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Motor Neuron Disease: The Clinical Syndrome

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Motor Neuron Disease

Abstract

The nomenclature of disorders of anterior horn cells is confused. This confusion reflects ignorance of the underlying causes of these syndromes; indeed, the current terminology is based on clinical or pathological descriptions of the syndromes themselves, rather than basic mechanisms. Rowland (1982, 1988) has pointed out the importance of distinguishing between motor neuron diseases and motor neuron disease in discussions of anterior horn cell disorders. The confusion in terminology can be resolved by appreciation of the historical development of the concept of anterior horn cell disease as a cause of muscle wasting.

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References

  • Achari AN, Anderson MS (1974) Myopathic changes in amyotrophic lateral sclerosis. Neurology 24: 477–481

    PubMed  CAS  Google Scholar 

  • Allen IV, Dermot E, Connolly JH, Hurwitz LJ (1971) A study of a patient with the amyotrophic form of Creutzfeldt-Jakob disease. Brain 94: 715–724

    Article  PubMed  CAS  Google Scholar 

  • Ben Hamida M, Hentati F, Chebbi N et al. (1988) Amyotrophies spinales proximales et chroniques en Tunisie. Rev Neurol 144: 737–747

    PubMed  CAS  Google Scholar 

  • Brain WR (1962) Diseases of the nervous system, 6th ed. Oxford University Press, Oxford, p 531

    Google Scholar 

  • Brain WR, Croft PB, Wilkinson M (1969) The course and outcome of motor neuron disease. In: Norris FG, Kurland LT (eds) Motor neuron disease. Grune and Stratton, New York, pp 20–27

    Google Scholar 

  • Brooks BR, Kurent J, Madden D, Sever J, Engel WK (1978) Cerebrospinal fluid antiviral antibody titres in ALS and late post-poliomyelitis progressive muscular atrophy (LPPPMA). Neurology 28: 388 (abstract)

    Google Scholar 

  • Brownell B, Oppenheimer DR, Hughes JT (1970) The central nervous system in motor neuron disease. J Neurol Neurosurg Psychiatr 33: 338–357

    Article  PubMed  CAS  Google Scholar 

  • Caroscio JT, Calhoun WF, Yahr MD (1984) Prognostic factors in motor neuron disease a prospective study of longevity. In: Rose FC (ed) Research progress in motor neuron disease. Pitman, London, pp 34–43

    Google Scholar 

  • Cha CH, Patten BM (1989) Amyotrophic lateral sclerosis: abnormalities of the tongue on magnetic resonance imaging. Ann Neurol 25: 468–472

    Article  PubMed  CAS  Google Scholar 

  • Chaine P, Bouche P, Leger JM et al. (1988) Atrophie musculaire progressive localisee a la main. Rev Neurol 144: 759–763

    PubMed  CAS  Google Scholar 

  • Chalmers D, Swash M (1987) Selective vulnerability of urinary ONUF motoneurons in Shy–Drager syndrome. J Neurol 234: 259–260

    Article  PubMed  CAS  Google Scholar 

  • Charcot JM, Joffroy A (1869) Deux cas d’atrophie musculaire progressive avec lesions de la substance grise et des faisceaux anterolateraux de la moelle epiniere. Arch Physiol Neurol Path 2: 744

    Google Scholar 

  • Chida K, Sakamaki S, Takasu T (1989) Alteration in autonomic function and cardiovascular regulation in amyotrophic lateral sclerosis. J Neurol 236: 127–130

    Article  PubMed  CAS  Google Scholar 

  • Chopra JS, Prabhakar S, Bannerjee AK (1984) The wasted leg syndrome: clinical, electrophysiological and histopathological studies. In: Rose FC (ed) Research progress in motor neuron disease. Pitman, London, pp 422–431

    Google Scholar 

  • Connolly JH, Allen IV, Dermott E (1988) Transmissible agent in the amyotrophic form of Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatr 51: 1459–1460

    Article  PubMed  CAS  Google Scholar 

  • Cruveilhier J (1852–1853) Sur la paralysie musculaire, progressive, atrophique. Bull Acad Med (Paris) 18:490, 546

    Google Scholar 

  • 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: 959–963

    Article  PubMed  CAS  Google Scholar 

  • David AS, Gillham RA (1986) Neuropsychological study of motor neuron disease. Psychosomatics 27: 441–445

    PubMed  CAS  Google Scholar 

  • Doherty, P, Dickson JG, Flawgan TP et al. (1986) Effects of amyotrophic lateral sclerosis serum shown on cultured spinal neurons. Neurology 36: 1330–1334

    PubMed  CAS  Google Scholar 

  • Duchenne de Boulogne GBA (1849) Recherches faites a l’orde des galvanisine sur l’etat de la contractilite et de la sensibilite electromusculaires dans les paralysies des membres superieurs. CR Acad Sci (Paris) 29: 667

    Google Scholar 

  • Duchenne de Boulogne GBA (1860) Paralysie musculaire progressive de la langue, du voile du palais et des levres: affection non encore decrite comme espece morbide distincte. Arch Gen Med 16: 283, 431

    Google Scholar 

  • Dyck PJ (1982) Are motor neuropathies and motor neuron diseases separable? In: Rowland LP (ed) Human motor neuron diseases. Raven Press, New York, pp 105–114

    Google Scholar 

  • Fleet WS, Watson RT (1986) From benign fasciculations and cramps to motor neuron disease. Neurology 36: 997–998

    PubMed  CAS  Google Scholar 

  • Gherardi R, Zuber M, Viard JP (1988) Mise en point: les neuropathies dysglobuliniques. Rev Neurol 144: 391–408

    PubMed  CAS  Google Scholar 

  • Greenfield JG, Matthews WB (1954) Post-encephalitic Parkinsonism and amyotrophy. J Neurol Neurosurg Psychiatr 17: 50–56

    Article  PubMed  CAS  Google Scholar 

  • Gubbay SS, Kahana E, Zilber N, Cooper G, Pintov S, Leibowitz Y (1985) Amyotrophic lateral sclerosis. A study of its presentation and prognosis. J Neurol 232: 295–300

    Google Scholar 

  • Harvey DG, Torack RM, Rosenbaum HE (1979) Amyotrophic lateral sclerosis with ophthalmoplegia: a clinicopathologic study. Arch Neurol 36: 615–617

    PubMed  CAS  Google Scholar 

  • Heath R, Schwartz MS, Brown IRF et al. (1983) Carbonic anhydrase III in neuromuscular disorders. J Neurol Sci 59: 383–388

    Article  PubMed  CAS  Google Scholar 

  • Hirayama K, Tsubaki T, Toyokura Y et al. (1963) Juvenile muscular atrophy of unilateral upper extremity. Neurology 13: 317–380

    Google Scholar 

  • Hoffman PM, Festoff BW, Giron LT Jr, Hallenbeck LC, Garruto RM, Ruscetti FW (1985) Isolation of LAV/HTLV III from a patient with amyotrophic lateral sclerosis. N Engl J Med 313: 324–325

    Article  PubMed  CAS  Google Scholar 

  • Hudson AJ (1981) Amyotrophic lateral sclerosis and its association with dementia in Parkinsonism and other neurological disorders: a review. Brain 104: 217–247

    Article  PubMed  CAS  Google Scholar 

  • Jablecki CF, Berg C, Leach J (1989) Survival prediction in amyotrophic lateral sclerosis. Neurology 12: 833–841

    CAS  Google Scholar 

  • Jacobs C, Bozran D, Heffner RR Jr et al. (1981) An eye movement disorder in amyotrophic lateral sclerosis. Neurology 31: 1282–1287

    PubMed  CAS  Google Scholar 

  • Jamal GA, Weir Al, Hansen S, Ballantyne JP (1985) Sensory involvement in motor neuron disease: further evidence from automated thermal threshold determination. J Neurol Neurosurg Psychiatr 48: 906–910

    Article  PubMed  CAS  Google Scholar 

  • Johnson RT (1987) Myelopathies and retroviral infections. Ann Neurol 21: 113–116

    Article  PubMed  CAS  Google Scholar 

  • Jokelainen M (1977) Amyotrophic lateral sclerosis in Finland. II. Clinical characteristics. Acta Neurol Scand 56: 194–204

    Article  PubMed  CAS  Google Scholar 

  • Juergens SM, Kurland LT, Okazaki H, Mulder DW (1980) ALS in Rochester, Minnesota; 1925–1977. Neurology (Minneap) 30: 463–470

    CAS  Google Scholar 

  • Kasarskis EJ, Winslow M (1989) When did Lou Gehrig’s personal illness begin? Neurology 39: 1243–1245

    PubMed  CAS  Google Scholar 

  • Kelly JJ Jr, Kyle RA, Miles JM, O’Brien PC, Dyck PJ (1981) The spectrum of peripheral neuropathy in myeloma. Neurology 31: 24–31

    PubMed  Google Scholar 

  • Kennedy PGE, Duchen LW (1985) A quantitative study of intermediolateral column cells in motor neurone disease and the Shy-Drager syndrome. J Neurol Neurosurg Psychiatr 48: 1103–1106

    Article  PubMed  CAS  Google Scholar 

  • Lebo CD, Sang KU, Norris FH Jr (1976) Cricopharyngeal myotomy in amyotrophic lateral sclerosis. Laryngoscope 86: 862–868

    Article  PubMed  CAS  Google Scholar 

  • Luys JB (1860) Atrophie musculaire progressive. Lesions histologiques de la substance grise de la moelle epiniere. Gaz Med (Paris) 15: 505

    Google Scholar 

  • Mackay RP (1963) Course and prognosis in amyotrophic lateral sclerosis. Arch Neurol 8: 117–127

    Google Scholar 

  • Mady S (1984) Surgery for dysphagia in motor neurone disease. In: Rose FC (ed) Research progress in motor neurone disease. Pitman Medical, London, pp 443–447

    Google Scholar 

  • Mannen T, Iwata M, Toyokura Y, Nagashima K (1977) Preservation of a certain motoneuron group of the sacral cord in amyotrophic lateral sclerosis: its clinical significance. J Neurol Neurosurg Psychiatr 40: 464–469

    Article  PubMed  CAS  Google Scholar 

  • McHenry LC (1969) Garrison’s history of neurology. CC Thomas, Springfield, Illinois. Meadows JC, Marsden CD (1969) A distal form of chronic spinal muscular atrophy. Neurology 19: 53–59

    Google Scholar 

  • Mitsumoto H, Sliman RJ, Schafer IA et al. (1985) Motor neuron disease and adult hexosaminidase A deficiency in two families; evidence for multisystem degeneration. Ann Neurol 17: 378–385

    Article  PubMed  CAS  Google Scholar 

  • Mortara P, Chio A, Rossa MG et al. (1984) Motor neuron disease in the province of Turin, Italy 1966–1980. J Neurol Sci 66: 165–173

    Article  PubMed  CAS  Google Scholar 

  • Ider DW (1982) Clinical limits of amyotrophic lateral sclerosis. In: Rowland LP (ed) Human motor neuron diseases. Raven Press, New York, pp 15–29

    Google Scholar 

  • Mulder DW, Howard FM (1976) Patient resistance and prognosis in amyotrophic lateral sclerosis. Mayo Clin Proc 51: 537–541

    PubMed  CAS  Google Scholar 

  • Mulder DW, Bushek W, Spring E et al. (1983) Motor neuron disease/ALS: Evaluation of detection thresholds of cutaneous sensation. Neurology 33: 1625–1627

    PubMed  CAS  Google Scholar 

  • Newrick PG, Langton Hewer R (1985) Pain in motor neuron disease. J Neurol Neurosurg Psychiatr 48: 838–840

    Article  PubMed  CAS  Google Scholar 

  • Norris F, Shepherd R, Denys EUK, Mukai E, Elias L, Holden D, Norris H (1993) Onset, natural history and outcome in idiopathic adult motor neuron disease. J Neurol Sci 118: 48–55

    Article  PubMed  CAS  Google Scholar 

  • Ono S, Toyokura Y, Mannen T et al. (1986) Amyotrophic lateral sclerosis: histologic, histochemical and ultrastructural abnormalities of skin. Neurology 36: 948–956

    PubMed  CAS  Google Scholar 

  • Patten BM, Engel WK (1982) Phosphate and parathyroid disorders associated with the syndrome of amyotrophic lateral sclerosis. In: Rowland LP (ed) Human motor neuron diseases. Raven Press, New York, pp 181–200

    Google Scholar 

  • Peiris JB, Seneviratne KN, Wickremasinghe HR et al. (1989) Non-familial juvenile distal spinal muscular atrophy of upper extremity. J Neurol Neurosurg Psychiatr 52: 314–319

    Article  PubMed  CAS  Google Scholar 

  • Poskanzer DC, Cantor HM, Kaplan GS (1969) The frequency of preceding poliomyelitis in ALS. In: Norris FH, Kurland LT (eds) Motor neuron diseases. Grune and Stratton, New York, pp 286–290

    Google Scholar 

  • Potts, CS (1903) A case of progressive muscular atrophy occurring in a man who had had acute poliomyelitis nineteen years previously. Univ Penn Med Bull 16: 31–37

    Google Scholar 

  • Reed DM, Kurland LT (1963) Muscle fasciculations in a healthy population. Arch Neurol 9: 363–367

    PubMed  CAS  Google Scholar 

  • Rowland LP (1982) Diverse forms of motor neuron disease. In: Rowland LP (ed) Human motor neuron diseases. Raven Press, New York, pp 1–13

    Google Scholar 

  • Rowland LP (1988) Research progress in motor neuron diseases. Rev Neurol 144: 623–629

    PubMed  CAS  Google Scholar 

  • Rowland LP, Defendini R, Sherman W et al. (1982) Macroglobulinemia with peripheral neuropathy simulating motor neuron disease. Ann Neurol 11: 532–536

    Article  PubMed  CAS  Google Scholar 

  • Sakata MS, Nakanishi T, Toyokura Y (1978) Anal muscle electromyograms differ in amyotrophic lateral sclerosis and Shy-Drager syndrome.

    Google Scholar 

  • Salazar AM (1982) Discussion. In: Rowland LP (ed) Human motor neuron diseases. Raven Press, New York, pp 179–180

    Google Scholar 

  • Salmon LA, Riley HA (1935) The relation between chronic anterior poliomyelitis or progressive spinal muscular atrophy and an antecedent attack of acute anterior poliomyelitis. Bull Neurol Inst NY 4: 35–63

    Google Scholar 

  • Sayeed ZA, Velmurugendran CU, Arjunds G et al. (1975) Anterior horn cell disease seen in South India. J Neurol Sci 26: 484–498

    Article  Google Scholar 

  • Schwartz MS, Sargeant M, Swash M (1976) Longitudinal fibre splitting in neurogenic muscular disorders: its relation to the pathogenesis of myopathic change. Brain 99: 617–636

    Article  PubMed  CAS  Google Scholar 

  • Shahani B, Davies-Jones GAB, Russell WR (1971) Motor neuron disease: further evidence for an abnormality of disease metabolism. J Neurol Neurosurg Psychiatr 34: 185–191

    Article  PubMed  CAS  Google Scholar 

  • Shy ME, Rowland LP, Smith TS et al. (1986) Motor neuron disease and plasma cell dyscrasia. Neurology 36: 1429–1436

    PubMed  CAS  Google Scholar 

  • Shy ME, Evans VA, Lublin FD et al. (1989) Antibodies to GM1 and GDlb in patients with motor neuron disease without plasma cell dyscrasia. Ann Neurol 25: 511–513

    Article  PubMed  CAS  Google Scholar 

  • Sobue I, Saito N, Iida M et al. (1978) Juvenile type of distal and segmental muscular atrophy of upper extremities. Ann Neurol 3: 429–432

    Article  PubMed  CAS  Google Scholar 

  • Spiller WG (1904) Primary degeneration of the pyramidal tracts: a study of eight cases with necrophy. Univ Pa Med Bull 17: 390–395

    Google Scholar 

  • Stalberg E, Sanders DB (1984) The motor unit in ALS studied with different neurophysiological techniques. In: Rose FC (ed) Research progress in motor neuron disease. Pitman, London, pp 105–122

    Google Scholar 

  • Summers BA, Swash M, Schwartz MS et al. (1987) Juvenile onset bulbospinal muscular atrophy with deafness: Vialetto-van Laere syndrome or Madras–type motor neurone disease? J Neurol 234: 440–442

    Article  PubMed  CAS  Google Scholar 

  • Swash M, Ingram DA (1988) Preclinical and subclinical events in motor neuron disease. J Neurol Neurosurg Psychiatr 51: 165–168

    Article  PubMed  CAS  Google Scholar 

  • Swash M, Schwartz MS (1984) Staging motor neurone disease: single fibre EMG studies of asymmetry, progression and compensatory reinnervation. In: Rose FC (ed) Research progress in motor neurone disease. Pitman, London, pp 123–140

    Google Scholar 

  • Swash M, Schwartz MS (1988) Neuromuscular diseases: a practical approach to diagnosis and management. Springer, Berlin Heidelberg New York, p 456

    Google Scholar 

  • Trojaborg W, Buchthal F (1965) Malignant and benign fasciculations. Acta Neurol Scand (Suppl 13 ) 41: 251–254

    Google Scholar 

  • Tyler JR (1979) Double–blind study of modified neurotoxin in motor neuron disease. Neurology 29: 77–81

    PubMed  CAS  Google Scholar 

  • Van Laere JE (1966) Paralysie bulbo-pontine chronique progressive familiale avec surdite. Rev Neurol 115: 289–295

    PubMed  Google Scholar 

  • Vialetto E (1936) Contributo alia forma ereditaria della paralisi bulbare progressiva. Riv Sper Frevi 40: 1–24

    Google Scholar 

  • Virmani V, Mohan PK (1985) Non–familial spinal segmental muscular atrophy in juvenile and young subjects. Acta Neurol Scand 72: 336–340

    Article  PubMed  CAS  Google Scholar 

  • Williams ER, Bruford A (1970) Creatine phosphokinase in motor neuron disease. Clin Chim Acta 27: 53–56

    Article  PubMed  CAS  Google Scholar 

  • Wilson SAK (1940) Neurology. Edward Arnold, London, pp 1006–1034

    Google Scholar 

  • Younger DS, Chou S, Hay SAP et al. (1988) Primary lateral sclerosis: a clinical diagnosis reemerges. Arch Neurol 45: 1304–1307

    PubMed  CAS  Google Scholar 

  • Zilkha KJ (1962) Discussion. Proc R Soc Med 55: 1028–1029

    CAS  Google Scholar 

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Authors
  1. M. Swash
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  2. M. S. Schwartz
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Editors and Affiliations

  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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Swash, M., Schwartz, M.S. (1995). Motor Neuron Disease: The Clinical Syndrome. In: Leigh, P.N., Swash, M. (eds) Motor Neuron Disease. Springer, London. https://doi.org/10.1007/978-1-4471-1871-8_1

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  • DOI: https://doi.org/10.1007/978-1-4471-1871-8_1

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  • Online ISBN: 978-1-4471-1871-8

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