- Email: [email protected]
TESTICULAR FEMINISATION AND INGUINAL HERNIA
740
Candida, Phycomycetes, and Aspergillus species frequent opportunistic invaders of burns, wounds, and other sites,’-5,11 these data suggest that topical silver sulphadiazine may be very useful in the treatment and prevention of mycotic infections.
TESTICULAR FEMINISATION AND INGUINAL HERNIA
Because
are
We are grateful to Dr M. Silva-Hutner for the cultures. The investigation was supported by a grant from Marion Laboratories, Inc., Kansas City, Missouri, and Dr Gerald L. Beckloff, of Marion Laboratories, who supplied the silver sulphadiazine.
H. S. R. has an award from the National Institute of General Medical Sciences. Department of Microbiology, College of Physicians and
Surgeons, Columbia University, New York, N.Y. 10032, U.S.A.
THEODORE J. WLODKOWSKI HERBERT S. ROSENKRANZ.
DERMATOGLYPHIC PATTERNS IN NEWBORNS
SIR,-Dermatoglyphic patterns in newborns are notoriously difficult to analyse. C:car prints are hard to obtain because of ridge hypoplasia and smudges caused by uncooperative subjects. We have found it convenient to use an otoscope to observe ridge patterns. This instrument concentrates a bright spot of light on a small area and magnifies clearly at a short working distance. Contrast between ridges and furrows is enhanced by lightly inking the hands before observation. Although permanent and precise records are not obtained, a general impression is possible, which is better than no impression at all. Department of Pathology, Monmouth Medical Center, Long Branch, New Jersey 07740, U.S.A.
BRUCE R. KORF BARBARA E. SCHUH.
T-LYMPHOCYTES IN HUMAN SPLEEN
SIR,-We have calculated the ratio of thymus-depending lymphocytes (T cells) in normal human spleen by using their ability to form spontaneously rosettes with sheep erythrocytes as their marker. The spleens we call normal had just been removed from 10 patients with operable cancer of the stomach, 1 with a splenic cyst, and 1 with pancreatitis. Lymphocytes from these spleens were isolated by the ’Isopaque-Ficoll’ method and incubated with washed sheep red cells.12 Preparations were stained with acridine-orange, and rosettes were counted from 200 lymphocytes by combined dark-field and fluorescence microscopy. The mean percentage of T cells was 263 54. Bone-marrow derived lymphocytes (B cells) were also examined from part of the samples by using their ability to bind activated complement as a marker. The percentage of cells capable of binding complement-coated sheep red cells was 58-08-9. The T/B ratio in the human spleen seems to be such that T cells form a minority, while in blood the situation is reversed (60-70% T cells have been reported 12). These percentages seem to be similar to those reported in mice, where T cells number 70% in blood and 30-35% in the spleen, as estimated by the theta-antigen marker.13 Department of Serology and Bacteriology, University of Helsinki,
R. VISAKORPI H. REPO.
Haartmanink. 3, 00290 Helsinki 29, Finland. 11.
H.
Pruitt, B. A. Archs Surg.
M., Nash, G., Foley, Bruck, 1971, 102, 476. 12. Stjernsward, J., Jondal, M., Vánky, F., Wigzell, H., Sealy, R. Lancet, 1972, i, 1352. 13. Raff, M. C., Owen, J. J. T. Eur. J. Immun. 1971, 1, 27. F. D.,
SIR,-We read with interest the article by German al.lI He recommends the analysis of sex-chromatin pattern in all prepubertal girls with inguinal hernia in order to detect patients with testicular feminisation syndrome, although his prospective study of 32 prepubertal girls with inguinal hernia revealed no patient with testicular et
feminisation. We wish to report that in our department during a 12-month period (August, 1972, to present), a buccalmucosal smear was taken on 17 prepubertal girls admitted to the hospital for repair of inguinal hernia. Of these patients, 2 were found to have a negative sex-chromatin Chromosome analysis, including quinacrinepattern. fluorescent study of dividing and non-dividing lymphocytes, confirmed a 46, XY karyotype in both cases. On biopsy, the inguinal masses contained only testicular tissue. The diagnosis of testicular feminisation before puberty raises important questions for clinical management and genetic counselling. Treatment involves the removal of the gonads in order to prevent neoplasia. When diagnosis is made after puberty, gonadectomy should be performed immediately. There is disagreement about when gonadectomy should be carried out in patients diagnosed before puberty. According to Rimoin and Schimke2 the consensus is that the testes should be left in situ until puberty, since female secondary sexual development does not occur in the absence of testes, and gonadal neoplasia is not a problem until after 25 years of age. On the other hand, Inhorn and Opitz3 recommend gonadectomy as early as possible, because oestrogenic function of the testes can be easily replaced, serious psychological trauma related to the adolescent patient’s knowledge of gonadectomy may be avoided, and the testes may undergo malignant transformation if not removed. We have followed the latter opinion, particularly in view of the recent report of testicular malignancy in an 18-month-old infant with testicular feminisation.4
Diagnosis of testicular feminisation through primary amenorrhoea enables the patient to be reared through childhood as a normal female. When this condition is recognised in infancy or early childhood, the doubt and anxiety of the parents regarding the sex and development of the child may have adverse affects on the psychosexual orientation of the child and presents a problem in counselling. At least
two
approaches
to
genetic counselling may be
considered. The parents could be fully informed that their phenotypically female child has a male chromosomal constitution and testes but that the infant must be raised female. Since no specific prenatal test for testicular feminisation exists, they could then choose to abort all further male (sex-chromatin negative) fetuses and go on to have only daughters, approximately 50% of whom would be carriers for testicular feminisation. Alternatively, our procedure for genetic counselling, similar to that of other genetic centres,2 is designed to minimise emotional problems of the parents and patient by advising them that the development of the gonads is not complete and removal is necessary because of their abnormal location and predisposition to malignant change. The parents are further informed that their daughter has one X chromosome and as a
German, J., Simpson, J. L., Morillo-Cucci, G., Passarge, E., De Mayo, A. P. Lancet, 1973, i, 891. 2. Rimoin, D. L., Schimke, R. N. Genetic Disorders of the Endocrine Glands; p. 300. St. Louis, 1971. 3. Inhorn, S. L., Opitz, J. M. in Endocrine Pathology (edited by J. M. B. Bloodworth, Jr.); p. 578. Baltimore, 1968. 4. Polani, P. E. Phil. Trans. R. Soc. 1970, 259, 187. 1.
741 " marker chromosome. The latter permits prenatal diagnosis in future pregnancies. Recognising that the recurrence risk for testicular feminisation may be 25’o,3one might ask whether prenatal diagnosis is warranted. Affected individuals, although infertile, are consistently feminine in their gender role and identity and lead relatively normal lives as females.5 Our experience with two infants with testicular feminisation emphasises the need for cooperation among obstetrician, "
one
pxdiatrician, surgeon, endocrinologist, house-staff, nursingstaff, and genetic counsellor to reinforce the counselling. We are concerned about the rights of the patient and parents as a result of this approach. At our hospital each patient receives a copy of the patient’s Bill of Rights which " The patient has the right to obtain from his current information concerning his diagnosis, treatment and prognosis in terms the patient can be reasonably expected to understand. When it is not medically advisable to give such information to the patient, the information should be made available to an appropriate person in his behalf." In testicular feminisation we have decided that if serious emotional problems are to be averted in the parents and patient, the actual diagnosis should be provided on behalf of the patient only to her states:
physician complete
physician.
Jewish-Hillside Medical Center, New Hyde Park, New York 11040, U.S.A.
EUGENE PERGAMENT AUDREY HEIMLER PULOMA SHAH.
TRANSIENT INCREASES IN INTRACRANIAL PRESSURE AND THE BLOOD-BRAIN BARRIER
SiR,-Previously published studies have shown that the transient changes in heart-rate, blood-pressure, respiration, and sometimes in central-venous pressure induced in anaesthetised cats by rapid infusion of saline into the supratentorial epidural space are often associated with petechial and confluent hxmorrhages in the cerebral hemispheres, diencephalon, midbrain, and pons.s6’7 The hsmorrhagic lesions originate from arterioles, capillaries, and venules, are mainly remote from the site of pressure induction, and are similar in distribution and nature to those described in human beings as a consequence of head injury and/or intracranial hypertension. 8-11 In a more recent series of experiments, transient increases in intracranial pressure were induced in cats anaesthetised with pentobarbitone by rapid injection of 0-5 ml. aliquots of buffered saline into the epidural space over the lateral gyrus in six trials. Evans-blue, 5 mg. per kg., was injected intravenously; the animals were killed and the central nervous system fixed in situ by perfusion through the left ventricle and aorta with buffered saline, followed by 8% buffered formalin after clamping of the descending aorta. Sectioning of the brain showed the haemorrhagic lesions as previously described; haemorrhages were also found in the cervical spinal cord of some animals. Evans-blue, which combines with plasma-albumin, Money, J., Ehrhardet, A. A., Masica, N. Johns Hopk. med. J. 1968, 123, 105. 6. Heck, A. F. Eur. Neurol. 1972, 8, 104. 7. Heck, A. F. in Intracranial Pressure (edited by M. Brock and H. Dietz); p. 200. Berlin, 1972. 8. Heck, A. F., Garcia, J., Reichl, W., Kawamura, J., Komijyo, Y., Hall, V. R. Microvasc. Res. 1973, 6, 123. 9. Heck, A. F., Reichl, W., Garcia, J., Kawamura, J., Komijyo, Y., Hall, V. R. Proc. int. Symp. Path. cerebr. Microcirc. Berlin, 1973 (in the press). 10. Moritz, A. R. Pathology of Trauma; p. 317. Philadelphia, 1954. 11. Rap. Z., Zaremba, J. Pol. med. J. 1971, 10, 188. 5.
These results indicate that transient increases in intracranial pressure as induced in this model alter the bloodbrain barrier for albumin at sites remote from that of the pressure induction. The presence of this phenomenon in the hypothalamus in particular seems significant in view of the not infrequent occurrence of inappropriate-antidiuretic-hormone syndrome and other disturbances of homceostatic functions in patients with head injury and intracranial hypertension. ALBERT F. HECK* WOLFRAM W. REICHL VERLAN R. HALL FRANK V. MCL. BOOTH.
Department of Neurology, University of Maryland, Baltimore, Maryland 21201.
LITHIUM IN SPASMODIC TORTICOLLIS
Division of Human Genetics,
Department of Pediatrics, Long Island
was present in the brain and spinal-cord parenchyma, rimming the hxmorrhagic lesions, but was also found in well-defined areas in the anterior hypothalamus, mamillary bodies, cerebral cortex, basal ganglia, central pons, and cervical spinal cord not associated with haemorrhages. Sham-operated controls subjected to anaesthesia for similar periods of time showed neither haemorrhages nor Evansblue in the brain and spinal cord.
SIR,- There have been reports of the use of lithium salts treatment of tardive dyskinesia, Huntington’s chorea,l and parkinsonism. These can be seen as diseases in which the transmitter balance in the basal ganglia is disturbed, and the hypothesis has been advanced that lithium acts by stabilising such amine-containing systems.3 In many respects spasmodic torticollis resembles the more generalised dyskinetic states and a similar anatomical basis in the
"
"
reasonable. It presents a considerable problem in treatment, with wide individual variations in response4 (possibly indicating heterogeneity of the syndrome). In the following case spasmodic torticollis of possible " " organic aetiology responded well to lithium when other medication had failed, the response being confirmed by a " reversal " comparison with an identical placebo tablet. seems
A 36-year-old woman had a long history of difficulty in coping with the stresses of social and marital life leading to frequent attendances at a clinic or hospital for treatment of depressive reactions. In 1964 she was found unconscious after exposure to town gas and required intensive resuscitation and treatment, remaining comatose for two days. After her recovery she was" described as " vague, easily distracted" and " forgetful (however her " amnesia " was inconsistent and selective). The pattern of day-hospital and inpatient care progressed and various
tranquillisers, including phenothiazines, were prescribed to control her impetuous behaviour. (However, phenothiazine medication was intermittent and in moderate dosage, antiparkinsonian drugs being prescribed concurrently.) In 1970 she began to notice that her " head kept turning to the right " and this progressed gradually, with the subsequent development of high back pain. Physical examination a year later revealed full passive movements, intermittent left sternomastoid spasm, facial grimacing associated with attempts at active correction, a mild choreiform movement of the upper limbs (later undetectable), but no other central or peripheral nervous defects. The progressive torticollis failed to respond to adequate trials of atropine sulphate (Kleeman’s method), anticholinergic drugs
(benzhexol, methixine, orphenadrine), diazepam, amantadine, and haloperidol (9 mg. per day) plus amantadine. Levodopa and * After Sept. 1, 1973, Professor Heck will be visiting professor at the Medizinische Hochschule Hannover, Neurochirurgische Klinik, 3 Hannover-Kleefeld, Roderbruch Strasse 101, West Germany. 1. Dalen, P. Lancet, 1973, i, 107. 2. Dalen, P., Steg, G. ibid. p. 936. 3. Medical Research Council Brain Metabolism Unit, ibid. 1972, ii, 573. 4. Shaw, K. M., Hunter, K. R., Stern, G. M. ibid. 1972, i, 1399. 5. Gilbert, G. J. New Engl. J. Med. 1971, 284, 896.
Candida, Phycomycetes, and Aspergillus species frequent opportunistic invaders of burns, wounds, and other sites,’-5,11 these data suggest that topical silver sulphadiazine may be very useful in the treatment and prevention of mycotic infections.
TESTICULAR FEMINISATION AND INGUINAL HERNIA
Because
are
We are grateful to Dr M. Silva-Hutner for the cultures. The investigation was supported by a grant from Marion Laboratories, Inc., Kansas City, Missouri, and Dr Gerald L. Beckloff, of Marion Laboratories, who supplied the silver sulphadiazine.
H. S. R. has an award from the National Institute of General Medical Sciences. Department of Microbiology, College of Physicians and
Surgeons, Columbia University, New York, N.Y. 10032, U.S.A.
THEODORE J. WLODKOWSKI HERBERT S. ROSENKRANZ.
DERMATOGLYPHIC PATTERNS IN NEWBORNS
SIR,-Dermatoglyphic patterns in newborns are notoriously difficult to analyse. C:car prints are hard to obtain because of ridge hypoplasia and smudges caused by uncooperative subjects. We have found it convenient to use an otoscope to observe ridge patterns. This instrument concentrates a bright spot of light on a small area and magnifies clearly at a short working distance. Contrast between ridges and furrows is enhanced by lightly inking the hands before observation. Although permanent and precise records are not obtained, a general impression is possible, which is better than no impression at all. Department of Pathology, Monmouth Medical Center, Long Branch, New Jersey 07740, U.S.A.
BRUCE R. KORF BARBARA E. SCHUH.
T-LYMPHOCYTES IN HUMAN SPLEEN
SIR,-We have calculated the ratio of thymus-depending lymphocytes (T cells) in normal human spleen by using their ability to form spontaneously rosettes with sheep erythrocytes as their marker. The spleens we call normal had just been removed from 10 patients with operable cancer of the stomach, 1 with a splenic cyst, and 1 with pancreatitis. Lymphocytes from these spleens were isolated by the ’Isopaque-Ficoll’ method and incubated with washed sheep red cells.12 Preparations were stained with acridine-orange, and rosettes were counted from 200 lymphocytes by combined dark-field and fluorescence microscopy. The mean percentage of T cells was 263 54. Bone-marrow derived lymphocytes (B cells) were also examined from part of the samples by using their ability to bind activated complement as a marker. The percentage of cells capable of binding complement-coated sheep red cells was 58-08-9. The T/B ratio in the human spleen seems to be such that T cells form a minority, while in blood the situation is reversed (60-70% T cells have been reported 12). These percentages seem to be similar to those reported in mice, where T cells number 70% in blood and 30-35% in the spleen, as estimated by the theta-antigen marker.13 Department of Serology and Bacteriology, University of Helsinki,
R. VISAKORPI H. REPO.
Haartmanink. 3, 00290 Helsinki 29, Finland. 11.
H.
Pruitt, B. A. Archs Surg.
M., Nash, G., Foley, Bruck, 1971, 102, 476. 12. Stjernsward, J., Jondal, M., Vánky, F., Wigzell, H., Sealy, R. Lancet, 1972, i, 1352. 13. Raff, M. C., Owen, J. J. T. Eur. J. Immun. 1971, 1, 27. F. D.,
SIR,-We read with interest the article by German al.lI He recommends the analysis of sex-chromatin pattern in all prepubertal girls with inguinal hernia in order to detect patients with testicular feminisation syndrome, although his prospective study of 32 prepubertal girls with inguinal hernia revealed no patient with testicular et
feminisation. We wish to report that in our department during a 12-month period (August, 1972, to present), a buccalmucosal smear was taken on 17 prepubertal girls admitted to the hospital for repair of inguinal hernia. Of these patients, 2 were found to have a negative sex-chromatin Chromosome analysis, including quinacrinepattern. fluorescent study of dividing and non-dividing lymphocytes, confirmed a 46, XY karyotype in both cases. On biopsy, the inguinal masses contained only testicular tissue. The diagnosis of testicular feminisation before puberty raises important questions for clinical management and genetic counselling. Treatment involves the removal of the gonads in order to prevent neoplasia. When diagnosis is made after puberty, gonadectomy should be performed immediately. There is disagreement about when gonadectomy should be carried out in patients diagnosed before puberty. According to Rimoin and Schimke2 the consensus is that the testes should be left in situ until puberty, since female secondary sexual development does not occur in the absence of testes, and gonadal neoplasia is not a problem until after 25 years of age. On the other hand, Inhorn and Opitz3 recommend gonadectomy as early as possible, because oestrogenic function of the testes can be easily replaced, serious psychological trauma related to the adolescent patient’s knowledge of gonadectomy may be avoided, and the testes may undergo malignant transformation if not removed. We have followed the latter opinion, particularly in view of the recent report of testicular malignancy in an 18-month-old infant with testicular feminisation.4
Diagnosis of testicular feminisation through primary amenorrhoea enables the patient to be reared through childhood as a normal female. When this condition is recognised in infancy or early childhood, the doubt and anxiety of the parents regarding the sex and development of the child may have adverse affects on the psychosexual orientation of the child and presents a problem in counselling. At least
two
approaches
to
genetic counselling may be
considered. The parents could be fully informed that their phenotypically female child has a male chromosomal constitution and testes but that the infant must be raised female. Since no specific prenatal test for testicular feminisation exists, they could then choose to abort all further male (sex-chromatin negative) fetuses and go on to have only daughters, approximately 50% of whom would be carriers for testicular feminisation. Alternatively, our procedure for genetic counselling, similar to that of other genetic centres,2 is designed to minimise emotional problems of the parents and patient by advising them that the development of the gonads is not complete and removal is necessary because of their abnormal location and predisposition to malignant change. The parents are further informed that their daughter has one X chromosome and as a
German, J., Simpson, J. L., Morillo-Cucci, G., Passarge, E., De Mayo, A. P. Lancet, 1973, i, 891. 2. Rimoin, D. L., Schimke, R. N. Genetic Disorders of the Endocrine Glands; p. 300. St. Louis, 1971. 3. Inhorn, S. L., Opitz, J. M. in Endocrine Pathology (edited by J. M. B. Bloodworth, Jr.); p. 578. Baltimore, 1968. 4. Polani, P. E. Phil. Trans. R. Soc. 1970, 259, 187. 1.
741 " marker chromosome. The latter permits prenatal diagnosis in future pregnancies. Recognising that the recurrence risk for testicular feminisation may be 25’o,3one might ask whether prenatal diagnosis is warranted. Affected individuals, although infertile, are consistently feminine in their gender role and identity and lead relatively normal lives as females.5 Our experience with two infants with testicular feminisation emphasises the need for cooperation among obstetrician, "
one
pxdiatrician, surgeon, endocrinologist, house-staff, nursingstaff, and genetic counsellor to reinforce the counselling. We are concerned about the rights of the patient and parents as a result of this approach. At our hospital each patient receives a copy of the patient’s Bill of Rights which " The patient has the right to obtain from his current information concerning his diagnosis, treatment and prognosis in terms the patient can be reasonably expected to understand. When it is not medically advisable to give such information to the patient, the information should be made available to an appropriate person in his behalf." In testicular feminisation we have decided that if serious emotional problems are to be averted in the parents and patient, the actual diagnosis should be provided on behalf of the patient only to her states:
physician complete
physician.
Jewish-Hillside Medical Center, New Hyde Park, New York 11040, U.S.A.
EUGENE PERGAMENT AUDREY HEIMLER PULOMA SHAH.
TRANSIENT INCREASES IN INTRACRANIAL PRESSURE AND THE BLOOD-BRAIN BARRIER
SiR,-Previously published studies have shown that the transient changes in heart-rate, blood-pressure, respiration, and sometimes in central-venous pressure induced in anaesthetised cats by rapid infusion of saline into the supratentorial epidural space are often associated with petechial and confluent hxmorrhages in the cerebral hemispheres, diencephalon, midbrain, and pons.s6’7 The hsmorrhagic lesions originate from arterioles, capillaries, and venules, are mainly remote from the site of pressure induction, and are similar in distribution and nature to those described in human beings as a consequence of head injury and/or intracranial hypertension. 8-11 In a more recent series of experiments, transient increases in intracranial pressure were induced in cats anaesthetised with pentobarbitone by rapid injection of 0-5 ml. aliquots of buffered saline into the epidural space over the lateral gyrus in six trials. Evans-blue, 5 mg. per kg., was injected intravenously; the animals were killed and the central nervous system fixed in situ by perfusion through the left ventricle and aorta with buffered saline, followed by 8% buffered formalin after clamping of the descending aorta. Sectioning of the brain showed the haemorrhagic lesions as previously described; haemorrhages were also found in the cervical spinal cord of some animals. Evans-blue, which combines with plasma-albumin, Money, J., Ehrhardet, A. A., Masica, N. Johns Hopk. med. J. 1968, 123, 105. 6. Heck, A. F. Eur. Neurol. 1972, 8, 104. 7. Heck, A. F. in Intracranial Pressure (edited by M. Brock and H. Dietz); p. 200. Berlin, 1972. 8. Heck, A. F., Garcia, J., Reichl, W., Kawamura, J., Komijyo, Y., Hall, V. R. Microvasc. Res. 1973, 6, 123. 9. Heck, A. F., Reichl, W., Garcia, J., Kawamura, J., Komijyo, Y., Hall, V. R. Proc. int. Symp. Path. cerebr. Microcirc. Berlin, 1973 (in the press). 10. Moritz, A. R. Pathology of Trauma; p. 317. Philadelphia, 1954. 11. Rap. Z., Zaremba, J. Pol. med. J. 1971, 10, 188. 5.
These results indicate that transient increases in intracranial pressure as induced in this model alter the bloodbrain barrier for albumin at sites remote from that of the pressure induction. The presence of this phenomenon in the hypothalamus in particular seems significant in view of the not infrequent occurrence of inappropriate-antidiuretic-hormone syndrome and other disturbances of homceostatic functions in patients with head injury and intracranial hypertension. ALBERT F. HECK* WOLFRAM W. REICHL VERLAN R. HALL FRANK V. MCL. BOOTH.
Department of Neurology, University of Maryland, Baltimore, Maryland 21201.
LITHIUM IN SPASMODIC TORTICOLLIS
Division of Human Genetics,
Department of Pediatrics, Long Island
was present in the brain and spinal-cord parenchyma, rimming the hxmorrhagic lesions, but was also found in well-defined areas in the anterior hypothalamus, mamillary bodies, cerebral cortex, basal ganglia, central pons, and cervical spinal cord not associated with haemorrhages. Sham-operated controls subjected to anaesthesia for similar periods of time showed neither haemorrhages nor Evansblue in the brain and spinal cord.
SIR,- There have been reports of the use of lithium salts treatment of tardive dyskinesia, Huntington’s chorea,l and parkinsonism. These can be seen as diseases in which the transmitter balance in the basal ganglia is disturbed, and the hypothesis has been advanced that lithium acts by stabilising such amine-containing systems.3 In many respects spasmodic torticollis resembles the more generalised dyskinetic states and a similar anatomical basis in the
"
"
reasonable. It presents a considerable problem in treatment, with wide individual variations in response4 (possibly indicating heterogeneity of the syndrome). In the following case spasmodic torticollis of possible " " organic aetiology responded well to lithium when other medication had failed, the response being confirmed by a " reversal " comparison with an identical placebo tablet. seems
A 36-year-old woman had a long history of difficulty in coping with the stresses of social and marital life leading to frequent attendances at a clinic or hospital for treatment of depressive reactions. In 1964 she was found unconscious after exposure to town gas and required intensive resuscitation and treatment, remaining comatose for two days. After her recovery she was" described as " vague, easily distracted" and " forgetful (however her " amnesia " was inconsistent and selective). The pattern of day-hospital and inpatient care progressed and various
tranquillisers, including phenothiazines, were prescribed to control her impetuous behaviour. (However, phenothiazine medication was intermittent and in moderate dosage, antiparkinsonian drugs being prescribed concurrently.) In 1970 she began to notice that her " head kept turning to the right " and this progressed gradually, with the subsequent development of high back pain. Physical examination a year later revealed full passive movements, intermittent left sternomastoid spasm, facial grimacing associated with attempts at active correction, a mild choreiform movement of the upper limbs (later undetectable), but no other central or peripheral nervous defects. The progressive torticollis failed to respond to adequate trials of atropine sulphate (Kleeman’s method), anticholinergic drugs
(benzhexol, methixine, orphenadrine), diazepam, amantadine, and haloperidol (9 mg. per day) plus amantadine. Levodopa and * After Sept. 1, 1973, Professor Heck will be visiting professor at the Medizinische Hochschule Hannover, Neurochirurgische Klinik, 3 Hannover-Kleefeld, Roderbruch Strasse 101, West Germany. 1. Dalen, P. Lancet, 1973, i, 107. 2. Dalen, P., Steg, G. ibid. p. 936. 3. Medical Research Council Brain Metabolism Unit, ibid. 1972, ii, 573. 4. Shaw, K. M., Hunter, K. R., Stern, G. M. ibid. 1972, i, 1399. 5. Gilbert, G. J. New Engl. J. Med. 1971, 284, 896.