1297 blame everyone but themselves-the Health Departments who simply outwitted them and the Review Body which has done precisely what it said it would do. A year ago I predicted what would happen and that, as a consequence, the Review Body system itself would be placed in danger.’ As your readers may know I have never been a supporter of the D.H.S.S. or the Review Body but in my view the real fault lies with our negotiators who chose to mislead the profession and repeatedly and deliberately ignored the warnings of the dangers they themselves were creating for us all. I will take no action whatsoever to save their faces or their necks: the profession’s negotiators should admit that the mistake was theirs and resign. to
Guy’s Hospital,
N. A. SIMMONS
London SE1
*** The British Medical Association’s reactions discussed by Body’s report dent on p. 1304.-ED.L. are
our
to
Parliamentary
the Review correspon-
PSYCHIATRIC DISORDERS FOLLOWING INFANTILE SPASMS
SIR,—Infantile spasms (West’s syndrome)2may present with comparatively mild symptoms usually in the first year of life. The attacks, often called "salaam" or "jack-knife" are brief, each lasting about a second and practically always occurring at intervals of seconds over a period of several minutes. When they appear in an otherwise healthy child these spasms may be dismissed as "colic" or "wind" by parents and sometimes by inexperienced doctors, until mental and motor regression becomes obvious, revealing the true gravity of the illness 3-y We would like to report some preliminary findings of a long-term follow-up of patients referred to the Department of Clinical Neurophysiology, Hospital for Sick Children, with the infantile-spasm syndrome, from July, 1960, to December, 1963. The first 100 patients traced by 1978 form the basis of this study; follow-up was 15-18 years, and the patients were classified as follows: Group A-24 were found to have died. Group B-31 patients in late adolescence were in permanent care in hospitals for the mentally subnormal. Group C-17 patients in essentially the same clinical state as in group B but being cared for at home by their parents. Group D-20 patients had made a reasonably good recovery though with various residual disabilities. Group E-Only 8 patients had made a good recovery. The question of early and appropriate treatment cannot be discussed in a brief note. No necropsies had been done on any of the 10 children who had died in institutions for the mentally subnormal while only 3 necropsies had been done in the remaining 14 cases of group A. The 48 patients in groups B and C were severely disabled, many bed-ridden or chair-bound, unable to speak, incontinent, incapable of any self care, and having to be fed. Some, who had originally recovered enough to be able to walk, were confined to bed or wheelchair because of deformities and contractures developed over the years despite surgery, splints, and other appliances. Some appeared blind and/or deaf but speech was invariably affected even though in 18 patients some degree of verbal comprehension and ability to communicate by gesture was preserved. In the more severely affected, however,
1. Simmons, N. A. On Call, June 8, 1978, p.7. 2. West, W. J. Lancet, 1841, i, 724. 3. Gibbs, E. L., Fleming, M. M., Gibbs, F. A. Pediatrics, 1954, 33, 66. 4.Sorel, L., Dusaucy-Bauloye, A. Acta neurol, psychiat. belg. 1958, 58, 130. 5. Jeavons, P. M., Bower, B. D. Clin. devel. Med. 1964, no. 15. 6. della Rovere, M., Hoare, R. D., Pampiglione, G. Devel. Med Child Neurol.
1964, 6, 149. E., Pampiglione, G. ibid. 1964, 6, 149. 8. Pampiglione, G., Moynahan, E. J., J. Neurol. Neurosurg. Psychiat. 1976, 39, 666. 9. Jeavons, P. M., Bower, B. D., Dimitrakoudi, M. Epilepsia, 1973, 14, 153.
7. Friedman, I.
there was little, if any, contact with surroundings or recognition of parents or nurses. It was difficult in these severely handicapped children to differentiate the psychiatric problems, which became more prominent with increasing age, from the persistent motor and mental defects. The 20 patients in group D had recovered from the spasms and other seizures with gradual resumption of developmental progress, even though this was delayed. The residual neurological handicap was limited to mild athetosis, clumsiness or some degree of spasticity. All were educable, attaining at least schooling for educationally subnormal and 10 reached the lower streams of normal schools. All were able to speak though many had slight stammering, echolalia, or word substitution. However, in group D psychiatric disorders were a distinct feature with greater or lesser hyperactivity, temper tantrums, aggressive/destructive behaviour, pica, bouts of head-banging or rocking, gaze avoidance, and hand regard, generally considered characteristic of autistic behaviour. Over the years, most patients in group D had been treated for emotional problems in child-guidance clinics. It seems probable that these psychiatric manifestations were caused by the same braindisease process which, at an earlier age, had appeared in the form of the "infantile spasms syndrome" with severe E.E.G. abnormalities. The general neurological features and the mental handicap in the present series were on the whole similar to those of previous studies (see Lacy and Penry’s review’O) and therefore not entirely unexpected. We had not, however, anticipated the large number of psychiatric sequelae in the less badly affected survivors, and we do not think that selective referral to this hospital could account for these findings. This aspect has received little attention in the literature. Our study suggests that the infantile-spasms syndrome is still insufficiently understood in respect of aetiology, epidemiology, evolution (including psychiatric manifestations), and biochemical and histopathological features. A prospective research programme should be considered to assess the incidence of the condition in Britain and its implications, to establish a national register, to provide facilities for early or even presymptomatic detection, and to rationalise treatment in the various phases of evolution. We thank our medical colleagues who answered our inquiries, convaluable information, and the medical records officers in many hospitals, especially Miss P. Corns.
tributing
Department of Clinical Neurophysiology, Hospital for Sick Children,
E. M. THORNTON G. PAMPIGLIONE
London WC1
ORAL PROSTAGLANDIN E2 IN PULMONARY ATRESIA
SIR,-Short-term intravenous prostaglandin E1
or
E2
is used in some centres to maintain the patency of the ductus arteriosus in neonates who have no other source of pulmonary blood-flow. It is generally used for 24-48 h while cardiac surgery is arranged. The increased pulmonary blood-flow results in improved tissue oxygenation, permitting correction of metabolic acidosis and making surgery less risky.’-’ The longest reported infusion of p.G.Ej has been 29 days.8 Long-term p.G.E may be beneficial in certain cases but there are practical limitations to intra-aortic or intravenous in-
(P.G.E1
10.
or
P.G.E2)
Lacy, J. R., Penry, J. K. Infantile Spasms, New York, 1976. Starling, M, B., Neutz, J. M. Lancet, 1975, i, 140.
1. Elliot, R., R, B.,
2. Christen, N C, Fabricus, J ibid 1975, ii, 406. 3. Olley, P. M. Coceani, F., Bodach, E Circulation, 1976, 53, 728. M. Pediatrics, 1977, 59, 325. 4. Heymann, M. A., Rudolph, A. 5. Moulaert, A., Senders, R., van Ertbruggen, I , Huysmans, H., Harinck, E.
Eur. J. Cardiol. 1977, 5, 321. J. M., Starling, M B., Elliot, 1977, 55, 238
6. Neutz 7.
8.
Lewis,. A. L., Takahashi, M.,
R B.,
Barrat-Boyes, B. G. Circulation,
Lurie, P. R. J. Pediat. 1978, 93, 481.
Lewis,. A. L., Lurie, P.R. Pediatrics,
1978, 61, 524.
Guy’s Hospital,
N. A. SIMMONS
London SE1
*** The British Medical Association’s reactions discussed by Body’s report dent on p. 1304.-ED.L. are
our
to
Parliamentary
the Review correspon-
PSYCHIATRIC DISORDERS FOLLOWING INFANTILE SPASMS
SIR,—Infantile spasms (West’s syndrome)2may present with comparatively mild symptoms usually in the first year of life. The attacks, often called "salaam" or "jack-knife" are brief, each lasting about a second and practically always occurring at intervals of seconds over a period of several minutes. When they appear in an otherwise healthy child these spasms may be dismissed as "colic" or "wind" by parents and sometimes by inexperienced doctors, until mental and motor regression becomes obvious, revealing the true gravity of the illness 3-y We would like to report some preliminary findings of a long-term follow-up of patients referred to the Department of Clinical Neurophysiology, Hospital for Sick Children, with the infantile-spasm syndrome, from July, 1960, to December, 1963. The first 100 patients traced by 1978 form the basis of this study; follow-up was 15-18 years, and the patients were classified as follows: Group A-24 were found to have died. Group B-31 patients in late adolescence were in permanent care in hospitals for the mentally subnormal. Group C-17 patients in essentially the same clinical state as in group B but being cared for at home by their parents. Group D-20 patients had made a reasonably good recovery though with various residual disabilities. Group E-Only 8 patients had made a good recovery. The question of early and appropriate treatment cannot be discussed in a brief note. No necropsies had been done on any of the 10 children who had died in institutions for the mentally subnormal while only 3 necropsies had been done in the remaining 14 cases of group A. The 48 patients in groups B and C were severely disabled, many bed-ridden or chair-bound, unable to speak, incontinent, incapable of any self care, and having to be fed. Some, who had originally recovered enough to be able to walk, were confined to bed or wheelchair because of deformities and contractures developed over the years despite surgery, splints, and other appliances. Some appeared blind and/or deaf but speech was invariably affected even though in 18 patients some degree of verbal comprehension and ability to communicate by gesture was preserved. In the more severely affected, however,
1. Simmons, N. A. On Call, June 8, 1978, p.7. 2. West, W. J. Lancet, 1841, i, 724. 3. Gibbs, E. L., Fleming, M. M., Gibbs, F. A. Pediatrics, 1954, 33, 66. 4.Sorel, L., Dusaucy-Bauloye, A. Acta neurol, psychiat. belg. 1958, 58, 130. 5. Jeavons, P. M., Bower, B. D. Clin. devel. Med. 1964, no. 15. 6. della Rovere, M., Hoare, R. D., Pampiglione, G. Devel. Med Child Neurol.
1964, 6, 149. E., Pampiglione, G. ibid. 1964, 6, 149. 8. Pampiglione, G., Moynahan, E. J., J. Neurol. Neurosurg. Psychiat. 1976, 39, 666. 9. Jeavons, P. M., Bower, B. D., Dimitrakoudi, M. Epilepsia, 1973, 14, 153.
7. Friedman, I.
there was little, if any, contact with surroundings or recognition of parents or nurses. It was difficult in these severely handicapped children to differentiate the psychiatric problems, which became more prominent with increasing age, from the persistent motor and mental defects. The 20 patients in group D had recovered from the spasms and other seizures with gradual resumption of developmental progress, even though this was delayed. The residual neurological handicap was limited to mild athetosis, clumsiness or some degree of spasticity. All were educable, attaining at least schooling for educationally subnormal and 10 reached the lower streams of normal schools. All were able to speak though many had slight stammering, echolalia, or word substitution. However, in group D psychiatric disorders were a distinct feature with greater or lesser hyperactivity, temper tantrums, aggressive/destructive behaviour, pica, bouts of head-banging or rocking, gaze avoidance, and hand regard, generally considered characteristic of autistic behaviour. Over the years, most patients in group D had been treated for emotional problems in child-guidance clinics. It seems probable that these psychiatric manifestations were caused by the same braindisease process which, at an earlier age, had appeared in the form of the "infantile spasms syndrome" with severe E.E.G. abnormalities. The general neurological features and the mental handicap in the present series were on the whole similar to those of previous studies (see Lacy and Penry’s review’O) and therefore not entirely unexpected. We had not, however, anticipated the large number of psychiatric sequelae in the less badly affected survivors, and we do not think that selective referral to this hospital could account for these findings. This aspect has received little attention in the literature. Our study suggests that the infantile-spasms syndrome is still insufficiently understood in respect of aetiology, epidemiology, evolution (including psychiatric manifestations), and biochemical and histopathological features. A prospective research programme should be considered to assess the incidence of the condition in Britain and its implications, to establish a national register, to provide facilities for early or even presymptomatic detection, and to rationalise treatment in the various phases of evolution. We thank our medical colleagues who answered our inquiries, convaluable information, and the medical records officers in many hospitals, especially Miss P. Corns.
tributing
Department of Clinical Neurophysiology, Hospital for Sick Children,
E. M. THORNTON G. PAMPIGLIONE
London WC1
ORAL PROSTAGLANDIN E2 IN PULMONARY ATRESIA
SIR,-Short-term intravenous prostaglandin E1
or
E2
is used in some centres to maintain the patency of the ductus arteriosus in neonates who have no other source of pulmonary blood-flow. It is generally used for 24-48 h while cardiac surgery is arranged. The increased pulmonary blood-flow results in improved tissue oxygenation, permitting correction of metabolic acidosis and making surgery less risky.’-’ The longest reported infusion of p.G.Ej has been 29 days.8 Long-term p.G.E may be beneficial in certain cases but there are practical limitations to intra-aortic or intravenous in-
(P.G.E1
10.
or
P.G.E2)
Lacy, J. R., Penry, J. K. Infantile Spasms, New York, 1976. Starling, M, B., Neutz, J. M. Lancet, 1975, i, 140.
1. Elliot, R., R, B.,
2. Christen, N C, Fabricus, J ibid 1975, ii, 406. 3. Olley, P. M. Coceani, F., Bodach, E Circulation, 1976, 53, 728. M. Pediatrics, 1977, 59, 325. 4. Heymann, M. A., Rudolph, A. 5. Moulaert, A., Senders, R., van Ertbruggen, I , Huysmans, H., Harinck, E.
Eur. J. Cardiol. 1977, 5, 321. J. M., Starling, M B., Elliot, 1977, 55, 238
6. Neutz 7.
8.
Lewis,. A. L., Takahashi, M.,
R B.,
Barrat-Boyes, B. G. Circulation,
Lurie, P. R. J. Pediat. 1978, 93, 481.
Lewis,. A. L., Lurie, P.R. Pediatrics,
1978, 61, 524.
