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Alemtuzumab (Lemtrada) is a humanized monoclonal antibody indicated for the treatment of adult patients with relapsing-remitting multiple sclerosis with active disease defined by clinical or imaging features. Alemtuzumab demonstrated superior efficacy over active comparator in both treatment naive patients and those with inadequate response to prior therapy. Alemtuzumab is associated with a consistent and manageable safety and tolerability profile. Treatment with alemtuzumab for multiple sclerosis increases the risk for autoimmune adverse events including immune thrombocytopenia (ITP). Complete blood counts with differential should be obtained prior to initiation of treatment and at monthly intervals thereafter for 48 months after the last infusion. After this period of time, testing should be performed based on clinical findings suggestive of ITP. If ITP onset is confirmed, appropriate medical intervention should be promptly initiated, including immediate referral to a specialist. This paper presents the consensus of Belgian multiple sclerosis specialists and hematologists to guide the treating physician with practical recommendations. ispartof: Acta Neurologica Belgica vol:118 issue:1 pages:7-11 ispartof: location:Italy status: published

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

Objective: Due to the growing number of older people with dementia (PWD) worldwide, there is an urgent need to improve undergraduate medical education on dementia and skills of future health professionals to provide the best quality care for PWD. The study aimed to determine attitudes and knowledge regarding dementia among medical students. Methods: This cross-sectional, survey- based study included 231 final-year medical students who completed the paper-and-pencil questionnaire with seven categories of questions including two dementia knowledge tests. Results: Students have relatively neutral attitudes towards working with PWD and a rather poor theoretical knowledge in the dementia knowledge exam. Students showed the best theoretical knowledge about the symptoms of Alzheimer's disease, while their knowledge was poor in the field of differential diagnoses, epidemiology, and pathogenesis of Alzheimer's disease. One in 11 sixth-year medical students has never had contact with a person with dementia. The opinion of most students was that they should have spent more time with PWD during their medical education and that there are too few classes in the field of dementia at the School of Medicine. The dementia knowledge test with 23 Multiple- Choice Questions showed a higher internal consistency compared to the Alzheimer's Disease Knowledge Scale. Conclusion: There is an urgent need to increase the interest and improve attitudes and knowledge towards dementia for all future health care professionals from the earliest student days to provide the best care for PWD.

The aim of this paper is to analyse the pathological background of very early Guillain-Barré (VEGBS) (≤ 4 days after onset) comparing it with initial stages of experimental autoimmune neuritis (EAN). The pathological hallmark of VEGBS is inflammatory oedema predominating in proximal nerve trunks. In EAN inflammatory oedema precedes the development of demyelination or axonal degeneration; such oedema may increase endoneurial fluid pressure (EFP) stretching the perineurium and constricting the transperineurial microcirculation. Centrofascicular or wedge-shaped areas of nerve ischemia have been reported in GBS and EAN. Additional support for proximal VEGBS pathology comes from electrophysiology showing alterations in late responses as the most frequent features, and ultrasonography illustrating that main changes rely on ventral rami of spinal nerves. Selective inefficiency of the blood-nerve barrier would explain the topography of changes in VEGBS. Increased serum neurofilament light chain concentration has recently been reported in VEGBS, with no difference between demyelinating and axonal subtypes. This is a marker of axonal damage, which could be correlated with endoneurial ischemia caused by increased EFP. Inflammatory oedema of proximal nerve trunks may be pathogenic in VEGBS, and consequently there is a pressing need for therapeutic strategies to stop its rapid impact on the axons.

Joob and Wiwanitkit [1] raised two points regarding our paper on neurocysticercosis in Western Europe [2]. First, they considered that there should be comparative data to support our suggestions that the prevalence of neurocysticercosis in Western Europe may be on the rise. This was based on the comparison of reported cases over the past four decades. As clearly stated on our paper, the number of publications and patients with neurocysticercosis steadily increased from 1970 to 2011. Of the 779 patients captured in our review, only 28 were published from 1970 to 1985, and the remaining 751 were reported from 1996 to date. Such an increase goes far beyond an enhanced interest of physicians for publishing neurocysticercosis cases, and clearly reflects an increase in its prevalence. Regarding the second topic raised by Joob and Wiwanitkit, the authors must have been confused. Taeniasis is a food-borne disease acquired by eating undercooked pork meat infested by cysticerci. In contrast, cysticercosis is mostly transmitted from person-to-person [3, 4]. It would be wise to review the life cycle of Taenia solium—clearly described more than one century ago—before commenting on this point, as it may create confusion among readers who are not familiar with the disease complex taeniasis/ cysticercosis. Humans become infected by cysticerci after ingesting Taenia eggs from a Taenia carrier.

Recent discovery of nusinersen, an antisense oligonucleotide drug, has provided encouragement for improving treatment of spinal muscular atrophy. No therapeutic options currently exist for this autosomal recessive motor neuron disorder. Nusinersen is developed for intrathecal use and binds to a specific sequence within the survival motor neuron 2 pre-messenger RNA, modifying the splicing process to promote expression of full-length survival motor neuron protein. We performed a MEDLINE and CENTRAL search to investigate the current evidence for treatment with nusinersen in patients with spinal muscular atrophy. Four papers were withheld, including two phase-3 randomized controlled trials, one phase-2 open-label clinical trial and one phase-1 open-label clinical trial. Outcome measures concerned improvement in motor function and milestones, as well as event-free survival and survival. Results of these trials are hopeful with significant and clinically meaningful improvement due to treatment with intrathecal nusinersen in patients with early- and later-onset spinal muscular atrophy, although this does not restore age-appropriate function. Intrathecal nusinersen has acceptable safety and tolerability. Further trials regarding long-term effects and safety aspects as well as trials including broader spinal muscular atrophy and age categories are required and ongoing.

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.

This paper proposes therapeutic guidelines for the management of some epileptic syndromes in infants, children, and adolescents, based on available medical literature and clinical practice in the French Community of Belgium. The guidelines address both epileptic encephalopathies (West syndrome, Lennox-Gastaut syndrome, and Dravet syndrome) and idiopathic epilepsies (typical absence seizures, epilepsy with centro-temporal spikes and juvenile myoclonic epilepsy).

In patients with Parkinson's disease with higher prevalence than in current population there appear pathological behaviours characterized by compulsion, repetitiveness and impulsivity, which are connected with material profit or pleasurable experience. They are, in particular, pathological gambling, hypersexuality, compulsive shopping and compulsive eating (in the literature they are collectively referred to as impulse control disorders). Pathological preoccupation with repeated mechanical activities (so-called punding) and excessive compulsive intake of dopaminergic medication (so-called dopamine dysregulation syndrome or also syndrome of hedonistic homeostatic dysregulation) are of similar nature. The paper treats briefly the risk factors and prevalence of these pathological behaviours. In current clinical practice, these psychiatric complications frequently escape doctors' attention, they are underdiagnosed. Although no generally valid recommendations for their therapy are currently available, they can be influenced medically. Of advantage can be modified dopaminergic medication (usually dose reduction ofdopaminergic agonists); multidisciplinary approach to the problem is appropriate. The pathological behaviours given above can frequently lead to considerable material losses and markedly aggravate patients' handicap in the social sphere; it can be expected that in the future they can become a problem also from the ethical and legal points of view.

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.

We read with interest the comments of Prof. Garraux on our recent case report describing a 71-year-old woman presenting with right-sided hemidystonia following an ischemic stroke. The initial title of our paper was: ‘‘Hemidystonia caused by a frontoparietal cortical infarction’’. However, we now receive the comment that according to the published computed tomography axial images of the brain, the causal lesion is located in the postcentral gyrus of the left parietal lobe. Indeed, we must agree that the lesion shown in image 1A of the manuscript is located in the left postcentral gyrus of the parietal lobe. Compliance with ethical standards

Dear Readers, On behalf of the editorial team of the Acta Neurologica Belgica I wish you and your family all the best for 2013. 2013 will be our 2nd ‘‘Springer’’ year and should further increase the success and visibility of the journal. To give you a brief overview of some ANB’s life, I shall pinpoint some of the figures presented by our editorial manager Paola Teti during the editorial board meeting of December 11, 2012. Submissions increased to 431 in 2012, an absolute record for the ANB. Only 30 % were accepted for publication: 101 articles were printed in volume 112, but 63 articles were in addition already published online at the end of 2012. ANB benefits from the world’s leading internet scientific information portal, ‘‘SpringerLink’’, and from the numerous consortia agreements for online deals by Springer. As by December 2012, 3,275 full text downloads were performed for ANB articles and 4,701 tables of contents (ToC) alerts were sent. The increase in visibility has generated an important supplementary workload for the editors and I take this opportunity to sincerely thank my co-editors. However, one other crucial step in the success chain of the journal is the peer review process. This is why we are especially grateful to our reviewers who are listed below with the number of papers they handled. We hope that we can continue to count on you for the rest of volume 113,.. and beyond. Thank you!