Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2016 Mar 26;6(1):56-64.
doi: 10.5662/wjm.v6.i1.56.

Quo vadis motor neuron disease?

Rubika Balendra  1 , Rickie Patani  1
Affiliations
Review

Quo vadis motor neuron disease?

Rubika Balendra et al. World J Methodol. .

Abstract

Motor neuron disease (MND), also known as amyotrophic lateral sclerosis, is a relentlessly progressive neurodegenerative condition that is invariably fatal, usually within 3 to 5 years of diagnosis. The aetio-pathogenesis of MND remains unresolved and no effective treatments exist. The only Food and Drug Administration approved disease modifying therapy is riluzole, a glutamate antagonist, which prolongs survival by up to 3 mo. Current management is largely symptomatic/supportive. There is therefore a desperate and unmet clinical need for discovery of disease mechanisms to guide novel therapeutic strategy. In this review, we start by introducing the organizational anatomy of the motor system, before providing a clinical overview of its dysfunction specifically in MND. We then summarize insights gained from pathological, genetic and animal models and conclude by speculating on optimal strategies to drive the step change in discovery, which is so desperately needed in this arena.

Keywords: Amyotrophic lateral sclerosis; Disease models; Motor neuron disease; Neurodegeneration.

PubMed Disclaimer

Figures

Figure 1
Figure 1
The motor columns of the spinal cord. The LMCs innervate the muscles of the upper and lower limbs, the MMC innervates axial musculature and the HMC and PGC are in the thoracic spinal cord and innervate the intercostal musculature and sympathetic ganglia respectively. LMC: Lateral motor column; MMC: Medial motor column; HMC: Hypaxial motor column; PGC: Preganglionic motor column; MNs: Motor neurons.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

References

    1. Burke RE, Levine DN, Tsairis P, Zajac FE. Physiological types and histochemical profiles in motor units of the cat gastrocnemius. J Physiol. 1973;234:723–748. - PMC - PubMed
    1. Kuffler SW, Hunt CC, Quilliam JP. Function of medullated small-nerve fibers in mammalian ventral roots; efferent muscle spindle innervation. J Neurophysiol. 1951;14:29–54. - PubMed
    1. Hunt CC, Kuffler SW. Stretch receptor discharges during muscle contraction. J Physiol. 1951;113:298–315. - PMC - PubMed
    1. Westbury DR. A comparison of the structures of alpha and gamma-spinal motoneurones of the cat. J Physiol. 1982;325:79–91. - PMC - PubMed
    1. Burke RE, Strick PL, Kanda K, Kim CC, Walmsley B. Anatomy of medial gastrocnemius and soleus motor nuclei in cat spinal cord. J Neurophysiol. 1977;40:667–680. - PubMed

LinkOut - more resources