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.
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.
