Clinical Neuroscience

[LONG-TERM APPLICATION OF THE MULTIPLE SCLEROSIS FUNCTIONAL COMPOSITE IN DEBRECEN]

MEZEI Zsolt, BERECZKI Dániel, CSIBA László, CSÉPÁNY Tünde

NOVEMBER 30, 2006

Clinical Neuroscience - 2006;59(11-12)

[Introducion - The multiple sclerosis functional composite (MSFC) has been recommended by the National Multiple Sclerosis Society as a new clinical outcome measure. It is based on measurements in three clinical dimensions: leg function/ambulation (timed 25-foot walk), arm function (9 hole peg test) and cognitive function (paced auditory serial addition test). Scores on component measures are converted to standard scores (Z-scores), that reflect patient performance. This method has not yet been introduced into routine clinical practice. Patients and methods - Since March 2000 over the five years period the MSFC calculation was applied to 17 patients with relapsing-remitting multiple sclerosis (age mean: 37.4±10.8 years, duration of the disease: 5.5±4.9 years, EDSS: 2.7±1.4) seen at the neuroimmunological outpatient clinic to evaluate its usefulness and its correlation with the traditionally applied expanded disability status scale (EDSS). Thirteen patients received immunomodulatory treatment (interferon beta and glatiramer acetate), one patient received immunosuppressant therapy (azathioprine), and there was a patient, to whom developed secondary - progressive phase and we changed the interferon treatment to mitoxantrone. MSFC and EDSS were measured at 3., 6., 9, 12., 18 and 60 months of follow-up. Results - The prospective study confirmed a strong correlation between EDSS and MSFC in multiple sclerosis. The MSFC was more sensitive to clinical changes than EDSS. Our results after 18 months of follow up are already published. After five years arm/hand function and leg function/ambulation were the most sensitive mesures for disease progression. In contrast with the literature we did not experience correlation with cognitive changes. Consequences - We demonstrated strong correlation between MSFC and EDSS after a longer period. MSFC is a simple method, suitable for follow-up of multiple sclerosis patients in everyday clinical practice.]

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Clinical Neuroscience

[THORACIC MENINGOCELE]

FEKETE Tamás Fülöp, VERES Róbert, NYÁRY István

[Herniation of the meninges through a defect of the spinal canal is a spinal meningocele, and is usually located dorsally in the lumbosacral region. Meningoceles are usually part of a complex developmental disorder, or of a systemic disease, or it can be iatrogenic, as well. We report a very rare case of a true anterior thoracic meningocele.]

Clinical Neuroscience

[Introduction]

VÉCSEI László

Clinical Neuroscience

[THE MODULATORY EFFECT OF ESTROGEN ON THE CAUDAL TRIGEMINAL NUCLEUS OF THE RAT IN AN ANIMAL MODEL OF MIGRAINE]

VARGA Hedvig, PÁRDUTZ Árpád, TAJTI János, VÉCSEI László, JEAN Schoenen

[Migraine is one of the most common neurological disorder affecting up to 14% of the population. The disease shows sexual dimorphism, thus gonadal steroids may play an important role in its patophysiology. One model of migraine headache is the systemic administration of nitric oxide (NO) donor nitroglycerin (NTG), which triggers a delayed attack without aura in many migraine patients but not in healthy volunteers. NTG is also able to activate the neurons of the caudal trigeminal nucleus in the rat. In our review we summarise the effect of NTG on the expression of some molecules, in the superficial laminae of the spinal portion of trigeminal nucleus caudalis, which play an important role in the pathomechanism of headaches, and the modulatory effect of chronic estradiol treatment. Our data show that NTG was able to modify all the examined substances in the caudal trigeminal nucleus, while chronic estradiol treatment abolished this effect. These data may help to understand the mechanisms by which estrogens influence trigeminal nociception and how nitric oxide triggers migraine attacks.]

Clinical Neuroscience

[ANIMAL MODELS OF HUNTINGTON’S DISEASE]

GÁRDIÁN Gabriella

[Huntington’s disease is an autosomal dominantly inherited progressive neurodegenerative disorder. The main symptoms are choreiform, involuntary movements, personality changes and dementia. Huntington’s disease is a member of a group of diseases caused by CAG repeat expansions. One research aim is to determine the earliest molecular changes associated with Huntington’s disease. There is no possibility for this in humans, but various early changes have been identified in an animal model of Huntington’s disease. They are constructed by excitotoxin causing striatal lesion, or mitochondrial toxins inducing energy impairment, or by generating transgenic mice.]

Clinical Neuroscience

[CLINICAL ANALYSIS OF PATIENTS WITH PERIPHERAL FACIAL PALSY]

ILNICZKY Sándor

[symptoms. In two thirds of the cases the cause is unknown, this is called “idiopathic peripheral facial palsy or Bell’s palsy”, but several different diseases have to be considered in the differential diagnosis. In this paper we reviewed the case histories of 110 patients treated for “peripheral facial palsy” in the Department of Neurology, Semmelweis University, Budapest in a five year period, 2000-2004. We studied the age, gender distribution, seasonal occurance, comorbidities, sidedness, symptoms, circumstances of referral to the hospital, the initial diagnoses and therapeutic options. We also discuss the probable causes and consequences of diagnostic failures. Results: the proportion of males and females was equal. There was no considerable difference between sexes regarding agedistribution. Of the 110 patients 106 was diagnosed with idiopathic Bell’s palsy, three cases with otic herpes zoster and one patient with Lyme disease. In our material, peripheral facial palsy was significantly more frequent in the cold period of late autumn, winter, and early spring. Diabetes mellitus and hypertension were more frequent than in the general population. 74% of the patients were admitted within two days from the onset of the symptoms. In 37% preliminary diagnosis was unavailable. In 15% cerebrovascular insult was the first, incorrect diagnosis, the correct diagnosis of “Bell’s palsy” was provided only in 16%. The probable causes of diagnostic failures may be the misleading symptoms and accompanying conditions. We examined the different therapies applied and reviewed the literature in this topic. We conclude that intravenous corticosteroid treatment in the early stage of the disease is the therapy of choice.]

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Clinical Neuroscience

[Health status and costs of ambulatory patients with multiple sclerosis in Hungary]

PÉNTEK Márta, GULÁCSI László, RÓZSA Csilla, SIMÓ Magdolna, ILJICSOV Anna, KOMOLY Sámuel, BRODSZKY Valentin

[Background and purpose - Data on disease burden of multiple sclerosis from Eastern-Central Europe are very limited. Our aim was to explore the quality of life, resource utilisation and costs of ambulating patients with multiple sclerosis in Hungary. Methods - Cross-sectional questionnaire survey was performed in two outpatient neurology centres in 2009. Clinical history, health care utilisation in the past 12 months were surveyed, the Expanded Disability Status Scale and the EQ-5D questionnaires were applied. Cost calculation was conducted from the societal perspective. Results - Sixty-eight patients (female 70.6%) aged 38.0 (SD 9.1) with disease duration of 7.8 (SD 6.7) years were involved. Fifty-five (80.9%) had relapsing-remitting form and 52 (76.5%) were taking immunomodulatory drug. The average scores were: Expanded Disability Status Scale 1.9 (SD 1.7), EQ-5D 0.67 (SD 0.28). Mean total cost amounted to 10 902 Euros/patient/year (direct medical 67%, direct nonmedical 13%, indirect costs 20%). Drugs, disability pension and informal care were the highest cost items. Costs of mild (Expanded Disability Status Scale 0-3.5) and moderate (Expanded Disability Status Scale 4.0-6.5) disease were 9 218 and 17 634 Euros/patient/year respectively (p<0.01), that is lower than results from Western European countries. Conclusion - Our study provides current inputs for policy making and contributes to understanding variation of costof- illness of multiple sclerosis in Europe.]

Clinical Neuroscience

[Family planning in multiple sclerosis: conception, pregnancy, breastfeeding]

RÓZSA Csilla

[Family planning is an exceptionally important question in multiple sclerosis, as women of childbearing age are the ones most often affected. Although it is proven that pregnancy does not worsen the long-term prognosis of relapsing-remitting multiple sclerosis, many patients are still doubtful about having children. This question is further complicated by the fact that patients – and often even doctors – are not sufficiently informed about how the ever-increasing number of available disease-modifying treatments affect pregnancies. Breastfeeding is an even less clear topic. Patients usually look to their neurologists first for answers concerning these matters. It falls to the neurologist to rationally evaluate the risks and benefits of contraception, pregnancy, assisted reproduction, childbirth, breastfeeding and disease modifying treatments, to inform patients about these, and then together come to a decision about the best possible therapeutic approach, taking the patients’ individual family plans into consideration. Here we present a review of relevant literature adhering to international guidelines on the topics of conception, pregnancy and breastfeeding, with a special focus on the applicability of approved disease modifying treatments during pregnancy and breastfeeding. The goal of this article is to provide clinicians involved in the care of MS patients with up-to-date information that they can utilize in their day-to-day clinical practice. ]

Clinical Neuroscience

Matrix metalloproteinases and their tissue inhibitors in relapsing remitting multiple sclerosis: Possible markers and treatment agents

SANLI Arzu, OZTURK Musa, SOYSAL Aysun, DOVENTAS Yasemin, BASOGLU Fulya, GOZUBATIK-CELIK R. Gokcen, BAYBAS Sevim

Matrix metalloproteinases (MMPs), which are synthesized by many cell groups and responsible for the destruction of matrix proteins, and endogen tissue inhibitors of MMPs (TIMPs) have a role in the pathogenesis of Multiple Sclerosis (MS) by affecting the blood-brain barrier. We aimed to investigate the role of MMPs and TIMPs in the immunopathogenesis and in the course of multiple sclerosis (MS). We enrolled 25 relapsing remitting MS patients, who had a definite MS diagnosis according to McDonald criteria and 25 healthy subjects similar for age and gender as control group. MMP-9- and TIMP-1 levels were measured twice in patient group (one time during an attack and one in remission) and once in healthy subjects. MMP-9- and TIMP-levels of patients during attack and remission period and MMP-9/TIMP-1 ratio were found significantly higher than in the control subjects. In patient group MMP-9 and TIMP-1 levels and MMP-9/TIMP-1 ratio during attacks were not significantly different than during remission period. However, when subdivided according to their number of attacks, patients with 2 attacks had significantly higher levels during attack period comparing to remission period (p<0.05); in case of patients with more than 2 attacks did not have a statistically significant difference in attack and remission periods. Matrix metalloproteinases are important actors in MS immunopathogenesis, particularly in the early period and inhibitor agents for these enzymes can be used as a treatment option.

Clinical Neuroscience

[MR imaging of acute disseminated encephalomyelitis and multiple sclerosis in children. A review (in English language)]

PATAY Zoltán

[Inflammatory diseases of the central nervous system (CNS) are relatively rare in children, but their relevance to public health is considerable due to frequent and significant long term morbidity and even mortality. As in adults, acute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS) and their variants are the most common entities in this group of pathologies in the pediatric patient population. Recent efforts have focused on establishing standardized diagnostic criteria schemes to facilitate the diagnosis and differential diagnosis of these diseases, however especially with multiple sclerosis those have not been fully validated yet for disease occurring in children. In recent decades the role of MRI has been constantly increasing in the diagnostic work-up of suspected inflammatory diseases of the CNS as well as in the follow-up of patients with confirmed disease. Currently, MRI is the first-line diagnostic imaging modality in ADEM and MS and is fully integrated in the most widely used diagnostic criteria schemes, but it has a key role in clinical therapeutic research trials as well. This paper provides an update on the current concepts and strategies of MRI in inflammatory diseases of the CNS, as well as a review of the imaging semiology of the various disease entities and variants with emphasis on clinical and imaging particularities relevant to the pediatric patient population.]

Clinical Neuroscience

The effects of the level of spinal cord injury on life satisfaction and disability

GULSAH Karatas, NESLIHAN Metli, ELIF Yalcin, RAMAZAN Gündüz, FATIH Karatas, MÜFIT Akyuz

Introduction - Spinal Cord Injury (SCI) may often lead to significant disability in affected individuals and reduce life satisfaction. Herein we aimed to investigate the effects of the level of injury on disability and life satisfaction as well as the relation between life satisfaction and disability. Methods - Patients with at least one-year history of SCI were included. Demographic-clinical data of patients were recorded. The Craig Handicap Assessment and Reporting Technique-Short Form (CHART-SF) was used for quantifying the degree of patients’ disability. Life satisfaction was assessed by the Satisfaction with Life Scale (SWLS). Results - Of the 76 patients, 21 (27.6%) were tetraplegic and 55 (72.4%) were paraplegic. SWLS was found to be similar in tetraplegic vs. paraplegic patients (P=0.59), whereas CHART parameters such as physical independence, mobility, occupation, and total CHART value were significantly higher in paraplegic patients (P=0.04, P=0.04, P=0.001 and P=0.01, respectively). Social integration was found similarly high in both groups. There was a positive correlation between elapsed time after the injury and CHART physical independence, occupation and the level of economic sufficiency (P<0.01, P<0.01, P=0.01). Excluding the economic sufficiency (P=0.02), there was not any other association between the rest of CHART parameters and SWLS. Conclusions - According to our findings, although the level of injury seems to be influential on disability, it seems to have no significant effect on life satisfaction. Since the only thing that positively affects life satisfaction is economic sufficiency, more emphasis should be placed on regulations that increase the return to work in patients.