186 a:striol levels. At birth he weighed 1-519 kg. He was apnoeic and hypotonic, and had a one-minute Apgar score of 1; the score was still only 4 at five minutes. He was noted to have an achondroplastic appearance and to have an extra left thumb and a bifurcated right thumb. Post-mortem examination confirmed the postaxial polydactyly, and revealed bossing of the forehead with a diminished volume to the posterior fossa of the skull, 11 achondroplasia ", and unaerated lungs. No abnormalities were detected in the cardiovascular, alimentary, genitourinary, endocrine, or lymphoreticular systems. Neither chromosome analysis nor radiographs were undertaken on this second case.
The family had moved since the birth of the first child and were again referred for genetic counselling, when I saw them. It is probable that, despite the differing nature of the digital anomaly, the two cases have the same malformation. (Variable digital anomaly has been reported in other disorders-for example, Conradi syndrome and some of its variants.) It is probable that the skull defect is the same in both cases. The small posterior fossa in the second case suggests that this may be due to a short base to the skull rather than a true hydrocephalus as was suggested in the first case. In view of the fact that two boys have been affected it must be assumed that this is a recessive disorder, but whether autosomal or sex-linked cannot be said. There was nothing in the mother’s family history to support sex-linkage, but on the other hand the different racial origin of the parents does not support an autosomal basis. The answer will have to come from further reports. I
grateful to genetic counselling am
discussion
on
Dr C. 0. Carter, who saw the parents for after the birth of the first child, for helpful
this familv.
University Department of Genetics, Leeds LS2 9JT.
M. D’A. CRAWFURD.
FAILURE OF CEPHALOSPORINS TO PREVENT BACTERIAL ENDOCARDITIS DURING LABOUR
SIR,-We wish to draw attention to another1 failure of antibiotic prophylaxis of endocarditis, the second2 to have occurred at Queen Charlotte’s Hospital. housewife was referred during her third pregAt another hospital, before her first pregnancy, mitral regurgitation, possibly of rheumatic origin, had been diagnosed because of cardiac enlargement, grade 6/6 pansystolic murmur at the apex, and third heart sound. She had been advised to take phenoxymethyl penicillin, 250 mg. twice daily, as a prophylactic against further streptococcal infection. This she had done for five years. She remained well and in sinus rhythm throughout the antenatal period. One hour after the spontaneous onset of labour at term, she was admitted to hospital, and cephaloridine 500 mg. intramuscularly was given as a prophylactic. The membranes ruptured fifteen minutes later, and two hours after the onset of labour she was delivered spontaneously of a healthy baby. She was given cephalexin 500 mg. six-hourly for the next five days and remained well. However, on the day after cephalexin was discontinued she developed a fever of 38-3°C. There was no clinical evidence to localise the site of the infection, and there were no peripheral signs of endocarditis. Microbiological examination of urine and high vaginal swab was normal, but Streptococcus mutans3 was recovered from blood-cultures after sixteen hours’ incubation. This alpha-haemolytic streptococcus would, formerly, have been reported as a streptococcus of the viridans type. The organism was resistant to cephaloridine and to streptomycin. Penicillin at a concentration of 0 15 mg. per litre was bactericidal. Combination of penicillin 2 megaunits intravenously four-hourly and streptomycin 0-5 g. intravenously twelve-hourly A
21-year-old
nancy.
1. Durack, D. T., Littler, W. A. Lancet, 1974, ii, 846. 2. Barnes, C. G., Hurley, R. Br. med. J. 1963, ii, 1205. 3. Colman, G., Williams, R. E. O. in Streptococci and Streptococcal Disease (edited by L. W. Wannamaker and J. M. Matsen). New York, 1972.
produced bactericidal levels in serum diluted to 1/8, immediately before injection, and in serum diluted to 1/500, thirty minutes after injection. The patient was treated for six weeks and has made
an
uneventful recovery.
A single injection of cephalexin or cephaloridine fails to prevent experimental streptococcal endocarditis in rabbits, and this report suggests that cephalosporins used alone are unsuitable agents for prophylaxis. Penicillins used alone are also not the best choice for prophylaxis.’ Durack5 recommends ampicillin 1-0 g. plus gentamicin 80 mg. intramuscularly thirty minutes before urethral, gynaecological, and other abdominal procedures, with cephazolin 1 g. plus gentamicin 80 mg. intramuscularly as In view of the experimental evidence 4 an alternative. and of our failures using penicillins2 or cephalosporins alone, we would advocate such a regimen during pregnancy
or labour. MICHAEL Queen Charlotte’s Hospital for Women London W6 0XG.
DE SWIET LOUVOIS ROSALINDE HURLEY.
JOHN
DE
PHOTIC FIT NEAR A HELICOPTER
SiR,—The production of an epileptic convulsion by flickering lights is well known, but how often is the photic stimulation of helicopter propellers responsible ? aged 18, without any past history of faints or fits, waiting to board a helicopter whose blades were revolving when he suddenly fell to the ground and an epileptic fit followed. The E.E.G. report by Dr June Dickson (Severalls Hospital) states: " There are no abnormalities at rest and during overbreathing when the E.E.G. contains a normal alpha rhythm and generalised fast activity. Photic stimulation, however, at many frequencies, particularly when the eyes are open, evoked general spike and wave complexes." A soldier,
was
Military Hospital, Colchester CO2 7UD.
H. FOSTER.
EXPERIMENTAL TRANSMISSION OF CREUTZFELDT-JAKOB DISEASE SIR,-We have attempted the experimental transmission of the agent of Creutzfeldt-Jakob disease (C.J.D.) to random-bred albino laboratory mice and report here our
preliminary findings.
_
The patient was a 63-year-old man with a typical history of c.J.D. Three months after the onset of symptoms, when he had become mute and quadriplegic, a biopsy specimen was taken from the right frontal lobe of the brain. Histological examination revealed neuronal degeneration, status spongiosus, and astrocytic proliferation. The patient died four months later, and postmortem examination of the brain showed severe atrophy of the cerebral and cerebellar grey matter, with neuronal degeneration, status spongiosus, and astrocytosis. The spinal cord showed vacuolar degeneration of anterior horn cells, and there was neurogenic atrophy of muscles, indicating an amyotrophic form of the disease.
Brain tissue removed at biopsy was inoculated intracerebrally into 1-day-old and 10-day-old mouse pups. Between 18 and 24 months’ post-inoculation four of the mice were selected for detailed neuropathological examina-
The first of these was selected because of the a bilateral hindlimb paralysis eighteen months after inoculation. The mouse showed astrocytic proliferation and mild status spongiosus in sections of the brain. Sections of the spinal cord at various levels showed a number of anterior horn cells undergoing vacuolar degeneration. In addition, there was loss of anterior horn tion.
development of
4. 5.
Durack, D. T., Petersdorf, R. G. J. clin. Invest. 1973, 52, 592. Durack, D. T. Br. Heart J. 1975, 37, 478.
The family had moved since the birth of the first child and were again referred for genetic counselling, when I saw them. It is probable that, despite the differing nature of the digital anomaly, the two cases have the same malformation. (Variable digital anomaly has been reported in other disorders-for example, Conradi syndrome and some of its variants.) It is probable that the skull defect is the same in both cases. The small posterior fossa in the second case suggests that this may be due to a short base to the skull rather than a true hydrocephalus as was suggested in the first case. In view of the fact that two boys have been affected it must be assumed that this is a recessive disorder, but whether autosomal or sex-linked cannot be said. There was nothing in the mother’s family history to support sex-linkage, but on the other hand the different racial origin of the parents does not support an autosomal basis. The answer will have to come from further reports. I
grateful to genetic counselling am
discussion
on
Dr C. 0. Carter, who saw the parents for after the birth of the first child, for helpful
this familv.
University Department of Genetics, Leeds LS2 9JT.
M. D’A. CRAWFURD.
FAILURE OF CEPHALOSPORINS TO PREVENT BACTERIAL ENDOCARDITIS DURING LABOUR
SIR,-We wish to draw attention to another1 failure of antibiotic prophylaxis of endocarditis, the second2 to have occurred at Queen Charlotte’s Hospital. housewife was referred during her third pregAt another hospital, before her first pregnancy, mitral regurgitation, possibly of rheumatic origin, had been diagnosed because of cardiac enlargement, grade 6/6 pansystolic murmur at the apex, and third heart sound. She had been advised to take phenoxymethyl penicillin, 250 mg. twice daily, as a prophylactic against further streptococcal infection. This she had done for five years. She remained well and in sinus rhythm throughout the antenatal period. One hour after the spontaneous onset of labour at term, she was admitted to hospital, and cephaloridine 500 mg. intramuscularly was given as a prophylactic. The membranes ruptured fifteen minutes later, and two hours after the onset of labour she was delivered spontaneously of a healthy baby. She was given cephalexin 500 mg. six-hourly for the next five days and remained well. However, on the day after cephalexin was discontinued she developed a fever of 38-3°C. There was no clinical evidence to localise the site of the infection, and there were no peripheral signs of endocarditis. Microbiological examination of urine and high vaginal swab was normal, but Streptococcus mutans3 was recovered from blood-cultures after sixteen hours’ incubation. This alpha-haemolytic streptococcus would, formerly, have been reported as a streptococcus of the viridans type. The organism was resistant to cephaloridine and to streptomycin. Penicillin at a concentration of 0 15 mg. per litre was bactericidal. Combination of penicillin 2 megaunits intravenously four-hourly and streptomycin 0-5 g. intravenously twelve-hourly A
21-year-old
nancy.
1. Durack, D. T., Littler, W. A. Lancet, 1974, ii, 846. 2. Barnes, C. G., Hurley, R. Br. med. J. 1963, ii, 1205. 3. Colman, G., Williams, R. E. O. in Streptococci and Streptococcal Disease (edited by L. W. Wannamaker and J. M. Matsen). New York, 1972.
produced bactericidal levels in serum diluted to 1/8, immediately before injection, and in serum diluted to 1/500, thirty minutes after injection. The patient was treated for six weeks and has made
an
uneventful recovery.
A single injection of cephalexin or cephaloridine fails to prevent experimental streptococcal endocarditis in rabbits, and this report suggests that cephalosporins used alone are unsuitable agents for prophylaxis. Penicillins used alone are also not the best choice for prophylaxis.’ Durack5 recommends ampicillin 1-0 g. plus gentamicin 80 mg. intramuscularly thirty minutes before urethral, gynaecological, and other abdominal procedures, with cephazolin 1 g. plus gentamicin 80 mg. intramuscularly as In view of the experimental evidence 4 an alternative. and of our failures using penicillins2 or cephalosporins alone, we would advocate such a regimen during pregnancy
or labour. MICHAEL Queen Charlotte’s Hospital for Women London W6 0XG.
DE SWIET LOUVOIS ROSALINDE HURLEY.
JOHN
DE
PHOTIC FIT NEAR A HELICOPTER
SiR,—The production of an epileptic convulsion by flickering lights is well known, but how often is the photic stimulation of helicopter propellers responsible ? aged 18, without any past history of faints or fits, waiting to board a helicopter whose blades were revolving when he suddenly fell to the ground and an epileptic fit followed. The E.E.G. report by Dr June Dickson (Severalls Hospital) states: " There are no abnormalities at rest and during overbreathing when the E.E.G. contains a normal alpha rhythm and generalised fast activity. Photic stimulation, however, at many frequencies, particularly when the eyes are open, evoked general spike and wave complexes." A soldier,
was
Military Hospital, Colchester CO2 7UD.
H. FOSTER.
EXPERIMENTAL TRANSMISSION OF CREUTZFELDT-JAKOB DISEASE SIR,-We have attempted the experimental transmission of the agent of Creutzfeldt-Jakob disease (C.J.D.) to random-bred albino laboratory mice and report here our
preliminary findings.
_
The patient was a 63-year-old man with a typical history of c.J.D. Three months after the onset of symptoms, when he had become mute and quadriplegic, a biopsy specimen was taken from the right frontal lobe of the brain. Histological examination revealed neuronal degeneration, status spongiosus, and astrocytic proliferation. The patient died four months later, and postmortem examination of the brain showed severe atrophy of the cerebral and cerebellar grey matter, with neuronal degeneration, status spongiosus, and astrocytosis. The spinal cord showed vacuolar degeneration of anterior horn cells, and there was neurogenic atrophy of muscles, indicating an amyotrophic form of the disease.
Brain tissue removed at biopsy was inoculated intracerebrally into 1-day-old and 10-day-old mouse pups. Between 18 and 24 months’ post-inoculation four of the mice were selected for detailed neuropathological examina-
The first of these was selected because of the a bilateral hindlimb paralysis eighteen months after inoculation. The mouse showed astrocytic proliferation and mild status spongiosus in sections of the brain. Sections of the spinal cord at various levels showed a number of anterior horn cells undergoing vacuolar degeneration. In addition, there was loss of anterior horn tion.
development of
4. 5.
Durack, D. T., Petersdorf, R. G. J. clin. Invest. 1973, 52, 592. Durack, D. T. Br. Heart J. 1975, 37, 478.
