Skip to main content

Advertisement

SpringerLink
Log in

Diagnostic investigation and multidisciplinary management in motor neuron disease

  • REVIEW
  • Published:
Journal of Neurology Aims and scope Submit manuscript

Abstract

Amyotrophic lateral sclerosis (ALS) is the most common adult-onset motor neuron disease. ALS is a progressive neurodegenerative disorder, involving motor neurons in the cerebral cortex, brainstem and spinal cord, presenting with a combination of upper and lower motor neuron signs. Etiology remains undetermined, although a multifactorial origin is widely accepted including genetic factors, auto-immunity, oxidative stress, glutamate excitotoxicity and abnormal neurofilament aggregation. The absence of specific diagnostic testing, and variable clinical presentations make the diagnosis of ALS challenging, relying upon correlation of clinical, electrophysiological and neuroimaging data. The disease is relentlessly progressive, with dysarthria, dysphagia, tetraparesis, and respiratory insufficiency due to ongoing respiratory muscle paresis. There is no specific treatment for ALS. Riluzole, a glutamate antagonist, is the only FDA approved drug for ALS, but has only a modest effect on survival. The multiplicity and progressiveness of the disabilities in ALS, highlights the need for a coordinated multidisciplinary rehabilitation program managing symptoms, respiratory care, dysphagia and nutrition, dysarthria and communication, physical and occupational therapy. The main goals are to prolong independence, prevent complications and improve quality of life.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Donaghy M (1999) Classification and clinical features of motor neuron diseases and motor neuropathies in adults. J Neurol 246:331–333

    PubMed  CAS  Google Scholar 

  2. Winderbank A (1994) Adult Motor neuron diseases. In: Munsat TL, Engel AG, Banker BQ (eds) Myology. McGrawHill, New York, pp 1854–1864

  3. David C (2002) Electrodiagnostic approach to the patient with suspected motor neuron disease. Neurol Clinics N Am 20:527–555

    Google Scholar 

  4. Lou J-S (2004) Amyotrophic lateral sclerosis. In: Gilman S (ed) Neurobase. Arbor Publishing, La Jolla, CA

  5. Han JJ, et al. (2003) The amyotrophic lateral sclerosis center: A model of multidisciplinary management. Crit Rev Phys Med Rehabil 15:21–40

    Google Scholar 

  6. Francis K, Bach JR, DeLisa JA (1999) Evaluation and rehabilitation of patients with adult motor neuron disease. Arch Phys Med Rehabil 80:951–963

    Article  PubMed  CAS  Google Scholar 

  7. Ross MA (1997) Acquired motor neuron disorders. Neurol Clinics N Am 15:481–500

    CAS  Google Scholar 

  8. Robberecht W, Van Den Bosch L (1998) The pathogenesis of amyotrophic lateral sclerosis. Neurosci Res Commun 23:67–75

    Article  Google Scholar 

  9. Bryant PR, et al. (2004) Stroke and neurodegenerative disorders. Arch Phys Med Rehabil 85(Suppl 1):21–33

    Article  Google Scholar 

  10. Haverkamp LJ, et al. (1995) Natural history of amyotrophic lateral sclerosis in a database population: validation of a scoring system and a model for survival prediction. Brain 118:707–719

    PubMed  Google Scholar 

  11. Aguila MA, et al. (2003) Prognosis in amyotrophic lateral sclerosis—a population based study. Neurology 60:813–819

    PubMed  Google Scholar 

  12. Leigh PN, et al. (2003) The management of motor neuron disease. J Neurol Neurosurg Psychiatry 74 (Suppl IV):32–47

    Google Scholar 

  13. Krakauer MD, Willems PJ, Hofman A (2003) Genetic epidemiology of amyotrophic lateral sclerosis. Clin Genet 63:83–101

    Google Scholar 

  14. 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

    Article  PubMed  CAS  Google Scholar 

  15. Cudkowicz ME, McKenna-Vasek D, Sapp PE, et al. (1997) Epidemiology of mutations in superoxide dismutase in amyotrophic lateral sclerosis. Ann Neurol 2:210–221

    Google Scholar 

  16. Yang Y, Hentati A, Deng HX, et al. (2001) 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 29:160–165

    Article  PubMed  CAS  Google Scholar 

  17. Hand CK, Khoris J, Salachas F, et al. (2001) A novel locus for familial ALS on chromosome 18q. Am J Hum Genet 70(1):251–256

    PubMed  Google Scholar 

  18. Rabin BA, Griffin JW, Crain BJ, Scavina M, Chance PF,Cornblath DR (1999) Autosomal dominant juvenile amyotrophic lateral sclerosis. Brain 122:1539–1550

    Article  PubMed  Google Scholar 

  19. Chen YZ, Benett CL, Huynh HM, et al. (2004) DNA/RNA helicase gene mutations in a form of juvenile amyotrophic sclerosis (ALS4). Am J Hum Genet 74:1128–1135

    PubMed  CAS  Google Scholar 

  20. Hentati A, Ouahci K, Pericak-Vance MA, Ahmad A, et al. (1997) Linkage of a common locus for recessive amyotrophic lateral sclerosis. Am J Hum Genet A61:A279

    Google Scholar 

  21. Hafezparast M, Ahmad-Annuar A, Hummerich H, et al. (2003) Paradigms for identification of new genes in motor neuron degeneration. Amyotroph Lateral Scler Other Motor Neuron Disord 4:249–257

    Article  PubMed  Google Scholar 

  22. Hentati A, Ouahci K, Pericak-Vance MA, et al. (1998) Linkage of a commoner form of recessive amyotrophic lateral sclerosis to chromossome 15q15-q22 markers. Neurogenetics 2(1):55–60

    Article  PubMed  CAS  Google Scholar 

  23. Abalkhail H, Mitchell J, Habgood J et al. A new familial amyotrphic lateral sclerosis locus on chromosome 16q12.1–16q12.2. Am J Hum Genet 73:383–389

  24. Sapp PC,Hosler BA, McKenna-Yasek D, et al. (2003) Identification of two novel loci for dominantly inherited familial Amyotrophic Lateral Sclerosis. Am J Hum Genet 73:397–403

    Article  PubMed  CAS  Google Scholar 

  25. Nishimura Al, Mitne-Neto M, Silva MC, et al. (2004) A mutations in the vesicle trafficking protein VAPB causes late-onset spinal muscular atrophy and amyotrophic lateral sclerosis. Am J Hum Genet 75:822–831

    Article  PubMed  CAS  Google Scholar 

  26. Hosler BA, Siddique T, Sapp PC, et al. (2000) Linkage of familial amyotrophic lateral sclerosis with frontotemporal dementia to chromosome 9q21-q22. JAMA 284:1664–1669

    Article  PubMed  CAS  Google Scholar 

  27. Wilhelmsen KC, Lynch T, Pavlou E, Higgins M, Nygaard TG (1994) Localization of disinhibition-dementia parkinsonism amyotrophy complex to 17q21–22. Am J Hum Genet 55:1159–1165

    PubMed  CAS  Google Scholar 

  28. Hutton M, Lendon CL, Rizzu P, et al. (1998) Association of missense and 5ƒ-splice-site mutations in tau with the inherited dementia FTDP-17. Nature 393:702–705

    Article  PubMed  CAS  Google Scholar 

  29. Munch C, Sedlmeier R, Meyer T, Homberg V, et al. (2004) point mutations of the p150 subunit of dynactin (DCTN1) gene in ALS. Neurology 63:724–726

    PubMed  CAS  Google Scholar 

  30. Storkebaum E, Lambrechts D, Carmeliet P (2004) VEGF: once regarded as a specific angiogenic factor, now implicated in neuroprotection. Bioessays 26(9):943–954

    Article  PubMed  CAS  Google Scholar 

  31. Terry PD, Kamel F, Umbach DM, et al. (2004) VEGF promoter haplotype and amyotrophic lateral sclerosis (ALS). J Neurogenet 18:429–434

    PubMed  CAS  Google Scholar 

  32. Figlewicz DA, Krizus A, Martinoli MG, et al. (1994) Variants of the heavy neurofilament subunit are associated with the development of amyotrophic lateral sclerosis. Hum Mol Genet 3:1757–1761

    PubMed  CAS  Google Scholar 

  33. Corbo M, Hays AP (1992) Peripherin and neurofilament protein coexist in spinal spheroids of motor neuron disease. J Neuropathol Exp Neurol 51:531–537

    PubMed  CAS  Google Scholar 

  34. Jackson M, Steers G, Leigh PN, et al. (1999) Polymorphisms in the Glutamate transporter gene EAAT2 in European ALS patients. J Neurol 246:1140–1144

    Article  PubMed  CAS  Google Scholar 

  35. Carriedo SG, Yin HZ, Weiss JH (1996) Motor neurons are selectively vulnerable to AMPA/kainate receptormediated injury in vitro. J Neurosci 16:4069–4079

    PubMed  CAS  Google Scholar 

  36. Lacomblez L, Doppler V, Beucher I, et al. (2002) ApoE: a potential marker of disease progression in ALS. Neurology 58:1112–1114

    PubMed  CAS  Google Scholar 

  37. Mui S, Rebeck GW, McKenna-Yasek D, Hyman BT, Brown RH Jr. (1995) Apolipoprotein E epsilon 4 allele is not associated with earlier age at onset in amyotrophic lateral sclerosis. Ann Neurol 38:460–463

    Article  PubMed  CAS  Google Scholar 

  38. Giess R, Beck M, Goetz R, et al. (2000) Potential role of LIF as a modifier gene in the pathogenesis of amyotrophic lateral sclerosis. Neurology 54:1003–1005

    PubMed  CAS  Google Scholar 

  39. Orrell RW, King AW, Lane RJ, de Belleroche JS (1995) Investigation of a null mutation of the CNTF gene in familial amyotrophic lateral sclerosis. J Neurol Sci 132:126–128

    Article  PubMed  CAS  Google Scholar 

  40. James CM, Daniels J, Wiles CM, Owen MJ (1994) Debrisoquine hydroxylase gene polymorphism in motor neuron disease. Neurodegeneration 3:149–152

    Google Scholar 

  41. Olkowski ZL (1998) Mutant AP endonuclease in patients with amyotrophic lateral sclerosis. Neuroreport Jan 26:9(2):239–242

    Google Scholar 

  42. Carri MT, et al. (2003) Neurodegeneration in amyotrophic lateral sclerosis: the role of oxidative stress and altered homeostasis of metals. Brain Res Bull 61:365–374

    PubMed  CAS  Google Scholar 

  43. Robert H, Phil B, Robberecht D (2001) Amyotrophic lateral sclerosis: Pathogenesis. Semin Neurol 8:131–139

    Google Scholar 

  44. Cudkowicz ME, McKenna-Vasek D, Sapp PE, et al. (1997) Epidemiology of mutations in superoxide dismutase in amyotrophic lateral sclerosis. Ann Neurol 2:210–221

    Google Scholar 

  45. Andersen PM, Nilsson P, Keränen M-L, et al. (1997) Phenotypic heterogeneity in MND-patients with CuZn-superoxide dismutase mutations in Scandinavia. Brain 10:1723–1737

    Google Scholar 

  46. Andersen MP (2001) Genetics of sporadic ALS. ALS and other motor neuron disorders 2(Suppl 1):S37-S41

    PubMed  CAS  Google Scholar 

  47. Rosen DR, Siddiique T, Petterson D, et al. (1993) Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature 362(6415):59–62

    Article  PubMed  CAS  Google Scholar 

  48. Jan HV, Van den Berg, Wokkle JH (2004) The future of motor neuron disease, the challenges is in the genes. J Neurol 251:491–450

    Google Scholar 

  49. Nelson LM, McGuire V, Longstreth WT, et al. (2000) Population-based case-control study of amyotrophic lateral sclerosis in western Washington State. I. Cigarette smoking and alcohol consumption. Am J Epidemiol 151:156–163

    PubMed  CAS  Google Scholar 

  50. Weisskopf MG, McCullough ML, Calle EE, et al. (2004) Prospective study of cigarette smoking and amyotrophic lateral sclerosis. Am J Epidemiol 160:26–33

    Article  PubMed  CAS  Google Scholar 

  51. Louvel E, Hugon J, Doble A (1997) Therapeutic advances in amyotrophic lateral sclerosis. TiPS 18:196–203

    PubMed  CAS  Google Scholar 

  52. Barbeito LH (2004) A role for astrocytes in motor neuron loss in amyotrophic lateral sclerosis. Brain Res Rev 1:12

    Google Scholar 

  53. Rothstein JD, Van Kammen M, Levey AI, Martin LJ, Kuncl RW (1995) Selective loss of glial glutamate transporter GLT 1 in amyotrophic lateral sclerosis. Ann Neurol 38:73–84

    Article  PubMed  CAS  Google Scholar 

  54. Stuerenburg HJ, Kunze K (1998) Tissue nerve growth factor concentrations in neuromuscular diseases. Eur J Neurol 5:487–490

    Article  PubMed  Google Scholar 

  55. Georgesco M, Salerno A, Camu W (1997) Somatosensory evoked potentials by stimulation of lower limbs nerves in amyotrophic lateral sclerosis. Electroencephalograph Clin Neurophysiol 104:333–342

    CAS  Google Scholar 

  56. Haganasi HA, et al. (2002) Cognitive impairment in amyotrophic lateral sclerosis: evidence from neuropsycological investigation and event related potentials. Cogn Brain Res 14:234–244

    Google Scholar 

  57. Brooks BR, Miller RG, Swash M, Munsat TL (1998) World Federation of Neurology group on Motor Neuron Diseases. El Escorial revisited: revised criteria for the diagnosis of Amyotrophic sclerosis. Philadelphia: FA Davis, pp 47–64

  58. Cornbloth DR, et al. (1992) Nerve conduction studies in amyotrophic sclerosis. Muscle Nerve 15:1111–1115

    Google Scholar 

  59. Rowland LP (1998) Diagnosis of amyotrophic lateral scleoris. J Neurol Sci 160(Suppl 1):S6-S24

    Article  PubMed  Google Scholar 

  60. Olney RK, Lewis RA, Putnam TD, et al. (2003) Consensus criteria for the diagnosis of multifocal motor neuropathy. Muscle Nerve 27:117–121

    Article  PubMed  Google Scholar 

  61. Daube JR (2000) Electrodiagnostic studies in amyotrophic lateral sclerosis and other motor neuron disorders. Muscle Nerve 23:1488–1502

    Article  PubMed  CAS  Google Scholar 

  62. Masseli RA, Wollman RL, Leung C (1993) Neuromuscular transmission in amyotrophic lateral sclerosis. Muscle Nerve 16:1193–1203

    Google Scholar 

  63. Iijima M, Arasaki K, Iwamoto H, et al. (1991) Maximal and minimal motor nerve conduction velocities in patients with motor neuron diseases. Muscle Nerve 14:1110–1115

    Article  PubMed  CAS  Google Scholar 

  64. Clifford R, et al. (2002) Normal aging, dementia, and neurodegenerative disease-amyotrophic lateral sclerosis. In: Atlas SW (ed) Magnetic resonance imaging of the brain and spine. Lippincott Wand W, Philadelphia, pp 1227–1229

  65. Osborn AG (1994) Infection, aquired metabolic,white matter, and degenerative diseases of the brain—amyotrophic lateral sclerosis. In: Osborn AG (ed) Diagnostic Neuroradiology. Mosby, St Louis, pp 778–779

  66. Grossman RI, Yousem DM (2003) Neurodegenerative diseases and hydrocephalus-amyotrophic lateral sclerosis. In: Grossman RI, Yousem DM (eds) Neuroradiology—The requisites.Mosby, USA, pp 388–389

  67. Comi G, Rovaris M, Leocani L (1999) Neuroimaging in amyotrophic lateral sclerosis. Eur J Neurol 6:629–637

    Article  PubMed  CAS  Google Scholar 

  68. Bowen BC, et al. (2000) MR imaging and localized proton spectroscopy of the precentral gyrus in amyotrophic lateral sclerosis. AJNR 21:647–648

    PubMed  CAS  Google Scholar 

  69. Kalra S, et al. (2003) Gabapentin therapy for amyotrophic lateral sclerosis: lack of improvement in neuronal integrity shown by MR spectroscopy. AJNR 24:476–480

    PubMed  Google Scholar 

  70. Ellis CM, et al. (1999) Diffusion tensor MRI assesses corticospinal tract damage in ALS. Neurology 53(5):1051–1058

    PubMed  CAS  Google Scholar 

  71. Ulug AM, et al. (1999) Clinical use of Diffusion-Tensor Imaging for diseases causing neuronal and axonal damage. AJNR 20:1044–1048

    PubMed  CAS  Google Scholar 

  72. Sundgren PC, et al. (2004) Diffusion tensor imaging of the brain: review of clinical applications. Neuroradiology 46:339–350

    Article  PubMed  CAS  Google Scholar 

  73. Kato, et al. (1997) Detection of pyramidal tract lesions in amyotrophic lateral sclerosis with magnetization-transfer measurements. AJNR 18:1541–1547

    PubMed  CAS  Google Scholar 

  74. Bensimon GL, Lacomblez L, Meininger V (1994) A control trial of riluzole in amyotrophic sclerosis. N Engl J Med 330:585–591

    Article  PubMed  CAS  Google Scholar 

  75. Borasio GD, et al. (1996) A placebo-controlled trial of insulin-like growth factor-I in amyotrophic lateral sclerosis. European ALS/IGF-I Study Group. J Neurol 243(Suppl 2):26

    Google Scholar 

  76. ALS CNTF Treatment Study (ACTS) Phase III (1996) Study Group A double-blind placebo-controlled trial of subcutaneous recombinant human ciliary neurotrophic factor (rHCNTF) in amyotrophic lateral sclerosis. Neurology 46:1244–1249

    Google Scholar 

  77. Gian Domenico Borasio, Raymond Voltz (1997) Palliative care in amyotrophic lateral sclerosis. J Neurol 244(Suppl 4):S11–S17

    Google Scholar 

  78. Ganzini L, Johnston S, Silveira MJ (2002) The final month in patients with ALS. Neurology 59:428–431

    PubMed  Google Scholar 

  79. Lyall RA, Donaldson N, Polkey MI, et al. (2001) Respiratory muscle strength and ventilatory failure in amyotrophic sclerosis. Brain 124(10):2000–2013

    Article  PubMed  CAS  Google Scholar 

  80. Fitting JW, Paillex R, Hirt L, et al. (1999) Sniff nasal pressure: a sensitive respiratory test to assess progression of amyotrophic lateral sclerosis. Ann Neurol 46(6):887–893

    Article  PubMed  CAS  Google Scholar 

  81. Morgan RK, McNally S, Alexander M, et al. (2005) Use of sniff-inspiratory force to predict survival in amyotrophic lateral sclerosis. Am J Resp Crit Care Med 171:269–274

    PubMed  Google Scholar 

  82. Miller RG, et al. (1999) “Practice parameter: the care of the patient with amyotrophic lateral sclerosis- an evidence-based review”, American Academy of Neurology-Guidelines for Care in ALS

  83. Krivickas LS (2000) Symptom management and rehabilitation in ALS. AAEM course handout. Update on Motor Neuron Dis

  84. Bach J (1995) respiratory muscle aids for prevention of morbidity and mortality. Scand Neurol 15:72–83

    CAS  Google Scholar 

  85. Schiffman PL, Belsh JM (1989) Effect of inspiratory resistance and theophylline on respiratory muscle strength in patients with amyotrophic lateral sclerosis. Am Rev Resp Dis 139:1418–1423

    PubMed  CAS  Google Scholar 

  86. Aboussouan LS, Khan SU, Banerjee M, et al. (2001) Objective measures of the efficacy of noninvasive positive-pressure ventilation in amyotrophic lateral sclerosis. Muscle Nerve 24:403–409

    Article  PubMed  CAS  Google Scholar 

  87. Francis K, DeLisa J (1999) Evaluation and rehabilitation of patients with adult motor neuron disease. Arch Phys Med Rehabil 80:951–963

    Article  PubMed  CAS  Google Scholar 

  88. Aboussouan LS, Khan SU, Meeker DP, Stelmach K, Mitsumoto H (1997) Effect of non-invasive positive-pressure ventilation on survival im amyotrophic lateral sclerosis. Ann Inter Med 127:450–453

    CAS  Google Scholar 

  89. Stambler N, Charantan M, Cedarbaum JM (2003) Prognostic indicators of survival in ALS. Neurology 60:1252–1258

    Google Scholar 

  90. Kasarskis EJ, Berryman S, et al. (2001) Nutritional status of patients with ALS: relation to proximity of death. Am J Clin Nutr 74:328–334

    Google Scholar 

  91. Mazzini L, Corra T, et al. (1995) Percutaneous endoscopic gastrostomy and enteral nutrition in amyotrophic lateral sclerosis. Neurology 242:695–698

    CAS  Google Scholar 

  92. Kasaskis EJ, Scarlata D, Hill R, et al. (1999) A retrospective study of percutaneous endoscopic gastrostomy in ALS patients during the BDNF and CNTF trials. J Neurol Sci 169:118–125

    Google Scholar 

  93. Thornton FJ, et al. (2002) Amyotrophic lateral sclerosis: enteral nutrition provision: endoscopic or radiologic gastrostomy? Radiology 224:713–717

    PubMed  Google Scholar 

  94. Drory VE, Goltsman E, Reznik JG (2001) The value of muscle exercise in patients with amyotrophic lateral sclerosis. J Neurol Sci 191:133–137

    Article  PubMed  CAS  Google Scholar 

  95. Brinkmann JR, Ringel SP (1991) Effectiveness of exercise in progressive neuromuscular disease. J Neurol Rehabil 5:195–199

    Google Scholar 

  96. Talbot K (2002) Motor neurone disease. Postgrad Med J 78:513–519

    Article  PubMed  CAS  Google Scholar 

  97. Oliver D (1996) The quality of care and symptom control-the effects on the terminal phase of MND/ALSJ. Neurol Sc 139(Suppl):134–136

    Google Scholar 

  98. World Health organization (1990) Cancer pain relief. Report of the WHO expert comitee. Technical report series 804. Geneva: World Health Organization

  99. Gallagher JP (1989) Pathologic laughter and crying in ALS: a search for their origin. Acta Neurol Scand 80:114–117

    Article  PubMed  CAS  Google Scholar 

  100. Borasio GD, Miller RG (2001) Clinical Characteristics and Management of ALS Semin Neurol 21(2):155–166

    Article  PubMed  CAS  Google Scholar 

  101. David Oliver (2002) Paliative care for motor neuron disease. Pract Neurol 2:68–80

    Google Scholar 

  102. Schiffer RB, Herndon RM, Rudick RA (1985) Treatment of pathologic laughing and weeping with amitriptyline. N Engl J Med 312:1480–1482

    Article  PubMed  CAS  Google Scholar 

  103. Borasio GD, Voltz R (1997) Palliative care in amyotrophic lateral sclerosis. J Neurol 244(Suppl 4):S11-S17

    PubMed  Google Scholar 

  104. Iannacone S, Strambi FL (1996) Pharmacological management of emotional lability. Clin Pharmacol 19:532–535

    Google Scholar 

  105. Brooks BR, Thisted RA, Appel SH (2004) Treatment of pseudobulbar affect in ALS with dextromethorphan/quinidine—A randomized trial. Neurology 63:1364–1370

    PubMed  CAS  Google Scholar 

  106. Bushara KO (1997) Sialorrhea in amyotrophic lateral sclerosis: a hypothesis of a new treatment: botulinum A injections of the parotid glands. Med Hypotheses 48:337–339

    Article  PubMed  CAS  Google Scholar 

  107. Bathia KP, Munchau A, Brown P (1999) Botulinum toxin is a useful treatment in excessive drooling in saliva. J Neurol Neurosurg Psychiatry 667:697

    Google Scholar 

  108. Benditt JO, Smith TS, Tonelli MR (2001) Empowering the individual with ALS at the end-of-life: disease-specific advance care planning. Muscle Nerve 24:1706–1709

    Article  PubMed  CAS  Google Scholar 

  109. Silverstein MD, Stocking CB, Antel JP (1991) Amyotrophic lateral sclerosis and life-sustaining therapy. Mayo Clin Proc 66:906–913

    PubMed  CAS  Google Scholar 

  110. Neudert C, Oliver D, Wasner M (2001) The course of the terminal phase in patients with amyotrophic lateral sclerosis. J Neurol 248:612–616

    Article  PubMed  CAS  Google Scholar 

  111. O’Brien T, Kelly M, Sunders C (1992) Motor neuron disease: a hospice perspective. BMJ 304:471–473

    PubMed  CAS  Google Scholar 

  112. Standards and Accreditation Comitee Medical Guidelines Task Force (1996) Medical guidelines for determining prognosis in selected non-cancer diseases. The National Hospice Organization, pp 24–26

  113. Krivickas LS, Shocley L, Mitsumoto H (1997) Homecare of patients with amyotrophic lateral sclerosis (ALS). J Neurol Sci 152(Supple 1):S82-S89

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Dept. of Physical Medicine and Rehabilitation, Senhora da Oliveira Hospital, SA Guimarães, Portugal

    J. A. Rocha

  2. Dept. of Neuroradiology, S. João Hospital, Porto, Portugal

    C. Reis & J. Fonseca

  3. Dept. of Neurology, S. João Hospital, Porto, Portugal

    F. Simões

  4. Dept. of Neurophysiology, S. João Hospital, Porto, Portugal

    J. Mendes Ribeiro

Authors
  1. J. A. Rocha
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. C. Reis
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. F. Simões
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. J. Fonseca
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. J. Mendes Ribeiro
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to J. A. Rocha.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Rocha, J.A., Reis, C., Simões, F. et al. Diagnostic investigation and multidisciplinary management in motor neuron disease. J Neurol 252, 1435–1447 (2005). https://doi.org/10.1007/s00415-005-0007-9

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00415-005-0007-9

Key words

Search

Navigation