Abstract
Diaphragmatic weakness implies a decrease in the strength of the diaphragm. Diaphragmatic paralysis is an extreme form of diaphragmatic weakness. Diaphragmatic paralysis is an uncommon clinical problem while diaphragmatic weakness, although uncommon, is probably frequently unrecognized because appropriate tests to detect its presence are not performed. Weakness of the diaphragm can result from abnormalities at any site along its neuromuscular axis, although it most frequently arises from diseases in the phrenic nerves or from myopathies affecting the diaphragm itself. Presence of diaphragmatic weakness may be suspected from the complaint of dyspnea (particularly on exertion) or orthopnea; the presence of rapid, shallow breathing or, more importantly, paradoxical inward motion of the abdomen during inspiration on physical examination; a restrictive pattern on lung function testing; an elevated hemidiaphragm on chest radiograph; paradoxical upward movement of 1 hemidiaphragm during fluoroscopic imaging; or reductions in maximal static inspiratory pressure. The diagnosis of diaphragmatic weakness is confirmed, however, by a reduction in maximal static transdiaphragmatic pressure (Pdimax). The diagnosis of diaphragmatic paralysis is confirmed by the absence of a compound diaphragm action potential on phrenic nerve stimulation. There are many causes of diaphragmatic weakness and paralysis. In this review we outline an approach we have found useful in attempting to determine a specific cause. Most frequently the cause is either a phrenic neuropathy or diaphragmatic myopathy. Often the neuropathy or myopathy affects other nerves or muscles that can be more easily investigated to determine the specific pathologic basis, and, by association, it is presumed that the diaphragmatic weakness or paralysis is secondary to the same disease process.
Similar content being viewed by others
References
Agostoni E, Sant’Ambrogio G (1970) The diaphragm. In: Campbell EJM, Agostoni E, Newsom-Davis J (eds) WB Saunders, Philadelphia, pp 145–147
Aldrich TK (1988) Respiratory muscle fatigue. Clin Chest Med 9:225–236
Alexander C (1966) Diaphragm movements and the diagnosis of diaphragmatic paralysis. Clin Radiol 17:79–83
Arborelins M, Lilja B, Senyk J (1975) Regional and total lung function studies in patients with hemidiaphragmatic paralysis. Respiration 32:253–264
Argov Z, Mastaglia FL (1979) Disorders of neuromuscular transmission caused by drugs. N Engl J Med 301:409–413
Asmussen E (1979) Muscle fatigue. Med Sci Sports 11:313–321
Black LF, Hyatt RE (1969) Maximal respiratory pressures: normal values and relationship to age and sex. Am Rev Respir Dis 99:696–702
Braun NMT, Arora NS, Rochester DF (1983) Respiratory muscle and pulmonary function in polymyositis and other proximal myopathies. Thorax 38:616–623
Bye PTP, Esau SA, Walley KR, Macklem PT, Pardy RL (1984) Ventilatory muscles during exercise in air and oxygen in normal man. J Appl Physiol 56:464–471
Cohen CA, Zagelbaum G, Gross D, Roussos C, Macklem PT (1982) Clinical manifestations of inspiratory muscle fatigue. Am J Med 73:308–314
Dail CW, Affeldt JE, Collier CR (1955) Clinical aspects of glossopharyngeal breathing. JAMA 158:445–449
Danon J, Druz WS, Goldberg NB, Sharp JT (1979) Function of the isolated paced diaphragm and the cervical accessory muscles in Cl quadriplegics. Am Rev Respir Dis 119:909–919
Delhez L (1965) Modalites chez l’homme normal de la réponsé électrique des nerfs phréniques par des chocs uniques. Arch Int Physiol Biochem 73:832–839
De Troyer A, Borenstein S, Cordier R (1980) Analysis of lung volume restriction in patients with respiratory muscle weakness. Thorax 35:603–610
De Troyer A, De Beyl DZ, Thirion M (1981) Function of the respiratory muscles in acute hemiplegia. Am Rev Respir Dis 123:631–632
De Troyer A, Estenne M (1981) Limitations of measurement of transdiaphragmatic pressure in detecting diaphragmatic weakness. Thorax 36:169–174
DeTroyer A, Pride NB (1985) The respiratory system in neuromuscular disease. In: Roussos C, Macklem PT (eds) The thorax, part B, vol. 29. Marcel Dekker, New York, pp 1089–1121
De Troyer A, Estenne M, Vincken W (1986) Rib cage motion and muscle use in high tetraplegics. Am Rev Respir Dis 133:1115–1119
Gandevia SC (1987) Assessment of hemidiaphragmatic “paralysis.” Am Rev Respir Dis 135:1214
Gandevia SC, McKenzie DK (1986) Human diaphragmatic EMG: changes with lung volume and posture during supramaximal phrenic stimulation. J Appl Physiol 60:1420–1428
Garrido H, Mazaira J, Gutierrez P, Gonzales E, Rivas J, Madrazo J (1987) Continuous respiratory support in quadriplegic children by bilateral phrenic nerve stimulation. Thorax 42:573–577
Gibson GJ, Pride NB, Newsom-Davis J, Loh LC (1977) Pulmonary mechanics in patients with respiratory muscle weakness. Am Rev Respir Dis 115:389–395
Gibson GJ, Clark E, Pride NB (1981) Static transdiaphragmatic pressures in normal subjects and in patients with chronic hyperinflation. Am Rev Respir Dis 124:685–689
Gibson GJ, Roussos C (1985) Clinical assessment of thoracic function. In: Roussos C, Macklem PT (eds) The thorax, part B, vol. 29. Marcel Dekker, New York, pp 1281–1299
Gilroy J, Cahalan JL, Berman R, Newman M (1963) Cardiac and pulmonary complications in Duchenne’s progressive muscular dystrophy. Circulation 27:484–493
Goldman M, Mead J (1973) Mechanical interaction between the diaphragm and rib cage. J Appl Physiol 35:197–203
Hapke EJ, Meek JC, Jacobs J (1972) Pulmonary function in progressive muscular dystrophy. Chest 61:41–47
Harrison BDW, Collins JV, Brown KGE, Clark TJH (1971) Respiratory failure in neuromuscular diseases. Thorax 26:579–584
Inkley SR, Oldenburg FC, Vignos PJ (1974) Pulmonary function in Duchenne muscular dystrophy related to stage of disease. Am J Med 56:297–306
Kaminski MJ, Young RR (1985) Neuromuscular and neurological disorders affecting respiration. In: Roussos C, Macklem PT (eds) The thorax, part B, vol. 29 Marcel Dekker, New York, pp 1023–1087
Kreitzer SM, Feldman NT, Saunders NA, Ingram RH Jr (1978) Bilateral diaphragmatic paralysis with hypercapnic respiratory failure. A physiologic assessment. Am J Med 65:89–95
Lachman T, Shahani BT, Young RR (1980) Late responses as aids to diagnosis in peripheral neuropathy. J Neurol Neurosurg Psychiatry 43:156–162
Lane DJ, Hazleman B, Nichols PJR (1974) Late onset respiratory failure in patients with previous poliomyelitis. Q J Med 43:551–568
Laporta D, Grassino A (1985) Assessment of transdiaphragmatic pressure in humans. J Appl Physiol 58:1469–1476
Leech JA, Ghezzo H, Stevens D, Becklake MR (1983) Respiratory pressures and function in young adults. Am Rev Respir Dis 128:17–23
Lisboa C, Pare P, Pertuze J, Contreras G, Moreno R, Guillemi S, Cruz E (1986) Inspiratory muscle function in unilateral diaphragmatic paralysis. Am Rev Respir Dis 134:488–492
Loh L, Goldman M, Newsom-Davis J (1977) The assessment of diaphragmatic function. Medicine 56:165–169
Loring SH, Mead J (1982) Action of the diaphragm on the rib cage inferred from a forcebalance analysis. J Appl Physiol 53:756–760
Loring SH, De Troyer A (1985) Actions of the respiratory muscle. In: Roussos C, Macklem PT (eds) The thorax, part A, vol. 29. Marcel Dekker, New York
MacLean IC, Mattioni TA (1981) Phrenic nerve conduction studies; a new technique and its application in quadriplegic patients. Arch Phys Med Rehab 62:70–73
Macklem PT, Gross D, Grassino A, Roussos C (1978) Partitioning of inspiratory pressure swings between the diaphragm and intercostal/accessory muscles. J Appl Physiol 44:200–208
Markand ON, Kincaid JC, Pourmand RA, Moorthy SS, King RD, Mahomed Y, Brown JW (1984) Electrophysiologic evaluation of diaphragm by transcutaneous phrenic nerve stimulation. Neurology 34:604–614
McCredie M, Lovejoy FW, Kaltredier NL (1962) Pulmonary function in diaphragmatic paralysis. Thorax 17:213–217
McElvaney G, Blackie S, Morrison NJ, Wilcox PG, Fairbarn MS, Pardy RL (1989) Maximum static respiratory pressures in the normal elderly. Am Rev Respir Dis. 139:277–281
McKenzie DK, Gandevia SC (1985) Phrenic nerve conduction times and twitch pressures of the human diaphragm. J Appl Physiol 58:1496–1504
Mead J (1979) Functional significance of the area of apposition of diaphragm to rib cage. Am Rev Respir Dis 119 (Part 2 Suppl):31–32
Mead J, Loring SH (1982) Analysis of volume displacement and length changes of the diaphragm during breathing. J Appl Physiol 53:750–755
Miller JM, Moxham J, Green M (1985) The maximal sniff in the assessment of diaphragm function in man. Clin Sci 69:91–96
Minh V, Dolan GF, Konopka RF, Moser KM (1976) Effect of hyperinflation on inspiratory function of the diaphragm. J Appl Physiol 40:67–73
Nakano KK, Bass H, Tyler HR, Carmel RJ (1976) Amyotrophic lateral sclerosis: a study of pulmonary function. Dis Neur Sys 37:32–35
Newsom-Davis J (1967) Phrenic nerve conduction in man. J Neurol Neurosurg Psychiatry 30:420–426
Newsom-Davis J, Goldman M, Loh L, Casson M (1976) Diaphragm function and alveolar hypoventilation. Q J Med 45:87–110
Newsom-Davis J, Loh L (1979) Alveolar hypoventilation and respiratory muscle weakness. Bull Eur Physiopathol Respir 15(suppl):45–51
Riley DJ, Santiago TV, Daniele RP, Schall B, Edelman NH (1977) Blunted respiratory drive in congenital myopathy. Am J Med 63:459–466
Ringquist T (1966) The ventilatory capacity in healthy subjects. Scand J Clin Invest 18(suppl):1–179
Rochester DF, Arora NS, Braun NMT (1981) Maximal contractile force of human diaphragm muscle determined in vivo. Trans Am Clin Climatol Assoc 93:200–206
Rochester DF (1988) Tests of respiratory muscle function. Clin Chest Med 9:249–262
Rosenow EC, Engel AG (1978) Acid maltase deficiency in adults presenting as respiratory failure. Am J Med 64:485–491
Roussos C (1982) The failing ventilatory pump. Lung 160:59–84
Roussos CS, Macklem PT (1977) Diaphragmatic fatigue in man. J Appl Physiol 43:189–197
Roussos C, Moxham J (1985) Respiratory muscle fatigue. In: Roussos C, Macklem PT (eds) The thorax, part B, vol. 29. Marcel Dekker, New York, pp 829–870
Simpson JA (1981) Myasthenia gravis and myasthenic syndromes. In: Walton JN (ed) Disorders of voluntary muscle, 4th ed. Churchill Livingstone, Edinburgh, pp 585–624
Skatrud J, Iber C, McHugh W, Rasmussen H, Nichols D (1980) Determinants of hypoventilation during wakefulness and sleep in diaphragmatic paralysis. Am Rev Respir Dis 121:587–593
Smyth RJ, Chapman KR, Rebuck AS (1984) Maximal inspiratory and expiratory pressures in adolescents. Chest 86:568–572
Stalberg E, Sanders DB (1981) Electrophysiological tests of neuromuscular transmission. In: Stalberg E, Young RR (eds) Clinical neurophysiology. Butterworths, London, pp 88–116
Vincken W, Ghezzo H, Cosio MG (1987) Maximal static respiratory pressures in adults: normal values and their relationship to determinants of respiratory function. Bull Eur Physiopathol Respir 23:435–439
Wicks AB, Menter RR (1986) Long-term outlook in quadriplegic patients with initial ventilatory dependency. Chest 90:406–410
Wilcox PG, Baile EM, Hards J, Muller NL, Dunn L, Pardy RL, Pare PD (1988) Phrenic nerve function and its relationship to atelectasis after coronary artery bypass surgery. Chest 93:693–698
Wilcox PG, Morrison NJ, Anzarut RA, Pardy RL (1988) Lambert Eaton myasthenic syndrome involving the diaphragm. Chest 93:604–606
Wilcox PG, Stein HB, Clarke, SD, Pare PD, Pardy RL (1988) Phrenic nerve function in patients with diaphragmatic weakness and systemic lupus erythematosus. Chest 93:352–358
Wynn-Williams N (1954) Hemidiaphragmatic paralysis and paresis of unknown aetiology without any marked rise in level. Thorax 9:299–303
Young RR, Bradley WG, Adams RD (1980) Approach to clinical myology. In: Isselbacker KJ, Adams RD, Braunwald E, et al (eds) Harrison’s principles of internal medicine. McGraw-Hill, New York, pp 2040–2053
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Wilcox, P.G., Pardy, R.L. Diaphragmatic weakness and paralysis. Lung 167, 323–341 (1989). https://doi.org/10.1007/BF02714961
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF02714961