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Diaphragmatic flutter with an electromyographic study
Volume 73 Number 5
Brief clinicat and laboratory observations
raises d o u b t r e g a r d i n g t h e statistical significance of these associations. T h e q u e s t i o n re-: m a i n s w h e t h e r other m a j o r or m i n o r disorders a r e m o r e f r e q u e n t in i n d i v i d u a l s w i t h nevus f l a m m e u s of the forehead. N e v u s flamm e u s n u c h a e deserves similar close scrutiny.
REFERENCES 1. Andrews, G. C., and Domonkos, A. N.: Diseases of the skin, Philadelphia, 1963, W. B. Saunders Company, p. 507.
Diaphragmatic flutter ith an electromyographic study Henrique Rigatto, M.D.,'* and Carlos Elysio C a s t r o C o r r e a , M.D. RIBE, IRAO
PRETO~
BRAZIL
T H E N A M e " d i a p h r a g m a t i c flutter" was i n t r o d u c e d by P o r t e r * in 1936 to describe a n u n u s u a l clonic d i s t u r b a n c e of d i a p h r a g m a t i c motility characterized by p a r o x y s m a l c o n t r a c tions w h i c h are g e n e r a l l y irregular~ a n d either s u p e r i m p o s e d o.n or r e p l a c i n g the, n o r m a l activity of the muscle. T h i s s y n d r o m e is particularly rare i n c h i l d h o o d ; one 9 - m o n t h - o l d i n f a n t has b e e n r e p o r t e d previously. 2 T h e p u r p o s e of this p a p e r is to p r e s e n t the case of a n 8-year-old boy w i t h d i a p h r a g m a t i c flutter, illustrated by e l e c t r o m y o g r a p h i c studies of the d i a p h r a g m a n d a b d o m i n a l muscles.
CASE REPORT This 8-year-old Caucasian boy was admitted to the University Hospital of Ribeirgo Preto in S~o Paulo in January, 1966, with the chief complaint of an uncontrollable coarse tremor of the
From the Department of Pediatrics, University of Sffo Paulo, Ribeirao Preto, Brazil. ~Present address: Department of Pediatrics University of California-San F~'ancisco Medical Center," S~n Francisco, Calif. 94122.
75 7
22 Taussig, H. B.: A study of the German outbreak of phocomelia, J. A. M. A. 180: 1106, 1962. 3. Patau, K., Smith, D. W., Therman, E., Inhorn, S. L., and Wagner, H. P.: Multiple congenital anomaly caused by an extra autosome, Lancet 1: 790, 1960. 4. Leao, J. C., et al.: New syndrome associated with partial deletion of short arms of chromosome No. 4, J. A. M. A. 202: 434, 1967. 5. Shafar, J., and Doig, A.: The "nape nevus," Lancet 1: 913, 1955.
upper abdominal wall which had been present since he was 3 years of age. These involuntary movements were exacerbated during anxiety and disappeared when he was relaxed and during sleep. Past history was irrelevant except for incision of a subcutaneous abscess in the right upper part of the abdomen at 2 years of age. Physical examination showed that he was in no acute distress, weighed 21.6 kilograms, was 120 cm. tall, and had a temperature of 36.5 ~ C. and a respiratory rate of 24 inspirations per minute. Auscultation of the chest revealed a lowfrequency m u r m u r unrelated to cardiac activity along the lower costal margin, more evident at tl~e midaxillary line. Coarse jerking movements were visible in the upper abdomen, mostly in the epigastrium but also involving all of the musculature around the diaphragmatic insertions, including the paravertebral regon. The movements were irregadar and occurred at a rate of approximately 100 per minute. The abdominal wall was tense; no abdominal masses were detected. The results of neurologic exainination were normal. The results of spirometry were also normal (vital capacity, 1,400 ml.; expiratory reserve volume, 300 ml.; inspiratory capacity, 1,100 ml.; maximal breathing capacity, 60 L per minute; forced expiratory volume, 90 per cent in I second), as were roentgenograms of the chest and abdomen. Pneumoperitoneum showed no adhesions between the diaphragmatic and abdominal muscles. Fluoroscopy confirmed the irregular tremor of both diaphragmatic leaves, each oscillation having an amplitude of less than 1 cm. Neither chlordiazepoxide hydrochloride nor carbon dioxide was effective in stopping the flutter. Blockade of the phrenic nerve was believed t o ' b e unjustified because the involvement was not severe. Electromyography. The technique used in
758
Brief clinical and laboratory observations
The Journal of Pediatrics November 1968
Fig. 1. Correlation between electrical activity of diaphragm and movements (solid line) of the chest (AI, A..) and abdominal wall (B1, B~, B~). A1, B~: Flutter absent, with muscular electrical activity restricted to inspiration; only the electrocardiogram is seen on expiration. A2: Flutter present (intense abnormal electrical potentials present in inspiration and expiration). B~, B3: Flutter present (abnormal electrical activity appears simultaneously with abnormal abdominal movements).
Fig. 2. Correlation between electromyographic recording and muscular movements (solid line) in a well-localized area of the upper right rectus abdominis. A: Flutter absent (no electrical potentials are seen and muscular record illustrates the respiratory movements). B: Start of flutter (minimal electrical potentials and muscular movements are becoming abnormal). C: Flutter evident (intense electrical potentials and muscular movements entirely abnormal, with disappearance of normal respiratory pattern).
electromyography of the diaphragm was similar to that described previously, a A catheter with four silver electrodes (3 mm. in diameter and 1 cm. apart) was introduced into the esophagus via the nose, with the patient in a semirecumbent position. The catheter was pushed down gently until a suitable electromyogram was obtained. Radiologic control was not used because the diaphragmatic potentials are very characteristic and there is only one position from which they can be recorded from the esophagus. In the present case the position corresponded to a distance of 44 cm. from the distal electrode to the nostril. The abdominal electrical activity was recorded
through a needle inserted in the right rectus abdominis muscle in the epigastrium, but on a few occasions was also checked in other involved abdominal muscles. Signals were amplified by an R-C amplifier (Grass P-5), recorded on a dual beam oscilloscope (Tektronix 502A) and photographed with a camera (Grass C-4). Muscular movements were recorded (Fig. 1) either with a pneumograph placed around the thorax or abdominal wall or, when analysis was in a more localized area (Fig. 2), with a funnel connected to a Statham transducer. In Fig. 2, C, the electrical potentials correspond to upward deflections in the muscular tracing. If these potentials .had
Volume 73 Number 5
9expressed isolated abdominal contraction, the muscular deflections would have been negative by the method employed; since they are positive, they represent the distension of the abdominal wall, which is most likely due to the diaphragmatic flutter. COMMENT I n the extensive review of the literature made by Rigatto and De Medeiros, 4 and in recent case reports, 4-7 there is no reference to the electrical nature of the disturbance in muscular activity of the d i a p h r a g m or abdominal muscles; furthermore, it is suggested t h a t the abdominal movements are passive. This is actually the most frequently observed sign and is generally the key to the diagnosis. I n the electrom:fographic study, two interesting facts were observed: (1) the intraesophageal potentials showed that the diaphragm is controlled by abnormal electrical activity (Fig. 1), and (2) the muscles surrounding the diaphragmatic insertion contract in an abnormally active way (Fig. 2). Although further experiments will be necessary to elucidate the basic failure of regulation which is present in flutter, this has been repeatedly attributed to an abnormal excitability of the phrenic nerve in its central origin or along its path. Since abdominal movements are also active, a question now arises in regard to the mechanism, since apparently no previous studies have shown any relationship between the phrenic branches and the abdominal muscles, s These abdominal movements might be: (1) a reaction to increased abdominal pressure caused by irregular movements of the diaphragm; (2) reflex contractions of abdominal muscles, particularly of the transversus abdominis, because its insertions are interposed with those of the diaphragm; (3) completely in-
Brie[ clinical and laboratory observations
759
dependent activity of the diaphragm and abdominal muscles. This third possibility seems to be least likely because abdominal movements occur simultaneously with diaphragmatic activity (Fig. 1, B.~ and B3). SUMMARY
A case of diaphragmatic flutter was demonstrated in an 8-year-old boy by fluoroscopy and by electromyographic study of the diap h r a g m and abdominal muscles, both of which showed an entirely abnormal electrical pattern. T h e observation that the abdominal movements are active suggests that abnormal excitability of the phrenic nerve cannot entirely explain the pathologic mechanism of this disease. We are grateful to Dr. Eduardo M. Krieger for his invaluable assistance in the electromyographic study. REFERENCES
1. Porter, W. B.: Diaphragmatic flutter with symptoms of angina pectorls, J. A. M. A. 106: 992, 1936. 2. Kulencamp, G.: Zwerchfeelltremor nach Grippe, Deutsch. Ztsehr. Nervenh. 94" 312, 1926. 3. Agostoni, E., Sant'Ambrogio, G., and Carrasco, I~. del P.: Electromyography of the diaphragm in man and transdiaphragmatic pressure, J. Appl . Physlol. 15" 1093, 1960. 4. Rigatto, M., and DeMedeiros, N. P.: Diaphragmatic flutter: Report of a case and review of literature, Am, J. Med. 32" 103, 1962. 5. Ting, E. Y., Karliner, J. S., and Williams, M. H., Jr.: Diaphragmatic flutter associated w~th apneusfic respiration, Am. Rev. Resp. Dis. 88" 833, 1963. 6. Dressier, W., Deduline, V., and Schub, H.: Diaphragmatic flutter associated with rheumatic heart disease, J. A. M. A. 186: 151, 1963. 7. Graber, A. L., and Sinclair-Smith, B. C.: Paroxysmal flutter of the diaphragm: report of five cases, Am. J. Cardiol. 15" 252, 1965. 8. Annotation: Diaphragmatic flutter, Lancet 1: 732, 1962.
Brief clinicat and laboratory observations
raises d o u b t r e g a r d i n g t h e statistical significance of these associations. T h e q u e s t i o n re-: m a i n s w h e t h e r other m a j o r or m i n o r disorders a r e m o r e f r e q u e n t in i n d i v i d u a l s w i t h nevus f l a m m e u s of the forehead. N e v u s flamm e u s n u c h a e deserves similar close scrutiny.
REFERENCES 1. Andrews, G. C., and Domonkos, A. N.: Diseases of the skin, Philadelphia, 1963, W. B. Saunders Company, p. 507.
Diaphragmatic flutter ith an electromyographic study Henrique Rigatto, M.D.,'* and Carlos Elysio C a s t r o C o r r e a , M.D. RIBE, IRAO
PRETO~
BRAZIL
T H E N A M e " d i a p h r a g m a t i c flutter" was i n t r o d u c e d by P o r t e r * in 1936 to describe a n u n u s u a l clonic d i s t u r b a n c e of d i a p h r a g m a t i c motility characterized by p a r o x y s m a l c o n t r a c tions w h i c h are g e n e r a l l y irregular~ a n d either s u p e r i m p o s e d o.n or r e p l a c i n g the, n o r m a l activity of the muscle. T h i s s y n d r o m e is particularly rare i n c h i l d h o o d ; one 9 - m o n t h - o l d i n f a n t has b e e n r e p o r t e d previously. 2 T h e p u r p o s e of this p a p e r is to p r e s e n t the case of a n 8-year-old boy w i t h d i a p h r a g m a t i c flutter, illustrated by e l e c t r o m y o g r a p h i c studies of the d i a p h r a g m a n d a b d o m i n a l muscles.
CASE REPORT This 8-year-old Caucasian boy was admitted to the University Hospital of Ribeirgo Preto in S~o Paulo in January, 1966, with the chief complaint of an uncontrollable coarse tremor of the
From the Department of Pediatrics, University of Sffo Paulo, Ribeirao Preto, Brazil. ~Present address: Department of Pediatrics University of California-San F~'ancisco Medical Center," S~n Francisco, Calif. 94122.
75 7
22 Taussig, H. B.: A study of the German outbreak of phocomelia, J. A. M. A. 180: 1106, 1962. 3. Patau, K., Smith, D. W., Therman, E., Inhorn, S. L., and Wagner, H. P.: Multiple congenital anomaly caused by an extra autosome, Lancet 1: 790, 1960. 4. Leao, J. C., et al.: New syndrome associated with partial deletion of short arms of chromosome No. 4, J. A. M. A. 202: 434, 1967. 5. Shafar, J., and Doig, A.: The "nape nevus," Lancet 1: 913, 1955.
upper abdominal wall which had been present since he was 3 years of age. These involuntary movements were exacerbated during anxiety and disappeared when he was relaxed and during sleep. Past history was irrelevant except for incision of a subcutaneous abscess in the right upper part of the abdomen at 2 years of age. Physical examination showed that he was in no acute distress, weighed 21.6 kilograms, was 120 cm. tall, and had a temperature of 36.5 ~ C. and a respiratory rate of 24 inspirations per minute. Auscultation of the chest revealed a lowfrequency m u r m u r unrelated to cardiac activity along the lower costal margin, more evident at tl~e midaxillary line. Coarse jerking movements were visible in the upper abdomen, mostly in the epigastrium but also involving all of the musculature around the diaphragmatic insertions, including the paravertebral regon. The movements were irregadar and occurred at a rate of approximately 100 per minute. The abdominal wall was tense; no abdominal masses were detected. The results of neurologic exainination were normal. The results of spirometry were also normal (vital capacity, 1,400 ml.; expiratory reserve volume, 300 ml.; inspiratory capacity, 1,100 ml.; maximal breathing capacity, 60 L per minute; forced expiratory volume, 90 per cent in I second), as were roentgenograms of the chest and abdomen. Pneumoperitoneum showed no adhesions between the diaphragmatic and abdominal muscles. Fluoroscopy confirmed the irregular tremor of both diaphragmatic leaves, each oscillation having an amplitude of less than 1 cm. Neither chlordiazepoxide hydrochloride nor carbon dioxide was effective in stopping the flutter. Blockade of the phrenic nerve was believed t o ' b e unjustified because the involvement was not severe. Electromyography. The technique used in
758
Brief clinical and laboratory observations
The Journal of Pediatrics November 1968
Fig. 1. Correlation between electrical activity of diaphragm and movements (solid line) of the chest (AI, A..) and abdominal wall (B1, B~, B~). A1, B~: Flutter absent, with muscular electrical activity restricted to inspiration; only the electrocardiogram is seen on expiration. A2: Flutter present (intense abnormal electrical potentials present in inspiration and expiration). B~, B3: Flutter present (abnormal electrical activity appears simultaneously with abnormal abdominal movements).
Fig. 2. Correlation between electromyographic recording and muscular movements (solid line) in a well-localized area of the upper right rectus abdominis. A: Flutter absent (no electrical potentials are seen and muscular record illustrates the respiratory movements). B: Start of flutter (minimal electrical potentials and muscular movements are becoming abnormal). C: Flutter evident (intense electrical potentials and muscular movements entirely abnormal, with disappearance of normal respiratory pattern).
electromyography of the diaphragm was similar to that described previously, a A catheter with four silver electrodes (3 mm. in diameter and 1 cm. apart) was introduced into the esophagus via the nose, with the patient in a semirecumbent position. The catheter was pushed down gently until a suitable electromyogram was obtained. Radiologic control was not used because the diaphragmatic potentials are very characteristic and there is only one position from which they can be recorded from the esophagus. In the present case the position corresponded to a distance of 44 cm. from the distal electrode to the nostril. The abdominal electrical activity was recorded
through a needle inserted in the right rectus abdominis muscle in the epigastrium, but on a few occasions was also checked in other involved abdominal muscles. Signals were amplified by an R-C amplifier (Grass P-5), recorded on a dual beam oscilloscope (Tektronix 502A) and photographed with a camera (Grass C-4). Muscular movements were recorded (Fig. 1) either with a pneumograph placed around the thorax or abdominal wall or, when analysis was in a more localized area (Fig. 2), with a funnel connected to a Statham transducer. In Fig. 2, C, the electrical potentials correspond to upward deflections in the muscular tracing. If these potentials .had
Volume 73 Number 5
9expressed isolated abdominal contraction, the muscular deflections would have been negative by the method employed; since they are positive, they represent the distension of the abdominal wall, which is most likely due to the diaphragmatic flutter. COMMENT I n the extensive review of the literature made by Rigatto and De Medeiros, 4 and in recent case reports, 4-7 there is no reference to the electrical nature of the disturbance in muscular activity of the d i a p h r a g m or abdominal muscles; furthermore, it is suggested t h a t the abdominal movements are passive. This is actually the most frequently observed sign and is generally the key to the diagnosis. I n the electrom:fographic study, two interesting facts were observed: (1) the intraesophageal potentials showed that the diaphragm is controlled by abnormal electrical activity (Fig. 1), and (2) the muscles surrounding the diaphragmatic insertion contract in an abnormally active way (Fig. 2). Although further experiments will be necessary to elucidate the basic failure of regulation which is present in flutter, this has been repeatedly attributed to an abnormal excitability of the phrenic nerve in its central origin or along its path. Since abdominal movements are also active, a question now arises in regard to the mechanism, since apparently no previous studies have shown any relationship between the phrenic branches and the abdominal muscles, s These abdominal movements might be: (1) a reaction to increased abdominal pressure caused by irregular movements of the diaphragm; (2) reflex contractions of abdominal muscles, particularly of the transversus abdominis, because its insertions are interposed with those of the diaphragm; (3) completely in-
Brie[ clinical and laboratory observations
759
dependent activity of the diaphragm and abdominal muscles. This third possibility seems to be least likely because abdominal movements occur simultaneously with diaphragmatic activity (Fig. 1, B.~ and B3). SUMMARY
A case of diaphragmatic flutter was demonstrated in an 8-year-old boy by fluoroscopy and by electromyographic study of the diap h r a g m and abdominal muscles, both of which showed an entirely abnormal electrical pattern. T h e observation that the abdominal movements are active suggests that abnormal excitability of the phrenic nerve cannot entirely explain the pathologic mechanism of this disease. We are grateful to Dr. Eduardo M. Krieger for his invaluable assistance in the electromyographic study. REFERENCES
1. Porter, W. B.: Diaphragmatic flutter with symptoms of angina pectorls, J. A. M. A. 106: 992, 1936. 2. Kulencamp, G.: Zwerchfeelltremor nach Grippe, Deutsch. Ztsehr. Nervenh. 94" 312, 1926. 3. Agostoni, E., Sant'Ambrogio, G., and Carrasco, I~. del P.: Electromyography of the diaphragm in man and transdiaphragmatic pressure, J. Appl . Physlol. 15" 1093, 1960. 4. Rigatto, M., and DeMedeiros, N. P.: Diaphragmatic flutter: Report of a case and review of literature, Am, J. Med. 32" 103, 1962. 5. Ting, E. Y., Karliner, J. S., and Williams, M. H., Jr.: Diaphragmatic flutter associated w~th apneusfic respiration, Am. Rev. Resp. Dis. 88" 833, 1963. 6. Dressier, W., Deduline, V., and Schub, H.: Diaphragmatic flutter associated with rheumatic heart disease, J. A. M. A. 186: 151, 1963. 7. Graber, A. L., and Sinclair-Smith, B. C.: Paroxysmal flutter of the diaphragm: report of five cases, Am. J. Cardiol. 15" 252, 1965. 8. Annotation: Diaphragmatic flutter, Lancet 1: 732, 1962.