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. 2002 Dec;78(926):736–741. doi: 10.1136/pmj.78.926.736

Management of motor neurone disease

R Howard 1, R Orrell 1
PMCID: PMC1757945  PMID: 12509691

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Selected References

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  1. Aboussouan L. S., Khan S. U., Meeker D. P., Stelmach K., Mitsumoto H. Effect of noninvasive positive-pressure ventilation on survival in amyotrophic lateral sclerosis. Ann Intern Med. 1997 Sep 15;127(6):450–453. doi: 10.7326/0003-4819-127-6-199709150-00006. [DOI] [PubMed] [Google Scholar]
  2. Albert S. M., Murphy P. L., Del Bene M. L., Rowland L. P. A prospective study of preferences and actual treatment choices in ALS. Neurology. 1999 Jul 22;53(2):278–283. doi: 10.1212/wnl.53.2.278. [DOI] [PubMed] [Google Scholar]
  3. Albert S. M., Murphy P. L., Del Bene M. L., Rowland L. P. Prospective study of palliative care in ALS: choice, timing, outcomes. J Neurol Sci. 1999 Oct 31;169(1-2):108–113. doi: 10.1016/s0022-510x(99)00227-0. [DOI] [PubMed] [Google Scholar]
  4. Bach J. R. Amyotrophic lateral sclerosis. Communication status and survival with ventilatory support. Am J Phys Med Rehabil. 1993 Dec;72(6):343–349. [PubMed] [Google Scholar]
  5. Bensimon G., Lacomblez L., Meininger V. A controlled trial of riluzole in amyotrophic lateral sclerosis. ALS/Riluzole Study Group. N Engl J Med. 1994 Mar 3;330(9):585–591. doi: 10.1056/NEJM199403033300901. [DOI] [PubMed] [Google Scholar]
  6. Blasco P. A., Stansbury J. C. Glycopyrrolate treatment of chronic drooling. Arch Pediatr Adolesc Med. 1996 Sep;150(9):932–935. doi: 10.1001/archpedi.1996.02170340046009. [DOI] [PubMed] [Google Scholar]
  7. Borasio G. D., Miller R. G. Clinical characteristics and management of ALS. Semin Neurol. 2001 Jun;21(2):155–166. doi: 10.1055/s-2001-15268. [DOI] [PubMed] [Google Scholar]
  8. Borasio G. D., Sloan R., Pongratz D. E. Breaking the news in amyotrophic lateral sclerosis. J Neurol Sci. 1998 Oct;160 (Suppl 1):S127–S133. doi: 10.1016/s0022-510x(98)00211-1. [DOI] [PubMed] [Google Scholar]
  9. Borasio G. D., Voltz R. Discontinuation of mechanical ventilation in patients with amyotrophic lateral sclerosis. J Neurol. 1998 Nov;245(11):717–722. doi: 10.1007/s004150050273. [DOI] [PubMed] [Google Scholar]
  10. Borasio G. D., Voltz R., Miller R. G. Palliative care in amyotrophic lateral sclerosis. Neurol Clin. 2001 Nov;19(4):829–847. doi: 10.1016/s0733-8619(05)70049-9. [DOI] [PubMed] [Google Scholar]
  11. Bradley W. G., Anderson F., Bromberg M., Gutmann L., Harati Y., Ross M., Miller R. G., ALS CARE Study Group Current management of ALS: comparison of the ALS CARE Database and the AAN Practice Parameter. The American Academy of Neurology. Neurology. 2001 Aug 14;57(3):500–504. doi: 10.1212/wnl.57.3.500. [DOI] [PubMed] [Google Scholar]
  12. Bradley W. G., Anderson F., Bromberg M., Gutmann L., Harati Y., Ross M., Miller R. G., ALS CARE Study Group Current management of ALS: comparison of the ALS CARE Database and the AAN Practice Parameter. The American Academy of Neurology. Neurology. 2001 Aug 14;57(3):500–504. doi: 10.1212/wnl.57.3.500. [DOI] [PubMed] [Google Scholar]
  13. Brooks B. R. Natural history of ALS: symptoms, strength, pulmonary function, and disability. Neurology. 1996 Oct;47(4 Suppl 2):S71–S82. doi: 10.1212/wnl.47.4_suppl_2.71s. [DOI] [PubMed] [Google Scholar]
  14. Carrey Z., Gottfried S. B., Levy R. D. Ventilatory muscle support in respiratory failure with nasal positive pressure ventilation. Chest. 1990 Jan;97(1):150–158. doi: 10.1378/chest.97.1.150. [DOI] [PubMed] [Google Scholar]
  15. Cazzolli P. A., Oppenheimer E. A. Home mechanical ventilation for amyotrophic lateral sclerosis: nasal compared to tracheostomy-intermittent positive pressure ventilation. J Neurol Sci. 1996 Aug;139 (Suppl):123–128. doi: 10.1016/0022-510x(96)00099-8. [DOI] [PubMed] [Google Scholar]
  16. David W. S., Bundlie S. R., Mahdavi Z. Polysomnographic studies in amyotrophic lateral sclerosis. J Neurol Sci. 1997 Oct;152 (Suppl 1):S29–S35. doi: 10.1016/s0022-510x(97)00241-4. [DOI] [PubMed] [Google Scholar]
  17. Demaerschalk BM, Strong MJ. Amyotrophic Lateral Sclerosis. Curr Treat Options Neurol. 2000 Jan;2(1):13–22. doi: 10.1007/s11940-000-0020-3. [DOI] [PubMed] [Google Scholar]
  18. Doyle D., O'Connell S. Breaking bad news: starting palliative care. J R Soc Med. 1996 Oct;89(10):590–591. doi: 10.1177/014107689608901016. [DOI] [PMC free article] [PubMed] [Google Scholar]
  19. Durelli L., Bongioanni M. R., Ferrero B., Ferri R., Imperiale D., Bradac G. B., Bergui M., Geuna M., Bergamini L., Bergamasco B. Interferon alpha-2a treatment of relapsing-remitting multiple sclerosis: disease activity resumes after stopping treatment. Neurology. 1996 Jul;47(1):123–129. doi: 10.1212/wnl.47.1.123. [DOI] [PubMed] [Google Scholar]
  20. Ellis C. M., Leary S., Payan J., Shaw C., Hu M., O'Brien M., Leigh P. N. Use of human intravenous immunoglobulin in lower motor neuron syndromes. J Neurol Neurosurg Psychiatry. 1999 Jul;67(1):15–19. doi: 10.1136/jnnp.67.1.15. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. Evangelista T., Carvalho M., Pinto A., Luís M. de L. Phrenic nerve conduction in amyotrophic lateral sclerosis. J Neurol Sci. 1995 May;129 (Suppl):35–37. doi: 10.1016/0022-510x(95)00057-9. [DOI] [PubMed] [Google Scholar]
  22. Gelinas D. F., O'Connor P., Miller R. G. Quality of life for ventilator-dependent ALS patients and their caregivers. J Neurol Sci. 1998 Oct;160 (Suppl 1):S134–S136. doi: 10.1016/s0022-510x(98)00212-3. [DOI] [PubMed] [Google Scholar]
  23. Giess R., Naumann M., Werner E., Riemann R., Beck M., Puls I., Reiners C., Toyka K. V. Injections of botulinum toxin A into the salivary glands improve sialorrhoea in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry. 2000 Jul;69(1):121–123. doi: 10.1136/jnnp.69.1.121. [DOI] [PMC free article] [PubMed] [Google Scholar]
  24. Goldstein L. H., Adamson M., Jeffrey L., Down K., Barby T., Wilson C., Leigh P. N. The psychological impact of MND on patients and carers. J Neurol Sci. 1998 Oct;160 (Suppl 1):S114–S121. doi: 10.1016/s0022-510x(98)00209-3. [DOI] [PubMed] [Google Scholar]
  25. Hadano S., Hand C. K., Osuga H., Yanagisawa Y., Otomo A., Devon R. S., Miyamoto N., Showguchi-Miyata J., Okada Y., Singaraja R. A gene encoding a putative GTPase regulator is mutated in familial amyotrophic lateral sclerosis 2. Nat Genet. 2001 Oct;29(2):166–173. doi: 10.1038/ng1001-166. [DOI] [PubMed] [Google Scholar]
  26. Hadjikoutis S., Eccles R., Wiles C. M. Coughing and choking in motor neuron disease. J Neurol Neurosurg Psychiatry. 2000 May;68(5):601–604. doi: 10.1136/jnnp.68.5.601. [DOI] [PMC free article] [PubMed] [Google Scholar]
  27. Hardiman O. Symptomatic treatment of respiratory and nutritional failure in amyotrophic lateral sclerosis. J Neurol. 2000 Apr;247(4):245–251. doi: 10.1007/s004150050578. [DOI] [PubMed] [Google Scholar]
  28. Haverkamp L. J., Appel V., Appel S. H. Natural history of amyotrophic lateral sclerosis in a database population. Validation of a scoring system and a model for survival prediction. Brain. 1995 Jun;118(Pt 3):707–719. doi: 10.1093/brain/118.3.707. [DOI] [PubMed] [Google Scholar]
  29. Hayashi H., Kato S., Kawada A. Amyotrophic lateral sclerosis patients living beyond respiratory failure. J Neurol Sci. 1991 Sep;105(1):73–78. doi: 10.1016/0022-510x(91)90121-m. [DOI] [PubMed] [Google Scholar]
  30. Hayashi H., Kato S. Total manifestations of amyotrophic lateral sclerosis. ALS in the totally locked-in state. J Neurol Sci. 1989 Oct;93(1):19–35. doi: 10.1016/0022-510x(89)90158-5. [DOI] [PubMed] [Google Scholar]
  31. Hayashi H. Ventilatory support: Japanese experience. J Neurol Sci. 1997 Oct;152 (Suppl 1):S97–100. doi: 10.1016/s0022-510x(97)00254-2. [DOI] [PubMed] [Google Scholar]
  32. Hill N. S. Noninvasive positive pressure ventilation in neuromuscular disease. Enough is enough! Chest. 1994 Feb;105(2):337–338. doi: 10.1378/chest.105.2.337. [DOI] [PubMed] [Google Scholar]
  33. Hillberg R. E., Johnson D. C. Noninvasive ventilation. N Engl J Med. 1997 Dec 11;337(24):1746–1752. doi: 10.1056/NEJM199712113372407. [DOI] [PubMed] [Google Scholar]
  34. Howard R. S., Wiles C. M., Loh L. Respiratory complications and their management in motor neuron disease. Brain. 1989 Oct;112(Pt 5):1155–1170. doi: 10.1093/brain/112.5.1155. [DOI] [PubMed] [Google Scholar]
  35. Iannaccone S., Ferini-Strambi L. Pharmacologic treatment of emotional lability. Clin Neuropharmacol. 1996 Dec;19(6):532–535. doi: 10.1097/00002826-199619060-00008. [DOI] [PubMed] [Google Scholar]
  36. Kasarskis E. J., Berryman S., Vanderleest J. G., Schneider A. R., McClain C. J. Nutritional status of patients with amyotrophic lateral sclerosis: relation to the proximity of death. Am J Clin Nutr. 1996 Jan;63(1):130–137. doi: 10.1093/ajcn/63.1.130. [DOI] [PubMed] [Google Scholar]
  37. Kimura K., Tachibana N., Kimura J., Shibasaki H. Sleep-disordered breathing at an early stage of amyotrophic lateral sclerosis. J Neurol Sci. 1999 Mar 15;164(1):37–43. doi: 10.1016/s0022-510x(99)00044-1. [DOI] [PubMed] [Google Scholar]
  38. Kleopa K. A., Sherman M., Neal B., Romano G. J., Heiman-Patterson T. Bipap improves survival and rate of pulmonary function decline in patients with ALS. J Neurol Sci. 1999 Mar 15;164(1):82–88. doi: 10.1016/s0022-510x(99)00045-3. [DOI] [PubMed] [Google Scholar]
  39. Lacomblez L., Bensimon G., Leigh P. N., Guillet P., Meininger V. Dose-ranging study of riluzole in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis/Riluzole Study Group II. Lancet. 1996 May 25;347(9013):1425–1431. doi: 10.1016/s0140-6736(96)91680-3. [DOI] [PubMed] [Google Scholar]
  40. Leighton S. E., Burton M. J., Lund W. S., Cochrane G. M. Swallowing in motor neurone disease. J R Soc Med. 1994 Dec;87(12):801–805. [PMC free article] [PubMed] [Google Scholar]
  41. Light V. L., Slezak F. A., Porter J. A., Gerson L. W., McCord G. Predictive factors for early mortality after percutaneous endoscopic gastrostomy. Gastrointest Endosc. 1995 Oct;42(4):330–335. doi: 10.1016/s0016-5107(95)70132-x. [DOI] [PubMed] [Google Scholar]
  42. Lyall R. A., Donaldson N., Polkey M. I., Leigh P. N., Moxham J. Respiratory muscle strength and ventilatory failure in amyotrophic lateral sclerosis. Brain. 2001 Oct;124(Pt 10):2000–2013. doi: 10.1093/brain/124.10.2000. [DOI] [PubMed] [Google Scholar]
  43. Mathus-Vliegen L. M., Louwerse L. S., Merkus M. P., Tytgat G. N., Vianney de Jong J. M. Percutaneous endoscopic gastrostomy in patients with amyotrophic lateral sclerosis and impaired pulmonary function. Gastrointest Endosc. 1994 Jul-Aug;40(4):463–469. doi: 10.1016/s0016-5107(94)70211-x. [DOI] [PubMed] [Google Scholar]
  44. Mazzini L., Corrà T., Zaccala M., Mora G., Del Piano M., Galante M. Percutaneous endoscopic gastrostomy and enteral nutrition in amyotrophic lateral sclerosis. J Neurol. 1995 Oct;242(10):695–698. doi: 10.1007/BF00866922. [DOI] [PubMed] [Google Scholar]
  45. Meininger V. Breaking bad news in amyotrophic lateral sclerosis. Palliat Med. 1993;7(4 Suppl):37–40. doi: 10.1177/0269216393007004S07. [DOI] [PubMed] [Google Scholar]
  46. Melo J., Homma A., Iturriaga E., Frierson L., Amato A., Anzueto A., Jackson C. Pulmonary evaluation and prevalence of non-invasive ventilation in patients with amyotrophic lateral sclerosis: a multicenter survey and proposal of a pulmonary protocol. J Neurol Sci. 1999 Oct 31;169(1-2):114–117. doi: 10.1016/s0022-510x(99)00228-2. [DOI] [PubMed] [Google Scholar]
  47. Miller R. G., Rosenberg J. A., Gelinas D. F., Mitsumoto H., Newman D., Sufit R., Borasio G. D., Bradley W. G., Bromberg M. B., Brooks B. R. Practice parameter: the care of the patient with amyotrophic lateral sclerosis (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology: ALS Practice Parameters Task Force. Neurology. 1999 Apr 22;52(7):1311–1323. doi: 10.1212/wnl.52.7.1311. [DOI] [PubMed] [Google Scholar]
  48. Moss A. H., Casey P., Stocking C. B., Roos R. P., Brooks B. R., Siegler M. Home ventilation for amyotrophic lateral sclerosis patients: outcomes, costs, and patient, family, and physician attitudes. Neurology. 1993 Feb;43(2):438–443. doi: 10.1212/wnl.43.2.438. [DOI] [PubMed] [Google Scholar]
  49. Moss A. H., Oppenheimer E. A., Casey P., Cazzolli P. A., Roos R. P., Stocking C. B., Siegler M. Patients with amyotrophic lateral sclerosis receiving long-term mechanical ventilation. Advance care planning and outcomes. Chest. 1996 Jul;110(1):249–255. doi: 10.1378/chest.110.1.249. [DOI] [PubMed] [Google Scholar]
  50. Neudert C., Oliver D., Wasner M., Borasio G. D. The course of the terminal phase in patients with amyotrophic lateral sclerosis. J Neurol. 2001 Jul;248(7):612–616. doi: 10.1007/s004150170140. [DOI] [PubMed] [Google Scholar]
  51. Newall A. R., Orser R., Hunt M. The control of oral secretions in bulbar ALS/MND. J Neurol Sci. 1996 Aug;139 (Suppl):43–44. doi: 10.1016/0022-510x(96)00104-9. [DOI] [PubMed] [Google Scholar]
  52. Newrick P. G., Langton-Hewer R. Pain in motor neuron disease. J Neurol Neurosurg Psychiatry. 1985 Aug;48(8):838–840. doi: 10.1136/jnnp.48.8.838. [DOI] [PMC free article] [PubMed] [Google Scholar]
  53. O'Brien T., Kelly M., Saunders C. Motor neurone disease: a hospice perspective. BMJ. 1992 Feb 22;304(6825):471–473. doi: 10.1136/bmj.304.6825.471. [DOI] [PMC free article] [PubMed] [Google Scholar]
  54. Oliver D. Opioid medication in the palliative care of motor neurone disease. Palliat Med. 1998 Mar;12(2):113–115. doi: 10.1191/026921698677326556. [DOI] [PubMed] [Google Scholar]
  55. Oliver D., Webb S. The involvement of specialist palliative care in the care of people with motor neurone disease. Palliat Med. 2000 Sep;14(5):427–428. doi: 10.1191/026921600701536264. [DOI] [PubMed] [Google Scholar]
  56. Oppenheimer E. A. Decision-making in the respiratory care of amyotrophic lateral sclerosis: should home mechanical ventilation be used? Palliat Med. 1993;7(4 Suppl):49–64. doi: 10.1177/0269216393007004S09. [DOI] [PubMed] [Google Scholar]
  57. Orrell R. W., Figlewicz D. A. Clinical implications of the genetics of ALS and other motor neuron diseases. Neurology. 2001 Jul 10;57(1):9–17. doi: 10.1212/wnl.57.1.9. [DOI] [PubMed] [Google Scholar]
  58. Pinto A. C., Evangelista T., Carvalho M., Alves M. A., Sales Luís M. L. Respiratory assistance with a non-invasive ventilator (Bipap) in MND/ALS patients: survival rates in a controlled trial. J Neurol Sci. 1995 May;129 (Suppl):19–26. doi: 10.1016/0022-510x(95)00052-4. [DOI] [PubMed] [Google Scholar]
  59. Ringel S. P., Murphy J. R., Alderson M. K., Bryan W., England J. D., Miller R. G., Petajan J. H., Smith S. A., Roelofs R. I., Ziter F. The natural history of amyotrophic lateral sclerosis. Neurology. 1993 Jul;43(7):1316–1322. doi: 10.1212/wnl.43.7.1316. [DOI] [PubMed] [Google Scholar]
  60. Riviere M., Meininger V., Zeisser P., Munsat T. An analysis of extended survival in patients with amyotrophic lateral sclerosis treated with riluzole. Arch Neurol. 1998 Apr;55(4):526–528. doi: 10.1001/archneur.55.4.526. [DOI] [PubMed] [Google Scholar]
  61. Robinson A. C., Khoury G. G., Robinson P. M. Role of irradiation in the suppression of parotid secretions. J Laryngol Otol. 1989 Jun;103(6):594–595. doi: 10.1017/s0022215100109430. [DOI] [PubMed] [Google Scholar]
  62. Schiffer R. B., Cash J., Herndon R. M. Treatment of emotional lability with low-dosage tricyclic antidepressants. Psychosomatics. 1983 Dec;24(12):1094–1096. doi: 10.1016/S0033-3182(83)73113-0. [DOI] [PubMed] [Google Scholar]
  63. Schiffer R. B., Herndon R. M., Rudick R. A. Treatment of pathologic laughing and weeping with amitriptyline. N Engl J Med. 1985 Jun 6;312(23):1480–1482. doi: 10.1056/NEJM198506063122303. [DOI] [PubMed] [Google Scholar]
  64. Schiffman P. L., Belsh J. M. Pulmonary function at diagnosis of amyotrophic lateral sclerosis. Rate of deterioration. Chest. 1993 Feb;103(2):508–513. doi: 10.1378/chest.103.2.508. [DOI] [PubMed] [Google Scholar]
  65. Stambler N., Charatan M., Cedarbaum J. M. Prognostic indicators of survival in ALS. ALS CNTF Treatment Study Group. Neurology. 1998 Jan;50(1):66–72. doi: 10.1212/wnl.50.1.66. [DOI] [PubMed] [Google Scholar]
  66. Stern L. M. Preliminary study of glycopyrrolate in the management of drooling. J Paediatr Child Health. 1997 Feb;33(1):52–54. doi: 10.1111/j.1440-1754.1997.tb00991.x. [DOI] [PubMed] [Google Scholar]
  67. Swash M. Pharmacoeconomics and motor neuron disease. J Neurol Neurosurg Psychiatry. 2000 Apr;68(4):401–403. doi: 10.1136/jnnp.68.4.401. [DOI] [PMC free article] [PubMed] [Google Scholar]
  68. Voltz R., Borasio G. D. Palliative therapy in the terminal stage of neurological disease. J Neurol. 1997 Oct;244 (Suppl 4):S2–10. doi: 10.1007/PL00007721. [DOI] [PubMed] [Google Scholar]
  69. Yang Y., Hentati A., Deng H. X., Dabbagh O., Sasaki T., Hirano M., Hung W. Y., Ouahchi K., Yan J., Azim A. C. The gene encoding alsin, a protein with three guanine-nucleotide exchange factor domains, is mutated in a form of recessive amyotrophic lateral sclerosis. Nat Genet. 2001 Oct;29(2):160–165. doi: 10.1038/ng1001-160. [DOI] [PubMed] [Google Scholar]

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