Clinical Neuroscience

Posterior reversible encephalopathy syndrome as an initial manifestation of systemic lupus erythematosus

AYAS Özözen Zeynep1, ÖCAL Öncel Ruhsen2, GÜNDOGDU Aksoy Asli1

NOVEMBER 30, 2017

Clinical Neuroscience - 2017;70(11-12)


Posterior reversible encephalopathy syndrome (PRES) is a disorder which is diagnosed with its characteristic clinical and radiological findings, typically resolves with treatment. The prevalence of PRES in systemic lupus erythematosus (SLE) patients is not exactly known. A systemic disorder frequently appears as a presenting symptom in SLE. However, in rare cases, the disease starts with a neurological manifestation. Here we report a 35-year-old woman presenting with a headache and blurred vision. She had neurologic symptoms and cerebral lesions on magnetic resonance imaging (MRI) suggesting PRES. The patient was diagnosed with SLE during the etiological investigation of PRES. In this article, we aimed to emphasize that PRES as an initial presentation of SLE.


  1. Department of Neurology, Sakarya University Training and Research Hospital, Sakarya, Turkey
  2. Department of Neurology, Başkent University Hospital, Ankara, Turkey



Further articles in this publication

Clinical Neuroscience

A case with reversible neurotoxicity induced by metronidazole

EREN Fulya, ALDAN Ali Mehmet, DOGAN Burcu Vasfiye, GUL Gunay, SELCUK Hatem Hakan, SOYSAL Aysun

Background - Metronidazole is a synthetic antibiotic, which has been commonly used for protozoal and anaerobic infections. It rarely causes dose - and duration - unrelated reversible neurotoxicity. It can induce hyperintense T2/FLAIR MRI lesions in several areas of the brain. Although the clinical status is catastrophic, it is completely reversible after discontinuation of the medicine. Case report - 36-year-old female patient who had recent brain abscess history was under treatment of metronidazole for 40 days. She admitted to Emergency Department with newly onset myalgia, nausea, vomiting, blurred vision and cerebellar signs. She had nystagmus in all directions of gaze, ataxia and incompetence in tandem walk. Bilateral hyperintense lesions in splenium of corpus callosum, mesencephalon and dentate nuclei were detected in T2/FLAIR MRI. Although lumbar puncture analysis was normal, her lesions were thought to be related to activation of the brain abscess and metronidazole was started to be given by intravenous way instead of oral. As lesions got bigger and clinical status got worse, metronidazole was stopped. After discontinuation of metronidazole, we detected a dramatic improvement in patient’s clinical status and MRI lesions reduced. Conclusion - Although metronidazole induced neurotoxicity is a very rare complication of the treatment, clinicians should be aware of this entity because its adverse effects are completely reversible after discontinuation of the treatment.

Clinical Neuroscience

[Role of peginterferon-β-1a in the therapy of multiplex sclerosis]

SIMÓ Magdolna, ILJICSOV Anna

[The subcutaneous peginterferon-b-1a is recently introduced in the therapy of relapsing-remitting multiplex sclerosis (RRMS) patients. Pegylation of IFN b-1a improved pharmacodynamic and pharmacokinetic properties, resulting in, increased biologic activity and a longer half-life. The efficacy of peginterferon-b-1a was proved by the ADVANCE study - a 2-year Phase 3, multicenter, randomized, double-blind study with a 1-year placebocontrolled period evaluating the efficacy and safety of subcutaneous peginterferon-b-1a administered every 2 or 4 weeks in patients with RRMS. Peginterferon-b-1a efficacy was maintained during the two years, with greater effects observed with every 2 week versus every 4 week dosing. Annualized relapse rate and confirmed disability progression was reduced comparing with patients on delayed treatment. Patients treated with continuous peginterferon-b-1a had fewer new or newly enlarging T2 lesions over 2 years than patients in the delayed treatment group. Adverse events were consistent with the known profiles of IFN b therapies in MS. The most commonly reported adverse events were injection site erythema, influenza-like illness. The less frequent administration is associated with fewer flu-like adverse events, which may improve patients’ compliance and adherence. Peginter-feron-b-1a could be an effective and safe treatment option for RRMS patients.]

Clinical Neuroscience

[Change of therapeutic algorithm in sclerosis multiplex based on two case reports]

BIERNACKI Tamás, BENCSIK Krisztina, KINCSES Zsigmond Tamás, SANDI Dániel, FRICSKA-NAGY Zsanett, FARAGÓ Péter, VÉCSEI László

[The aim of our case reports is to demonstrate the therapeutic use and possibilities one has with alemtuzumab, should it be used either as a first or second line therapy. Our first patient's disease in the beginning seemed to be benign. It was not the case however, over several years the diesase showed high activity both radiologically and clinically, she was treated with alemtuzumab as part of an esclationbased therapeutic strategy. The second patient's disease on the other hand showed formidable activity since the very beginning both radiologically and clinically. Therefore we were facing a very disastrous prognosis on the long run, accordingly he received alemtuzumab treatment very early into his illness.]

Clinical Neuroscience

Validation of the Hungarian version of Carlson’s Work-Family Conflict Scale


Background and purpose - Work-family conflict has been associated with adverse individual (e.g., cardiovascular diseases, anxiety disorders), organizational (e.g., absenteeism, lower productivity), and societal outcomes (e.g., increased use of healthcare services). However, lack of standardized measurement has hindered the comparison of data across various cultures. The purpose of this study was to develop the Hungarian version of Carlson et al.’s multidimensional Work-Family Conflict Scale and establish its reliability and validity. Methods - In a sample of 557 employees (145 men and 412 women), we conducted confirmatory factor analysis to investigate the factor structure and factorial invariance of the instrument across sex and data collection points and evaluated the tool's validity by assessing relationships between its dimensions and scales measuring general, marital, and job-related stress, depressive symptomatology, vital exhaustion, functional somatic symptoms, and social support. Results - Our results showed that a six-factor model, similarly to that of the original instrument, fit the data best. Internal consistency of the six dimensions and the whole instrument was adequate. Convergent and divergent validity of the instrument and discriminant validity of the dimensions were also supported by our data. Conclusions - This study provides empirical support for the validity and reliability of the Hungarian version of the multidimensional Work-Family Conflict Scale. Deployment of this measure may allow for the generation of data that can be compared to those obtained in different cultural settings with the same instrument and hence advance our understanding of cross-cultural aspects of work-family conflict.

Clinical Neuroscience

Effect of maternal migraine on children’s quality of sleep

GÜNGEN Dogan Belma, YILDIRIM Ahmet, ARAS Guzey Yesim, ARAS Atılgan Bilgehan, TEKESIN Aysel, AYAZ Burcu Ayse

Backround and aim - Sleep disorders are common problems associated with migraine. These sleep disorders are known to have a debilitating impact on daily lives of migraine patients. The purpose of this study is to assess the effects of sleep disorders experienced by individuals suffering from migraine on their children as well as the presence of sleep disorders in their children. Materials and methods - This study included 96 mothers diagnosed with migraine and their 96 healthy children, and a control group formed of 74 healthy mothers and their children. Exclusion criteria were chronic systemic disease or central nervous system disease or a history of smoking/alcohol use for mothers, and chronic disease or regularly occurring headaches or recurrent abdominal pain for children. For maternal evaluation, the Visual Analogue Scale (VAS), Migraine Disability Assessment Scale (MIDAS), Pittsburgh Sleep Quality Index (PSQI), Beck Depression Index (BDI) and Beck Anxiety Index (BAI) were used and for the assessment of the children’s quality of sleep, the Children’s Sleep Habits Questionnaire (CSHQ) was used. The SPSS 21.0 program was employed for statistical analysis, with statistical significance set at p<0.05. Findings - The mean age of the group with migraine was 36.6±7.1 years, while that of the control group was 38.01±4.7. Mood and sleep disorders were more frequently observed in the participants with migraine (p<0.05). Sleep disorders were significantly low in children with migraineur mothers (p=0.02); and child sleep anxiety is significantly high in control group (p=0.048). Maternal BAI scores had a significant influence on their children’s quality of sleep. Discussion and conclusion - In our study, the presence of migraine-type headache in mothers was observed to have a positive effect on reducing sleep disorders in the children. Recurrent headaches of the migraineur mothers with or without sleep disorders and psychiatric comorbidities did not influence the quality of sleep in their children directly, but the sleep anxiety of the children may have had an impact on it.

All articles in the issue

Related contents

Hungarian Immunology

[MCP-1 (monocyte chemoattractant protein-1) G/A and T-bet (T-helper promoter factor) C/G polymorphisms in primary Sjögren’s syndrome and systemic lupus erythematosus]

KOVÁCS Attila, KONCZ Ágnes, ENDREFFY Emőke, ARANKA László, PETRI Ildikó, ELLER József, SZALAI Csaba

[INTRODUCTION - Monocyte chemoattractant protein- 1 (MCP-1) is a β-chemokine involved in the attraction and accumulation of mononuclear granulocytes towards the site of inflammation. One of the transcriptional factors of T-cells is called T-bet. PATIENTS AND METHODS - The authors investigated the MCP-1-2518 G/A and T-bet 310 C/G (His33Gln) polymorphisms evaluating the distribution of the specific genotypes in 45 patients with primary Sjögren's syndrome (pSS), 51 patients with systemic lupus erythematosus (SLE), and in 320 healthy blood donors as the control group. MCP-1-2518 G/A and T-bet 310 C/G polymorphisms were detected with molecular genetic methods from the purified genomic DNA. RESULTS - The frequency of the MCP-1-2518 AG heterozygous genotype decreased tendentiously only in SLE patients, while the frequency of the MCP-1 AA homozygous genotype increased comparing to the control group (13.7% vs. 5.9%; Pearson’s χ2 test=6.125, ns.). Analyzing the genotype frequency for the MCP-1 wild (GG) and AA homozygous genotypes in pSS group, the MCP-1 AA homozygous genotype proved to be more frequent comparing to the control group (82.8%:17.2% vs. 90.7%:9.3%; Pearson’s χ2 test 1.755, ns). These relations showed only tendentious association in the SLE group (81.6%:18.7% vs. 90.7%:9.3%; Pearson’s χ2 2.811, p=0.094, ns.) There was not any significant correlation between the investigated MCP-1- 2518 G/A and the T-bet 310 C/G polymorphisms and the TNF-α -308 G/A and -238 allele polymorphisms. The frequency of T-bet was equal in relation with heterozygous (CG) to wild CC genotype in the investigated two autoimmune disorders. The GG homozygous genotype for T-bet could not be found in SLE and pSS groups, likely to be a protective factor. CONCLUSIONS - The above mentioned polymorphisms didn’t show any significant correlation with TNF-α -308 and -238 allele polymorphisms. The further research of the MCP-1 G/A and T-bet C/G polymorphisms is important, because of their possible prognostic importance for SLE and pSS.]

Lege Artis Medicinae

[Analysis of short-term and long-term survival and causes of death in patients with systemic lupus erythematosus]

TARR Tünde, KISS Emese, SZEGEDI Gyula, ZEHER Margit

[INTRODUCTION - In systemic lupus erythematosus (SLE), both short-term and long-term survival rates have improved worldwide. We analysed retrospectively the short-term and long-term survival data and causes of death at a single center. These data were compared with previous survival data recorded at the same centre and published in international studies. PATIENTS AND METHOD - The data of 550 patients with SLE were analysed between 1970 and 2009. We examined the effect of clinical symptoms, age, severity and onset of the disease and the applied immunosuppressive treatment on survival, using the Kaplan-Meier method. RESULTS - Survival rates at 5, 10, 15 and 20 years after the diagnosis were 98%, 94%, 90% and 89%, respectively. Late onset, neuropsychiatric symptoms and severe SLE were found to be prognostic factors. Manifestations affecting other organs and the applied immunosuppressive therapy did not influence survival rates. During the study period, 57 out of the 550 patients (10.4%) died. The main causes of death were cardiovascular complications (50.9%), infections (21%), and malignancies (12.3%). CONCLUSIONS - Our results show that among patients with SLE, it is mostly longterm survival that has increased, owing to the close control of patients. The increase in cardiovascular mortality highlights the importance of regular screening.]

Hungarian Immunology

[SS-A(Ro) and SS-B(La) autoantibodies in systemic lupus erythematosus]

SALLAI Krisztina, NAGY Eszter, GERGELY Péter

[OBJECTIVE - To assess the relation between clinical features and the presence of SS-A(Ro) and SS-B(La) autoantibodies in systemic lupus erythematosus. PATIENTS - The data of 200 patients with definite systemic lupus erythematosus were analysed. SSA( Ro) and SS-B(La) antibodies were assessed by enzyme immunoassay. RESULTS - 40.5% of systemic lupus erythematosus' patients were SS-A(Ro) and/or SS-B(La) antibody positive (’positive group’); the majority of such patients displayed both antibodies, 16.5% had SSA( Ro) antibodies alone, while only 2% has SS-B(La) antibodies alone. There were no differences in the occurrence of arthritis, secondary antiphospholipid syndrome and hematologic manifestations between the positive and negative groups; serositis was more common in the positive group. Skin manifestations, in particular subacute cutaneous lupus erythematosus and urticaria vasculitis were more frequent in the positive group, while kidney and central nervous system involvation, in particular severe forms were less frequent. Secondary Sjögren's syndrome occurred exclusively in antibody positive patients. Sm, RNP and Scl-70 antibodies were more frequently found in the positive group. CONCLUSIONS - The presence of SS-A(Ro) and/or SS-B(La) antibodies in systemic lupus erythematosus has some prognostic significance; in antibody-positive patients there is an increased risk for skin lesions (in particular subacute cutaneous lupus erythematosus and urticaria vasculitis) and secondary Sjögren’s syndrome and a decreased risk for severe nephritis or central nervous system involvement.]

Lege Artis Medicinae



[Systemic lupus erythematosus is an autoimmune disorder. It stands at the focus of medical interest: basic scientists, clinicians and innovative biotechnologists all pay attention to lupus. Authors of this article present the novel scientific results on the etiopathogenesis, clinical and laboratory characteristics of SLE. Furthermore, authors discuss diagnostic problems and the possible therapeutic modalities. One of the most important results is the characterisation and mapping of the susceptibility genes as well as the analysis of their functional features. More and more is known about the relationship between natural and adaptive immunity, about the cooperation of T and B cells. The abnormalities of intracellular biochemical processes and signal transduction pathways have been cleared. The importance of cytokine network and infective agents in the pathogenesis of SLE has largely been investigated recently. With regards to the outcome of the disease, there is growing attention paid on chronic organ damages, such as end-stage renal disease, osteoporosis and atherosclerosis - in connection with the increased life expectancy. Evidence accumulates on the importance of immune-inflammatory processes in the initiation and perpetuation of osteoporosis and atherosclerosis. There is an urgent need for validated biomarkers which can predict the susceptibility, prognosis, clinical manifestations, activity and severity of SLE. To follow and measure the effectiveness of treatment is also required. Although the principals of lupus management have not changed, novel immune modulators, biological therapy and non-medical treatments (e.g. stem-cell transplantation) have become available. Further research and clinical observations may help to find the real place of such therapeutics.]

Clinical Neuroscience


ILNICZKY Sándor, KAMONDI Anita, ARÁNYI Zsuzsanna, VÁRALLYAY György, GAAL Barbara, SZIRMAI Imre, NAGY György

[Systemic lupus erythematosus is a frequent autoimmune disease, affecting several organs, including the brain, spinal cord and nerves. Cerebral vasculitis, transverse myelitis and polyneuropathy are the most common neurological manifestations. We report a case of a 46 years old woman who suffered incomplete transverse myelitis in her age of 44. After 2 years the second relapse presented with arthralgias, painful paraesthesias and weakness of the lower limbs. Neurological signs suggested involvement of the central and the peripheral nervous system. Based upon clinical and laboratory findings systemic lupus erythematosus was diagnosed. Magnetic resonance imaging revealed two hyperintense lesions on T2 weighted scans within the cervical spinal cord. The brain scan was normal. Protein content was slightly elevated in the cerebrospinal fluid, with normal cell count. Electrophysiological examinations diagnosed a subacute sensory-motor axonal polyneuropathy. On methylprednisolone treatment her condition improved. Simultaneous development of central and peripheral lesions of the nervous system in cases with systemic lupus erythematosus may lead to a challenge to establish the diagnosis.]