608 A sister of the proband was phenotypically normal except for her hair, which was light coloured, coarse, and frizzy. Hair samples were taken randomly from this child and from the maternal grandmother and great-grandmother. Hairs from this kindred and from unrelated controls were examined "blind" under the microscope by C.M.M. Pili torti were identified in approximately 50% of the hairs from the mother and in all the proband’s sample, while 30% of the hairs taken from the sibling and from each grandmother were also affected (figure). None of the control hairs had pili torti. We postulate that brittle hair with pili torti in heterozygotes represents inactivation of the normal maternal X chromosome8 areas.
-i.e., the abnormal hair is the product of cells whose active X chromosome carries the Menkes gene. Selective sampling from discrete cutaneous areas is planned and biochemical studies of individual hairs are under way. These studies may reveal two distinct tissue types with respect to copper metabolism and thus biochemically identify the heterozygous state. In performing this test a complete search of the scalp should be carried out, including areas thought normal as well as those said by the patient to be abnormal. Experienced observers must inspect because hair morphology is, a difficult subject. Furthermore, no examination can completely rule out the presence of an abnormal gene; it can only lessen its likelihood. Department of Pediatrics, University of Texas Medical School at Houston, Texas Medical Center, Houston, Texas 77030, U.S.A.
WILLIAM R. COLLIE CHARLEEN M. MOORE THOMAS J. GOKA R. RODNEY HOWELL
VASOPRESSIN IN AMNESIA
SIR,-De Wied et al. demonstrated that vasopressin affects memory consolidation, and Dr Oliveros and his colleagues (Jan. 7, p. 42) suggested that the amnestic syndrome (posttraumatic in three patients, probably alcohol induced in one) responds to low-dose intranasal vasopressin. We have treated two patient with an alcohol-induced amnestic state as pan of the Wernicke-Korsakoff syndrome, with vasopressin nasal spray (Sandoz). The first patient, male aged 62, known to have drunk excessively for many months, presented with a global confusional state, horizontal nystagmus, and truncal ataxia. As his confusional state regressed a Korsakoff psychosis developed with retrograde and anterograde amnesia in association with confabulatory episodes. He was treated for 3 weeks with intravenous thiamine (Parentrovite’). The truncal ataxia and nystagrnus cleared, but there was no improvement in the amnestic state. 3 months later vasopressin (four nasal puffs a day, supplying 16 i.u. daily) was prescribed. After 15 days treatment, psychometric testing showed no improvement in any of the indices that had led to the diagnosis of the amnestic syndrome, and his condition remains unchanged. The second patient, female aged 61, with a long history of alcohol abuse, presented with a global confusional state, horizontal nystagmus, conjugate gaze palsy, truncal ataxia, and mild hepatocellular dysfunction. High-dose thiamine was prescribed. Her global confusional state cleared in 7 days leaving a Korsakoff type amnestic syndrome. Vasopressin (16 I.u. day) was prescribed and continued for 21 days. There was no improvement in her anterograde or retrograde amnesia and she remains unable to maintain an independent existence. These two cases do not support the suggestion that a severe amnestic syndrome induced by alcohol is responsive to vaso-
pressin. Department of Medicine, Newcastle General Hospital, Newcastle upon Tyne NE4 6BE 8. Lyon, M. Nature, 1961, 190, 372. 1. De Wied, D., van Wimersma Greidanus, T.
W.H. Progr.Brain Res. 1976, 45, 181.
D. R. BLAKE M. J. DODD J. GRIMLEY EVANS
B., Bohus, B., Urban, I., Gispen,
FOOD ALLERGY
SIR,-Dr Finn and Dr Cohen (Feb. 25, p. 426) provide cogent evidence of the detrimental effect of common foodstuffs and reintroduce the term "food allergy" in its original, non-
immunological sense. I have suggested’ that large amounts of high-molecularweight breakdown products (B.D.P.) of dietary protein pass through the gut wall. My laboratory studies have shown that B.D.P. of IgGs, wheat gliadin, ha9moglobin, and ferritin pass the gut wall of rabbits, rats, guineapigs, and piglets,2-’ and a survey of older studies6 suggests that this also happens in man. Much of the B.D.P. retains its original antigenicity when in the tissue after ingestion and passage through the circulation’ and direct reactions with cellular antibody are thus possible. The enormous variety of protein structures which must arise from partial cleavage of dietary proteins may produce pharmacological and toxicological effects. Among the peptic degradation products of a-gliadin, one polypeptide has endorphinlike activity.8 The psychological symptoms observed by Finn and Cohen are not unexpected, since B.D.P. appear to pass the blood/brain barrier.9,lo These fragments, especially those of a-gliadin, may be directly cytotoxic for neural cells, have subtle pharmacological gical actions, or trigger a cerebral allergic reaction if antibody of the appropriate class is also present in the brain. The references cited by Finn and Cohen include many cases of severe psychiatric illness apparently caused by foodstuffs. The underlying mechanism in all these cases may be the absorption of B.D.P. of dietary protein through the gut wall and their dissemination throughout the tissues of the body. A.R.C.
Immunology Group, Department of Zoology, University College of North Wales, Brambell Laboratories, Bangor, Caernarvonshire LL57 2UW
W. A. HEMMINGS
WAYS OUT OF WAITING-LISTS
SIR,--Your Parliamentary correspondent asks (Feb. 18, p. 397) if there is any way out of the waiting-lists. Yes, there is. are due to lack of operating-theatre time or first step could be a moratorium on tonsillectomy ; there is no evidence that the operation does any good or that it is done at the right time on the right cases, and in two years most children grow out of the waiting-list anyway making the saving an absolute one. In so far as they are due to lack of beds, patients "awaiting electroencephalograms" could be allowed home until an outpatient reading makes it clear that they need to come in. When will the D.H.S.S. realise that there will be an inverse relationship between bed-turnover and bed-occupancy as long as the nursing establishment is geared to the latter and not the former? The Health Service is not very efficient, but it could be made more so if efficient units were not penalised for their efforts.
In
so
far
as
anaesthetists
they a
University Department of Child Health, St. Mary’s Hospital, Manchester M13 0JH
JOHN A. DAVIS
1. Hemmings, W. A. Lancet, 1976, i, 697. 2. Hemmings, W. A. I.R.C.S. med Sci. 1975, 3, 382. 3. Hemmings, W. A., Williams, E. W. Proc. R. Soc. B. 1977, 197, 425. 4. Hemmings, C., Hemmings, W. A., Patey, A. L., Wood, C. ibid. 198, 439. 5. Hemmings, W. A. in Antigen Absorption by the gut (edited by W. A. Hemmings) Lancaster, 1978 (in the press). 6. Hemmings, W. A. in The Biological Basis of Schizophrenia (edited by G. and W. A. Hemmings) Lancaster, (in the press). 7. Hemmings, W. A., Williams, E. W. Gut, (in the press). 8. Klee, W. A. Unpublished. 9. Hemmings, W. A. J. orthomol. Psychiat. 1977, 6, 309. 10. Hemmings, W. A. Proc. R. Soc. B. 1978, 200, 175.
-i.e., the abnormal hair is the product of cells whose active X chromosome carries the Menkes gene. Selective sampling from discrete cutaneous areas is planned and biochemical studies of individual hairs are under way. These studies may reveal two distinct tissue types with respect to copper metabolism and thus biochemically identify the heterozygous state. In performing this test a complete search of the scalp should be carried out, including areas thought normal as well as those said by the patient to be abnormal. Experienced observers must inspect because hair morphology is, a difficult subject. Furthermore, no examination can completely rule out the presence of an abnormal gene; it can only lessen its likelihood. Department of Pediatrics, University of Texas Medical School at Houston, Texas Medical Center, Houston, Texas 77030, U.S.A.
WILLIAM R. COLLIE CHARLEEN M. MOORE THOMAS J. GOKA R. RODNEY HOWELL
VASOPRESSIN IN AMNESIA
SIR,-De Wied et al. demonstrated that vasopressin affects memory consolidation, and Dr Oliveros and his colleagues (Jan. 7, p. 42) suggested that the amnestic syndrome (posttraumatic in three patients, probably alcohol induced in one) responds to low-dose intranasal vasopressin. We have treated two patient with an alcohol-induced amnestic state as pan of the Wernicke-Korsakoff syndrome, with vasopressin nasal spray (Sandoz). The first patient, male aged 62, known to have drunk excessively for many months, presented with a global confusional state, horizontal nystagmus, and truncal ataxia. As his confusional state regressed a Korsakoff psychosis developed with retrograde and anterograde amnesia in association with confabulatory episodes. He was treated for 3 weeks with intravenous thiamine (Parentrovite’). The truncal ataxia and nystagrnus cleared, but there was no improvement in the amnestic state. 3 months later vasopressin (four nasal puffs a day, supplying 16 i.u. daily) was prescribed. After 15 days treatment, psychometric testing showed no improvement in any of the indices that had led to the diagnosis of the amnestic syndrome, and his condition remains unchanged. The second patient, female aged 61, with a long history of alcohol abuse, presented with a global confusional state, horizontal nystagmus, conjugate gaze palsy, truncal ataxia, and mild hepatocellular dysfunction. High-dose thiamine was prescribed. Her global confusional state cleared in 7 days leaving a Korsakoff type amnestic syndrome. Vasopressin (16 I.u. day) was prescribed and continued for 21 days. There was no improvement in her anterograde or retrograde amnesia and she remains unable to maintain an independent existence. These two cases do not support the suggestion that a severe amnestic syndrome induced by alcohol is responsive to vaso-
pressin. Department of Medicine, Newcastle General Hospital, Newcastle upon Tyne NE4 6BE 8. Lyon, M. Nature, 1961, 190, 372. 1. De Wied, D., van Wimersma Greidanus, T.
W.H. Progr.Brain Res. 1976, 45, 181.
D. R. BLAKE M. J. DODD J. GRIMLEY EVANS
B., Bohus, B., Urban, I., Gispen,
FOOD ALLERGY
SIR,-Dr Finn and Dr Cohen (Feb. 25, p. 426) provide cogent evidence of the detrimental effect of common foodstuffs and reintroduce the term "food allergy" in its original, non-
immunological sense. I have suggested’ that large amounts of high-molecularweight breakdown products (B.D.P.) of dietary protein pass through the gut wall. My laboratory studies have shown that B.D.P. of IgGs, wheat gliadin, ha9moglobin, and ferritin pass the gut wall of rabbits, rats, guineapigs, and piglets,2-’ and a survey of older studies6 suggests that this also happens in man. Much of the B.D.P. retains its original antigenicity when in the tissue after ingestion and passage through the circulation’ and direct reactions with cellular antibody are thus possible. The enormous variety of protein structures which must arise from partial cleavage of dietary proteins may produce pharmacological and toxicological effects. Among the peptic degradation products of a-gliadin, one polypeptide has endorphinlike activity.8 The psychological symptoms observed by Finn and Cohen are not unexpected, since B.D.P. appear to pass the blood/brain barrier.9,lo These fragments, especially those of a-gliadin, may be directly cytotoxic for neural cells, have subtle pharmacological gical actions, or trigger a cerebral allergic reaction if antibody of the appropriate class is also present in the brain. The references cited by Finn and Cohen include many cases of severe psychiatric illness apparently caused by foodstuffs. The underlying mechanism in all these cases may be the absorption of B.D.P. of dietary protein through the gut wall and their dissemination throughout the tissues of the body. A.R.C.
Immunology Group, Department of Zoology, University College of North Wales, Brambell Laboratories, Bangor, Caernarvonshire LL57 2UW
W. A. HEMMINGS
WAYS OUT OF WAITING-LISTS
SIR,--Your Parliamentary correspondent asks (Feb. 18, p. 397) if there is any way out of the waiting-lists. Yes, there is. are due to lack of operating-theatre time or first step could be a moratorium on tonsillectomy ; there is no evidence that the operation does any good or that it is done at the right time on the right cases, and in two years most children grow out of the waiting-list anyway making the saving an absolute one. In so far as they are due to lack of beds, patients "awaiting electroencephalograms" could be allowed home until an outpatient reading makes it clear that they need to come in. When will the D.H.S.S. realise that there will be an inverse relationship between bed-turnover and bed-occupancy as long as the nursing establishment is geared to the latter and not the former? The Health Service is not very efficient, but it could be made more so if efficient units were not penalised for their efforts.
In
so
far
as
anaesthetists
they a
University Department of Child Health, St. Mary’s Hospital, Manchester M13 0JH
JOHN A. DAVIS
1. Hemmings, W. A. Lancet, 1976, i, 697. 2. Hemmings, W. A. I.R.C.S. med Sci. 1975, 3, 382. 3. Hemmings, W. A., Williams, E. W. Proc. R. Soc. B. 1977, 197, 425. 4. Hemmings, C., Hemmings, W. A., Patey, A. L., Wood, C. ibid. 198, 439. 5. Hemmings, W. A. in Antigen Absorption by the gut (edited by W. A. Hemmings) Lancaster, 1978 (in the press). 6. Hemmings, W. A. in The Biological Basis of Schizophrenia (edited by G. and W. A. Hemmings) Lancaster, (in the press). 7. Hemmings, W. A., Williams, E. W. Gut, (in the press). 8. Klee, W. A. Unpublished. 9. Hemmings, W. A. J. orthomol. Psychiat. 1977, 6, 309. 10. Hemmings, W. A. Proc. R. Soc. B. 1978, 200, 175.
