Clinical Neuroscience

[Diagnosis of multiple sclerosis: A review of the 2017 revisions of the McDonald criteria]


SEPTEMBER 30, 2018

Clinical Neuroscience - 2018;71(09-10)


[The revolutionary progress of research in neuroimmu­nology has led to the introduction of disease modifying therapies in multiple sclerosis at the end of the last century. The International Panel on Diagnosis of Multiple Sclerosis originally proposed the 2001 McDonald criteria to facilitate the diagnosis of MS in patients with the first objective neurological symptom(s) suggesting demyelinating event, when magnetic resonance imaging is integrated with clinical and other paraclinical diagnostic methods. New terms have been introduced to substitute clinical information by MRI: dissemination in space - indicating a multifocal central demyelinating process and dissemination in time - indicating the development of new CNS lesions over time. The criteria for diagnosis of Multiple Sclerosis have continuously evolved, they were modified in 2005 and 2010 allowing for an earlier and more accurate diagnosis of MS over time, and they provided the most up-to-date guidance for clinicians and researchers. The last recommended revisions relied entirely on available evidence, and not on expert opinion thereby reducing the risk of the misdiagnosis. The 2017 McDonald criteria continue to apply primarily to patients experiencing a typical, clinically isolated syndrome. In this review, we provide an overview of the recent 2017 revisions to the criteria of dissemination in space and time with the importance of the presence of CSF-specific oligoclonal bands; keeping fully in mind that there is no better explanation for symptoms than diagnosis of MS. In the future, validation of the 2017 McDonald criteria will be needed in diverse populations. Further investigations are required on the value of new MRI approaches, on optic nerve involvement, on evoked potential and optical coherence tomography, in order to assess their possible contribution to diagnostic criteria.]



Further articles in this publication

Clinical Neuroscience

A rare condition mimicking stroke: Diabetic uremic encephalopathy

TEKESIN Aysel, ERDAL Yuksel, MAHMUTOGLU Soydan Abdullah, HAKYEMEZ Ahmet, EMRE Ufuk

Uremic encephalopathy (UE) is a metabolic disorder associated with acute or chronic renal failure. It is characterized by the acute or subacute onset of reversible neurological symptoms and specific imaging findings. It is uncommon for uremic encephalopathy to be associated with acute bilateral lesions of the basal ganglia in diabetic uremic patients, and this can be seen most often in Asian patients. Here, we report a patient with diabetic uremic encephalopathy and bilateral basal ganglia lesions who developed acute onset dysarthria. The clinical and magnetic resonance brain imaging findings resolved after hemodialysis treatment.

Clinical Neuroscience

[Minimally invasive, extrapleural-anterolateral approach to the spine]

SZABÓ Viktor, BÜKI András, DÓCZI Tamás, SCHWARCZ Attila

[In spine surgery, minimally invasive approaches (MIS) are getting accepted and more popular worldwide during the last decades. It is due to the reduced intraoperative blood loss, decreased infection rate, less postoperative pain and earlier discharge from hospital compared to traditional approaches. The present paper puts forward a minimally invasive extrapleural approach to the thoracic spine that is not applied in Hungary. This new approach, in contrast to the standard costotransversectomy, provides direct visual control over the ventral surface of the dural sac. Furthermore, contrary to the transthoracic way, following minimally invasive extrapleural surgery thoracic drainage and intensive care are not necessary. The approach can be applied safely in treatment of ventral or ventrolateral pathologies of the thoracic spine. ]

Clinical Neuroscience

[The evaluation of paroxysmal events in neonates and infants]

NAGY Eszter, FARKAS Nelli, HOLLÓDY Katalin

[Introduction - Differential diagnosis of neonatal and infantile seizures based only on inspection poses a challenge even for specialists. Aims - To investigate the evaluations of neonatal and infantile paroxysmal events based only on inspection. Research question - Is there any difference in the opinion of neonatologists, paediatric neurologists and neurologists about the assessment of common paroxysmal events in infancy? Patients and methods - Video recordings about paroxysmal movements of 15 neonates or infants (aged 2 days- 5 months) were displayed for 47 paediatric neurologists, 35 neonatologists and nurses working in Neonatal or Perinatal Intensive Care Units and 43 neurologists. They had to decide without knowing the past medical history or EEG results whether events presented were epileptic or nonepileptic in nature. Results - Answers of neonatologists and paediatric neurologists were correct in 67% of cases (824/1230), no significant difference was found between their results. The largest uncertainty was in the judgement of discrete hand movements and very rapid clonus with epileptic origin, they were judged correctly by only one third of participants. The result of neurologists was only slightly, but not significantly different from that of paediatric neurologists. Conclusion - In most cases, the correct diagnosis of neonatal and infantile paroxysmal events requires video-EEG recording. No significant difference was revealed between the evaluation of neonatologists and paediatric neurologists about the differential diagnosis of movements. The ongoing cooperation of paediatric neurologists and neurologists going back to several decades facilitates the shaping of a common perspective.]

Clinical Neuroscience

A multidisciplinary clinical approach to facioscapulohumeral muscular dystrophy

CAKMAK Öztop Özgür, EREN Ilker, ASLANGER Ayca, GÜNERBÜYÜK Caner, KAYSERILI Hülya, OFLAZER Piraye, SAR Cüneyt, DEMIRHAN Mehmet, ÖZDEMIR Gürsoy Yasemin

Background - Impaired shoulder function is the most disabling problem for daily life of Fascioscapulohumeral muscular dystrophy (FSHD) patients. Scapulothoracic arthrodesis can give a high impact to the functionality of patients. Here we report our experience with scapulothoracic arthrodesis and spinal stenosis surgery in FSHD patients. Patients and methods - 32 FSHD patients were collected between 2015-2016. Demographical and clinical features were documented. All the patients were neurologically examined. The Medical Research Council (MRC) and the FSHD evaluation scale was used to assess muscle involvement1. Scapulothoracic arthrodesis and spinal stenosis surgeries were performed in eligible patients. Results - There were 16 male and 16 female (mean age 34.4 years; range 12-73) patients. 6 shoulders of 4 patients aged between 2132 years underwent scapulothoracic arthrodesis (two bilateral, one left and one right sided). Only one 63 years old female patient with severe hyperlordosis had spinal fusion surgery. All of the patients undergoing these corrective surgeries have better functionality in daily life, as well as superior shoulder elevation. Conclusion - Until the emergence and clinical use of novel therapeutics, surgical interventions are indicated in carefully selected patients with FSHD to improve arm movements, the posture and the quality of life of patients in general. Scapulothorosic arthrodesis is a management with good clinical results and patient satisfaction. In selected cases other corrective orthopedic surgeries like spinal fusion may also be considered.

Clinical Neuroscience

Acute motor and sensory axonal neuropathy associated with Sjögren’s syndrome


Sjögren’s syndrome (SS) is an autoimmune disease with mononuclear cell infiltration and destruction of the lacrimal gland and salivary glands, which cause dryness of the eyes and mouth. The most common neurological condition seen in SS is peripheral neuropathy. Initial manifestation of SS as an acute fulminant peripheral neuropathy is extremely rare. We report a 42-year-old patient presenting with acute motor sensory-axonal neuropathy in the presence of SS. She showed partial response to intravenous immunoglobulin but favourable clinical improvement was seen after initiation of corticosteroid treatment.

All articles in the issue

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

RÓZSA Csilla

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Clinical Neuroscience

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Clinical Neuroscience

[Teriflunomide: new oral immunmodulant drug in therapy of multiple sclerosis]

BENCSIK Krisztina, RÓZSA Csilla, VÉCSEI László

[Multiple sclerosis (MS) is the autoimmune, demyelinating, neurodegenerative disorder of the central nervous system (CNS). There are nine drugs available in Hungary reimbursed by the National Health Insurance Fund of Hungary (OEP) to reduce the activity of the disease, from which seven can be used as first line therapies. We have approximately 20 years of experience with the interferon b-1a/1b and glatiramer-acetate products. Though in case of approximately 30% of the patients using one of the first line drugs, the disease remains active, that we call break-through disease. The reasons for break-through disease could be the insufficient adherence and compliance, the appearance of neutralizing antibodies or the high activity of the disease. One of the oral immunomodulating drugs for MS, teriflunomide, was registered in Europe in 2013. Because of the anti-proliferative and anti-inflammatory effect of teriflunomide, it can be used for the reduction of the disease activity in the relapsing-remitting course of MS. The effect of teriflunomide was proved in one Phase II. and four Phase III. (TEMSO, TOWER, TENERE, TOPIC) studies. Teriflunomide 14 mg once daily was able to demonstrate in two consecutive placebo-controlled phase 3 clinical trials that significantly reduces the relapse rate (31.5% and 36.3%) and in both studies significantly reduces the sustained disability progression (29.8% and 31.5%) moreover delays the appearance of the clinically definitive MS in patients with clinically isolated syndrome (CIS). According to the TENERE study there were no significant differences observed between teriflunomide 14 mg and IFNb-1a s.c. in time to failure and annualized relapse rate but the treatment satisfaction domains of global satisfaction, side-effects and convenience were significantly improved with teriflunomide compared with s.c. IFNb-1a. ]

Clinical Neuroscience

[Fingolimod therapy in multiple sclerosis - the issue of the pathomechanism]

TAR Lilla, VÉCSEI László

[Multiple sclerosis is an autoimmune inflammatory disease of the central nervous system with neurodegenerative chararacteristics. The newly discovered per os administrable drug fingolimod (FTY720) has a different mechanism of action than the current disease-modifying therapies. In vivo the drug binds to four out of the five sphingosine-1-phosphate receptors after phosphorylation. Fingolimod-phosphate (FTY720-P) causes internalization and degradation of the sphingosine-1-phosphate receptors in the membrane of lymphocytes thus in contrast to sphingosine-1-phosphate it acts like a functional antagonist. In experimental autoimmune encephalomyelitis - an animal model of multiple sclerosis - fingolimod blocks the sphingosine-1-phosphate gradient controlled lymphocyte egress from the lymph nodes and therefore reduces the peripheral lymphocyte count especially the encephalitogenic Th17 subset is reduced. Modulation of the sinus lining and blood-brainbarrier constructing endothelial cells also contributes to the complex mechanism of action. Additionally due to its liphohilic nature fingolimod is able to penetrate the blood brain barrier thus, beside its peripheral effects the drug can probably modulate the cells of the central nervous system directly. Presumably it can reduce neurodegeneration caused by astrogliosis through modification of astrocyte and oligodendrocyte activity. The results of current clinical studies are holding out with bright prospective in the aspect of either the favourable effects or the well tolerated side effects.]

Hungarian Immunology

[β-endorphin concentrations in the cerebrospinal fluid and serum in systemic lupus erythematosus and multiple sclerosis patients]

BARACZKA Krisztina, BENDER Tamás, BARNA István, GÉHER Pál

[INTRODUCTION - The aim of the present study was to investigate the cerebrospinal fluid and serum β- endorphin levels in several diseases characterized by central nervous system demyelinisation. PATIENTS AND METHODS - Ten patients with systemic lupus erythematosus complicated with demyelinating syndrome and ten patients with chronic progressive form of multiple sclerosis were selected. Concentrations of β-endorphin were measured using a high sensitive, specific radioimmunoassay. Statistical significance (Wilcoxon test, two variable t test) and correlations (Spearman and Pearson correlations coefficients) were calculated. RESULTS - β-endorphin concentration in the cerebrospinal fluid did not differ in multiple sclerosis and systemic lupus erythematosus patients compared to the controls.]