Correction to: Longitudinal multi-modal muscle-based biomarker assessment in motor neuron disease

Jenkins, Thomas M.; Alix, James J P; Fingret, Jacob; Esmail, Taniya; Hoggard, Nigel; Baster, Kathleen; McDermott, Christopher J.; Wilkinson, Iain D.; Shaw, Pamela J.

doi:10.1007/s00415-019-09648-8

Cited by 1 publication

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“…Upper limb onset is most commonly in the dominant hand [53], with thenar muscles being more affected than hypothenar muscles (which is referred to as the split‐hand syndrome) [54], with early involvement of the first interosseous muscle and finger extensors more affected than finger flexors [55]. In the lower limb the anterior tibial muscle is typically affected earlier in the disease course than the gastrocnemius muscle, the hamstrings before the quadriceps muscles [56].…”

Section: Clinical Featuresmentioning

confidence: 99%

Amyotrophic lateral sclerosis: a clinical review

Masrori

Damme

2020

Euro J of Neurology

627

503

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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 sporadic ALS. These expansions are also a frequent cause of frontotemporal dementia, emphasizing the molecular overlap between ALS and FTD. To this day there is no cure or effective treatment for ALS and the cornerstone of treatment remains multidisciplinary care, including nutritional and respiratory support and symptom management. In this review, different aspects of ALS are discussed, including epidemiology, aetiology, pathogenesis, clinical features, differential diagnosis, investigations, treatment and future prospects.

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Section: Clinical Featuresmentioning

confidence: 99%