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

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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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[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. ]

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Clinical Neuroscience

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Clinical Neuroscience

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

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[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.]