**7. References**


Prevention of aspiration and pneumonia, and adequate management of bronchial secretions are two other important issues. Adequate treatment of sialorrhea and dysphagia are important in the reduction of pneumonia risk. Insufficient cough is a condition that can be diagnosed by measuring peak cough flow and should, whenever present, be treated in patients with ALS. There is some evidence that the MI-E device could be of help in cough assistance, except for patients with severe bulbar dysfunction, but further research is needed, as well as randomized trials that compare the MI-E with other techniques of

Aboussouan, LS; Khan, SU; Meeker, DP; Stelmach, K; Mitsumoto, H. (1997). Effect of

Aboussouan, LS; Khan, Su; Banerjee, M; Arroliga, AC; Mitsumoto, H. (2001). Objective

American Thoracic Society. (2005). Guidelines for the management of adults with hospital-

Andersen, PM; Borasio, GD; Dengler, R; Hardiman, O; Kollewe, K; Leigh, PN; Pradat, PF;

Andersen, PM; Borasio, GD; Dengler, R; Hardiman, O; Kollewe, K; Leigh, PN; Pradat, PF;

Ankrom, M; Zelesnick, L; Barofsky, I; Georas, S; Finucane, TE; Greenough, WB 3rd. (2001).

Arnulf, I; Similowski, T; Salachas, F; Garma, L; Mehiri, S; Attali, V; Behin-Bellhesen, V;

Bach, JR. (1993). Mechanical insufflation-exsufflation: Comparison of peak expiratory flow

Bach, JR; Smith, WH; Michaels, J; Saporito, L; Alba, AS; Dayal, R; Pan, J. (1993). Airway

Bach, JR. (1994). Update and perspective on noninvasive respiratory muscle aids: Part 2. The

Bach, JR & Saporito, LR. (1996). Criteria for extubation and tracheostomy tube removal for

patients with ALS. *Am. J. Respir. Crit. Care Med.,* vol.161:849-856.

assisted individuals. *Arch. Phys. Med. Rehabil.*, vol.74:170-177.

expiratory aids. *Chest,* vol.105:1538-1544.

noninvasive positive pressure ventilation on survival in ALS. *Ann. Intern. Med.,*

measures of the efficacy of non-invasive positive-pressure ventilation in ALS.

acquired, ventilator-associated, and healthcare-associated pneumonia. *Am. J. Respir.* 

Silani, V; Tomik, B; EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral Sclerosis. (2005). EFNS Task Force on managment of amyotrophic lateral sclerosis: guidelines for diagnosing and clinical care of patients

Silani, V; Tomik, B; EALSC Working Group. (2007). Good practice in the management of amyotrophic lateral sclerosis: clinical guidelines. An evidencebased review with good practice points. EALSC Working Group. *Amyotroph. Lateral* 

Elective discontinuation of life-sustaining mechanical ventilation on a chronic

Meininger, V; Derenne, JP. (2000). Sleep disorders and diaphragmatic function in

with manually assisted and unassisted coughing techniques. *Chest,* vol.104:1553-

secretion clearance by mechanical exsufflation for post-poliomyelitis ventilator

patients with ventilatory failure: a different approach to weaning. *Chest,*

assisted coughing.

**7. References** 

vol.127:450-453.

*Scler.*, vol.8:195-213.

vol.110:1566-1571.

1562.

*Muscle Nerve,* vol.24:403-409.

*Crit. Care Med.*, vol.171:388-416*.*

and relatives. *Eur. J. Neurol.,* vol.12:921-938.

ventilator unit. *J. Am. Geriatr. Soc.,* vol.49:1549-1554.


Assessment and Management of

vol.26:890-896.

Respiratory Dysfunction in Patients with Amyotrophic Lateral Sclerosis 591

Hayashi, H & Oppenheimer, EA. (2003). ALS patients on TPPV. Totally locked-in state, neurologic findings and ethical implications. *Neurology,* vol.61:135-137. Heffernan, C; Jenkinson, C; Holmes, T; Macleod, H; Kinnear, W; Oliver, D; Leigh, N;

Heiman-Patterson, TD & Miller, RG. (2006). NIPPV: A treatment for ALS whose time has

Jackson, CE; Rosenfeld, J; Moore, DH; Bryan, WW; Barohn, RJ; Wrench, M; Myers, D;

Kaub-Wittemer, D; von Steinbüchel, N; Wasner, M; Laier-Groenveld, G; and Borasio, GD.

Kim, SM; Lee, KM; Hong, YH; Park, KS; Yang, JH; Nam, HW; Sung, JJ; Lee, KW. (2007).

Kim, SM; Park, KS; Nam, H; Ahn, SW; Kim, S; Sung, JJ; Lee, KW. (March 2011).

King, M; Brock, G; Lundell, C. (1985). Clearance of mucus by simulated cough. *J. Appl.* 

Kirilloff, LH; Owens, GR; Rogers, RM; Mazzocco, MC. (1985). Does chest physical therapy

Kleopa, KA; Sherman, M; Bettle, N; Romano, CJ; Heiman-Patterson, T. (1999). BiPap

Lange, DJ; Lechtzin, N; Davey, C; David, W; Heiman-Patterson, T; Gelinas, D; Becker, B;

with amyotrophic lateral sclerosis. *Amyotroph. Lateral Scler.,* vol.5:9-15. Lechtzin, N; Lange, DJ; Davey, C; Becker, B; Mitsumoto, H. (2007). Measures of dyspnea in patients with amyotrophic lateral sclerosis. *Muscle Nerve,* vol.35:98-102. Lechtzin, N; Scott, Y; Busse, AM; Clawson, LL; Kimball, R; Wiener, CM. (2007). Early use of

hypoventilation in ALS/MND patients. *J. Neurol. Sci.,* vol.191:75-78. Just, N; Bautin, N; Danel-Brunaud, V; Debroucker, V; Matran, R; Perez, T. (2010). The Borg

evidence based review. *Amyotroph. Lateral Scler.,* vol.7:5-15.

amyotrophic lateral sclerosis. *Eur. Respir. J*., vol.35:353-360.

*Neurol. Neurosurg. Psychiatry,* vol.78:1387-1389.

vol.6(3):e17893. [Epub ahead of print].

*Physiol.,* vol.58:1776-1782.

work? *Chest,* vol.88:436-444.

*Neurol. Sci.,* vol.164:82-88.

*Scler.,* vol.8:185-188.

3):iii1-55.

come. *Neurology,* vol.67:736-737.

Ampong, MA. (2006). Management of respiration in MND/ALS patients: an

Heberlin, L; King, R; Smith, J; Gelinas, D; Miller, RG. (2001). A preliminary evaluation of a prospective study of pulmonary function studies and symptoms of

dyspnoea score: a relevant clinical marker of inspiratory muscle weakness in

(2003). Quality of life and psychososcial issues in ventilated patients with amyotrophic lateral sclerosis and their caregivers. *J. Pain and Symptom Manage.,*

Relationship between cognitive dysfunction and reduced vital capacity in ALS. *J.* 

Capnography for assessing nocturnal hypoventilation and predicting compliance with subsequent noninvasive ventilation in patients with ALS. *PLoS One*,

improves survival and rate of pulmonary function decline in patients with ALS. *J.* 

Mitsumoto, H; HFCWO Study Group. (2006). High-frequency chest wall oscillation in ALS: an exploratory randomized, controlled trial. *Neurology,* vol.67:991-997. Lechtzin, N; Wiener, CM; Clawson, L; Davidson, MC; Anderson, F; Gowda, N; Diette, GB;

and the ALS CARE Study Group. (2004). Use of noninvasive ventilation in patients

non-invasive ventilation prolongs survival in subjects with ALS. *Amyotroph. Lateral* 

community acquired pneumonia in adults: update 2009. *Thorax,* vol.64(Suppl.

Lim WS, Baudouin SV, George RC, *et al*. (2009). BTS guidelines for the management of


Chaisson, KM; Walsh, S; Simmons, Z; Vender, RL. (2006). A clinical pilot study: high

Chaudri, MB; Liu, C; Watson, L; Jefferson, D; Kinnear, WJ. (2000). Sniff nasal inspiratory

Chaudri, MB; Liu, C; Hubbard, R; Jefferson, D; Kinnear, WJ. (2002). Relationship between

Cheah, BC; Boland, RA; Brodaty, NE; Zoing, MC; Jeffery, SE; McKenzie, DK; Kiernan, MC.

Clemens, KE & Klaschik, E. (2008). Morphine in the management of dyspnoea in ALS. A

Czaplinski, A; Yen, AA; Appel, SH. (2006). Forced vital capacity (FVC) as an indicator of

De Carvalho, M ; Matias, T ; Coelho, F ; Evangelista, T ; Pinto, A ; Luis, ML. (1996). Motor

Fallat, RJ; Jewitt, B, Bass, M; Kamm, B; Norris, F. (1979). Spirometry in amyotrophic lateral

Farrero, E; Prats, E; Povedano, M; Martinez-Matos, JA; Manresa, F; Escabrill, J. (2005).

Ferguson, KA; Strong, MJ; Ahmad, D; George, FP. (1996). Sleep-disordered breathing in

Fitting, JW; Paillex, R; Hirt, L; Aebischer, P; Schluep, M. (1999). Sniff nasal pressure: A

Gay, PC; Westbrook, PR; Daube, JR; Litchy, WJ; Windebank, AJ; Iverson, R. (1991). Effects of

Gay, PC & Edmonds, LC. (1995). Severe hypercapnia after low-flow oxygen therapy in

Gelinas, DF; O'Connor, P; Miller, RG. (1998). Quality of life for ventilator-dependent ALS patients and their caregivers. *J. Neurol. Sci.,* vol.160(Suppl. 1):S134-S136. Goncalves, MR & Bach, JR. (2005). Mechanical insullation.exsufflation improves outcomes

right direction (Commentary). *Am. J. Phys. Med. Rehabil.,* vol.84:89-91. Gruis, KL; Brown, DL; Schoennemann, A; Zebarah, VA; Feldman, EL. (2005). Predictors of

lateral sclerosis. *Amyotroph. Lateral Scler.,* vol.7:107-11.

sclerosis. *Amyotroph. Lateral Scler*., vol.28:1-9.

pilot study. *Eur. J. Neurol*., vol.15:445-450.

vol.15:539-542.

*Respir. J.,* vol.19:434-438.

*Psychiatry,* vol.77:390-392.

vol.139(Suppl.):117-122.

vol.127:2132-2138.

*Neurol.,* vol.46:887-893.

*Proc*., vol.70:327-330.

*Muscle Nerve,* vol.32:808-811.

sclerosis. *Arch. Neurol.,* vol.36:74-80.

ALS. *Mayo Clin. Proc.,* vol.66:686-694.

amyotrophic lateral sclerosis. *Chest,* vol.110:664-669.

frequency chest wall oscillation airway clearance in patients with amyotrophic

pressure as a marker of respiratory function in motor neuron disease. *Eur. Respir. J.,*

supramaximal flow during cough and mortality in motor neuron disease. *Eur.* 

(2009). INSPIRATIonAL - INSPIRAtory muscle Training In Amyotrophic Lateral

survival and disease progression in an ALS clinic population. *J. Neurol. Neurosurg.* 

neuron disease presenting with respiratory failure. *J. Neurol. Sci.,*

Survival in amyotrophic lateral sclerosis with home mechanical ventilation. The impact of systematic respiratory assessment and bulbar involvement. *Chest,*

sensitive respiratory test to assess progression of amyotrophic lateral sclerosis. *Ann.* 

alterations in pulmonary function and sleep variables on survival in patients with

patients with neuromuscular disease and diaphragmatic dysfunction. *Mayo Clin.* 

for neuromuscular disease patients with respiratory tract infections. A step in the

noninvasive ventilation tolerance in patients with amyotrophic lateral sclerosis.


Assessment and Management of

1233.

*Neurology*, vol.73:1218-1226.

*Am. J. Respir. Crit. Care Med.,* vol.171:269-274.

the course of ALS. *Neurology*, vol.65:1774-1777.

attitudes. *Neurology,* vol.43:438-443.

*Psychiatry,* vol.71:482-487.

vol.304:471-473.

Respiratory Dysfunction in Patients with Amyotrophic Lateral Sclerosis 593

Miller, RG; Jackson, CE; Kasarskis, EJ; England, JD; Forshew, D; Johnston, W; Kalra, S; Katz,

Morgan, RK; McNally, S; Alexander, M; Conroy, R; Hardiman, O; Costello, RW. (2005). Use

Moss, AH; Casey, P; Stocking, CB; Roos, RP; Brooks, BR; Siegler, M. (1993). Home

Moss, AH; Oppenheimer, EA; Casey, P; Cazzolli, PA; Roos, RP; Stocking, CB; Siegler, M.

Neudert, C; Oliver, D; Wasner, M; Borasio, G. (2001). The course of the terminal phase in patients with amyotrophic lateral sclerosis. *J. Neurol.,* vol.248:612-616. Newson-Davis, IC; Lyall, RA; Leigh, PN; Morham, J; Goldstein, LH. (2001). The effect of

O'Brien, T; Kelly, M; Saunders, C. (1992). Motor neuron disease: a hospice perspective. *BMJ*,

Olney, RK; Murphy, J; Forshew, D; Garwood, E; Miller, BL; Langmore, S; Kohn, MA;

O'Mahony, S; McHugh, M; Zallman, L; Selwyn, P. (2003). Ventilator withdrawal: procedures

Peysson, S; Vandenberghe, N; Philit, F; Vial, C; Petitjean, T; Bouhour, F; Bayle, JY; Broussolle

Pinto, A; de Carvalho, M; Evangelista, T; Lopes, A; Sales-Luis, L. (2003). Nocturnal pulse

Pinto, AC; Evangelista, T; Carvalho, M; Alves, MA; Sales Luis, ML. (1995). Respiratory

palliative care service. *J. Pain Symptom Manage.*, vol.26:954-961.

ventilation in ALS patients. *Amyotroph. Lateral Scler.,* vol.4:31-35.

amyotrophic lateral sclerosis. *Eur. Neurol*., vol.59:164-171.

ventilation: advance care planning and outcomes. *Chest,* vol.110:249-255. Munsat, TL; Andres, PL; Finison, L; Conlon, T; Thibodeau, L. (1988). The natural history of motorneuron loss in amyotrophic lateral sclerosis. *Neurology,* vol.38:409-413. Mustfa, N; Walsh, E; Bryant, V; Lyall, RA; Addington-Hall, J; Goldstein, LH; Donaldson, N;

ALS patients and their caregivers. *Neurology,* vol.66:1211-1217.

Quality Standards Subcommittee of the American Academy of Neurology.

JS; Mitsumoto, H; Rosenfeld, J; Shoesmith, C; Strong, MJ; Woolley, SC; Quality Standards Subcommittee of the American Academy of Neurology. (2009). Practice parameter update: The care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. *Neurology*, vol.73:1227-

of Sniff nasal-inspiratory force to predict survival in amyotrophic lateral sclerosis.

ventilation for ALS patients: outcomes, costs, and patient, family, and physician

(1996). Patients with amyotrophic lateral sclerosis receiving long-term mechanical

Polkey, MI; Moxham, J; Leigh, PN. (2006). The effect of noninvasive ventilation on

NIPPV on cognitive function in ALS: a prospective study. *J. Neurol. Neurosur.* 

Lomen-Hoerth, C. (2005). The effects of executive and behavioral dysfunction on

and outcomes. Report of a collaboration between a critical care division and a

E. (2008). Factors predicting survival following noninvasive ventilation in

oximetry: a new approach to estabilish the appropriate time for non-invasive

assistance with a non-invasive ventilator (BiPaP) in motor neuron disease/ALS patients: survival rates in a controlled trial. *J. Neurol. Sci.,* vol.129(Suppl.):19-26.


Lo Coco, D; Marchese, S; Corrao, S; Pesco, MC; La Bella, V; Piccoli, F; Lo Coco, A. (2006).

Lo Coco, D; Marchese, S; Pesco, MC; La Bella, V; Piccoli, F; Lo Coco, A. (2006). Noninvasive

Lo Coco, D; Marchese, S; La Bella, V; Piccoli, T; Lo Coco, A. (2007). The amyotrophic lateral

sclerosis patients on invasive mechanical ventilation. *Chest,* vol.132:64-69. Lo Coco, D; Marchese, S; Lo Coco, A. (2008). Recent advances in respiratory care for Motor

269, Nova Science Publishers, Inc., ISBN 978-60456-155-5, New York, U.S.A. Lo Coco, D; Mattaliano, P; Spataro, R; Mattaliano, A; La Bella, V. (2011). Sleep-wake

Lomen-Hoerth, C. (2005). The effects of executive and behavioral dysfunction on the course

Lyall, RA; Donaldson, N; Fleming, T; Wood, C; Newsom-Davis, I; Polkey, MI; Leigh, PN;

Lyall, RA; Donaldson, N; Polkey, MI; Leigh, PN; Moxham, J. (2001). Respiratory muscle

Marchese, S; Lo Coco, D; Lo Coco, A. (2008). Outcome and attitudes toward home

Marti-Fabregas, J; Dourado, M; Sanchis, J; Miralda, R; Pradas, J; Illa, I. (1995). Respiratory

Massery, M & Frownfelter, D. (1990). Assisted cough techniques: There's more than one way

Miller, RG; Rosenberg, JA; Gelinas, DF; Mitsumoto, H; Newman, D; Sufit, R; Borasio, GD;

Miller, RG; Anderson, FA Jr; Bradley, WG; Brooks, BR; Mitsumoto, H; Munsat, TL; Ringel,

Miller, RG; Mitchell, JD; Lyon, M, Moore, DH. (2007). Riluzole for amyotrophic lateral

Miller, RG; Jackson, CE; Kasarskis, EJ; England, JD; Forshew, D; Johnston, W; Kalra, S; Katz,

with noninvasive ventilation. *Neurology,* vol.57:153-156.

lateral sclerosis. *Acta Neurol. Scand.,* vol.92:261-264.

to cough. *Phys. Ther. Forum.,* vol.9:1-4.

review). *Neurology,* vol.52:1311-1323.

C.A.R.E. Study Group. *Neurology,* vol.54:53-57.

*Respir. Med.,* vol.100:1028-1036.

*Neurology,* vol.67:761-765.

*Psychiatry*, vol.82:839-842.

2013.

vol.102:430-436.

vol.1:CD001447.

of ALS. *Neurology,* vol.65:1774-1777.

Development of chronic hypoventilation in Amyotrophic Lateral Sclerosis patients.

positive-pressure ventilation in ALS. Predictors of tolerance and survival.

sclerosis functional rating scale predicts survival time in amyotrophic lateral

Neuron Disease, In: *Motor Neuron Disease Research Progress*, R.L. Mancini, (Ed.), 253-

disturbances in patients with amyotrophic lateral sclerosis. *J. Neurol. Neurosurg.* 

Moxham, J. (2001). A prospective study of quality of life in ALS patients treated

strength and ventilatory failure in amyotrophic lateral sclerosis. *Brain,* vol.124:2000-

tracheostomy ventilation of consecutive patients: a 10-year experience. *Respir. Med*.,

function deterioration is not time-linked with upper-limb onset in amyotrophic

Bradley, WG; Bromberg, MB; Brooks, BR; Kasarskis, EJ; Munsat, TL; Oppenheimer, EA. (1999). Practice parameter. The care of the patient with ALS (an evidence based

SP. (2000). The ALS patient care database: goals, design, and early results. ALS

sclerosis (ALS)/motor neuron disease (MND). *Cochrane Database Syst. Rev.,* 

JS; Mitsumoto, H; Rosenfeld, J; Shoesmith, C; Strong, MJ; Woolley, SC; Quality Standards Subcommittee of the American Academy of Neurology. (2009). Practice parameter update: The care of the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory therapies (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. *Neurology*, vol.73:1218-1226.


**26** 

*Brazil* 

**Nutritional Care in Amyotrophic Lateral** 

**the Nutritional State** 

Luciano Bruno de Carvalho-Silva

*(NEPA), State University of Campinas (UNICAMP)* 

**Sclerosis: An Alternative for the Maximization of** 

*Federal University of Alfenas- MG (UNIFAL-MG); Center for Food Security Studies* 

Amyotrophic lateral sclerosis (ALS) is characterized by progressive paralysis secondary to the impairment of the motor neurons, upper motor neuron and lower motor neuron. The most common symptoms and signs are atrophy and muscle weakness, fasciculations, cramps, hypertonia and hyperreflexia. In more advanced stages, decreased respiratory muscle strength, progressive loss of body weight and changes in food intake are observed (Nelson et al., 2000). Several factors are inherent to the food intake in ALS, such as: lack of appetite, dysphagia, weakness, dyspnoea, and depression (Stanich et al., 2004; Nelson et al., 2000; Kasarskis et al., 1996; Welnetz, 1990; Slowie et al., 1983). ALS patients usually have rapid weight loss associated with reduced food intake, increased feeding time, fatigue, dehydration and depression (Wright et al., 2005). The combination of these factors may result in increased

Different studies confirm the correlation between the reduced Body Mass Index (BMI) and the decreased survival in subjects with ALS and the decreased food intake and decrease in tricipital skinfold (TSF) (Kasarskis et al., 1996; Desport et al., 2003; Desport et al., 1999; Desport et al., 2001; Heffernan et al., 2004; Slowie et al. 1983). They also show a high percentage of

Dysphagia, a common symptom with the disease progression, is a factor that makes difficult the maintenance of oral feeding, increasing the respiratory complications, with initiation of invasive ventilation, difficulty to move the patient for the outpatient care and depression

In this context, taking the nutritiontal impairment experienced by ALS subjects into account, this chapter aims to discuss the key strategies of nutritional care of patients with ALS, with a

Few studies on food intake in patients with ALS are available in the literature. Among these studies, Kasarskis et al. (1996) studies stand out, which found that 70% of the subjects

weight loss, greater than 15% (Mazzini et al. 1995), and prevalence of malnutrition.

**1. Introduction** 

(Mazzini et al., 1995).

**2.1 Food intake in ALS** 

tool for maximizing the nutritional status.

**2. Amyotrophic lateral sclerosis and nutritional status** 

energy expenditure and therefore hypercatabolism.

