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Acta Neurologica Scandinavica

Fabio Fierini, Anna Poggesi, Emilia Salvadori, Manlio Acquafresca, Enrico Fainardi, Marco Moretti, Leonardo Pantoni
OBJECTIVES: To investigate clinical and demographic characteristics of patients with intracranial arterial dolichoectasia (IADE) and describe the possible coexistence of cerebral small vessel disease (SVD) and systemic arteriopathy. MATERIAL AND METHODS: From January 2015 to March 2016, all the patients attending an out-patient service for chronic cerebrovascular diseases were screened for suspected IADE. Identified patients underwent a predefined protocol including: brain MR angiography for the diagnosis of IADE; brain MRI with visual rating of SVD features; whole-body CT angiography to assess signs of systemic arteriopathy; neuropsychological examination...
October 18, 2018: Acta Neurologica Scandinavica
Deepa Dash, Sanjay Pandey
Movement disorders are one of the common clinical feature of neurological disease associated with neuronal antibodies. which is a group of potentially reversible disorder. They can present with hypokinetic or hyperkinetic types of involuntary movements and may have other associated neurological symptoms. The spectrum of abnormal movements associated with neuronal antibodies is widening. Some specific phenomenology of movement disorders are likely to give clue about the type of antibody, for instance presence of paroxysmal dystonia (facio-brachial dystonic seizures) are a pointer towards presence of LGI-1 antibodies, and orofacial lingual dyskinesia is associated with NMDAR associated encephalitis...
October 18, 2018: Acta Neurologica Scandinavica
Trygve Holmøy, Egil Røsjø, Henrik Zetterberg, Kaj Blennow, Jonas Christoffer Lindstrøm, Linn Hofsøy Steffensen, Margitta T Kampman
OBJECTIVES: The effect of vitamin D supplementation on the disease course of multiple sclerosis (MS) is not established. Neurofilament light chain (NFL) is a sensitive marker of axonal degeneration. The aim of this study was to establish whether high-dose vitamin D supplementation reduces serum levels of NFL. MATERIALS AND METHODS: We have performed a 96 weeks placebo-controlled randomized study of weekly supplementation with 20.000 IU vitamin D3 in 71 patients with relapsing remitting MS (RRMS)...
October 13, 2018: Acta Neurologica Scandinavica
Josef Finsterer
With interest we read the article by Hovsepian et al. about a 46 years-old female with MELAS syndrome due to the common variant m.3243A>G in the tRNA(Leu) gene [1]. The patient experienced a stroke-like episode (SLE), which was treated with L-arginine (0.5g/kg/d) intravenously during 7 days, followed by oral administration thereafter. Therapy with L-arginine not only resulted in clinical improvement but also in improvement of cerebral imaging and the NAA/Cho ratio and cerebral lactate levels on MR-spectroscopy (MRS) [1]...
October 9, 2018: Acta Neurologica Scandinavica
Josef Finsterer, Stephan Iglseder, Julia Wanschitz, Raffi Topakian, Wolfgang N Löscher, Wolfgang Grisold
Hereditary transthyretin(TTR)-related amyloidosis (ATTRm amyloidosis) is an endemic/non-endemic, autosomal-dominant, early- and late-onset, rare, progressive disorder, predominantly manifesting as length-dependent, small-fiber dominant, axonal polyneuropathy and frequently associated with cardiac disorders and other multisystem diseases. ATTRm amyloidosis is due to variants in the TTR gene, with the substitution Val30Met as the most frequent mutation. TTR mutations lead to destabilisation and dissociation of TTR tetramers into variant TTR monomers, and formation of amyloid fibrils, which are consecutively deposited extra-cellularly in various tissues, such as nerves, heart, brain, eyes, intestines, kidneys...
October 8, 2018: Acta Neurologica Scandinavica
Vlasta Vukovic Cvetkovic, Rigmor Højland Jensen
Small subsets of patients who fail to respond to pharmacological treatment may benefit from alternative treatment methods. In the last decade, neurostimulation is being explored as a potential treatment option for the patients with chronic, severely disabling refractory primary headaches. To alleviate pain, specific nerves and brain areas have been stimulated, and various methods have been explored: deep brain stimulation, occipital nerve stimulation and sphenopalatine ganglion stimulation are among the more invasive ones, whereas transcranial magnetic stimulation, supraorbital nerve stimulation are non-invasive...
October 6, 2018: Acta Neurologica Scandinavica
Mariana Lunardi, Katia Lin, Roger Walz, Peter Wolf
OBJECTIVES: To investigate the significance of "subtherapeutic" versus "therapeutic" antiepileptic drug (AED) plasma levels with respect to treatment adherence. MATERIAL & METHODS: 170 patients with refractory temporal lobe epilepsy who underwent video-EEG monitoring in view of a surgical indication had their AEDs (carbamazepine, phenobarbital, phenytoin and valproate) rapidly withdrawn following a standardized schedule. Plasma levels were measured at admission, and during the two days of drug withdrawal...
September 26, 2018: Acta Neurologica Scandinavica
Ofira Zloto, Aharon Lubetsky, Iris Ben-Bassat Mizrachi, Anat Kesler, Peter A Quiros, Ruth Huna-Baron
OBJECTIVES: To examine the clinical characteristics and prognosis of cerebral venous sinus thrombosis (CVST) that presented as pseudotumor cerebri (PTC) patients with JAK2V617F mutation. METHODS: Medical records of all consecutive patients that presented with PTC and a JAK2V617F mutation who were treated were retrospectively reviewed. Data regarding demographics and ocular presenting symptoms and signs, neurological signs, hematological factors treatment and prognosis were collected...
September 25, 2018: Acta Neurologica Scandinavica
Carrai Riccardo, Scarpino Maenia, Lolli Francesco, Spalletti Maddalena, Lanzo Giovanni, Peris Adriano, Lazzeri Chiara, Amantini Aldo, Grippo Antonello
OBJECTIVES: The bilateral absence of cortical Somatosensory Evoked Potentials(SEPs), after cardiac arrest(CA), is a high reliable predictor of poor outcome but it is present in no more than 40% of patients. An amplitude reduction of cortical SEPs was found in about 30% of subjects but few papers analysed its prognostic significance. The aim of our study is to identify a value of SEP amplitude reduction below which all the CA patients had poor outcome and the relationship between SEP and Electroencephalogram(EEG) patterns...
September 19, 2018: Acta Neurologica Scandinavica
Rodolfo Savica, Thomas G Beach, Joseph G Hentz, Marwan N Sabbagh, Geidy E Serrano, Lucia I Sue, Brittany N Dugger, Holly A Shill, Erika Driver-Dunckley, John N Caviness, Shyamal H Mehta, Sandra A Jacobson, Christine M Belden, Kathryn J Davis, Edward Zamrini, David R Shprecher, Charles H Adler
OBJECTIVE: Identify clinical features predictive of Lewy body pathology in AD patients in an ongoing longitudinal clinicopathologic study. MATERIAL & METHODS: We queried the Arizona Study of Aging and Neurodegenerative Disorders (AZSAND) database for dementia cases with AD pathology (1997-2015). Subjects received longitudinal comprehensive clinical evaluations including motor/neuropsychological assessment and Apo-E4 genotyping. All cases were autopsied and had standard neuropathological assessments for AD and Lewy-type synucleinopathy (LTS)...
September 19, 2018: Acta Neurologica Scandinavica
Halvor Naess, Lars Thomassen, Ulrike Waje-Andreassen, Solveig Glad, Christopher E Kvistad
BACKGROUND AND PURPOSE: We aimed to evaluate factors associated with neurological worsening among patients with lacunar or non-lacunar infarction admitted within 3 hours and between 3 and 24 hours after stroke onset. METHODS: All patients admitted to Haukeland university hospital between 2006 and 2016 with acute cerebral infarction on MRI and admission within 24 hours were included. Repeated National Institute of Health Stroke Scale (NIHSS) scoring was performed in all patients whenever possible...
September 19, 2018: Acta Neurologica Scandinavica
Dominic A Hovsepian, Alexandra Galati, Robert A Chong, Rajarshi Mazumder, Christopher M DeGiorgio, Shri Mishra, Catherine Yim
BACKGROUND: To assess the utility of Magnetic Resonance Spectroscopy (MRS) as a biomarker of response to L-arginine in mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS). AIMS: To describe a case of MELAS treated with L-arginine that showed improvement clinically and on serial MRS. METHODS: MRS was performed on a 1.5-Tesla scanner to evaluate a MELAS patient before, during, and after intravenous (IV) L-arginine therapy for the treatment of stroke-like episodes...
September 14, 2018: Acta Neurologica Scandinavica
Margret Jona Einarsdottir, Elias Olafsson, Helga Agusta Sigurjonsdottir
OBJECTIVE: Studies in children have shown an increased frequency of central hypothyroidism (CH) with long-term use of antiepileptic drugs (AEDs). The aim of this study was to search for CH in adults treated with AEDs and find whether the type of AEDs used matters. MATERIALS AND METHODS: Adult epileptic patients treated at the neurology outpatient clinic at Landspitali University Hospital (LSH) from 1998 to 2011 were included. Patients were invited for a blood test if serum levels for TSH (s-TSH) or free-T4 (s-fT4 ) had not already been obtained...
September 8, 2018: Acta Neurologica Scandinavica
Simona Lattanzi, Gaetano Zaccara, Fabio Giovannelli, Elisabetta Grillo, Raffaele Nardone, Mauro Silvestrini, Eugen Trinka, Francesco Brigo
Second and third generation AEDs have been directly compared to controlled-release carbamazepine (CBZ-CR) as initial monotherapy for new-onset focal epilepsy. Conversely, no head-to-head trials have been performed. The aim of this study was to estimate the comparative efficacy and tolerability of the antiepileptic monotherapies in adults with newly diagnosed focal epilepsy through a network meta-analysis (NMA). Randomized, double-blinded, parallel group, monotherapy studies comparing any AED to CBZ-CR in adults with newly diagnosed untreated epilepsy with focal-onset seizures was identified...
September 8, 2018: Acta Neurologica Scandinavica
Johannes Finnsson, Mark Lubberink, Irina Savitcheva, David Fällmar, Atle Melberg, Eva Kumlien, Raili Raininko
OBJECTIVE: LMNB1-related autosomal dominant leukodystrophy is caused by an overexpression of the protein lamin B1, usually due to a duplication of the LMNB1 gene. Symptoms start in 5th to 6th decade. This slowly progressive disease terminates with death. We studied brain glucose metabolism in this disease using 18 F-fluorodeoxyglucose positron emission tomography (PET). METHODS: We examined 8 patients, aged 48-64 years, in varying stages of clinical symptomatology...
September 7, 2018: Acta Neurologica Scandinavica
Juan F Vázquez-Costa, Sara Carratalà-Boscà, José I Tembl, Victoria Fornés-Ferrer, Jordi Pérez-Tur, Luis Martí-Bonmatí, Teresa Sevilla
OBJECTIVES: An enlarged width of the third ventricle (WTV) has been described in amyotrophic lateral sclerosis (ALS) patients, although its clinical meaning is unknown. The aims of this study were to evaluate the contribution of demographical, clinical and genetic factors to the WTV in different motor neuron disease (MND) phenotypes and to assess its brain structural correlates. MATERIALS AND METHODS: The WTV was measured by transcranial ultrasound in 107 MND patients (82 diagnosed with classical ALS, 16 with progressive muscular atrophy and 9 with primary lateral sclerosis) and 25 controls...
September 5, 2018: Acta Neurologica Scandinavica
Ilias Thomas, Mevludin Memedi, Jerker Westin, Dag Nyholm
OBJECTIVE: The aim of this retrospective study was to investigate whether patients with Parkinson's disease, who are treated with levodopa-carbidopa intestinal gel (LCIG), clinically worsen during the afternoon hours and if so, to evaluate whether this occurs in all LCIG-treated patients or in a subgroup of patients. METHODS: Three published studies were identified and included in the analysis. All studies provided individual response data assessed on the treatment response scale (TRS), and patients were treated with continuous LCIG...
September 4, 2018: Acta Neurologica Scandinavica
Martin Holtkamp, Norman Delanty, Francisco Sales, Jose Serratosa, Rob McMurray, Vicente Villanueva
OBJECTIVES: To assess the effectiveness and safety/tolerability of eslicarbazepine acetate (ESL) monotherapy in clinical practice in Europe. MATERIALS AND METHODS: Euro-Esli was a pooled analysis of 14 European clinical practice studies. Responder rate (≥50% seizure frequency reduction) and seizure freedom rate (seizure freedom at least since prior visit) were assessed after 3, 6 and 12 months of ESL treatment and at last visit. Adverse events (AEs) and AEs leading to ESL discontinuation were assessed throughout follow-up...
September 3, 2018: Acta Neurologica Scandinavica
Elisabet Westerberg, Anne-Marie Landtblom, Anna Rostedt Punga
PURPOSE: To evaluate disease-specific differences between Myasthenia Gravis (MG) subgroups and compare patterns of lifestyle between MG patients and population controls. METHODS: All MG patients (n = 70) in Jönköping County, Sweden, were invited to answer a disease-specific questionnaire, containing questions about disease-specific data, lifestyle, comorbidity, and mental fatigue. The patients were clinically evaluated. Four hundred age- and gender-matched population controls were invited to answer the nondisease-specific part of the questionnaire...
August 28, 2018: Acta Neurologica Scandinavica
Lukas Holmegaard, Christer Jensen, Petra Redfors, Christian Blomstrand, Christina Jern, Katarina Jood
OBJECTIVES: Studies on long-term progression of white matter hyperintensities (WMH) after ischemic stroke are scarce. Here, we sought to investigate this progression and its predictors in a cohort presenting with ischemic stroke before 70 years of age. MATERIALS AND METHODS: Participants in the Sahlgrenska Academy Study on Ischemic Stroke who underwent magnetic resonance imaging (MRI) of the brain at index stroke were examined by MRI again after 7 years (n = 188, mean age 53 years at index stroke, 35% females)...
August 28, 2018: Acta Neurologica Scandinavica
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