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Recent surveys show a steady increase in infectious syphilis incidence among sexual transmitted infection clinic visitors in many industrialized countries. Untreated, 30% of patients develop symptomatic neurosyphilis, possibly presenting with a variety of behavioral en neurological symptoms. Neurosyphilis is diagnosed on specific abnormalities in cerebrospinal fluid (CSF).
Case reports on neurosyphilis in Dutch patients prompted us to explore the epidemiology of neurosyphilis with psychiatric co morbidity.
To estimate the incidence of neurosyphilis and psychiatric co morbidity in the Netherlands based on data of hospitalizations in general hospitals from 1999-2007.
Hospitalization data were collected from the Dutch National Medical Register. We included all records on hospitalizations with any kind of neurosyphilis, except congenital neurosyphilis, as discharge- or secondary diagnosis (ICD9-code 094 and subcategory codes).
Between 1999 and 2007, 542 cases of neurosyphilis were registered, approximately 60 cases per year. Of all cases, 76% were male. Co-infection with HIV was seen in 74 (14%) of hospitalized cases of whom 91% were male. In 35 (6%) cases one or more concurrent psychiatric disorders were diagnosed, including 10 organic psychiatric disorders, 8 dementia cases, 6 substance abuses, 4 affective disorders, 7 psychotic disorders, 2 personality disorders and 8 miscellaneous diagnoses.
Neurosyphilis is still diagnosed in patients admitted to Dutch general hospitals. Under diagnosis due to unfamiliarity with the recent syphilis surge is possible. Over reporting is also possible due to incorrect diagnosis not based on CSF results. The reemerging syphilis epidemic may result in an increasing neurosyphilis incidence.
Neurosyphilis is a complication of syphilis, due to invasion into the central nervous system of Treponema pallidum, subspecies pallidus. Although neurosyphilis may remain asymptomatic, symptoms can develop at any time after initial infection. Early forms include syphilitic meningitis, meningovasculitis and ocular disease. Dementia paralytica, or “general paralysis of the insane”, and tabes dorsalis used to be the most frequent forms of late neurosyphilis. Several reports, however, suggest a changing clinical presentation of neurosyphilis. Atypical neuropsychiatric syndromes seem to be more prevalent than in the past. Inadvertent treatment with antibiotics prescribed for unrelated diseases and improved serological methods are probably the most important contributing factors. Accelerated progression from syphilis to neurosyphilis in HIV seropositive patients has also been reported.
This prospective study aims to investigate the clinical spectrum of neurosyphilis in patients whose cerebrospinal fluid was tested positive for neurosyphilis by the Laboratory for Infectious Diseases, a large reference laboratory in the North-East part of the Netherlands.
From 2004 to 2013 all patients with a new diagnosis of neurosyphilis, proven by cerebrospinal fluid serology, were included. Each patient’s clinical record was reviewed for demographic, clinical and laboratory data.
Our inclusion criteria were met by 24 patients, all male. The clinical spectrum ranged from asymptomatic to dementia paralytica. Age varied from 31 to 71. At the time of neurosyfilis diagnosis, 7 patients were HIV seropositive.
Further analysis is currently being done and definite results and conclusions will be presented at the meeting.
Acute intermittent porphyria (AIP) is a rare autosomal dominant inherited metabolic disease characterized by mutations in the porphobilinogen deaminase gene. This mutation may provoke neurotoxic levels of delta-aminolevulinic-acid and porphobilinogen, potentially resulting in an acute life-threatening clinical syndrome, characterized by psychiatric, in particular atypical psychotic, symptoms as well as severe neurological and gastrointestinal symptoms. Since the clinical presentation varies and symptoms are nonspecific, diagnosis is often made late.
Naming of alarm symptoms based on a recent case study.
Description of a recent case supplemented with data from the literature.
The patient is a 46 year old woman who was admitted in 2007 with abdominal pain, an epileptic seizure and weakness, interpreted as a Guillain-Barre syndrome. In 2011 she was readmitted with severe abdominal pain, diarrhea, volatile psychotic symptoms and seizures, following a short period of excessive alcohol consumption. During admission she developed progressive weakness in the upper arms, shooting pains in the limbs and a feeling of tightness. Impaired abdominal breathing was suspected. Again, Guillain-Barré syndrome was considered, but additional studies did not support this diagnosis. Because of the recurrent character of symptomatology following alcohol abuse, acute (intermittent) porphyria was considered diagnostically. The dark-colored urine indeed contained significantly increased delta-aminolevulinic-acid and porphobilinogen concentrations. Additional (genetic) diagnosis follows.
A recurrent disease course with severe gastrointestinal, neurological and psychiatric symptoms, following alcoholabuse, is suspect for AIP.
Dementia paralytica, a late neuropsychiatric complication of syphilis, was common in Europe at the beginning of the 20th century. in the course of the subsequent century, the number of patients decreased dramatically, partly due to improved treatment options. One of the great therapeutic breakthroughs was the discovery of malaria fever therapy by Wagner von Jauregg.
This retrospective cohort-study was designed to detect prognostic factors regarding the treatment of dementia paralytica with malaria fever therapy.
Inpatients of the Vincent van Gogh psychiatric hospital in Venray were included in this study if they had a discharge diagnosis of dementia paralytica (general paralysis of the insane) and had been treated with malaria fever therapy in the period from 1907 to 1970. Patients were identified using annual reports and death registries from the hospital. Each patient's clinical record was reviewed for demographic, clinical, therapeutical and laboratory data. We measured survival after malaria fever therapy. Treatment response was categorized as: complete remission, incomplete remission but no hospitalization required, incomplete remission and permanent hospitalization required, unimproved or death during or shortly after treatment. the cause of death was classified as related or not related to dementia paralytica.
Preliminary results suggest that 70 % of the patients were male. Many patients died within 2 months of hospitalisation, but some survived for more than 20 years.
Further analysis is currently being done and definite results and conclusions will be presented at the meeting.
General Paralysis of the Insane (GPI), or Dementia Paralytica, was once a fatal complication of syphilitic infection and a major reason for psychiatric hospitalisation. Syphilis re-emerged worldwide at the turn of the 20th to 21st century, and, considering the incubation period of 10-30 years, a revival of GPI may be expected within the next one or two decades. Early diagnosis and treatment improve prognosis and, therefore, a renewed clinical awareness of signs and symptoms of GPI is needed.
This historical cohort study aims to investigate the clinical presentation of GPI in patients deceased at the Vincent van Gogh psychiatric hospital in Venray, the Netherlands, in the period 1924-1954.
Annual hospital reports and individual patient's records were used for identification of patients with an established diagnosis of GPI, usually based on a combination of clinical data and positive syphilis serology.
Individual clinical records of 105 patients (91 men; 14 women) with GPI were available for evaluation. Median age for men (50.4 years; range 31.5 to 82.1 years) was higher than for women (43.1 years; range 34.4 to 58.4 years): p = 0.01. Most patients completed elementary school only and belonged to the lower working class. Lack of judgement and insight, hyperactivity, and confusion were the most frequently documented psychiatric symptoms, whereas speech disorders, pupillary abnormalities, micturition disorders, dementia, and cranial nerve involvement were the most frequently reported neurological symptoms.
The clinical presentation of GPI displays a wide range of cognitive, affective, psychotic and focal neurological symptoms.
Neurosyphilis is caused by dissemination into the central nervous system of Treponema pallidum. Although the incidence of syphilis in the Netherlands has declined since the mid-1980s, syphilis has re-emerged, mainly in the urban centres. It is not known whether this also holds true for neurosyphilis.
The epidemiology of neurosyphilis in Dutch general hospitals in the period 1999–2010 was studied in a retrospective cohort study. Data from the Dutch sexually transmitted infection (STI) clinics were used to analyse the number of patients diagnosed with syphilis in this period.
An incidence of neurosyphilis of 0.47 per 100 000 adults was calculated, corresponding with about 60 new cases per year. This incidence was higher in the western (urbanised) part of the Netherlands, as compared with the more rural areas (0.6 and 0.4, respectively). The number of patients diagnosed with syphilis in STI clinics increased from 150 to 700 cases in 2004 and decreased to 500 new cases in 2010. The sex ratio was in favour of men, yielding a percentage of 90% of the syphilis cases and of 75% of the neurosyphilitic cases. The incidence of neurosyphilis was highest in men aged 35–65 years, and in women aged 75 years and above. The most frequently reported clinical manifestation of neurosyphilis was tabes dorsalis. In this study, 15% of the patients were HIV seropositive.
The incidence of neurosyphilis in a mixed urban–rural community such as the Netherlands is comparable to that in other European countries. Most patients are young, urban and men, and given the frequent atypical manifestations of the disease reintroduction of screening for neurosyphilis has to be considered.
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