1346
fall in
patients in remission was noted in report16 though IgA antibody levels in a proportion of long-term survivors remained consistent to
N.P.c.
one
Recurrent or metastatic tumour was some of these cases but others have remained. apparently well. In another account,t5 relapse-free survivors were tested 1-12 years after radiotherapy and most of them proved to have detectable levels of viral IgA antibody in their sera. It was concluded that the antibody titres "remained relatively unchanged in individual NPC patients after radiotherapy, regardless of the disease evolution". Although unlikely to be useful in assessing the course of the disease, these IgA antibodies are valuable in the diagnosis of patients with suspected N.P.C.—particularly those with an occult primary lesion who present with infiltrative tumour, causing cranial-nerve palsies, and metastatic disease in cervical lymph-nodes. or even rose.
later found in
Further
have revealed E.B. viralantibodies in concentrated saliva and in capsid IgA throat-washings from a high proportion of N.P.C. patients from Hong Kong and Tunisia.17,18 In many instances these antibodies seemed to interfere with the isolation of E.B. virus. 18 No virus-specific IgA antibodies were found in throat-washings from patients with other E.B.-virus-associated conditions (Burkitt lymphoma, infectious mononucleosis), nor in patients with other carcinomas or immune deficiency states, nor in healthy controls.
investigations
These various rigorously controlled investigations provide convincing evidence that increased levels of IgA antibodies directed against E.B. virus capsid antigen are "an outstanding feature"" of N.P.c. The diagnostic value of IgA antibody levels in N.P.C. patients with an occult primary tumour has already been noted, and another use is illustrated by cases where high levels of IgA antibody have been detected up to 61 months before the onset of any clinical symptoms.16,18,19 Raised IgA antibody titres have been reported in some ostensibly healthy members of the families of N.P.C. patients in Hong Kong-a finding which has epidemiological implications for screening of high-risk individuals, families, and populations.I4,15 The distribution of N.P.C. patients’ siblings with IgA antibodies suggests an autosomal trait,t5 consistent with genetic involvement in the IgA antibody response to E.B. viral-capsid antigen. An association between viral IgA antibody production and the distinctive HLA phenotype described in N.P.C.
Henle, W., Ho, J. H. C., Henle, G., Chau, J. C. W., Kwan, H. C. Int. J. Cancer, 1977, 20, 663. 17. Ho, H. C., Ng, M. H., Kwan, H. C. Br. J. Cancer, 1977, 35, 888. 18. Desgranges, C., De-Thé, G., Ho, H. C., Ellouz, R. Int. J. Cancer, 1977, 19, 16.
627. 19. Ho, H. C., Kwan, H.
C., Ng, M. H., De-Thé, G. Lancet, 1978, i, 436.
from Hong Kong20,21 should be explored. ’ The persistence of E.B. viral-capsid IgA antibodies in N.P.C. patients in prolonged remission is puzzling, and the proposalls that "the IgA antiVCA response is very sensitive in NPC, such that it is elicited whether the disease is clinical or subclinical" needs clarification. Another question is the source of the IgA antibodies: how much is produced locally in the oropharynx and how much systemically ? The presence of the secretory piece of the IgA molecule is crucial, but such evidence as we have is conflicting.1S,17 IgA-containing plasma cells accumulate in the vicinity of N.P.C. where they outnumber IgG-containing cells,22 but the specificity of the IgA is unknown. The parotid and other salivary glands are an additional source of IgA in the mouth and oropharynx, but specific IgA antibody synthesis by plasma cells in close contact with epithelial cells infected and transformed by E.B. virus is an attractive proposal.
patients
CLOSE ENCOUNTERS WITH CHILDHOOD EPILEPSY
"Epilepsy, cancer and leprosy-these are the three medical terms which rouse the greatest fear in the untutored". So the words of Graham Greene conclude a study of some social consequences of epilepsy in childhood. Nearly 1% of children have epilepsy, so most children’s doctors will have to help a child and its family to come to terms with it. How well do we do? Ward and Bower! from Oxford have investigated what happens after the diagnosis is made and control of fits attempted. Although most patients were attending a teaching-hospital psediatric neurology clinic they were believed to be representative of the local childhood epileptic population and their occupational-class distribution was similar to that of the region. Two groups of 30 and 51 patients were chosen--one of children diagnosed in the six months immediately preceding the study, the other of patients diagnosed up to 4 years previously. Therefore the immediate impact of epilepsy and later adjustments and attitudes could be assessed. Although most parents had previous experience of fits, many thought their child was dying in the first fit and it is unsurprising that fears about further fits and their complications were commonly and forcefully expressed. Prospects for employment and the possibility of mental handicap or personality change were more of a worry to parents than the dulling effects of drugs and the origins or inheritance of epilepsy. Most parents seemed to understand the diagnosis and could discuss it freely with the interviewer and 90% had explained to the child or siblings the reason for medication, but only a 20. Simons, M. J., Wee, G. B., Day, N. E., Morris, P. J., Shanmugaratnam, K., De-Thé, G. Int. J. Cancer, 1974, 13, 122. 21. Simons, M. J., Wee, G. B., Chan, S. H., Shanmugaratnam, K., Day, N. E., de Thé, G. Lancet, 1975, i, 142. 22. Ho, H. C., Kwan, H. C., Ng, M. H. Br. J. Cancer, 1978, 37, 514. 1.
Ward, F., Bower, B. D. Devel. Med. Chld Neurol. 1978, 20, suppl. 39.
1347 used the term epilepsy or fits in conversation. Ward and Bower do not mention parents’ groups or the British Epilepsy Association, both of which may have helped parents to come to terms with the condition. 37 patients had a background of family discord (broken marriage, unemployment, parental illness); in some this predated the fits but in 14 epilepsy was the alleged cause of the disruption. As often happens in other chronic childhood illnesses or handicap, epilepsy exposed rather than precipitated difficulties in some families. 29 mothers reported that their epileptic child was difficult to handle; in most of these the fits were incompletely controlled. It is the fear of fits that provokes most anxiety and when they are persistent and unpredictable, family life can be severely dislocated. Those children who were stable had no more restrictions on activity than would be appropriate for any child of the same age, and further prohibitions were more a reflection of dis-
minority
proportionate parental anxiety The attitudes of people and institutions outside the are sometimes more difficult to accept. Most parents confided in their own parents and they and other relatives would mind the child and deal with fits that occurred. Similarly of the 80% of parents who told acquaintances, most were met with understanding and help. Despite some parents’ fears, there was no excessive teasing of epileptic children; a few even seemed to revel in their newfound notoriety. 73 children attended school, nursery, or playgroup and most of these establishments accepted the epileptic child unreservedly, were prepared to deal with fits, and gave unobtrusive educational help where necessary. This positive attitude was seen as a powerful influence on the parents and their adjustments to epilepsy. The only worries were of exposure to physical danger in the gymnasium or the laboratory should a fit occur, and the tendency of a few schools to keep the academic pressure off these children. These conclusions are encouraging to doctors who manage childhood epilepsy since hitherto the decision about whether to confide in teachers was difficult. Frankness occasionally brought unfortunate and degrading consequences to the child: let us hope that the attitudes in Oxford reflect those in the rest of the country.
family
The main conclusion from this study is that reliable control of childhood epilepsy should allow satisfactory adjustment by the child, his parents, and the outside world and if this does not happen other sources of discord may be responsible. Conversely, the child whose fits are difficult to control may be the centre of anxiety and disharmony between parents, school, and doctors. It would be interesting to compare the anxieties of parents of "normal" children to see how much social disability exists in the community and whether chronic illness enhances or suppresses such background anxiety. Ward and Bower emphasise the importance of letting parents talk about their fears and uncertainties. Early involvement of a social worker and health visitor is suggested for families with problems, but routine referral is not advocated. Multidisciplinary teamwork is fine for some patients but since medical treatment determines the social prognosis the attentions of an unhurried and unchanging doctor are likely to be more effective-if less fashionable.
THE EXPERIENCE OF DYING PERHAPS Lazarus was the first man to be required to die twice, but the event of resuscitation after cardiac and respiratory arrest and other near-death experiences has become commonplace, and there are now many survivors. Several popular works, such as Moody’s bookl have presented anecdotes from these survivors as "exciting evidence of the survival of the human spirit beyond death", and even as proving the existence of an afterlife. Only a deliberate use of obsolete definitions of death can enable one to claim that anybody has, under clinical conditions, returned to tell us what lies beyond death, for by working definition, periodically updated, death is just beyond the point from which anyone can return to tell us anything. Collected accountsl-6volunteered by survivors do, however, bear striking similarities. Amongst the experiences many have described are an initial period of distress followed by profound calm and joy; out-of-the body experiences with the sense of watching resuscitation events from a distance; the sensation of moving rapidly down a tunnel or along a road, accompanied by a loud buzzing or ringing noise or hearing beautiful music; recognising friends and relatives who have died previously; a rapid visual review of pleasant incidents from throughout the life as a panoramic playback (in perhaps 12% of cases6); a sense of approaching a border or frontier and being sent back; and being annoyed or disappointed at having to return from such a pleasant experience-"I tried not to come back", in one patient’s words. Some describe frank transcendant experiences and many state that they will never fear death again. Similar stories have been reported from the victims of accidents, falls, drowning, anaphylaxis, and cardiac or respiratory arrest.
The
complete descriptions tend to come from rapidly afflicted and perceive their demise as inescapable and imminent. Where escape is possible but difficult, many or all features are missing, and with the concentration on avoiding death, panic and fear are more notable.6 The experiences are probably psychologically determined and defensive in function, and seem more dependent on the patient’s perception of his situation than on his physical or biochemical state. More careful collection of data and cross-cultural comparisons 7 may throw light on this matter. Of the less pleasant manifestations, Dlin et al. have described the "I am Dead" syndrome in which survivors of cardiac arrest may for some days after recovery claim that they are already dead. Cleveland and Johnson,9 investigating patients recovering from coronary thromboses, showed major discrepancies between the disturbing fantasies about death, as disclosed by detailed psychological testing, and the unperturbed front the patients maintained in what they said about themselves. most
those who
are
1. Moody, R. Life After Life. Atlanta, 1975. 2. Hunter, R. C. A. Am. J. Psychiat., 1967, 124, 84. 3. Noyes, R. Psychiatry, 1972, 35, 174. 4. Heim, A. Yearbook of the Swiss Alpine Club, 1892, 27, 327. 5. Pfister, O. Int. Z. Psychoanal. 1930, 16, 430. 6. Kalish, R. A. Death and Bereavement (edited by A. H. Kutscher); Springfield, Illinois, 1969. 7. Osis, K., Haraldsson, E. At the Hour of Death. New York, 1977. 8. Dlin, M. D., Stern, A., Poliakoff, S. J. Psychosomatics, 1974, 15, 61. 9. Cleveland, S. E., Johnson, D. L. Psychosom. Med. 1962, 24, 600.
p. 89.
fall in
patients in remission was noted in report16 though IgA antibody levels in a proportion of long-term survivors remained consistent to
N.P.c.
one
Recurrent or metastatic tumour was some of these cases but others have remained. apparently well. In another account,t5 relapse-free survivors were tested 1-12 years after radiotherapy and most of them proved to have detectable levels of viral IgA antibody in their sera. It was concluded that the antibody titres "remained relatively unchanged in individual NPC patients after radiotherapy, regardless of the disease evolution". Although unlikely to be useful in assessing the course of the disease, these IgA antibodies are valuable in the diagnosis of patients with suspected N.P.C.—particularly those with an occult primary lesion who present with infiltrative tumour, causing cranial-nerve palsies, and metastatic disease in cervical lymph-nodes. or even rose.
later found in
Further
have revealed E.B. viralantibodies in concentrated saliva and in capsid IgA throat-washings from a high proportion of N.P.C. patients from Hong Kong and Tunisia.17,18 In many instances these antibodies seemed to interfere with the isolation of E.B. virus. 18 No virus-specific IgA antibodies were found in throat-washings from patients with other E.B.-virus-associated conditions (Burkitt lymphoma, infectious mononucleosis), nor in patients with other carcinomas or immune deficiency states, nor in healthy controls.
investigations
These various rigorously controlled investigations provide convincing evidence that increased levels of IgA antibodies directed against E.B. virus capsid antigen are "an outstanding feature"" of N.P.c. The diagnostic value of IgA antibody levels in N.P.C. patients with an occult primary tumour has already been noted, and another use is illustrated by cases where high levels of IgA antibody have been detected up to 61 months before the onset of any clinical symptoms.16,18,19 Raised IgA antibody titres have been reported in some ostensibly healthy members of the families of N.P.C. patients in Hong Kong-a finding which has epidemiological implications for screening of high-risk individuals, families, and populations.I4,15 The distribution of N.P.C. patients’ siblings with IgA antibodies suggests an autosomal trait,t5 consistent with genetic involvement in the IgA antibody response to E.B. viral-capsid antigen. An association between viral IgA antibody production and the distinctive HLA phenotype described in N.P.C.
Henle, W., Ho, J. H. C., Henle, G., Chau, J. C. W., Kwan, H. C. Int. J. Cancer, 1977, 20, 663. 17. Ho, H. C., Ng, M. H., Kwan, H. C. Br. J. Cancer, 1977, 35, 888. 18. Desgranges, C., De-Thé, G., Ho, H. C., Ellouz, R. Int. J. Cancer, 1977, 19, 16.
627. 19. Ho, H. C., Kwan, H.
C., Ng, M. H., De-Thé, G. Lancet, 1978, i, 436.
from Hong Kong20,21 should be explored. ’ The persistence of E.B. viral-capsid IgA antibodies in N.P.C. patients in prolonged remission is puzzling, and the proposalls that "the IgA antiVCA response is very sensitive in NPC, such that it is elicited whether the disease is clinical or subclinical" needs clarification. Another question is the source of the IgA antibodies: how much is produced locally in the oropharynx and how much systemically ? The presence of the secretory piece of the IgA molecule is crucial, but such evidence as we have is conflicting.1S,17 IgA-containing plasma cells accumulate in the vicinity of N.P.C. where they outnumber IgG-containing cells,22 but the specificity of the IgA is unknown. The parotid and other salivary glands are an additional source of IgA in the mouth and oropharynx, but specific IgA antibody synthesis by plasma cells in close contact with epithelial cells infected and transformed by E.B. virus is an attractive proposal.
patients
CLOSE ENCOUNTERS WITH CHILDHOOD EPILEPSY
"Epilepsy, cancer and leprosy-these are the three medical terms which rouse the greatest fear in the untutored". So the words of Graham Greene conclude a study of some social consequences of epilepsy in childhood. Nearly 1% of children have epilepsy, so most children’s doctors will have to help a child and its family to come to terms with it. How well do we do? Ward and Bower! from Oxford have investigated what happens after the diagnosis is made and control of fits attempted. Although most patients were attending a teaching-hospital psediatric neurology clinic they were believed to be representative of the local childhood epileptic population and their occupational-class distribution was similar to that of the region. Two groups of 30 and 51 patients were chosen--one of children diagnosed in the six months immediately preceding the study, the other of patients diagnosed up to 4 years previously. Therefore the immediate impact of epilepsy and later adjustments and attitudes could be assessed. Although most parents had previous experience of fits, many thought their child was dying in the first fit and it is unsurprising that fears about further fits and their complications were commonly and forcefully expressed. Prospects for employment and the possibility of mental handicap or personality change were more of a worry to parents than the dulling effects of drugs and the origins or inheritance of epilepsy. Most parents seemed to understand the diagnosis and could discuss it freely with the interviewer and 90% had explained to the child or siblings the reason for medication, but only a 20. Simons, M. J., Wee, G. B., Day, N. E., Morris, P. J., Shanmugaratnam, K., De-Thé, G. Int. J. Cancer, 1974, 13, 122. 21. Simons, M. J., Wee, G. B., Chan, S. H., Shanmugaratnam, K., Day, N. E., de Thé, G. Lancet, 1975, i, 142. 22. Ho, H. C., Kwan, H. C., Ng, M. H. Br. J. Cancer, 1978, 37, 514. 1.
Ward, F., Bower, B. D. Devel. Med. Chld Neurol. 1978, 20, suppl. 39.
1347 used the term epilepsy or fits in conversation. Ward and Bower do not mention parents’ groups or the British Epilepsy Association, both of which may have helped parents to come to terms with the condition. 37 patients had a background of family discord (broken marriage, unemployment, parental illness); in some this predated the fits but in 14 epilepsy was the alleged cause of the disruption. As often happens in other chronic childhood illnesses or handicap, epilepsy exposed rather than precipitated difficulties in some families. 29 mothers reported that their epileptic child was difficult to handle; in most of these the fits were incompletely controlled. It is the fear of fits that provokes most anxiety and when they are persistent and unpredictable, family life can be severely dislocated. Those children who were stable had no more restrictions on activity than would be appropriate for any child of the same age, and further prohibitions were more a reflection of dis-
minority
proportionate parental anxiety The attitudes of people and institutions outside the are sometimes more difficult to accept. Most parents confided in their own parents and they and other relatives would mind the child and deal with fits that occurred. Similarly of the 80% of parents who told acquaintances, most were met with understanding and help. Despite some parents’ fears, there was no excessive teasing of epileptic children; a few even seemed to revel in their newfound notoriety. 73 children attended school, nursery, or playgroup and most of these establishments accepted the epileptic child unreservedly, were prepared to deal with fits, and gave unobtrusive educational help where necessary. This positive attitude was seen as a powerful influence on the parents and their adjustments to epilepsy. The only worries were of exposure to physical danger in the gymnasium or the laboratory should a fit occur, and the tendency of a few schools to keep the academic pressure off these children. These conclusions are encouraging to doctors who manage childhood epilepsy since hitherto the decision about whether to confide in teachers was difficult. Frankness occasionally brought unfortunate and degrading consequences to the child: let us hope that the attitudes in Oxford reflect those in the rest of the country.
family
The main conclusion from this study is that reliable control of childhood epilepsy should allow satisfactory adjustment by the child, his parents, and the outside world and if this does not happen other sources of discord may be responsible. Conversely, the child whose fits are difficult to control may be the centre of anxiety and disharmony between parents, school, and doctors. It would be interesting to compare the anxieties of parents of "normal" children to see how much social disability exists in the community and whether chronic illness enhances or suppresses such background anxiety. Ward and Bower emphasise the importance of letting parents talk about their fears and uncertainties. Early involvement of a social worker and health visitor is suggested for families with problems, but routine referral is not advocated. Multidisciplinary teamwork is fine for some patients but since medical treatment determines the social prognosis the attentions of an unhurried and unchanging doctor are likely to be more effective-if less fashionable.
THE EXPERIENCE OF DYING PERHAPS Lazarus was the first man to be required to die twice, but the event of resuscitation after cardiac and respiratory arrest and other near-death experiences has become commonplace, and there are now many survivors. Several popular works, such as Moody’s bookl have presented anecdotes from these survivors as "exciting evidence of the survival of the human spirit beyond death", and even as proving the existence of an afterlife. Only a deliberate use of obsolete definitions of death can enable one to claim that anybody has, under clinical conditions, returned to tell us what lies beyond death, for by working definition, periodically updated, death is just beyond the point from which anyone can return to tell us anything. Collected accountsl-6volunteered by survivors do, however, bear striking similarities. Amongst the experiences many have described are an initial period of distress followed by profound calm and joy; out-of-the body experiences with the sense of watching resuscitation events from a distance; the sensation of moving rapidly down a tunnel or along a road, accompanied by a loud buzzing or ringing noise or hearing beautiful music; recognising friends and relatives who have died previously; a rapid visual review of pleasant incidents from throughout the life as a panoramic playback (in perhaps 12% of cases6); a sense of approaching a border or frontier and being sent back; and being annoyed or disappointed at having to return from such a pleasant experience-"I tried not to come back", in one patient’s words. Some describe frank transcendant experiences and many state that they will never fear death again. Similar stories have been reported from the victims of accidents, falls, drowning, anaphylaxis, and cardiac or respiratory arrest.
The
complete descriptions tend to come from rapidly afflicted and perceive their demise as inescapable and imminent. Where escape is possible but difficult, many or all features are missing, and with the concentration on avoiding death, panic and fear are more notable.6 The experiences are probably psychologically determined and defensive in function, and seem more dependent on the patient’s perception of his situation than on his physical or biochemical state. More careful collection of data and cross-cultural comparisons 7 may throw light on this matter. Of the less pleasant manifestations, Dlin et al. have described the "I am Dead" syndrome in which survivors of cardiac arrest may for some days after recovery claim that they are already dead. Cleveland and Johnson,9 investigating patients recovering from coronary thromboses, showed major discrepancies between the disturbing fantasies about death, as disclosed by detailed psychological testing, and the unperturbed front the patients maintained in what they said about themselves. most
those who
are
1. Moody, R. Life After Life. Atlanta, 1975. 2. Hunter, R. C. A. Am. J. Psychiat., 1967, 124, 84. 3. Noyes, R. Psychiatry, 1972, 35, 174. 4. Heim, A. Yearbook of the Swiss Alpine Club, 1892, 27, 327. 5. Pfister, O. Int. Z. Psychoanal. 1930, 16, 430. 6. Kalish, R. A. Death and Bereavement (edited by A. H. Kutscher); Springfield, Illinois, 1969. 7. Osis, K., Haraldsson, E. At the Hour of Death. New York, 1977. 8. Dlin, M. D., Stern, A., Poliakoff, S. J. Psychosomatics, 1974, 15, 61. 9. Cleveland, S. E., Johnson, D. L. Psychosom. Med. 1962, 24, 600.
p. 89.
