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This paper deals with thyroid disease that can occur after treatment with alemtuzumab (humanized monoclonal anti-CD52) for relapsing–remitting multiple sclerosis (MS). The 5-year incidence of thyroid adverse events in phase 3 clinical trials is up to 40.7%. In most cases, the thyroid dysfunction is mild and easily manageable and only few serious thyroid adverse events have been reported. The need for patient education on the risk of thyroid dysfunction, as well as regular clinical and biochemical thyroid function screening is well described. However, practical clinical guidance in case of abnormal thyroid-related findings prior to or after alemtuzumab treatment is currently lacking. Therefore, a Belgian taskforce consisting of MS and thyroid experts was created in 2016, with the objective of issuing a clinical thyroid management algorithm based on available scientific evidence and personal experience with regard to alemtuzumab treatment-related thyroid adverse events., SCOPUS: ar.j, info:eu-repo/semantics/published

To make assessment of neurocognitive decline in patients with brain metastases more reliable and feasible, Brainlab AG developed an application 'Cognition' for the iPad by gamifying validated paper and pencil tests. This study aims at validating the computerized tests. We assessed reliability and comparability of 'Cognition' with similar well-established paper and pencil tests in two consecutive sessions per participant. The electronic tests used the same assignments with different stimuli than the paper and pencil tests. Domains involved are learning and memory, attention and processing speed, verbal fluency and executive functions. In total 5 employees and 25 cancer patients without disease in the CNS participated, of whom 24 completed both sessions. Reliability was found satisfying for the domains learning and memory (p = 0.08; p = 0.612; p = 0.4445) and verbal fluency (p = 0.064). A learning effect showed for attention and processing speed (p = 0.001) while executive functioning showed a significant decline, possibly due to radiotherapy-related fatigue (p = 0.013). Concerning comparability between electronic and paper results, a significant correlation was found for attention and processing speed (p = 0.000), for verbal fluency (p = 0.03), for executive functions (p = 0.000), but not for learning and memory (p = 0.41; p = 0.25). Overall 'Cognition' showed moderate comparability, probably caused by the consecution of tests during sessions and the unfamiliarity with electronic test in older patients. After improving its functionality, the application needs to be validated in patients with brain metastases before it can detect cognitive decline and possible early radiation toxicity or relapses.

Spinal epidural angiolipomas are rare lesions composed of mature lipomatous and angiomatous elements. In this paper, the authors review a surgical series of ten patients with epidural angiolipomas. All patients had performed preoperative and postoperative magnetic resonance imaging. The diagnosis of angiolipoma was based on pathology. All the follow-up data were obtained during office visits. There were 5 males and 5 females with a mean age of 53.6 years. One tumor was located in the cervicothoracic, six in the thoracic, and three in the lumbar spine. The most common symptom was progressive motor deficit. Gross total resection of the tumor was achieved in nine cases, and subtotal resection was obtained in one case. No recurrence or regrowth of the residual tumor was observed during a follow-up period of 50.8 months. At the last follow-up, 90% of patients experienced improvement in the neurological function. Epidural angiolipomas are benign but clinically progressive lesions. Early surgery should be performed to prevent irreversible neurological deficits. Postoperative radiotherapy is not recommended. The risk of long-term recurrence/regrowth of the lesions is low, and a good clinical outcome after total or subtotal removal can be expected.

Primary Sjögren's syndrome (pSS) is a chronic autoimmune disorder characterized by lymphocytic infiltrates of the exocrine glands, particularly the salivary and lacrimal glands, resulting in oral and ocular dryness. pSS has been linked to various neurological manifestations, including cerebellar dysfunction. We aimed to provide a comprehensive analysis of the currently available evidence regarding pSS-related cerebellar ataxia.A systematic literature search in the PubMed database was performed and 19 papers were eligible to be included in this paper.The pooled prevalence of cerebellar ataxia in pSS is estimated to be 1.5% (95% CI 0.3-6.8%). pSS patients with cerebellar involvement have a female-to-male ratio of 6:1. Although most of the patients are adults in their fifth decade of life when diagnosed, cases of children with pSS and cerebellar involvement have been reported. Typical cerebellar ataxia related to pSS manifests with vermian dysfunction, namely gait ataxia and/or cerebellar speech. Cerebellar ataxia due to pSS may also mimic degenerative cerebellar ataxia, especially when the onset is progressive.The diagnostic approach to a patient with cerebellar ataxia of unknown etiology should include evaluation for an underlying pSS. A thorough history and clinical examination, antibody testing, brain MRI imaging and/or MRS of the cerebellum will assist in establishing the diagnosis. Setting up a joint neuro-rheumatology clinic can be valuable given that rheumatic and neurological diseases share comorbidities.

Patients with refractory epilepsy can suffer from psychological and physical problems. Epilepsy can also be associated with menstruation abnormalities in women. In this paper, we aim to survey women with focal refractory epilepsy who also suffer from menstrual abnormalities. This cross-sectional study was performed in 2018 in Tertiary Epilepsy Center in Kashani Hospital, Isfahan, Iran and Milad hospital, Isfahan, Iran. A total number of 381 patients diagnosed with refractory epilepsy joined the study based on an inclusion and exclusion criteria. Clinical characteristics of epilepsy, menstrual issues and brain MRI findings were assessed. Among 381 women with refractory epilepsy, 250 had refractory focal epilepsy (65.61%), and the others (34.39%) had generalized refractory epilepsy. 132 patients out of 381 had menstrual abnormalities (34.65%). Among 132 women with coexisting refractory epilepsy and menstrual abnormalities, 86 (65.15%) were diagnosed with refractory focal epilepsy, and 46 (34.86%) had generalized refractory epilepsy (p value = 0.041). Oligomenorrhea–hypomenorrhea was the most common type of menstrual abnormality in women with refractory epilepsy seen in 83 (62.87%) (p value = 0.039). The most common brain MRI pathology in women with refractory focal epilepsy was mesial temporal sclerosis (MTS) (39.20%, p = 0.00). We report that almost one-third of women with refractory epilepsy suffer from menstrual disorders, and the most common disorder was Oligomenorrhea–hypomenorrhea, and the most common brain pathology was MTS. It has also been a hypothesis that reproductive dysfunctions are connected to temporal lobe malfunctions, and more specific studies are required in this issue.

Hypoxia as one of the most common clinical disturbances in pregnancy period can cause destructive changes in motor sensory cortex and can lead to imperfect organization in motor reactions. Crocin, a water-soluble carotenoid, is the most active ingredients of saffron and a lot of studies declare its positive effectiveness on improving motor activity. Since the hypoxia intensity affects its malicious amount on movement, in this paper, we have studied the effect of crocin in three maternal hypoxia protocols with different oxygen intensities on motor activity and balance in rat offspring. In this experiment, female rats (Wistar) were used on the 20th day of pregnancy. The rats were randomly divided into eight experimental groups: sham, crocin, hypoxia with three different intensities: 10% oxygen and 90% nitrogen for 1 h (hypoxia-ɪ), 7% oxygen and 93% nitrogen for 1 h (hypoxia-ɪɪ), 7% oxygen and 93% nitrogen for 3 h (hypoxia-ɪɪɪ) and treated-crocin hypoxia groups. To produce hypoxia, pregnant rats were placed in a hypoxia box. In crocin group, rat offspring received 30 mg/kg crocin via IP injection at P14–28. Control group also received saline injection at the same time. Finally, balance and motor activity in offspring were measured respectively by rotarod and open-field devices. Results showed that motor activity significantly decreased in hypoxia-ɪɪɪ group as compared with sham group (p

While the influence of deep brain stimulation (DBS) of the subthalamic nucleus (STN) on the comprehension of pragmatic language in Parkinson's disease (PD) has been the focus of studies, its impact on production, however, has yet to be elucidated. (1) Investigating the inf luence of DBS STN on pragmatic language production in spontaneous speech by comparing different stimulation conditions and (2) evaluating the effect of asymmetric dopaminergic denervation. This paper included 18 patients with advanced idiopathic PD with STN DBS. [Ten PD patients with predominantly left hemispheric dopamine denervation (PD-left) and eight PD patients with predominantly right-hemispheric dopamine denervation (PD-right).] The pragmatic components 'communicative functions' and 'conversational skills' were evaluated by analysing the spontaneous language production in four stimulation conditions. STN stimulation did not appear to influence the pragmatic production skills. Only when asymmetric dopamine depletion was taken into account the parameter 'giving an explanation' interaction was detectable. STN DBS appears to have some influence on the production of pragmatic language depending on asymmetric dopaminergic denervation. Suggestions are made for further research of pragmatic production in Parkinson's disease.

The aim of this study was to analyse the frequency of genotypes and alleles of single-nucleotide polymorphism (SNP)-G2677T/A (rs2032582) of MDR1 gene and to investigate the significance this genetic variation exerts on drug-resistant epilepsy (DRE) in the Polish adult population. The study comprised 340 patients treated for DRE and 240 patients treated for drug-responsive epilepsy. Three hundred and sixty disease-free individuals were used as controls. Genomic DNA was isolated, and the SNP G2677T/A of MDR1 was determined by High-Resolution Melter technique. Odds ratios (ORs) and 95% confidence intervals (CIs) were calculated for each genotype and allele. In this paper, we have demonstrated that A allele of SNP G2677T/A of MDR1 may reduce the risk of DRE (OR 0.44; 95% CI 0.33-0.58, p 0.0001), whereas allele G itself may be a risk factor for this disease. No differences were found in the distribution of the genotypes and alleles in the studied groups, depending on sex as well as on concomitant diseases (p 0.05). G2677T/A polymorphism of MDR1 may play a significant role in the development of DRE in the Polish patients.

The aim of this study is to present a diagnostic and therapeutic approach in patients with LETM. In a period between June 2008 and June 2010, all patients who fulfilled criteria for LETM were included in the study. All patients underwent a standardized protocol of investigations presented in this paper. Ten patients were included (5 male, 5 female, with the age distribution from 24 to 70 years). Four patients were diagnosed with NMO/spatially limited NMO spectrum disorder, three patients were diagnosed with spinal cord ADEM, two multiple sclerosis (MS) and one patient with copper deficiency myelopathy. Laboratory support for the diagnosis of NMO was positive NMO-IgG antibody ; for the diagnosis of ADEM signs of peripheral nervous system involvement on electromyoneurography ; and for the diagnosis of MS brain MRI lesions typical for MS, as well as positive oligoclonal bands (OCB) in the cerebrospinal fluid (CSF). All cases with inflammatory myelopathy were treated either with steroids or plasma exchange and copper replacement was started in the case of copper deficiency. The mean time from the first symptom until the final diagnosis was 16.3 months (range 1 month to 7 years). As each of idiopathic inflammatory demyelinating diseases that can present with LETM have specific therapy, the postponement in making the correct diagnosis can lead to a poor recovery. In patients with LETM, a standardized diagnostic approach can result in a correct diagnosis and appropriate treatment.

In patients with drug resistant epilepsy it is accepted to try different lines of dual therapy before adding a third antiepileptic drug (AED). The usefulness of triple therapy is questionable since it is not likely to improve symptoms but it can increase adverse effects and drug interactions [1]. Despite this, in regular clinical practice it is still used in selected patients. Our paper approaches this controversy from a different angle: in our case series we analyze the outcome after withdrawal of one AED in patients with triple therapy. Our objective is to analyze the change in seizure frequency after reduction of antiepileptic polytherapy from three to two drugs (AEDs). We have analyzed in the database of our Outpatient Epilepsy Clinic the data

A decade ago, stroke was the first leading cause of morbidity and mortality in Croatia. Nowadays, we record reduction in stroke incidence, as well as stroke consequences—invalidity and mortality. These are due to long-term planned actions in the field of public health as well as actions performed by professional organizations. Today, we can be satisfied with improvement in that field, but there are still things we can improve, at the first place improvement of the emergency medicine network due to Croatian-specific topographical characteristics to reduce onset-to- door time. In this paper, we evaluated results from 11 Croatian hospitals in the period 11/2005– 11/2012. To find out about the past and present state in applying thrombolytic therapy in Croatia and to plan further actions in light of new studies and efforts in Europe and in the world, all with the aim of improvement in stroke prevention and acute treatment resulting in reduction of stroke morbidity, mortality and symptomatic intracerebral hemorrhage as well as better functional outcome. Our results have shown that we improved stroke treatment in the last decade, but further actions should be performed to raise public stroke awareness and to improve emergency medicine network as well as in hospital protocols.

The prevalence of restless legs syndrome (RLS) is increased in gluten sensitive enteropathy (GSE); but prevalence of GSE is not known in RLS. 96 RLS patients and 97 healthy controls, both with or without iron deficiency were enrolled. All secondary RLS patients except iron deficiency were excluded. Subjects underwent a thorough biochemistry and routine blood analyses, and tissue transglutaminase antibodies (TTGA), endomysium antibodies (EMA) and gliadin antibodies (AGA) were also tested. In RLS patients positivity rates of all GSE antibodies were similar to those in controls. The rate of iron deficiency anaemia in RLS patients with at least one positive GSE antibody was significantly higher than that of RLS patients whose GSE antibodies were all negative. The prevalence of GSE antibodies in RLS patients is not increased. GSE might have a role in the aetiology of RLS in association with iron deficiency anaemia. Since the prevalence of GSE antibodies is not increased in RLS, it seems unlikely that GSE is involved in the aetiology of RLS through different mechanisms (e.g. immunological mechanisms) other than iron deficiency as proposed in some published papers.

Focal cortical dysplasia is a malformation caused by abnormalities of cortical development. It is characterized by no dysmorphic or ballon cells (type I), dysmorphic neurons witout or with ballon cells (type II). It is the main cause of pharmacoresistant epilepsy. The combination of clinical and neurophysiological findings provided by VEEG and MRI had lead to an improvement in the diagnosis and outcome of FCD. This paper describes our experience in the form of a retrospective study conducted on the patients affected by FCD who were treated in the Clinical Neurophysiology Service at University Hospital of Lille in France.

While the most common neurological disorder associated with Human T lymphotropic virus type-1 (HTLV-1) infection in the endemic areas is HTLV-1 associated myelopathy also know as tropical spastic paraparesis (HAM/TSP), other disorders such as optic neuropathy, peripheral neuropathy and cerebellar diseases have also been reported in patient with this infection. In this paper, we studied the prevalence of peripheral nerve involvement in patients with HAM/TSP.Seventy three patients diagnosed with HAM/TSP in accordance to criteria set by the World Health Organization (WHO) were evaluated in this cross-sectional study. Clinical and electrodiagnostic criteria were used for the diagnosis of peripheral neuropathy.Electrodiagnostic studies showed that 30.1% of patients with HAM/TSP had peripheral nerve involvement. All patients had predominantly axonal neuropathy with sensory-motor polyneuropathy being the most common neuropathy observed in our patients.Peripheral neuropathy may be more common than previously thought and should be checked systematically in all patients with HAM/TSP.

Several abnormal synkinetic eye movements during jaw movements may often be seen after trauma or congenitally in the Marcus Gunn jaw winking phenomenon. The most frequent type consists of unilateral ptosis and retraction of the ptotic lid upon moving the jaw. The authors describe a case with isolated simultaneous adduction of the left eye upon jaw movement. This paper presents a rare case of Marcus Gunn jaw winking with trigemino-oculomotor synkinesis.

Epilepsy is one of the most frequent neurological disorders. Despite the advances and improvements in treatment of seizure disorders, immunologic alterations related to anticonvulsant drugs have been described. The aim of this paper is to assess the effect of some antiepileptic drugs on serum immunoglobulin levels in epileptic patients. Seventy-one patients with epilepsy were included in the study. Participants were divided into three groups based on their treatment with carbamazepine (n=33), sodium valproate (n=22) or phenobarbital (n=16) as monotherapy. Three samples were taken from each patient and serum immunoglobulin levels were measured before treatment, 3 months and 6 months after therapy. Overall, eleven patients out of 71 (15.5%) had a decrease in at least one serum immunoglobulin level (more than 2SD below age-matched control). In the patients receiving carbamazepine, 8 patients (24.2%) showed significant decline in at least one immunoglobulin (3 cases in IgA and 5 cases in IgG). In the group of treated with sodium valproate, 2 patients showed significant decrease in serum IgA level. Results of the last group indicated a significant reduction in serum IgG concentration only in one patient. No patient at all showed significant decrease in serum IgM level. This study suggests that anti-epileptic drugs could reduce serum immunoglobulins, especially IgA and IgG; among them carbamazepine effect is of more concern.

The spinal cord, brain, optic nerves and peripheral nerves may be affected by vitamin B12 (cobalamin) deficiency. Deficiency of vitamin B12 also causes megaloblastic anaemia, meaning that the red blood cells are usually larger than normal. In this paper we report a 16-year old girl who was referred to us for the evaluation of mild paraparesis and paresthesias marked by tingling "pins and needles" feelings and general weakness. The patient, her parents and sisters were on a strict vegan diet, which made us believe that vitamin B12 deficiency may be the possible cause of the neurologic clinical manifestations. The serum level of vitamin B12 was low, but there was no macrocytosis in the routine blood examination. The electrophoresis of haemoglobin was pathologic, there was 3.7% of HbA2 and 11.6% of HbF (heterozygous form of beta-thalassaemia). When megaloblastic anaemia occurs in combination with a condition that gives rise to microcytic anaemia, many megaloblastic features may be masked. Instead of being macrocytic, the anaemia could be normocytic or even microcytic. Vitamin B12 deficiency is a diagnosis that must not be overlooked. This case report turns the light on the fact that increased MCV is a hallmark in vitamin B12 deficiency, but it is not an obligatory sign.

Approximately 20% of people with epilepsy are of childbearing potential and about 3 to 5 births per thousand will be to women with epilepsy. Both epilepsy and antiepileptic drugs can cause specific problems in women and embryos (less than 8 weeks of gestational age) or foetuses (more than 8 weeks of gestational age). The aim of this paper is to discuss therapeutic issues for the management of women with epilepsy: initiation of antiepileptic therapy, contraception, pregnancy, breast feeding and menopause. Some fertility issues are also discussed.

Vagus nerve stimulation (VNS) is an established treatment for patients with medically refractory epilepsy who are unsuitable candidates for conventional epilepsy surgery. VNS requires an initial financial investment but apart from our own previous study there are no reports on cost-benefit published to date. The purpose of this paper is to assess prospectively the cost-benefit ratio of VNS in a series of patients with long term follow-up.Our experience with VNS comprises 25 patients of whom 20 with sufficient follow-up will be further discussed. These 20 patients have a mean post-implantation follow-up of 26 months (range: 6-50 months). Mean age was 30 years (range: 12-45 years); mean duration of epilepsy 17 years (range: 5-35 years). We prospectively assessed seizure frequency, prescribed AEDs, number of hospital admission days and side effects and calculated the epilepsy related direct medical cost and compared this with pre-implantation data.Mean seizure frequency decreased from 14 seizures/month (range: 2-40) to 9 seizures/month (range: 0-30) (p = 0.0003). The mean yearly epilepsy related direct medical costs per patient dropped from 6,682 USD (range: 829-21,888 USD) to 3,635 USD (range: 684-12,486 USD) (p = 0.0046). The mean number of hospital admission days was reduced from 16 days/year (range: 0-60) to 4 days/year (range: 0-30) (p = 0.0029).VNS is an efficacious and cost-beneficial treatment for refractory partial seizures.

The purpose of this paper is to demonstrate the diagnostic efficacy and therapeutic relevance of video-EEG monitoring in an large patient population with long-term follow-up.Between October 1990 and May 1997, 400 patients were monitored at the Epilepsy Monitoring Unit (EMU) of the University Hospital in Gent. In all patients, the following parameters were retrospectively examined: reason for referral, tentative diagnosis, prescribed antiepileptic drugs (AEDs), seizure frequency, number of admission days, number of recorded seizures, ictal and interictal EEG, clinical and electroencephalographic diagnosis following the monitoring session. During follow-up visits at the Epilepsy Clinic, we prospectively collected data on different types of treatment and post-monitoring seizure control.255/400 (64%) patients were referred for refractory epilepsy. 145/400 (36%) patients were evaluated for attacks of uncertain origin. Mean follow-up, available in 225 patients, was 28 months (range: 6-80 months). Mean duration of a single monitoring session was 4 days (range: 2-7 days). Prolonged interictal EEG was recorded in all patients and ictal EEG in 258 (65%) patients. Following the monitoring session, the diagnosis of epilepsy was confirmed in 217 patients. Pseudoseizures were diagnosed in 31 patients (8%). AEDs were started in 19 patients, stopped in 6 and left unchanged in 110. The type and/or number of AEDs was changed in 111 patients. Sixty patients underwent epilepsy surgery. In 48 surgery patients, follow-up data were available, 29 of whom became seizure-free, and 16 of whom experienced a greater than 90% seizure reduction. Vagus nerve stimulation was performed in 11 patients, 2 became seizure-free, and 7 improved markedly. Of the non-invasively treated patients in whom follow-up was available (n = 135), 70 became seizure-free or experienced a greater than 50% reduction in seizure frequency; 51 patients experienced no change in seizure frequency. Outcome was unrelated to the availability of ictal video-EEG recording. In patients with complex partial seizures, seizure control was significantly improved when a well-defined ictal onset zone could be defined during video-EEG monitoring.Prolonged interictal EEG monitoring is mandatory in the successful management of patients with refractory epilepsy. Ictal video-EEG monitoring is very helpful but not indispensable, except in patients enrolled for presurgical evaluation or suspected of having pseudoseizures.

This paper evaluates the laboratory and environmental factors that induce seizures in photosensitive epileptic patients. The aims are (i) to identify those factors that trigger seizures so that the patients could take preventive measures, (ii) to advise the patients on appropriate preventive measures that could be taken to avoid spontaneous seizures. Thirty-six photosensitive epileptic patients were studied. The electroencephalographic laboratory seizure-inducing factors identified were intermittent photic stimulation (IPS), flickering lights from both the TV and the visual display unit (VDU) at various flashes per second, grid patterns (vertical, horizontal lines, squares, black and white lines) of various cycles per degree, and blank screens. In the environmental factor investigation, 10 out of 36 patients had series of seizures induced by narrow stripes of black and white, or white and red striped coloured dresses especially, when stepping outdoors on sunny days. Other environmental factors identified include moving escalators; 'moving' trees, bushes, and houses when travelling in a vehicle, including trains; faceted patterns in the bathroom windows with light passing through; swirling light patterns especially on displayed objects of black and white colours; telegraphic post, gantry post, and luminance variance when stepping outdoors from the dark in bright sunlight. The effective preventive measures were: (i) patients should occlude one eye when travelling in a vehicle or using computers or when stepping outdoors on a sunny day, or when there are various patterns before them, (ii) patients should sit at least 3 metres away from the TV when watching a programme, (iii) they should avoid any object that transmits luminance variance; if this is not possible, they should occlude one eye.

The paper gives a brief historical survey of craniostenosis and the first operative approaches. The authors describe a classification of various types of cranio-facio-stenosis and facio-cranio-stenosis and present a detailed study of their experience in this area. Operative results are discussed in function of the selected surgical procedure, the immediate per- and post-operative complications and the short and long term morbidity. The authors present their current attitude for each dysmorphic type. They underline the heavy nature of this long-lasting and bleeding surgical procedure that concerns small children. They have recorded 31 incidents in relation with surgical procedure, 10 complications at short and long term, and 1 death. Such surgery requires an experienced and vigilant team, associating surgeons, anesthetists and intensive-care physicians.

In two separate papers submitted to a symposium in Barcelona in December 1987 on primary, generalized grand mal epilepsy becoming manifest in adulthood, the findings were so identical that the authors thought it appropriate to make a joint publication. L. Oller Daurella, after studying 90 cases, and L. Sorel, after studying 80 cases, propose the definition of a particular form of benign grand mal epilepsy in the adult. The various characteristics of this new entity are described.