Amyotrophic lateral sclerosis: a clinical review.

European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies
P Masrori, P Van Damme

Abstract

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder affecting primarily the motor system, but in which extra-motor manifestations are increasingly recognized. The loss of upper and lower motor neurons in the motor cortex, the brain stem nuclei and the anterior horn of the spinal cord gives rise to progressive muscle weakness and wasting. ALS often has a focal onset but subsequently spreads to different body regions, where failure of respiratory muscles typically limits survival to 2-5 years after disease onset. In up to 50% of cases, there are extra-motor manifestations such as changes in behaviour, executive dysfunction and language problems. In 10%-15% of patients, these problems are severe enough to meet the clinical criteria of frontotemporal dementia (FTD). In 10% of ALS patients, the family history suggests an autosomal dominant inheritance pattern. The remaining 90% have no affected family members and are classified as sporadic ALS. The causes of ALS appear to be heterogeneous and are only partially understood. To date, more than 20 genes have been associated with ALS. The most common genetic cause is a hexanucleotide repeat expansion in the C9orf72 gene, responsible for 30%-50% of familial ALS and 7% of ...Continue Reading

References

Apr 1, 1992·Brain : a Journal of Neurology·C E PringleG C Ebers
Mar 3, 1994·The New England Journal of Medicine·G BensimonV Meininger
Jan 14, 2000·Archives of Neurology·B J TraynorO Hardiman
Jul 24, 2001·Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders : Official Publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases·B R BrooksUNKNOWN World Federation of Neurology Research Group on Motor Neuron Diseases
Aug 28, 2001·Brain : a Journal of Neurology·J ParviziA R Damasio
Aug 1, 2006·The Journal of Clinical Investigation·Richard A SmithDon W Cleveland
Jan 16, 2007·Journal of Neurology·Clare A JohnstonAmmar Al-Chalabi
Jun 22, 2007·Journal of the Neurological Sciences·Letizia MazziniFranca Fagioli
Oct 20, 2007·Lancet Neurology·Julie PhukanOrla Hardiman
Jan 1, 2008·Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology·Mamede de CarvalhoMichael Swash
Mar 1, 2008·Science·Jemeen SreedharanChristopher E Shaw
Aug 28, 2009·Journal of Neurology, Neurosurgery, and Psychiatry·Giancarlo LogroscinoUNKNOWN EURALS
Mar 17, 2010·Amyotrophic Lateral Sclerosis : Official Publication of the World Federation of Neurology Research Group on Motor Neuron Diseases·Zita R ManjalyAmmar Al-Chalabi
Apr 29, 2010·The FEBS Journal·Emanuele BurattiFrancisco Baralle
Apr 30, 2010·Nature·Hirofumi MaruyamaHideshi Kawakami
May 14, 2010·Journal of Neurology, Neurosurgery, and Psychiatry·Kerry R Mills
Jun 22, 2010·Journal of Neurology, Neurosurgery, and Psychiatry·M R TurnerA Al-Chalabi
Sep 24, 2010·Journal of Neurology, Neurosurgery, and Psychiatry·A Al-ChalabiF Rijsdijk
Sep 25, 2010·Lancet Neurology·Ian Ra MackenzieManuela Neumann
Dec 15, 2010·Neuron·Janel O JohnsonBryan J Traynor
Mar 16, 2011·Journal of Neurology, Neurosurgery, and Psychiatry·Adriano ChiòUNKNOWN PARALS study group
Mar 26, 2011·Annals of Neurology·Maarten SchrootenPhilip Van Damme
Aug 16, 2011·Brain : a Journal of Neurology·James R BurrellJohn R Hodges
Sep 15, 2011·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies· Markus Weber
Oct 13, 2011·Nature Reviews. Neurology·Orla HardimanMatthew C Kiernan
Nov 16, 2011·Archives of Neurology·Faisal FectoTeepu Siddique
Nov 29, 2011·Neurobiology of Aging·Frank P DiekstraJan H Veldink
May 31, 2012·Human Molecular Genetics·Marka van BlitterswijkLeonard H van den Berg
Oct 16, 2013·Nature Reviews. Neurology·Ammar Al-Chalabi, Orla Hardiman
Aug 15, 2014·Neurology·Marco PaganiAngelina Cistaro

❮ Previous
Next ❯

Citations

Aug 21, 2020·Medicinal Research Reviews·Michail S KukharskyVladimir L Buchman
Jan 4, 2021·Annals of Neurology·Matthieu MoisseUNKNOWN Project MinE Sequencing Consortium
Dec 15, 2020·Respiratory Medicine·Giulia Michela PellegrinoFranco Laghi
Dec 18, 2020·Frontiers in Neurology·Michael J StrongKathryn Volkening
Oct 30, 2020·International Journal of Molecular Sciences·Giada CipollinaMarco Peviani
Feb 7, 2021·Diagnostics·Ivana Štětkářová, Edvard Ehler
Mar 27, 2021·Journal of Neurology·Anna Bartoletti-StellaUNKNOWN BoReALS
Mar 22, 2021·Developmental Dynamics : an Official Publication of the American Association of Anatomists·Kathryn Russo, Kristi A Wharton
Apr 4, 2021·International Journal of Molecular Sciences·Isabel Lastres-BeckerÁngeles Martin-Requero
Apr 4, 2021·Diagnostics·Robert RusinaRose Bruffaerts
Apr 13, 2021·Frontiers in Cellular Neuroscience·Thomas Ernest James Phillips, Emily Maguire
Apr 20, 2021·Expert Opinion on Drug Discovery·Kayla Farrell, Timothy J Jarome
May 1, 2021·Diagnostics·Andrea BarpValeria Ada Sansone
Apr 27, 2021·Frontiers in Aging Neuroscience·Chao HuXiao-Ping Dong
Jun 3, 2021·Antioxidants·Matan SollZeev Gross
Jun 28, 2021·Current Opinion in Chemical Biology·Stuart P MoonMatthew R Pratt
Jul 13, 2021·Frontiers in Molecular Neuroscience·Agnes Molnar-KaszaBirgit Hutter-Paier
Jul 3, 2021·International Journal of Molecular Sciences·Elena ObradorJosé M Estrela
Jul 9, 2021·Frontiers in Neuroscience·Federico VerdeVincenzo Silani
Jul 8, 2021·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·Hatice TankisiHugh Bostock
Jul 21, 2021·Acta Neuropathologica Communications·Alyssa N Coyne, Jeffrey D Rothstein
Jul 21, 2021·Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology·Miguel Oliveira SantosMamede de Carvalho
Jul 30, 2021·Cellular and Molecular Life Sciences : CMLS·Francesco LiguoriCinzia Volonté
Aug 8, 2021·International Journal of Molecular Sciences·Mengmeng JinAndreas Hermann
Aug 7, 2021·Journal of Personalized Medicine·Oihane Pikatza-MenoioSonia Alonso-Martín
Apr 30, 2021·G3 : Genes - Genomes - Genetics·Marko MelnickMercedes Prudencio
Aug 28, 2021·Biomedicines·Elena ObradorJosé M Estrela
Jun 27, 2021·Brain : a Journal of Neurology·Michael ZechFelix Distelmaier
Sep 28, 2021·Frontiers in Cardiovascular Medicine·Ana Catarina Pronto-LaborinhoFilomena A Carvalho
May 15, 2021·Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics·Giulia FisconPaola Paci
Nov 28, 2021·Experimental & Molecular Medicine·Byeong Eun LeeJae-Ick Kim

❮ Previous
Next ❯

Methods Mentioned

BETA
ubiquitination
antisense oligonucleotides

Related Concepts

Related Feeds

ALS: Genetics

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterized by muscle weakness. Here is the latest research investigating genetic alterations in this genetically heterogeneous disorder.

ALS: Genetics

Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disorder characterized by muscle weakness. ALS is a genetically heterogeneous disorder with several causative genes. Here are the latest discoveries pertaining to the genetics of this disease.

Epidemiology of Aging

This feed focuses on epidemiology of aging and aging-related conditions, including Alzheimer’s disease, dementia, and age-associated cognitive impairment. Here is the latest research.

Amyloid Lateral Sclerosis

Amyotrophic Lateral Sclerosis (ALS) is a progressive nervous system disease associated with the death of neurons that control voluntary muscles. Discover the latest research on ALS here.

Related Papers

Nederlands tijdschrift voor geneeskunde
Michael A van EsL H van den Berg
Dementia and Geriatric Cognitive Disorders
Catherine Lomen-Hoerth
Current Opinion in Neurology
Mamede de Carvalho, M Swash
International Journal of Molecular Sciences
Francesca TrojsiGioacchino Tedeschi
© 2022 Meta ULC. All rights reserved