blood tests results, negative hepatitis and HIV serology ruled out metabolic and infectious causes Conflict of Interest: No conflict of interest was declared by of dementia. Serologic tests of serum and CSF for the authors. syphilis were found positive. Clinical findings and Financial Disclosure: The authors declared that this study positive serologic results were compatible with gen- has received no financial support. eral paresis, one of the forms of neurosyphilis. Tertiary syphilis is a form of progressive demen- REFERENCES tia, also termed as general paresis, paretic neuro- syphilis or dementia paralytica. Generally it devel- 1. Bharucha NE. Infections of the nervous system. In: Bradley ops 10-25 years after onset of the infection. In early WG, Daroff RB, Fenichel GM (editors). Neurology in Clini- phases of the disease, general paresis is associated cal Practice. 3th edition. London: Butterworth - Heinemann with amnesia and personality changes. Most fre- 2000;1334-5. quently problems related to memory and reasoning 2. Adams RD, Victor M, Ropper AH (editors). Principles of th have been encountered. Less frequently, symptoms Neurology. 7 edition, New York: Mc Graw-Hill Companies as depression, mania and psychosis are seen. Neu- 2000;722-8. rological examination may be normal or there are 3. Schmidt RP. Neurosyphilis. In: Joynt RJ (editor). Clinical Neu- rology Vol.2 Revised edition. Philadelphia: Lippincott Williams some pathological findings such as dysarthria, Ar- & Wilkins 1992;1-23. gyl Robertson pupil defects, hypotonia, intentional 4. Berger JR. Neurosyphilis in human immunodeficiency virus tremor and reflex abnormalities [9]. Literature re- type 1-seropositive individuals. A prospective study. Arch Neu- ports indicate that outcome of patients with general rol 1991;48:700-2. CrossRef paresis is poor and death occurred within nearly 2.5 5. Birnbaum NR, Goldschmidt RH, Buffett WO. Resolving years before the
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