International Journal of Gerontology
Volume 2, Issue 2 , Pages 72-75, June 2008

Acute Respiratory Failure as Initial Presentation of Amyotrophic Lateral Sclerosis Onset

  • Chien-Tsun Chou

      Affiliations

    • Department of Emergency Medicine, Hsinchu Branch, Taiwan
    • ,
  • Chun-Hao Lin

      Affiliations

    • Department of Neurology, Mackay Memorial Hospital, Hsinchu Branch, Taiwan
    • ,
  • Wai-Mau Choi

      Affiliations

    • Department of Emergency Medicine, Hsinchu Branch, Taiwan
    • Corresponding Author InformationCorrespondence to: Dr Wai-Mau Choi, Department of Emergency Medicine, Mackay Memorial Hospital, Hsinchu Branch, 690, Section 2, Guangfu Road, Hsinchu City 300, Taiwan

Accepted 20 January 2008.

Article Outline

Summary 

Amyotrophic lateral sclerosis (ALS) is a progressive, unrelenting and presently incurable neurodegenerative disorder. Respiratory muscle involvement is often a late complication of ALS. Only 1–3% of patients with ALS presenting to a tertiary care center had respiratory symptoms as their initial clinical symptom. In addition, only 14% of these individuals presented acutely and required emergency intubation. We report a 75-year-old female whose initial presentation at our emergency department was dyspnea. Dyspnea worsened and progressed to acute respiratory failure. There was no definite cause of respiratory failure found initially. However, ALS was diagnosed after physical and neurologic examination and electrodiagnostic studies. Respiratory muscle weakness was her first presentation of ALS.

Key Words:  amyotrophic lateral sclerosis , respiratory failure

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References 

  1. Charcot JM , Joffroy A . Deux cas d'atrophie musculaire progressive avec lesions de la substance grise et des faisceaux antero-lateraux de la moelle epiniere . Arch Physiol Neurol Pathol . 1869;2:744–754
  2. Traynor BJ , Codd MB , Corr B , Forde C , Frost E , Hardiman O . Incidence and prevalence of ALS in Ireland, 1995–1997: a population-based study . Neurology . 1999;52:504–509
  3. Piemonte and Valle d'Aosta Register for Amyotrophic Lateral Sclerosis (PARALS)  . Incidence of ALS in Italy: evidence for a uniform frequency in Western countries . Neurology . 2001;56:239–244
  4. Worms PM . The epidemiology of motor neuron diseases: a review of recent studies . J Neurol Sci . 2001;191:3–9
  5. Shoesmith CL , Findlater K , Rowe A , Strong MJ . Prognosis of amyotrophic lateral sclerosis with respiratory onset . J Neurol Neurosurg Psychiatry . 2007;78:629–631
  6. Rowland LP . Diagnosis of amyotrophic lateral sclerosis . J Neurol Sci . 1998;160(Suppl 1):S6–24
  7. Rowland LP . What's in a name? Amyotrophic lateral sclerosis, motor neuron disease, and allelic heterogeneity . Ann Neurol . 1998;43:691–694
  8. Adamek D , Tomik B , Pichór A , Kauza J , Szczudlik A . The heterogeneity of neuropathological changes in amyotrophic lateral sclerosis. A review of own autopsy material . Folia Neuropathol . 2002;40:119–124
  9. Ince PG , Evans J , Knopp M , Forster G , Hamdalla HH , Wharton SB , et al.   Corticospinal tract degeneration in the progressive muscular atrophy variant of ALS . Neurology . 2003;60:1252–1258
  10. Ince PG , Lowe J , Shaw PJ . Amyotrophic lateral sclerosis: current issues in classification, pathogenesis and molecular pathology . Neuropathol Appl Neurobiol . 1998;24:104–117
  11. Rowland LP . Amyotrophic lateral sclerosis . Curr Opin Neurol . 1994;7:310–315
  12. Talbot K . Motor neurone disease . Postgrad Med J . 2002;78:513–519
  13. Howard RS , Orrell RW . Management of motor neurone disease . Postgrad Med J . 2002;78:736–741
  14. Davenport RJ , Swingler RJ , Chancellor AM , Warlow CP . Avoiding false positive diagnoses of motor neuron disease: lessons from the Scottish Motor Neuron Disease Register . J Neurol Neurosurg Psychiatry . 1996;60:147–151
  15. Traynor BJ , Codd MB , Corr B , Forde C , Frost E , Hardiman O . Amyotrophic lateral sclerosis mimic syndromes: a population-based study . Arch Neurol . 2000;57:109–113
  16. Pestronk A . Invited review: motor neuropathies, motor neuron disorders, and antiglycolipid antibodies . Muscle Nerve . 1991;14:927–936
  17. Pestronk A , Cornblath DR , Ilyas AA , Baba H , Quarles RH , Griffin JW , et al.   A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside . Ann Neurol . 1988;24:73–78
  18. Daube JR . Electrodiagnostic studies in amyotrophic lateral sclerosis and other motor neuron disorders . Muscle Nerve . 2000;23:1488–1502
  19. Krivickas LS . Amyotrophic lateral sclerosis and other motor neuron diseases . Phys Med Rehabil Clin N Am . 2003;14:327–345
  20. de Carvalho M , Swash M . Nerve conduction studies in amyotrophic lateral sclerosis . Muscle Nerve . 2000;23:344–352
  21. Bensimon G , Lacomblez L , Meininger V . A controlled trial of riluzole in amyotrophic lateral sclerosis. ALS/ Riluzole Study Group . N Engl J Med . 1994;330:585–591
  22. Mustfa N , Walsh E , Bryant V , Lyall RA , Addington-Hall J , Goldstein LH , et al.   The effect of noninvasive ventilation on ALS patients and their caregivers . Neurology . 2006;66:1211–1217
  23. Lo Coco D , Marchese S , Pesco MC , La Bella V , Piccoli F , Lo Coco A . Noninvasive positive-pressure ventilation in ALS: predictors of tolerance and survival . Neurology . 2006;67:761–765
  24. Moss AH , Oppenheimer EA , Casey P , Cazzolli PA , Roos RP , Stocking CB , et al.   Patients with amyotrophic lateral sclerosis receiving long-term mechanical ventilation: advance care planning and outcomes . Chest . 1996;110:249–255
  25. Millul A , Beghi E , Logroscino G , Micheli A , Vitelli E , Zardi A . Survival of patients with amyotrophic lateral sclerosis in a population-based registry . Neuroepidemiology . 2005;25:114–119

PII: S1873-9598(08)70014-0

doi:10.1016/S1873-9598(08)70014-0

International Journal of Gerontology
Volume 2, Issue 2 , Pages 72-75, June 2008

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