1040 with dietary fat intake2 and fatty-acid changes in the serum3 is well known. Baker et al. found a tendency to greater saturation of fatty acids in the lecithins of unaffected cerebral white-matter in patients with M.S. than in controls, and Gul et al. demonstrated reduced relative levels of the fatty acid linoleate (expressed as percentage of the five principal fatty acids) in platelets and erythrocytes in M.S. patients. These studies suggest the possibility of faulty lipid metabolism in mt.s. Our case supports this hypothesis. Perhaps there is a continuum of small-intestinal changes in M.S., extending from normal ultrastructure and villous architecture to abnormal ultrastructure, villous atrophy, and clinical malabsorption. Division of Laboratory Medicine, Cleveland Clinic Foundation, Cleveland, Ohio 44106 U.S.A.

FLOYD J. FANTELLI HIROSHI MITSUMOTO BRUCE A. SEBEK

and brainstem auditory evoked response (B.A.E.R.)’"’" tests and blink reflex 12 gave unexpected abnormal results (clinically silent plaques). 3 patients were eventually found to have another neurological disease: no unexpected lesion was found among them. 11 patients remained undiagnosed but 3 of them are considered as probable M.S. (according to McAlpine’s clinical criteria); in these 3 patients, silent lesions were demonstrated in the optic nerve and/or brainstem. No unexpected lesion was discovered in the other 8 patients. Although our series is small, we feel that pattern reversal and B.A.E.R. are the most efficient tests for detecting clinically silent lesions. We thank Professor Coers, Dr these patients. Service Revalidation,

Hôpital Brugmann, 1020 Bruxelles, Belgium

IMMUNE COMPLEXES IN AMYOTROPHIC LATERAL SCLEROSIS

SiR,—The aetiology of amyotrophic lateral sclerosis (A.L.S.) remains unknown. Immunofluorescence microscopy has shown immune complexes in kidney6 and jejunal mucosa of patients with A.L.S. To determine whether similar deposits are found in voluntary muscle, muscle biopsy specimens of 20 patients with

examined using immunohistological methods. meeting clinical criteria for diagnosis, patients showed electromyographic evidence of denervation in all four extremities with normal nerve-conduction velocities. Muscle biopsy was from either biceps brachii or vastus lateralis as determined by electromyographic examination. Muscle was prepared and examined by standard techniques.8 Specimens for immunofluorescence microscopy were frozen in isopentane and cooled in liquid nitrogen to -160°C. The unfixed sections were washed in phosphate-buffered saline and incubated for 30 min with commercially available (Hyland) fluorescein-conjugated antisera to IgG, IgA, IgM, C3, C4, albumin, or A.L.S. were

In addition to

fibrinogen. No muscle specimen showed immunofluorescent staining any component of muscle fibre, connective tissue, or vascular structures. If immunological mechanisms are significant in the pathogenesis of amyotrophic lateral sclerosis, immune complex deposition in muscle does not appear to be involved.

against

Dent Neurologic Institute and State University of New York, Buffalo, N.Y., U.S.A.

STEPHEN A. BARRON REID R.

HEFFNER, JR

EVOKED POTENTIALS IN THE EARLY DIAGNOSIS OF MULTIPLE SCLEROSIS

SIR,-According to Professor Matthews (Feb. 25, p. 443), somatosensory evoked responses after retrobulbar neuritis seem to be of little help in the prediction of multiple sclerosis (M.S.). Our own observations suggest that the investigation of several afferent pathways with the evoked responses method may be useful in the detection of clinically silent lesions in the central nervous system. In a series of 28 patients in the initial phase of an undiagnosed neurological disease which was possibly M.S., a combination of four evoked responses9 was used to search for clinically silent plaques. 14 of the patients were later found to have M. s. In 7 of them the combination of visual (pattern reversal), somatosensory, 4. 5. 6. 7.

Baker, R. W. R., Thompson, R. H. S., Zilkha, K. J. Lancet, 1963, i, 26. Gul, S., Smith, A. D., Thompson, R. H. S., Payling Wright, H., Zilkha, K. J.J. Neurol. Neurosurg. Psychiat. 1970, 33, 506. Oldstone, M. B. A., Wilson, C. B., Perrin, L. H., Norris, F. H. Lancet, 1976, ii, 169. Pertschuk, L. P., Broome, J. D., Brigati, D. J., Cook, A. W., Vuletin, J. C., Rainford, E. A., Gupta, J. K., Kim, D. S., Nidsgorski, F. ibid. 1977, i, Oxenhandler, R., Adelstein, E. H., Hart, M. N. Hum. Path. 1977, 8, 321. Robinson, K., Rudge, P. Lancet, 1975, i, 1164.

Dr Monseu for

referring

P. DELTENRE C. VAN NECHEL P. KETELAER

DERMATOMYOSITIS AND VACCINATION

SIR,-Dr Kass and his colleagues (April 8, p. 772) ask if others have shared their experience of an association between dermatomyositis and immunisations. I first met this problem back in 1950 and since then have collected and read of further cases.

A 10-year-old boy was vaccinated against diphtheria and scarlet fever. 5 h later he collapsed and generalised weakness and an uneasy feeling started. The boy’s eyes were swollen and he felt feverish. The boy’s weakness necessitated hospital treatment, and dermatomyositis was diagnosed by muscle biopsy. A 6-year-old girl was immunised against diphtheria. 5 days later a small swelling was noted on the vaccination site; this enlarged and became painful to touch. 6 weeks later further subcutaneous infiltrations on other parts of the body followed. Dermatomyositis was diagnosed with typical course of the disease.

A 13-year-old boy had had whooping-cough despite pertussis immunisations. Before going to a children’s camp a diphtheria immunisation was asked for. Unfortunately the doctor gave diphtheria-pertussis-tetanus (D.P.T.). A few hours after immunisation they boy had pain in the vaccinated extremity which increased in the following days, with swelling. On the 10th day after the immunisation oedema of the lips and around the eyes started. 1years previously the boy had occasionally had red spots around the eyes. Muscle biopsy supported the diagnosis of dermatomyositis. The disease may have been aggravated by the pertussis component of the vaccine given to an immune individual. Thieffry et al.1 published a case of dermatomyositis in a 4-year-old girl, starting approximately 14 days after a D.P.T.polio immunisation. Daeschner2 saw a 5-year-old Black boy with a facial rash which had developed 2 weeks after smallpox vaccination; this was the first symptom of dermatomyositis. Winkelmann’ saw a 47-year-old female patient with onset of dermatomyositis after a cold vaccine. Winkelmann felt that the immunisation was a precipitating factor. An 11-year-old child had been vaccinated for the second time against poliomyelitis (Salk). 26 days later swelling of the dorsal aspects of the hand was observed; this was the first symptom of dermatomyositis.1 Gotoff et al. described a 17-year-old boy with agammaglo10

Robinson, K., Rudge, P. in Auditory Evoked Potentials in Man (edited by J. E. Desmedt); p. 58. Basle, 1975. 11. Kimura, J. Brain, 1975, 98, 413. 12. McDonald, W. I., Halliday, A. M. Br. med. Bull. 1977, 33, p. 4. 1. Thieffry, S., Arthus, M., Martin, C., Sorrel-Dejerine, J., Benhamida, M. Sem. Hôp. 1967, 43, 2202. 2. Bitnum, S., Daeschner, C. W. Jr., Travis, L. B., Dodge, W. F., Hopps, H. C. J. Pediat. 1964, 64, 101; and Daeschner, C. W. Jr., Personal communication.

3. Winkelmann, R. K., Mulder, D. W., Lambert, E. H., Howard, F. M., Diessner, G. R. Mayo Clin. Proc. 1968, 43, 545; and Winkelmann, R. K. Personal communication.

1119. 8. 9.

Capon, and

4. 5.

Pichler, E. Personal communication. Gotoff, S. P., Smith, R. D., Sugar, O. Am.J. Dis. Child. 1972,123,53.

1041 bulinaemia and dermatomyositis who, despite D.P.T. immunisations, remained Schick positive; he also lacked delayed-type

hypersensitivity

after

B.C.G.

vaccination. Gotoff

et

al.

won-

dered if a disorder of cell-mediated immunity might be causally related to the development of dermatomyositis. Up to 1960 approximately two hundred cases of dermatomyositis had been published in children. The close temporal relationship between onset of symptoms and the vaccinations could support a causal connection. I believe that doctors may not have paid sufficient attention to this possibility when

checking the case-histories of patients with dermatomyositis. Institute for

Vaccinology and Virology 2000 Hamburg 26, West Germany

W. EHRENGUT

Surely Dunn does not suggest that we should disregard our findings because they fail to confirm the experience of others in different populations and circumstances. We endorse Dunn’s criticisms of the dorsal position and did so in our paper. The clear demonstration of the dangers of this

position rested on hard clinical data and should now be universally recognised. The calls from Dunn and others for widespread adoption of an upright posture for labour and delivery may yet be shown to be wise, but will need to be supported by much stronger evidence than has so far been advanced.

University Department of Obstetrics Royal Maternity Hospital, Glasgow G4 0NA

upright maternal posture for labour and delivery.’ His views are supported by studies of parturients in other countries2,3 and by evidence that primitive peoples adopted an upright’position during labour and delivery. We can see no reason why the practices of primitive peoples should necessarily be regarded as correct. Further, in our report we drew attention to possible sources of error in the methods used in the most widely cited of the previous studies, that of Mendez-Bauer et al. Their patients were all managed in the same way and acted as their own controls. The stimulatory effect of the very frequent vaginal examinations which were made may have favourably influenced the course of labour. Dunn criticises our work on three main grounds. Firstly, he suggests that the absence of data on gestational age and birthweight precludes meaningful interpretation. The design of the study by Mitre3 was very similar to our own, and the results suggest a clear benefit from an upright posture. This report contained even less data on gestational age and birthweight than ours, but this does not discourage Dunn from citing it in support of his own views. In our study no baby weighed less than 2500 g, and there was no significant difference between the birthweights in the different groups. As stated in our paper, the gestational age was in every case 38 weeks or more and the cervix was invariably ripe (i.e., the patients were all at term and on the threshold of spontaneous labour). Secondly, the validity of our study is questioned because the labours were induced. Induced labour was studied for the reasons outlined in our paper, and we accept that spontaneous labour may be different in many respects. For this reason we are now doing a similar study on spontaneous labour. One major difference between spontaneous and induced labour often concerns the timing of amniotomy, but in this our patients differed little from those of Mendez-Bauer et awl. whose membranes were ruptured when the cervix was only 2-3 cm dilated. The influence of the obstetric abnormalities leading to induction of labour is also advanced as a source of bias, but all our patients were in low-risk categories with indications for induction such as postmaturity and poor maternal weight gain. In any case, our trial was randomised and the indications for induction were similar in the different groups. Dunn is surprised that against the background of previous studies which claimed to show benefit from an upright posture we should have drawn the conclusions we did. We did our study in response to reports from other centres claiming to show an important benefit from an upright posture both to mother and child. We found no such benefit in our population. 1. Dunn,

P. M. Lancet, 1976, i, 790.

2. Mendez-Bauer, C., Arroyo, J., Garcia-Ramos, C., Menendez, A., Lavilla, M., Izqierdo, F., Villa Elizaga, I., Zamarriego, J. J. perinat. Med. 1975,

3, 89. 3. Mitre, I. N. Int.J. Gynœc. Obstet. 1974,12,

181.

Gynaecology,

T. J. McMANUS A. A. CALDER

CLASSIFICATION OF OSTEOGENESIS IMPERFECTA

POSTURE IN LABOUR

SIR,-Our study of maternal posture during induced labour (Jan. 14, p. 78), in which we found no benefit associated with an upright posture, has been severely criticised by Dr Dunn (March 4, p. 496). Dunn is a well-known advocate of an

and

SIR,-Dr Levin and colleagues (Feb. 11, p. 332) propose a subclassification of the dominantly inherited syndrome of osteogenesis imperfecta (0.1.) associated with distinctly blue sclera’ and presenile otosclerotic deafness. This classification was based on the presence or absence of dentiogenesis imperfecta. Levin et al. call for a comprehensive classification of 0.1. syndromes which takes into account cases with newborn presentation, lethal cases, and pedigrees with autosomal recessive inheritance. A genetic and epidemiological study of 0.1. in Victoria, Australia identified four distinct syndromes,1,2 types l-iv. Three other groups have called attention to this genetic heterogeneity in o.i. and proposed that there were at least four syndromes

commonly designated osteogenesis imperfecta.3-5 The largest group (0.1. type I) had dominant inheritance of distinctly blue sclerae, bone fragility, and presenile deafness or a family history of deafness. Rarely baliies with this syndrome had multiple fractures at birth. However, these infants were of normal birth weight and length. There did not appear to be an excessive mortality from complications of 0.1. in this group. Severe long-bone deformity was uncommon and scoliosis, usually of moderate severity, was found in 20% of adolescents and adults. 40% of 65 patients of all ages examined had impaired hearing, and 40% of adults wore a hearing aid. (This syndrome is also known as van der Hoeve’s syndrome, 0.1. with mild long-bone disease, 0.1. tarda levis, among others.) Patients with 0.1. type 11 were either stillborn or died in the newborn period. These comprised the most frequent form of 0.1. in the newborn and all were small for gestational age, with short bowed limbs. Radiographs revealed concertina-like femora, beaded ribs, platyspondyly, and poor ossification of the cranial vault. This syndrome (also called lethal 0.1. or 0.1. letalis Vrolik) is not uncommon in North America and Australia. o.i. type 11 appears to be inherited in an autosomal recessive fashion. o.i. type 111 was characterised by progressive deformity of long bones and spine through childhood into adult life, bluish sclerae in infancy, and normal or pale blue sclerae by adolescence. This syndrome is also inherited in an autosomal recèssive fashion. All patients had fractures at birth (two-thirds) or during the first year of life. Patients with the dominantly inherited forms of o.i. occasionally show similar skeletal deformities but may be distinguished on the basis of family history. 0.1. type ill has been described by others as osteopsathyrosis idiopathica of Lobstein, 0.1. congenita, Vrolik’s disease, 0.1. tarda gravis, 0.1. tarda type i, la maladie de Porak et Durante, and o.i. with severe long-bone disease. A fourth group of patients (0.1. type iv) had dominant inherSillence, D. O., Senn, A., Danks, D. M. Excerpta med. int. Congr. Ser. 1977, no. 426, p. 43. 2. Sillence, D. O., Danks, D. M. Clin. Res. 1978, 26, 178A. 3. Cocchi, V. in Humangenetik (edited by Becker); vol. II, p. 151. Stuttgart, 1.

1964. 4. 5.

Ibsen, K. H. Clin. Orthoped. rel. Res. 1967, 50, 279. Lievre, I. A. Rev. Rhum. 1959, 26, 420.

Dermatomyositis and vaccination.

1040 with dietary fat intake2 and fatty-acid changes in the serum3 is well known. Baker et al. found a tendency to greater saturation of fatty acids i...
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