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Motor neurone disease
Published in Ibrahim Natalwala, Ammar Natalwala, E Glucksman, MCQs in Neurology and Neurosurgery for Medical Students, 2022
Ibrahim Natalwala, Ammar Natalwala, E Glucksman
Patients with ALS display both upper and lower motor neurone signs. The patient in the scenario exhibits fasciculations, which is a lower motor neurone sign, and increased tone, which is an upper motor neurone sign. Another distinguishing feature of this disease process is the absence of sensory deficits.4 Riluzole has been proven to effectively lengthen survival rates by 2–3 months (by slowing down disease progression) when used for 18 months.5 It is a neuroprotective drug that inhibits the release of glutamate and blocks the effects of it on N-methyl-D-aspartate (NMDA) receptors, thereby preventing excito-toxicity-mediated damage to neurones.6
Neuroprotection and Repair after Spinal Cord Injury
Published in Jacques Corcos, Gilles Karsenty, Thomas Kessler, David Ginsberg, Essentials of the Adult Neurogenic Bladder, 2020
The results of recently completed early phase trials have provided the rationale for further study through randomized controlled human studies.7–9 Riluzole, a sodium channel blocker currently used in the treatment of amyotrophic lateral sclerosis, is thought to reduce both aberrant sodium channel activation and glutamate release following SCI.10 Clinical trial showed efficacy and safety of riluzole, particularly in patients with incomplete cervical injury.7,11,12
Central Nervous System Effects of Essential Oil Compounds
Published in K. Hüsnü Can Başer, Gerhard Buchbauer, Handbook of Essential Oils, 2020
Elaine Elisabetsky, Domingos S. Nunes
Though the symptoms of neurodegenerative disorders are treated with various drugs, few are genuine neuroprotectors. Riluzole, used in amyotrophic lateral sclerosis, is often used as a positive control for neuroprotection; it acts by inhibiting the release of glutamate and blocking postsynaptic glutamate receptors (NMDA and kainate).
Pharmacological management of secondary spinal cord injury
Published in Expert Opinion on Pharmacotherapy, 2021
Alice Baroncini, Nicola Maffulli, Jörg Eschweiler, Markus Tingart, Filippo Migliorini
Riluzole is a benzothiazole anti-epileptic drug, and is approved in the management of amyotrophic lateral sclerosis. This compound acts as sodium channel antagonist, inhibiting the continuous activation of sodium channels and thus preventing intracellular edema [21]. Furthermore, riluzole can reduce cytotoxicity by inhibiting the release of glutamate [21]. One phase I clinical trial investigated the pharmacokinetics and safety of riluzole in SCI with positive results [22]. Another phase I clinical trial showed a significant improvement of motor score in patients treated with riluzole, without any serious adverse events related to its use [23]. A stage II/III randomized clinical trial on the safety of a 14-days treatment with riluzole and on its effect on motor scores is ongoing (NCT01597518) [24], along with another stage II investigating the possible improvement of spasticity in patients with SCI (NCT02859792).
A Phase 2, Double-Blind, Randomized, Dose-Ranging Trial Of Reldesemtiv In Patients With ALS
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2021
Jeremy M. Shefner, Jinsy A. Andrews, Angela Genge, Carlayne Jackson, Noah Lechtzin, Timothy M. Miller, Bettina M. Cockroft, Lisa Meng, Jenny Wei, Andrew A. Wolff, Fady I. Malik, Cynthia Bodkin, Benjamin R. Brooks, James Caress, Annie Dionne, Dominic Fee, Stephen A. Goutman, Namita A. Goyal, Orla Hardiman, Ghazala Hayat, Terry Heiman-Patterson, Daragh Heitzman, Robert D. Henderson, Wendy Johnston, Chafic Karam, Matthew C. Kiernan, Stephen J. Kolb, Lawrence Korngut, Shafeeq Ladha, Genevieve Matte, Jesus S. Mora, Merrilee Needham, Bjorn Oskarsson, Gary L. Pattee, Erik P. Pioro, Michael Pulley, Dianna Quan, Kourosh Rezania, Kerri L. Schellenberg, David Schultz, Christen Shoesmith, Zachary Simmons, Jeffrey Statland, Shumaila Sultan, Andrea Swenson, Leonard H. Van Den Berg, Tuan Vu, Steve Vucic, Michael Weiss, Ashley Whyte-Rayson, James Wymer, Lorne Zinman, Stacy A. Rudnicki
This randomized, double-blind, multicentre, dose-ranging, placebo-controlled, phase 2b trial recruited patients from 65 clinical trial sites in the United States, Canada, Ireland, Spain, the Netherlands, and Australia. Patients were between 18 and 80 years of age, and diagnosed within 24 months with possible, laboratory-supported probable, probable, or definite ALS according to the revised El Escorial criteria (9). An upright SVC ≥60% predicted for age, height, sex, and ethnic group at screening was required for inclusion. Patients on riluzole must have taken it for ≥30 days prior to screening. Following protocol amendment 2 (August 10, 2017), patients on edaravone were eligible to enroll in the trial and must have completed ≥2 cycles prior to screening. Exclusion criteria included prior use of reldesemtiv or tirasemtiv or receipt of stem cell or gene therapy for ALS.
Progression of cognitive and behavioral disturbances in motor neuron diseases assessed using standard and computer-based batteries
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2021
Veronica Castelnovo, Elisa Canu, Nilo Riva, Barbara Poletti, Camilla Cividini, Andrea Fontana, Federica Solca, Vincenzo Silani, Massimo Filippi, Federica Agosta
The study was performed retrospectively in a sample of MND patients recruited between 2009 and 2017 at two centers in Milan, Italy (IRCCS San Raffaele Scientific Institute and IRCCS Istituto Auxologico Italiano). We initially selected patients who had undergone at least two visits (6 months apart) within 12 months, each including a clinical evaluation and a standard neuropsychological assessment (minimum dataset at follow up: Mini Mental State Examination [MMSE], Fluency tests and indices, Beck Depression Inventory [BDI]). Then, ALS, PLS and PMA groups were age- and education matched. Finally, the subgroup of MND patients who underwent also a computer-based battery (TAP) was identified. See Figure 1 for a schematic representation of the sample selection. Additional inclusion criteria were: native Italian-speaking; no significant respiratory failure; no significant medical illnesses or substance abuse that could interfere with cognitive functioning; no any major systemic, psychiatric, or (other) neurological illnesses; and no (other) causes of focal or diffuse brain damage, including cerebrovascular disease at conventional MRI scans. All patients were in treatment with Riluzole at study entry. Disease severity was assessed using the ALS Functional Rating Scale-revised (ALSFRS-R) (33).