Abstract
In this chapter, we will review the epidemiology, clinical phenotype, genetics, causes, and management of amyotrophic lateral sclerosis (ALS). ALS is a devastating and invariably fatal adult-onset neurodegenerative disorder. It remains incurable, killing about 1,200 people in the UK each year. It has an annual incidence of 1-2 per 100,000, a figure that appears to be consistent in most populations studied around the world. ALS is also known as “Lou Gehrig’s disease” in the USA and motor neuron disease (MND) in the UK.
| Original language | English |
|---|---|
| Title of host publication | Neuroscience in the 21st Century: From Basic to Clinical, Second Edition |
| Publisher | Springer New York |
| Pages | 3799-3841 |
| Number of pages | 43 |
| ISBN (Electronic) | 9781493934744 |
| ISBN (Print) | 9781493934737 |
| DOIs | |
| Publication status | Published - 1 Jan 2016 |
Keywords
- Alsin
- Amyotrophic lateral sclerosis (ALS)
- ANG
- Apoptosis
- Axonal transport failure
- DAO
- DCTN1
- El escorial research diagnostic criteria
- Electrical impedance myography (EIM)
- Electromyography
- Excitotoxicity and calcium homeostasis
- FIG4
- Flail leg syndrome
- FUS/TLS
- Genome-wide association studies (GWAS)
- Glial cells and non-cell autonomy
- Lower motor neurons (LMNs)
- Mitochondrial dysfunction and apoptosis
- Motor neuron diseases
- Motor unit number estimation (MUNE)
- Nerve conduction studies (NCS)
- Neurofilamentous aggregates
- Neurogenic abnormities
- OPTN
- Paraoxonase enzymes
- Peripherin
- Primary lateral sclerosis (PLS)
- Progressive muscular atrophy (PMA)
- Protein aggregation
- Reactive oxygen species (ROS)
- RNA processing and transcriptional regulation
- SETX
- SOD1
- SPG11
- TARDBP
- TDP-43
- Transcranial magnetic stimulation (TMS)
- VAPB
- Vulpian-Bernhardt syndrome