Amyotrophic lateral sclerosis natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]
Overview
Natural History, Complications, and Prognosis
Natural History
- The symptoms of Amyotrophic lateral sclerosis usually develop in the fifth decade of life.[1][2]
- Only 5% of the cases have an onset <30 years of age.[1][2]
Complications
- With the progression of ALS, patients develop the distinctive feature of a combination of upper motor and LMN degeneration signs within the same CNS region.
- Common complications of ALS include:
- Pulmonary complications:
- Dyspnea
- Orthopnea
- Hypoventilation
- Pneumonia
- The main cause of death in ALS is respiratory failure as the result of pulmonary complications.
- Cognitive dysfunction
- Weight loss, which is an indicative of a poor prognosis
- Pulmonary complications:
Prognosis
- Prognosis is generally poor, and the 30 months survival rate of patients withALS is approximately 5%.[3]
- Only 20% of the patients survive between 5 and 10 years after symptoms onset.[3]
- Depending on the extent of the [tumor/disease progression] at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor/good/excellent.
- The presence of [characteristic of disease] is associated with a particularly [good/poor] prognosis among patients with [disease/malignancy].
- [Subtype of disease/malignancy] is associated with the most favorable prognosis.
- The prognosis varies with the [characteristic] of tumor; [subtype of disease/malignancy] have the most favorable prognosis.
. .[285] Reduced survival to the disease is related to the older age of symptom onset, early respiratory muscle dysfunction, and bulbar onset disease. On the other hand, limb-onset disease, younger age at presentation of the disease and longer diagnostic delay are independent predictors of prolonged survival.[312]
Some ALS subtypes vary according to prognosis. LMN form of ALS, which includes flail-limb variant and PMA, shows a slower progression than other forms of ALS.[285,312] A prognosis of 2–4 years is seen in the pure bulbar palsy phenotype, which usually affects women older than 65 years of age. In this type of ALS, the disease remains localized to the oropharyngeal musculature and UMN features predominate.[285]
References
- ↑ 1.0 1.1 Logroscino G, Traynor BJ, Hardiman O, Chio' A, Couratier P, Mitchell JD; et al. (2008). "Descriptive epidemiology of amyotrophic lateral sclerosis: new evidence and unsolved issues". J Neurol Neurosurg Psychiatry. 79 (1): 6–11. doi:10.1136/jnnp.2006.104828. PMID 18079297.
- ↑ 2.0 2.1 Zarei S, Carr K, Reiley L, Diaz K, Guerra O, Altamirano PF; et al. (2015). "A comprehensive review of amyotrophic lateral sclerosis". Surg Neurol Int. 6: 171. doi:10.4103/2152-7806.169561. PMC 4653353. PMID 26629397.
- ↑ 3.0 3.1 Talbot K (2009). "Motor neuron disease: the bare essentials". Pract Neurol. 9 (5): 303–9. doi:10.1136/jnnp.2009.188151. PMID 19762894.