MND-AMYOTROPHIC LATERAL SCLEROSIS (ALS)

SUMMARY

1. Progressive destruction of pyramidal cells (UMN) & anterior horn cells (LMN).

2. The ascending sensory tracts & nucleus of Onuf (controlling striated muscles of the pelvic floor, bowel & bladder sphincters) are spared.

3. Approximately 3-10% with ALS have frontotemporal dementia; 30-50% have cognitive impairment.

4. Presentation is varied with combination of UMN & LMN signs.

5. ALS is relentlessly progressive, involving upper and lower extremities, truncal and bulbar musculature, and is terminal typically within 3-5 years after diagnosis.

6. Differential: post-polio syndrome.

Reference(s)

Wilkinson, I., Furmedge, D. and Sinharay, R. (2017). Oxford handbook of clinical medicine. Oxford: Oxford University Press. Get it on Amazon.
Feather, A., Randall, D. and Waterhouse, M. (2020). Kumar And Clark’s Clinical Medicine. 10th ed. S.L.: Elsevier Health Sciences. Get it on Amazon.
Hannaman, R. A., Bullock, L., Hatchell, C. A., & Yoffe, M. (2016). Internal medicine review core curriculum, 2017-2018. CO Springs, CO: MedStudy.
Therapeutic Guidelines. Melbourne: Therapeutic Guidelines Limited. https://www.tg.org.au [Accessed 2021].