Clinical Neuroscience

[Selection of the optimal device-aided therapy in Parkinson’s disease]

KOVÁCS Norbert1,2, ASCHERMANN Zsuzsanna1, JUHÁSZ Annamária1, HARMAT Márk1, PINTÉR Dávid1, JANSZKY József1,2

JANUARY 30, 2019

Clinical Neuroscience - 2019;72(01-02)

DOI: https://doi.org/10.18071/isz.72.0005

[For the treatment of advanced Parkinson’s disease the deep brain stimulation (DBS) and the levodopa/carbidopa intestinal gel (LCIG) therapies are available in Hungary. Although they may have similar impact on the health-related quality of life and disabilities associated with the disease, they have different indications, and inclusion- and exclusion criteria. Consequently, the patient population treated with DBS and LCIG may be different. In the present review, the authors try to help the process of selection of the optimal device-aided therapy for the patients with advanced Parkinson’s disease. ]

AFFILIATIONS

  1. Pécsi Tudományegyetem, Neurológiai Klinika, Pécs
  2. MTA-PTE Klinikai Idegtudományi Képalkotó Kutatócsoport, Pécs

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Additional value of tau protein measurement in the diagnosis of Creutzfeldt-Jakob disease

CSEH Katalin Edina, VERES Gábor, DANICS Krisztina, SZALÁRDY Levente, NÁNÁSI Nikolett, KLIVÉNYI Péter, VÉCSEI László, ZÁDORI Dénes

Since the definite diagnosis of Creutzfeldt-Jakob disease (CJD) can currently only be provided by autopsy, there is a special need for fine diagnostic tools in live patients to achieve accurate diagnosis as early as possible. The aim of this study was to perform a preliminary retrospective analysis on the utility of the measurement of total Tau (tTau) and some other biomarkers from the cerebrospinal fluid (CSF) of patients with rapidly progressive dementia in the diagnostic work up of CJD. Beside the assessment of relevant clinical data and the findings of electroencephalography and brain magnetic resonance imaging, the presence of 14-3-3 protein and the levels of tTau were determined by Western blot technique and enzyme-linked immunosorbent assay from the CSF of 19 patients diagnosed with rapidly progressive dementia between the period of 2004-2017 at the Department of Neurology, University of Szeged. This preliminary study provided 100% sensitivity for 14-3-3, and interestingly, only 40% specificity to support the clinical diagnosis of CJD. Regarding tTau, the sensitivity values were calculated to be 100% or 83%, whereas the specificity values were 71% or 86%, depending on the applied cut-off levels. The poor specificity of 14-3-3 is not in line with literature data and may be the result of the small number of patients in the cohort with non-prion disease, predominantly consisting of disorders with considerable tissue damage, whereas tTau presented good sensitivity and specificity values. The combined application of these and novel chemical biomarkers may increase both sensitivity and specificity to a desired level.

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Axillary mononeuropathy after herpes zoster infection misdiagnosed as neuropathic pain

GÜL Sinem Sidika, AKARSU Oguz Emel

Zoster-associated extremity paresis is a rare complication of herpes zoster (HZ) and is usually due to zoster-associated mononeuropathy. Complaints of a 77-year-old man started with pain in his right arm and 4 days later he developed itchy red HZ lesions in the same area. One week later, the patient developed weakness in his right arm. The patient was diagnosed with isolated axillary mononeuropathy by physical examination and electromyography. Here, we present a case of axillary mononeuropathy which is a rare complication of HZ infection and needs particular attention.

Clinical Neuroscience

[Multiple ischemic stroke in Osler-Rendu-Weber disease]

SALAMON András, FARAGÓ Péter, NÉMETH Viola Luca, SZÉPFALUSI Noémi, HORVÁTH Emese, VASS Andrea, BERECZKY Zsuzsanna, TAJTI János, VÉCSEI László, KLIVÉNYI Péter, ZÁDORI Dénes

[Hereditary hemorrhagic teleangiectasia (HHT, Osler-Rendu-Weber disease) is an autosomal dominantly inherited disorder caused by the mutation of several possible genes and characterized by malformations of the arteriovenous system in multiple organs. The clinical diagnosis is based on the Curaçao criteria ((1) spontaneous, recurrent epistaxis; (2) teleangiectasias in characteristic sites (lips, oral cavity, nose, fingers); (3) visceral lesions (gastrointestinal, pulmonary, cerebral, spinal); (4) affected first degree relative). The aim of this study is to present the first genetically confirmed Hungarian case of hereditary hemorrhagic teleangiectasia with multiple ischemic strokes. Our 70-year-old woman has been suffering from severe epistaxis since her childhood and presented gastrointestinal bleeding during her adulthood as well. The characteristic skin lesions developed in the 5th decade of life. She was admitted to our department with loss of consciousness and fluctuating speech and swallowing problems. MRI of the brain supplemented with angiography revealed multiple arteriovenous malformations and multiple subacute ischemic lesions. The EEG demonstrated slowing of electric activity in the left frontal lobe. The neuropsychological assessment showed deficits in anterograde memory and executive functions. The diagnostic work-up for other characteristic alterations identified an arteriovenous malformation in the left lung. The genetic analysis demonstrated a heterozygous mutation in the 7th exon of the ENG gene at position 834 resulting in a thymine duplication and an early stop codon by a frame shift. The present case is largely similar to those already described in literature and draws the attention to the importance of multidisciplinary collaboration in the care of HHT patients.]

Clinical Neuroscience

[Tension type headache and its treatment possibilities]

ERTSEY Csaba, MAGYAR Máté, GYÜRE Tamás, BALOGH Eszter, BOZSIK György

[Tension type headache, the most common type of primary headaches, affects approximately 80% of the population. Mainly because of its high prevalence, the socio-economic consequences of tension type headache are significant. The pain in tension type headache is usually bilateral, mild to moderate, is of a pressing or tightening quality, and is not accompanied by other symptoms. Patients with frequent or daily occurrence of tension type headache may experience significant distress because of the condition. The two main therapeutic avenues of tension type headache are acute and prophylactic treatment. Simple or combined analgesics are the mainstay of acute treatment. Prophylactic treatment is needed in case of attacks that are frequent and/or difficult to treat. The first drugs of choice as preventatives of tension type headache are tricyclic antidepressants, with a special focus on amitriptyline, the efficacy of which having been documented in multiple double-blind, placebo-controlled studies. Among other antidepressants, the efficacy of mirtazapine and venlafaxine has been documented. There is weaker evidence about the efficacy of gabapentine, topiramate, and tizanidin. Non-pharmacological prophylactic methods of tension type headache with a documented efficacy include certain types of psychotherapy and acupuncture. ]

Clinical Neuroscience

Evaluation of neutrophil-to-lymphocyte ratio and platelet-to-lymphocyte ratio in essential tremor

TAK Zeynal Abidin Ali, SENGUL Yildizhan

Introduction - Although essential tremor (ET) is the most common cause of tremor, the pathology and underlying mechanisms have not fully understood yet. In addition to kinetic tremor, patients may present several types of tremor, gait ataxia, hearing deficits and eye movement abnormalities. Non-motor symptoms and signs have also added to definition of ET. There is significant evidence indicating the neurodegenerative nature of the disease. New studies indicate that inflammation may have a place in the etiology. The neutrophil-to lymphocyte ratio (NLR) and the platelet-to lymphocyte ratio (PLR) have recently begun to be used as a marker of systemic inflammation. Our study aims at finding a clue for systemic inflammation in ET. Methods - 67 patients with ET and 40 healthy controls were recruited for the study. The total white blood cells (WBC), absolute neutrophil count, lymphocyte count and platelet count were retrieved. The NLR was calculated by dividing the neutrophil count by the lymphocyte count and the PLR was calculated by dividing the platelet count by the lymphocyte count. Results - Patient and control groups were similar in terms of age and gender. The mean age of patient group was 25.29 ± 8.24 years and that of control group was 26.77 ± 6.73 years. The NLRs were 1.85 ± 0.58 in the patient group and 1.96 ± 0.53 in the control group. For the patient group and the control group the PLRs were 103.52 ±32.80 and 91.26 ± 31.57 respectively. There were no statistically significant differences between the participants for both NLR and PLR. Conclusion - The pathophysiological mechanism for essential tremor (ET) remains unclear. However, there is an increasing amount of research being conducted on the subject. Discussions about ET’s definition as a neurodegenerative disease are ongoing. Although previous studies showed that neuroinflammation could be a part of etiology of disease, this study has failed to demonstrate systemic inflammation in ET.

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Vestibular evoked myogenic potential responses in Parkinson’s disease

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Background - Our objectives were to determine the differences in the vestibular evoked myogenic potential (VEMP) responses in patients diagnosed with early staged idiopathic Parkinson’s disease (PD) compared to the normal population and evaluate the vestibular system disorder causing balance-posture disorders. Second aim of this study was to investigate caloric test responses particularly in early staged PD compared to normal popu­lation. Material and methods - Thirty patients (14 females and 16 males; mean age, 60.6 ± 13.1 years) diagnosed with idiopathic PD and 28 healthy subjects (20 males and 8 females; mean age, 59.1 ± 6.4 years) were included. The patient and control groups were subdivided according to their age, gender and the patient group was subdivided according to onset time of the Parkinson symptoms, Hoehn-Yahr staging. The subgroups were compared for VEMP and caloric test responses. Results - There were no significant differences between the study and control groups for right and left VEMP measurements. Patients over 60 years and under 60 years did not show significant differences in terms of right and left mean VEMP measurements. However, P1 amplitude was significantly lower in patients over 60 years old (P = .004). Gender, disease duration, BERG balance scale and Hoehn-Yahr stage had no effect on the VEMP amplitudes. There was no significant correlation with the side of Parkinsonian symptoms to the side of canal paresis (P = .566) and the side on which no VEMP response was obtained in caloric test. Conclusion - VEMP responses were not different between PD and healthy subjects. VEMP P1 amplitude was decreased with age in PD group. Canal paresis and symptoms side were not statistically correlated in caloric test.

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[Earlier and more efficiently: the role of deep brain stimulation for parkinson’s disease preserving the working capabilities]

DELI Gabriella, BALÁS István, KOMOLY Sámuel, DÓCZI Tamás, JANSZKY József, ASCHERMANN Zsuzsanna, NAGY Ferenc, BOSNYÁK Edit, KOVÁCS Norbert

[Background – The recently published “EarlyStim” study demonstrated that deep brain stimulation (DBS) for the treatment of Parkinson’s disease (PD) with early fluctuations is superior to the optimal pharmacological treatment in improving the quality of life and motor symptoms, and preserving sociocultural position. Our retrospective investigation aimed to evaluate if DBS therapy was able to preserve the working capabilities of our patients. Methods – We reviewed the data of 39 young (<60 years-old) PD patients who underwent subthalamic DBS implantation at University of Pécs and had at least two years follow-up. Patients were categorized into two groups based on their working capabilities: Patients with active job (“Job+” group, n=15) and retired patients (without active job, “Job-” group, n=24). Severity of motor symptoms (UPDRS part 3), quality of life (EQ-5D) and presence of active job were evaluated one and two years after the operation. Results – As far as the severity of motor symptoms were concerned, similar (approximately 50%) improvement was achieved in both groups. However, the postoperative quality of life was significantly better in the Job+ group. Majority (12/15, 80%) of Job+ group members were able to preserve their job two years after the operation. However, only a minimal portion (1/24, 4.2%) of the Job- group members was able to return to the world of active employees (p<0.01, McNemar test). Conclusion – Although our retrospective study has several limitations, our results fit well with the conclusions of “EarlyStim” study. Both of them suggest that with optimal timing of DBS implantation we may preserve the working capabilities of our patients.]

Clinical Neuroscience

[UPDATES IN PRACTICAL NEUROLOGY - I. THE PRINCIPLES OF MODERN LEVODOPA THERAPY IN PARKINSON’S DISEASE]

KLIVÉNYI Péter, VÉCSEI László

[Despite the levodopa is used for the treatment of Parkinson’s disease for a long time, recently many questions raised about its clinical use. New issues emerged based on the clinical trials, on latest neuroimaging data and on better understanding the pathomechanism of motor complications. These observations have changed the routine clinical use of levodopa. In this review we summarize the evidences and practical implications of levodopa therapy.]

Clinical Neuroscience

[Efficacy of deep brain stimulation in our patients with Parkinson’s disease]

GERTRÚD Tamás, TAKÁTS Annamária, RADICS Péter, RÓZSA Ildikó, CSIBRI Éva, RUDAS Gábor, GOLOPENCZA Péter, ENTZ László, FABÓ Dániel, ERÕSS Loránd

[Background and purposes - In advanced Parkinson’s disease, medically refractory motor fluctuation or medically resistant tremor considerably affects quality of life. However, these symptoms can be mostly successfully treated by deep brain stimulation. We analyzed the efficacy of bilateral subthalamic stimulation in our patients with Parkinson’s disease. Methods - We assessed the clinical data of ten patients who have been treated in the Department of Neurology, Semmelweis University and have been operated in the National Institute of Neurosciences between 2008 and 2011. The Hoehn-Yahr scale score, the Unified Parkinson’s Disease Rating Scale score and the Parkinson’s Disease Questionnaire 39, as well as the dose of antiparkinson medication were documented prior to and one year after surgery. Results - Patient condition improved according to the Hoehn-Yahr scale, approximately by two stages. The dose of antiparkinson medication could be reduced by 63.4% (p=0.005) post operation. Unified Parkinson’s Disease Rating Scale scores decreased by 70.9% (p=0.005). 12 hours after medication withdrawal, execution of daily activity improved by 57.1% (p<0.01) and motor functions developed by 79.1% (p<0.01). Duration of dyskinesias decreased by 62.5% (p=0.018), duration of akinesia diminished by 87.5% (p=0.005). Quality of life rose by 41.6% (p<0.01). Neuropsychological tests detected improvement in verbal memory. Conclusion - With deep brain stimulation, the dosage of antiparkinson medication could be significantly reduced, with considerable improvements in motor function and quality of life. Although the number of patients is still low, good results have been established by careful patient selection, precise neurosurgical procedure and by appropriate programming and patient care.]

LAM KID

[Effect of zoledronic acid treatment on pain and quality of life in patients with metastatic bone disease suffering from breast and prostate cancer - Multicenter, prospective, observational study]

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[INTRODUCTION - Metastatic bone disease is frequently associated with breast and prostate cancer. Bisphosphonate treatment of bone metastases is palliative: its primary goal is to relieve pain, while it's also important to decrease the risk of bone fractures, prolong survival and maintain physical activity of the patients. Pain is the most common symptom of bone metastases. PATIENTS AND METHODS - In total 845 patients were enrolled in our open, multicenter, prospective, observational study, the first of its kind in Hungary. The agent tested was zoledronic acid (Zometa®). Duration of the study was 20 months and its primary goal was to assess the correlation between pain and quality of life during the treatment of patients with bone metastases from breast or prostate carcinoma. RESULTS - During the 18 months of the study, the average intensity of pain, measured on the visual analog scale showed a 42% reduction (p<0.0001). By the end of the 18. month, the ratio of patients free of symptoms has increased by 15% and the number of patients with substantial complaints has decreased by 73%. CONCLUSION - Our study supports the observation published in the international literature that in patients with bone metastases from breast and prostate cancer, zoledronic acid treatment is beneficial for reducing pain and thus for improving quality of life.]