Motor Neuron Diseases


Motor neuron disease is an umbrella term referring to a group of diseases in which there is death of the lower motor neurons and/or the upper motor neurons. Some of these diseases are genetic, but most occur sporadically, that is unpredictably without any identified environmental or genetic cause.

Keywords: amyotrophic lateral sclerosis; spinal muscular atrophy; primary lateral sclerosis; glutamate toxicity

Figure 1.

Schematic representation showing the course of the pyramidal tract, the homuncular organization of the motor cortex in the precentral gyrus, the concentration of the motor output within the internal capsule, and the decussation of the pyramidal tract in the medulla oblongata. (From Donaghy M (1997) Neurology, Oxford University Press.)


Further Reading

Amato AA, Prior TW, Barohn RJ et al. (1993) Kennedy's disease: a clinico‐pathologic correlation with mutations in the androgen receptor gene. Neurology 43: 791–794.

Dalakas MC, Elder G, Hallett M et al. (1986) A long‐term follow‐up study of patients with post‐poliomyelitis neuromuscular symptoms. New England Journal of Medicine 314: 959–963.

Donaghy M (1995) Motor neuron diseases in adults. In: Kennard C (ed.) Recent Advances in Clinical Neurology 8, pp. 73–88. Edinburgh, UK: Churchill Livingstone.

Donaghy M (1999) Classification and clinical features of motor neurone diseases and motor neuropathies in adults. Journal of Neurology 246: 331–333.

Gordon PH, Rowland LP, Younger DS et al. (1997) Lymphoproliferative disorders and motor neurone disease. Neurology 48: 1671–1678.

Gurney ME, Pu H, Chiu AY et al. (1994) Motor neuron degeneration in mice that express a human Cn, Zn superoxide dismutase mutation. Science 164: 1772–1775.

Lefebvre S, Burglen L, Frezal J et al. (1998) The role of the SMN gene in proximal spinal muscular atrophy. Human Molecular Genetics 7: 1531–1536.

Pringle CE, Hudson AJ, Munoz DG et al. (1992) Primary lateral sclerosis. Clinical features, neuropathology and diagnostic criteria. Brain 115: 495–520.

Rosen DR, Siddique T, Patterson D et al. (1993) Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature 362: 59–62.

Rothstein JD, Martin LJ and Kuncl RW (1992) Decreased glutamate transport by the brain and spinal cord in amyotrophic lateral sclerosis. New England Journal of Medicine 326: 1464–1468.

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How to Cite close
Donaghy, Michael(Jun 2001) Motor Neuron Diseases. In: eLS. John Wiley & Sons Ltd, Chichester. [doi: 10.1038/npg.els.0002317]