eMedicine Specialties > Emergency Medicine > Neurology
Amyotrophic Lateral Sclerosis: Follow-up
Updated: Sep 15, 2009
Follow-up
Further Inpatient Care
- Physical and occupational therapy and speech pathology consultations assist the patient with amyotrophic lateral sclerosis (ALS) in maintaining strength, daily living activities, and communication skills.
- When forced vital capacity (FVC) reaches about 50% of predicted levels, supplemental respiratory support may be needed. (The first symptom may be disturbed sleep.) Use of noninvasive positive pressure ventilation improves quality of life; further respiratory deterioration may necessitate tracheostomy and ventilator support.
- Patients with progressive weight loss, symptomatic dysphagia, or aspiration should be considered for percutaneous gastrostomy placement. Risk of percutaneous endoscopic gastrostomy (PEG) placement increases with declining respiratory function. PEG is best placed before FVC falls to less than 50% of predicted levels.
- Advance directives should be addressed by the patient's neurologist early in the course and should be discussed at regular intervals. Hospice referral may be appropriate to aid in care during the terminal phase of the disease.
Further Outpatient Care
- Early in the treatment course of ALS, encourage patients to continue routine activities.
- Patients are best cared for in a designated ALS center. Many mechanical aids can help overcome disabilities.
- Patients' length of survival and quality of life are enhanced by night-time breathing assistance early in the course of the disease and by aggressive application of alternate feeding options to ensure good nutrition once swallowing becomes difficult.6
Inpatient & Outpatient Medications
- Medications are patient specific.
Complications
- Pneumonia
- Urosepsis
- Constipation
- Depression
- Muscle cramps
- Immobility-related problems
Prognosis
- ALS is a fatal disease. Younger patients may have a slower rate of progression. Patients with bulbar onset, particularly the lower motor neuron type, have a poorer prognosis.
Patient Education
- Patients with ALS are eligible for participation in a Muscular Dystrophy Association clinic.
- Informational web sites include the following:
- For excellent patient education resources, visit eMedicine's Brain and Nervous System Center. Also, see eMedicine's patient education articles Amyotrophic Lateral Sclerosis (Lou Gehrig Disease) and Advance Directives.
Miscellaneous
Medicolegal Pitfalls
- Refer patients to a neurologist. In a patient with an established diagnosis of ALS, follow advance directives.
More on Amyotrophic Lateral Sclerosis |
| Overview: Amyotrophic Lateral Sclerosis |
| Differential Diagnoses & Workup: Amyotrophic Lateral Sclerosis |
| Treatment & Medication: Amyotrophic Lateral Sclerosis |
Follow-up: Amyotrophic Lateral Sclerosis |
| Multimedia: Amyotrophic Lateral Sclerosis |
| References |
| Further Reading |
| « Previous Page | Next Page » |
References
Kimura F, Fujimura C, Ishida S, et al. Progression rate of ALSFRS-R at time of diagnosis predicts survival time in ALS. Neurology. Jan 24 2006;66(2):265-7. [Medline].
Murphy J, Henry R, Lomen-Hoerth C. Establishing subtypes of the continuum of frontal lobe impairment in amyotrophic lateral sclerosis. Arch Neurol. Mar 2007;64(3):330-4. [Medline].
Sabel CE, Boyle PJ, Loytonen M, et al. Spatial clustering of amyotrophic lateral sclerosis in Finland at place of birth and place of death. Am J Epidemiol. May 15 2003;157(10):898-905. [Medline].
Hudson AJ. The motor neuron diseases and related disorders. In: Clinical Neurology. Vol. 4. 1996:11-14.
[Guideline] Andersen PM, Borasio GD, Dengler R, et al. EFNS task force on management of amyotrophic lateral sclerosis: guidelines for diagnosing and clinical care of patients and relatives. Eur J Neurol. Dec 2005;12(12):921-38. [Medline]. [Full Text].
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. Sep 12 2006;67(5):761-5. [Medline].
Abramowitz M. Riluzole for amyotrophic lateral sclerosis. Med Lett Drugs Ther. 1995;37.
Carter GT, Miller RG. Comprehensive management of amyotrophic lateral sclerosis. Phys Med Rehabil Clin N Am. Feb 1998;9(1):271-84, viii-ix. [Medline].
Eisen A, Schulzer M, MacNeil M, Pant B, Mak E. Duration of amyotrophic lateral sclerosis is age dependent. Muscle Nerve. Jan 1993;16(1):27-32. [Medline].
Goldblatt D. Caring for patients with amyotrophic lateral sclerosis. In: Smith RA, ed. Handbook of Amyotrophic Lateral Sclerosis. Marcel Dekker; 1992.
Gourie-Devi M, Nalini A, Subbakrishna DK. Temporary amelioration of symptoms with intravenous cyclophosphamide in amyotrophic lateral sclerosis. J Neurol Sci. Sep 10 1997;150(2):167-72. [Medline].
Gubbay SS, Kahana E, Zilber N, Cooper G, Pintov S, Leibowitz Y. Amyotrophic lateral sclerosis. A study of its presentation and prognosis. J Neurol. 1985;232(5):295-300. [Medline].
Househam E, Swash M. Diagnostic delay in amyotrophic lateral sclerosis: what scope for improvement?. J Neurol Sci. Nov 1 2000;180(1-2):76-81. [Medline].
Ince PG, Lowe J, Shaw PJ. Amyotrophic lateral sclerosis: current issues in classification, pathogenesis and molecular pathology. Neuropathol Appl Neurobiol. Apr 1998;24(2):104-17. [Medline].
Jablecki CK, Berry C, Leach J. Survival prediction in amyotrophic lateral sclerosis. Muscle Nerve. Oct 1989;12(10):833-41. [Medline].
Kaufmann P, Pullman SL, Shungu DC, et al. Objective tests for upper motor neuron involvement in amyotrophic lateral sclerosis (ALS). Neurology. May 25 2004;62(10):1753-7. [Medline].
[Guideline] Miller RG, Rosenberg JA, Gelinas DF, et al. 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. Apr 22 1999;52(7):1311-23. [Medline].
Ringholz GM, Appel SH, Bradshaw M, Cooke NA, Mosnik DM, Schulz PE. Prevalence and patterns of cognitive impairment in sporadic ALS. Neurology. Aug 23 2005;65(4):586-90. [Medline].
Rippon GA, Scarmeas N, Gordon PH, Murphy PL, Albert SM, Mitsumoto H. An observational study of cognitive impairment in amyotrophic lateral sclerosis. Arch Neurol. Mar 2006;63(3):345-52. [Medline].
[Best Evidence] Rowland LP. Diagnosis of amyotrophic lateral sclerosis. J Neurol Sci. Oct 1998;160 Suppl 1:S6-24. [Medline].
Rowland LP. What's in a name? Amyotrophic lateral sclerosis, motor neuron disease, and allelic heterogeneity. Ann Neurol. Jun 1998;43(6):691-4. [Medline].
Shefner JM. Amyotrophic lateral sclerosis. In: Gilchrist JM, ed. Prognosis in Neurology. Boston: Butterworth-Heinemann; 1998:135-138.
Tandan R. Disorders of the upper and lower motor neurons. In: Bradley WG, Daroff RB, Fenichel GM, Marsden CD, eds. Neurology in Clinical Practice. Boston: Butterworth-Heinemann; 1996:1823-1852.
Tandan R, Bradley WG. Amyotrophic lateral sclerosis: Part 1. Clinical features, pathology, and ethical issues in management. Ann Neurol. Sep 1985;18(3):271-80. [Medline].
Further Reading
Clinical trials
Safety and Efficacy of TRO19622 as Add-on Therapy to Riluzole Versus Placebo in Treatment of Patients Suffering From Amyotrophic Lateral Sclerosis (ALS)
Olanzapine for the Treatment of Amyotrophic Lateral Sclerosis (ALS) Cachexia
Efficacy and Tolerability of Tauroursodeoxycholic Acid in Amyotrophic Lateral Sclerosis
Combination Therapy in Amyotrophic Lateral Sclerosis (ALS)
Pilot Placebo-Controlled Trial of Early Noninvasive Ventilation for ALS
A Long-Term Study in Patients With Amyotrophic Lateral Sclerosis (ALS)
Keywords
ALS, Lou Gehrig disease, Lou Gehrig's disease, amyotrophic lateral sclerosis, Charcot disease, Charcot's disease, motor neuron disease
Follow-up: Amyotrophic Lateral Sclerosis