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Pathology of Motor System Disorder

  • Chapter
Motor Neuron Disease

Abstract

Motor neuron disease (MND) or amyotrophic lateral sclerosis (ALS), the commonest form of motor system degeneration in man, occurs sporadically in middle or late adult life and can be divided into three clinical subtypes irrespective of bulbar or spinal predominance. This classification, convenient for conceptualization of the pathology germane to MND, includes: (1) lower motor neuron type, or primary muscular atrophy (PMA) (Aran’s disease 1850); (2) upper motor neuron type, or primary lateral sclerosis (PLS) (Erb’s disease 1891); and (3) upper-lower motor neuron type, or classic ALS. On the basis of some 20 clinical and 5 autopsy cases, Charcot (1869, 1885) coined the term “la sclerose laterale amyotrophique”, recognizing that the disease started in the lateral columns of the spinal cord, hence “lateral sclerosis”, propagated to the bulbar gray and anterior horns, and secondarily produced muscle atrophy as shown by the term “amyotrophic”. Many atypical forms of MND, all with a high familial incidence, have been recently added. They include: (a) familial ALS, (b) Guamanian ALS, (c) Kii-peninsula ALS in Japan, (d) ALS in Auyu and Jaki people of West New Guinea, and (e) familial juvenile ALS. Gowers (1888) believed that the degeneration in both upper and lower motor neurons might occur simultaneously or independently and, following this concept, pure lower motor neuron diseases have been regarded as separate entities. With this notion, one is obliged to compare the pathology of ALS with other MNDs such as Werdnig-Hoffmann disease, Fazio-Londe disease, Kugelberg-Welander disease, familial spastic paraplegia of Striimpell and the neuronal type of Charcot-Marie-Tooth disease. Striimpell disease is chosen for comparison in this chapter.

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  1. Samuel M. Chou
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  1. University Department of Neurology, Institute of Psychiatry and Kings College School of Medicine and Dentistry, De Crespigny Park, London, SE5 8AF, UK

    P. N. Leigh BSc, PhD, FRCP (Professor) (Professor)

  2. Department of Neurology, The Royal London Hospital, Whitechapel, London, E1 1BB, UK

    Michael Swash MD, FRCP, FRCPath (Consultant Neurologist) (Consultant Neurologist)

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© 1995 Springer-Verlag London Limited

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Chou, S.M. (1995). Pathology of Motor System Disorder. In: Leigh, P.N., Swash, M. (eds) Motor Neuron Disease. Springer, London. https://doi.org/10.1007/978-1-4471-1871-8_4

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  • DOI: https://doi.org/10.1007/978-1-4471-1871-8_4

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-4471-1873-2

  • Online ISBN: 978-1-4471-1871-8

  • eBook Packages: Springer Book Archive

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