Disorders of the Anterior Horn Cell

  • J. D. Mitchell


In terms of the totality of disease anterior horn cell disease is rare. Even in neurological practice spinal muscular atrophy is an uncommon condition. Motor neurone disease (MND) is the most common of the motor system diseases and arguably one of the most unpleasant diseases known to medical science. A large proportion of the work of neurological departments is devoted to the investigation of those suspected of having this disorder or in attempts to relieve the suffering of those in whom this diagnosis has been established.


Amyotrophic Lateral Sclerosis Spinal Muscular Atrophy Neurol Neurosurg Psychiatry Motor Neurone Disease Motor Neurone 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. Alter M, Kurland LT, Molgaard CA (1982) Late progressive muscular atrophy and antecedent poliomyelitis. In: Rowland LP (ed) Human motor neuron disease. Raven Press, New York, pp 303–309Google Scholar
  2. Antel J, Medof M, Richman D et al. (1979) Immunological considerations in amyotrophic lateral sclerosis.In Rose FC (ed) Clinical neuroimmunology. Blackwell Scientific, Oxford, pp 227–244Google Scholar
  3. Aran FA (1850) Recherches sur une maladie non encore d!ecrite du systeme musculaire. Arch Gén Méd (4me Sér) 24:1–35Google Scholar
  4. Askmark H, Aquilonius S-M, Gillberg P-G et al. (1993) A pilot trial of dextromethorphan in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 56:197–200PubMedGoogle Scholar
  5. Auerback P, Crocker P (1982) Regular involvement of Clarke’s nucleus in amyotrophic lateral sclerosis. Arch Neurol 39:155–156Google Scholar
  6. Bartfield H, Dham C, Donnenfeld H et al. (1982) Immunological profile of amyotrophic lateral sclerosis patients and their cell-mediated responses to viral and CNS antigens. Clin Exp Immunol 48:137–147Google Scholar
  7. Behan PO, Behan WM, Bell E et al. (1977) Possible persistent virus in motor neuron disease. Lancet ii:1176Google Scholar
  8. Bell C (1836) The nervous system of the human body, 3rd edn. Longmans, London, pp 432–434Google Scholar
  9. Bensimon G, Lacomblez L, Meininger V et al. (1994) A controlled trial of riluzole in amyotrophic lateral sclerosis. N Engl J Med 330:585–592PubMedGoogle Scholar
  10. Blundell G, Mitchell JD (1985) The use of intrathecal IgG synthetic rates in diagnosis. In: Peeters H (ed) Protides of the biological fluids, Vol. 32. Pergamon Press, Oxford, pp 189–190Google Scholar
  11. Bradley WG, Krasin F (1982) A new hypothesis of the etiology of amyotrophic lateral sclerosis - the DNA hypothesis. Arch Neurol 39:677–680.PubMedGoogle Scholar
  12. Brain WR, Croft PB, Wilkinson M (1965) Motor neurone disease as an manifestation of neoplasm. Brain 88:479–500PubMedGoogle Scholar
  13. Brooke MH, Florence JM, Heller SM et al. (1986) Controlled trial of thyrotropin releasing hormone in amyotrophic lateral sclerosis. Neurology 36:146–151PubMedGoogle Scholar
  14. Brooks BR (1994) World Federation of Neurology Sub Committee on Neuromuscular Diseases. El Escorial Criteria for the diagnosis of amyotrophic lateral sclerosis. J Neurol Sci 124 (Suppl):96–108PubMedGoogle Scholar
  15. Buckley J, Warlow C, Smith P et al. (1983) Motor neuron disease in England and Wales, 1959–1979. J Neurol Neurosurg Psychiatry 46:197–205PubMedGoogle Scholar
  16. Calne DB, Eisen A (1989) The relationship between Alzheimer’s disease, Parkinson’s disease and motor neurone disease. Can J Neurol Sci 16:547–550PubMedGoogle Scholar
  17. Campbell AMG, Williams ER, Barltrop D (1970) Motor neurone disease and exposure to lead. J Neurol Neurosurg Psychiatry 33:877–885PubMedGoogle Scholar
  18. Caroscio, JT (1986) Amyotrophic lateral sclerosis: the disease. In: Caroscio JT (ed) Amyotrophic lateral sclerosis. Thieme, New York, pp 3–15Google Scholar
  19. Caroscio JT, Cohen JA, Zawodiniak J et al. (1986) A double-blind, placebo-controlled trial of TRH in amyotrophic lateral sclerosis. Neurology 36:141–145PubMedGoogle Scholar
  20. Chad D, Mitsumoto H, Adelman LS et al. (1982) Conjugal motor neurone disease. Neurology 32:306–307PubMedGoogle Scholar
  21. Chalmers NC, Mitchell JD (1987) Optico-acoustic atrophy in distal spinal muscular atrophy. J Neurol Neurosurg Psychiatry 50, 238–239PubMedGoogle Scholar
  22. Charcot JM (1872, 1873) Leçons sur les maladies du système nerveux fâites à la Salpetrière. Paris: Delahaye. Translated 1881, New Sydenham Society, London pp 180–191Google Scholar
  23. Dalakas MC, Elder G, Hallett M et al. (1986) A long-term follow-up study of patients with post-poliomyelitis neuromuscular symptoms. N Engl J Med 314:959–963PubMedGoogle Scholar
  24. Davidson TJ Hartmann HA (1981) RNA content and volume of motor neurones in amyotrophic lateral sclerosis. J Neuropathol Exp Neurol 40:187–192PubMedGoogle Scholar
  25. Decaux G, Szyper M, Ectors M et al. (1980) Central nervous system complications of Mycoplasma pneumoniae. J Neurol Neurosurg Psychiatry 43:883–887PubMedGoogle Scholar
  26. Durrleman S, Alprerovitch A (1989) Increasing trend of ALS in France and elsewhere: are the changes real. Neurology 39:768–773PubMedGoogle Scholar
  27. Emery AEH, Holloway SM (1982) Familial motor neuron disease. In: Rowland LP (ed) Human motor neuron diseases. Raven Press, New York, pp. 139–147Google Scholar
  28. Engel WK, Siddique T, Nicoloff JT et al. (1983a) TRH levels are reduced in CSF of amyotrophic lateral sclerosis and other spastic patients and rise with intravenous treatment. Neurology 33 (Suppl. 2): 176Google Scholar
  29. Engel WK, Siddique T, Nicoloff JT (1983b) Effect on weakness and spasticity in amyotrophic lateral sclerosis of thyrotropin releasing hormone. Lancet ii:73–75Google Scholar
  30. Farrell DF, Starr A (1968) Delayed neurological sequelae of electrical injuries. Neurology 18:601–606PubMedGoogle Scholar
  31. Feigenbaum JA, Munsat TL (1970) A neuromuscular syndrome of scapuloperoneal distribution. Bull Los Angeles Neurosurg Soc 35:47–57Google Scholar
  32. Fraser KB, Shirodaria PV, Haire M (1979) Jejunal biopsy in multiple sclerosis. In: Behan PO, Rose FC (eds) Progress in neurological research. Pitman Medical, Tunbridge Wells, pp 73–78Google Scholar
  33. Garruto RM, Swyt C, Fiori CE et al. (1985) Intraneuronal deposition of calcium and aluminium in amyotrophic lateral sclerosis of Guam. Lancet ii:1353Google Scholar
  34. Gordon EE, Janusko DM, Kaufman L (1967) A critical survey of the stiff man syndrome. Am J Med 42:582PubMedGoogle Scholar
  35. Gowers WR (1902) A lecture on abiotrophy: disease from defect of life. Lancet ii:1003–1007Google Scholar
  36. Harding, AE (1984) The hereditary ataxias and related disorders. Churchill Livingstone, EdinburghGoogle Scholar
  37. Harding AE, Thomas PK, Baraitser M et al. (1982) X-linked bulbospinal neuronopathy: a report of ten cases. J Neurol Neurosurg Psychiatry 45:1012–1019PubMedGoogle Scholar
  38. Harding AE, Bradbury PG, Murray NMF (1983) Chronic asymmetrical spinal muscular atrophy. J Neurol Sci 59:69–83PubMedGoogle Scholar
  39. Harnisch R, Dworsky RL, Henderson BE (1976) A search for clues to the cause of amyotrophic lateral sclerosis. Arch Neurol 33:456–457Google Scholar
  40. Hawkes CH, Fox J (1981) Motor neurone disease in leather workers. Lancet i:507Google Scholar
  41. Hirayama K, Toyokura Y, Tsubaki T (1959) Juvenile muscular atrophy of unilateral upper extremity: a new clinical entity. Psychiatry Neurol Jpn 61:2190.Google Scholar
  42. Holbrook LA, Beach FXM, Silver JR (1970) Delayed myelopathy: a rare complication of severe electrical burns. Br Med J 4:659–660.PubMedGoogle Scholar
  43. Holloway SM, Emery AEH (1982) The epidemiology of motor neuron disease in Scotland. Muscle Nerve 5:131–133PubMedGoogle Scholar
  44. Holloway SM, Mitchell JD (1986) Motor neurone disease in the Lothian Region of Scotland 1961–1981. J Epidemiol Community Health 40:344–350PubMedGoogle Scholar
  45. Holt S, Hudgins D, Krishnan KR et al. (1976) Diffuse myelitis associated with rubella vaccination. Br Med J 2:1037–1038PubMedGoogle Scholar
  46. Iwashira H, Inoue N, Araki S et al. (1970) Optic atrophy, neurol deafness and distal neurogenic atrophy. Arch Neurol 22:357–364Google Scholar
  47. Jamal GA, Weir Al, Hansen S et al. (1985) Sensory involvement in motor neuron disease: further evidence from automated thermal threshold determination. J Neurol Neurosurg Psychiatry 48:906–910.PubMedGoogle Scholar
  48. Jennekens FGI, Busch HFM, van Hemel NM et al. (1975) Inflammatory myopathy in scapulo-ilioperoneal atrophy with cardiopathy. A study of two families. Brain 98:709–722PubMedGoogle Scholar
  49. Jones CT, Brock DJH, Chancellor AM et al. (1993) Cu/Zn superoxide dismutase (SOD1) mutations and sporadic amyotrophic lateral sclerosis. Lancet 342; 1050–1051PubMedGoogle Scholar
  50. Kaeser HE (1965) Scapuloperoneal muscular atrophy. Brain 88:407PubMedGoogle Scholar
  51. Kilness AW, Hochberg FH (1977) Amyotrophic lateral sclerosis in a high selenium environment. JAMA 237:2843–2844PubMedGoogle Scholar
  52. Kono R, Miyamura K, Tajiri E et al. (1974) Neurologic complications associated with acute haemorrhagic conjunctivitis virus infection and its serologic confirmation. J Infect Dis 129:590–593PubMedGoogle Scholar
  53. Kugelberg E (1975) Chronic proximal (pseudomyopathic) spinal muscular atrophy. Kugelberg Welander syndrome. In: Vinken PJ, Bruyn GW (eds) Handbook of clinical neurology, Vol 22 (II). Elsevier, New York, pp 67–80Google Scholar
  54. Leigh PN, Anderton BH, Dodson A et al. (1988) Ubiquitin deposits in anterior horn cells in motor neurone disease. Neurosci Lett 93:197–203PubMedGoogle Scholar
  55. Levine DP, Lerner AM (1978) The clinical spectrum of Mycoplasma pneumoniae infections. Med Clin North Am 62:961–978PubMedGoogle Scholar
  56. Lilienfeld DE, Chan E, Ehland J et al. (1989) Increasing mortality from motor neurone disease in the United States during the past two decades. Lancet i:710–713Google Scholar
  57. Lowe J (1994) New pathological findings in amyotrophic lateral sclerosis. J Neurol Sci 124 (Suppl):38–51PubMedGoogle Scholar
  58. Mann DMA, Yates PO (1974) Motor neuron disease - the nature of the pathogenic mechanism. J Neurol Neurosurg Psychiatry 37:1036–1046Google Scholar
  59. Martyn CN, Barker DJP, Osmond C (1988) Motoneuron disease and past poliomyelitis in England and Wales. Lancet i:1319–1322Google Scholar
  60. Mawatari S, Katayama K (1973) Scapuloperoneal muscular atrophy with cardiopathy. An X-linked recessive trait, Arch Neurol 28:55–59PubMedGoogle Scholar
  61. Mayor GH, Remedi RF, Sprague SM et al. (1980) Central nervous system manifestations of aluminium: effect of parathyroid hormone. Neurotoxicology 1:33–42Google Scholar
  62. McComas AJ, Upton ARM, Sica REP (1973) Motoneurone disease and ageing. Lancet ii: 1477–1480Google Scholar
  63. McLeod JG, Prineas JW (1971) Distal type of chronic spinal atrophy: clinical electro-physiological and pathological studies. Brain 94:703–714PubMedGoogle Scholar
  64. Mitchell JD (1987) Heavy metals and trace elements in amyotrophic lateral sclerosis. Neurol Clin 5:43–60PubMedGoogle Scholar
  65. Mitchell JD, Jackson MJ (1992) Free radicals, amyotrophic lateral sclerosis and neurodegenerative disease. In: Smith RA (ed) Handbook of amyotrophic lateral sclerosis. Marcel Dekker, New York, pp 533–541Google Scholar
  66. Mitchell DM, Olczak SA (1979) Remission of a syndrome clinically indistinguishable from motor neurone disease after resection of bronchial carcinoma. B Med J 2:176–177Google Scholar
  67. Mitchell JD, East BW, Harris IA et al. (1986) Trace elements in the spinal cord and other tissues in motor neuron disease. J Neurol Neurosurg Psychiatry 49:211–215PubMedGoogle Scholar
  68. Mitchell JD, Gibson H, Gatrell AC (1990) Amyotrophic lateral sclerosis in Lancashire and South Cumbria 1976–1986: a geographic study. Arch Neurol 47:875–880PubMedGoogle Scholar
  69. Mitchell JD, East BW, Harris I A, Pentland B (1991) Manganese, selenium and other trace elements in spinal cord, liver and bone in motor neurone disease. Eur Neurol 31:7–11PubMedGoogle Scholar
  70. Mitchell JD, Gatt J A, Phillips TM et al. (1993) Cu/Zn superoxide dismutase, free radicals and motoneuron disease. Lancet 342;1051–1052PubMedGoogle Scholar
  71. Mitchell JD, Houghton E, Rostron G et al. (1995) Serial studies of free radical and antioxidant activity in motor neurone disease and the effect of selegline. Neurodegeneration 4:233–235PubMedGoogle Scholar
  72. Mitsumoto H, Salgado ED, Negroski D et al. (1986) Amyotrophic lateral sclerosis: effects of intravenous and subcutaneous administration of thyrotropin-releasing hormone in controlled trials. Neurology 36:152–159PubMedGoogle Scholar
  73. Miyata S, Nakamura S, Nagata H et al. (1983) Increased manganese level in spinal cords of amyotrophic lateral sclerosis determined by radiochemical neutron activation analysis. J Neurol Sci 61:283–293PubMedGoogle Scholar
  74. Morrison KE, Harding AE (1994) Disorders of the motor neurone. Baillieres Clin Neurol 3:431–445PubMedGoogle Scholar
  75. Nagata H, Miyata S, Nakamura S et al. (1985) Heavy metal concentration in blood cells in patients with amyotrophic lateral sclerosis. J Neurol Sci 67:173–178PubMedGoogle Scholar
  76. Nakagawa S, Yoshida S, Suematsu C et al. (1977) The calcium-magnesium deficient rat: a study on the distribution of calcium in the spinal cord using the electron probe microanalyser. Experientia 33:1225–1226PubMedGoogle Scholar
  77. Neilson S, Robinson I, Clifford Rose F et al. (1993) Rising mortality from motor neurone disease: an explanation. Acta Neurol Scand 87:184–191PubMedGoogle Scholar
  78. Neilson S, Gunnarsson L-G, Robinson I (1994) Rising mortality from motor neurone disease in Sweden 1961–1990: the relative role of increased population life expectancy and environmental factors. Acta Neurol Scand 90:150–159PubMedGoogle Scholar
  79. Noronha ABC, Antel JP, Ross RP et al. (1981) Circulating immune complexes in neurologic disease. Neurology 31:1402–1407PubMedGoogle Scholar
  80. Norris FH, Engel WK (1965) Carcinomatous amyotrophic lateral sclerosis. In: Brain WR, Norris FH (eds.) The remote effects of cancer on the nervous system. Grune & Stratton, New York, pp 81–82Google Scholar
  81. Norris FH, U KS (1978) Amyotrophic lateral sclerosis and low urinary selenium levels. JAMA 239:404PubMedGoogle Scholar
  82. Oldstone MBA, Wilson CB, Perrin LH et al. (1976) Evidence for immune-complex formation in amyotrophic lateral sclerosis. Lancet ii: 169–172Google Scholar
  83. Panse F (1970) Electrical lesions of the nervous system. In: Vinken PJ, Bruyn GW (eds) Handbook of clinical neurology, Vol 7. North Holland, Amsterdam, pp 344–387Google Scholar
  84. Parry GJ, Holtz SJ, Ben-Zeev D et al. (1986) Gammopathy with proximal motor axonopathy simulating motor neuron disease. Neurology 36:273–276PubMedGoogle Scholar
  85. Pederson L, Platz P, Jerseld C et al. (1977) HLA (SD and LD) in patients with amyotrophic lateral sclerosis. J Neurol Sci 31:313–318Google Scholar
  86. Peters HA, Clatanoff DV (1968) Spinal muscular atrophy secondary to macroglobulinaemia. Neurology 18:101–118Google Scholar
  87. Price TML, Allott EH (1958) The stiff man syndrome. B Med J 1:682Google Scholar
  88. Riise T, Gronning M, Klauber MR et al. (1991) Clustering of residence of multiple sclerosis patients at age 13 to 20 years in Hordaland, Norway. Am J Epidemiol 133:932–939PubMedGoogle Scholar
  89. Romberg MH (1851) Lehrbuch der Nervenkrankheiten des Menschen. 2nd edn. Dunker, Berlin. Translated and edited Sieveking (1853) Sydenham Society, London, pp 371–375Google Scholar
  90. Rosen DR, Siddique T, Patterson D et al. (1993) Mutations in Cu/Zn superoxide gene are associated with familial amyotrophic lateral sclerosis. Nature 362:59–62PubMedGoogle Scholar
  91. Schluep M, Steck A J (1988) Immunostaining of motor nerve terminals by IgM M protein with activity against gangliosides GM1 and GDlb from a patient with motor neuron disease. Neurology 38:1890–18902PubMedGoogle Scholar
  92. Schmidt NJ, Lennette EH, Ho HH (1974) An apparently new enterovirus isolated from patients with disease of the central nervous system. J Infect Dis 129:304–309PubMedGoogle Scholar
  93. Schwartz MS, Stålberg E, Swash M (1980) Pattern of segmental motor involvement in syringomyelia: a single fibre EMG study. J Neurol Neurosurg Psychiatry 43:150–155PubMedGoogle Scholar
  94. Serratrice GT, Toga M, Pellisier JF (1983) Chronic spinal muscular atrophy and pallidonigral degeneration: report of a case. Neurology 33:306–310PubMedGoogle Scholar
  95. Shaw P (1994) Excitotoxicity and motor neurone disease: a review of the evidence. J Neurol Sci 124 (Suppl): 6–13PubMedGoogle Scholar
  96. Shaw IC, Fitzmaurice PS, Mitchell JD et al. (1995) Studies on cellular free radical protection mechanisms in the anterior horn from patients with amyotrophic lateral sclerosis. Neurodegeneration 4:391–396PubMedGoogle Scholar
  97. Siddique T, Figlewicz DA, Pericak-Vance JL et al. (1991) Linkage of a gene causing familial amyotrophic lateral sclerosis to chromosome 21 and evidence of genetic locus heterogeneity. N Engl J Med 324:1381–1384PubMedGoogle Scholar
  98. Urbanek K, Jansa P (1974) Amyotrophic lateral sclerosis - abnormal cellular inflammatory response. Arch Neurol 30:186–187PubMedGoogle Scholar
  99. Vijayalaxmi, Pentland B, Newton MS et al. (1985) Spontaneous and mutagen induced sister chromatid exchange in motor neurone disease. Mutat Res 150:355–358PubMedGoogle Scholar
  100. Viola MV, Lazarus M, Antel J et al. (1982) Nucleic acid probes in the study of amyotrophic lateral sclerosis. In Rowland LP (ed) Human motor neuron diseases. Raven Press, New York, pp 317–329Google Scholar
  101. Young ID, Harper PS (1980) Herditary distal spinal muscular atrophy with vocal cord paralysis. J Neurol Neurosurg Psychiatry 43:413–418PubMedGoogle Scholar

Copyright information

© Springer-Verlag London Limited 1997

Authors and Affiliations

  • J. D. Mitchell

There are no affiliations available

Personalised recommendations