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CEREBROSPINAL FLUID AND SERUM IGM IN SUBACUTE SCLEROSING PANENCEPHALITIS
864 tion. This became worse, and on Feb. 3 she had an acute retention together with a fresh crop of vesicles in the perianal region. She was admitted to hospital under the care of Mr R. E. Williams for catheterisation and cystoscopy. Cystoscopy showed oedema of the trigone but no herpetic lesions in the bladder. When she was last seen (March 18) the only trouble was occasional slight stress incontinence. Blood had been taken for virological studies when the eruption first appeared and 2 and 3 iweeks later. The titres for Herpesvirus hominis and zoster were less than 16 at onset, and each rose to 128 in 2 weeks and 256 at 3zweeks. Her father, with whom she had spent Christmas, had had herpes zoster at that time. The serology confirmed zoster as the cause because zoster would account for the rise in both titres whereas hominis would not have caused the zoster titre to increase to this extent.
striking changes in c.s.F. levels of gammaand globulins, IgG, IgM in patients with S.S.P.E. the C.S.F. IgM titres would also be expected to fluctuate. The mechantibody anisms resulting in changes in the c.s.F. concentration of immunoglobulins, gamma-globulins, and the number and density of M-components in the c.s.F. gamma-globulin field reflect one of the crucial aspects in the pathogenesis of S.S.P.E.
Health Leeds LS2 9J T
early
University
Service,
D. MCCRACKEN
CEREBROSPINAL FLUID AND SERUM IGM IN SUBACUTE SCLEROSING PANENCEPHALITIS
SIR,-Kiessling et al.’ indicated that in subacute sclerosing panencephalitis (S.S.P.E.) measles-virus-specific IgM antibodies in the cerebrospinal fluid (c.s.F.) reflect persistence of the virus in the central nervous system. In 8 of 20 S.S.P.E. cases, the serum/c.s.F. ratio of measles-specific IgM antibodies was <1. During the past eight years, we have examined c.s.F. and serum from 25 S.S.P.E. patients (7 females and 18 males, with an average age of nine years). In 4 patients, immunoelectrophoresis revealed IgM precipitate in the c.s.F. c.s.F. was reexamined in 3 patients but only 1 had IgM precipitate in the repeat samples two months later. However, in this patient immunoelectrophoretic abnormalities in c.s.F. albumin and a2 macroglobulin precipitates and increased concentration of c.s.F. total proteins indicated an altered blood/c.s.F. barrier. Only 1 patient displayed increased IgM concentration in the corresponding serum specimen. In additon, in 4 patients with S.S.P.E., the serum-IgM was raised in absence of c.s.F. IgM
Because of the
probably
Department of Neurology, Indiana University Medical Center, Indianapolis, Indiana 46202, U.S.A.
OLDRICH J. KOLAR
NEW CUTANEOUS PHENOTYPE IN FAMILIAL MALIGNANT MELANOMA
SIR,-Familial malignant melanoma is characterised by an age of onset, an excess of multiple primary melanomas, and, in certain kindreds, an increased frequency of other
histological varieties of cancers.The frequency of the familial variety of this disease amongst all melanoma cases ranges from 3 to 10%. The mode of genetic transmission is consistent with an autosomal dominant factor. Unfortunately, evidence for melanoma susceptibility-save light pigmentation, red hair, or freckling, often Celtic ancestry, and excessive sun exposure-is lacking.1 Clark has described a cutaneous phenotype characterised 1. 2.
Lynch, H. T., and others. Surgery Gynec. Obstet. 1975, 141, 77. Clark, W. H. Discussion at course on Advanced Dermatopathology held at meetings of the American Academy of Dermatology, ember, 1976.
in
Chicago, in Dec-
precipitate. In our S.S.P.E. patients, the occurrence of the did not correlate with c.s.F. and/or
cipitate
c.s.F. serum
IgM premeasles
haemagglutination-inhibiting antibody titres c.s.F. total protein, the number of M-components in the c.s.F. gamma-globulin field, or c.s.F. gamma-globulin concentration (determined by cellulose-polyacetate electrophoresis) and the c.s.F. or serum IgG. IgM precipitates in the c.s.F. may be also demonstrated in 10-14% of patients with multiple sclerosis.23 The c.s.F. IgM in S.S.P.E., as in multiple sclerosis, probably represents antibodies against various antigenic determinants. For example, IgM antibodies against a water-soluble brain extract found in serum of multiple-sclerosis patients4 may have their equivalent in the c.s.F. In S.S.P.E. autoantibodies against -
water-soluble brain extract were demonstrated in the C.S.F.s IgG and/or IgM antibodies with possible cross-reactivity between measles virus and the encephalitogenic factor6 should be also considered. c.s.F. IgM measles antibody in S.S.P.E. seems to be a less reliable indicator of activity of the C.N.S. involvement than is, for example, the presence of C.S.F. IgM in neurosyphilis.’ The clinical significance of the c.s.F. IgM measles antibody titres may be studied by periodic c.s.F. examinations during the course of S.S.P.E. a
c.s.F.
W. R., Hall, W. W., Yung, L. L., ter Muelen, V. Lancet, 1977, i, 324. 2. Kolar, O., Ross, A. T., Herman, J. Neurology, Minneapolis, 1970, 20, 1052. 3. Dencker, S. J. Acta neurol. scandi. 1964, 40 suppl. 10, p. 57. 4. Ross, J., Schumacher, K., Mancete, E. Klin. Wschr. 1965, 43, 1324. 5. Kolar, O. Neurology, Minneapolis, 1968, 18, no. 1, part 2, p. 107. 6. McDermott, J. R., Field, E. J. Caspary, E. A. J. Neurol Neurosurg. Psychiat. 1974, 37, 382. 7. Oxelius, V. A., Rorsman, H., Laurell, A. B. Br. J. vener. Dis. 1969, 45, 121. 1.
Kiessling,
Fig. I-Skin of back of 37-year-old White female with cutaneous malignant melanoma of right malar surface. Note
numerous
reddish-brown
in
large irregularly shaped moles which were a stnking
colour.
striking changes in c.s.F. levels of gammaand globulins, IgG, IgM in patients with S.S.P.E. the C.S.F. IgM titres would also be expected to fluctuate. The mechantibody anisms resulting in changes in the c.s.F. concentration of immunoglobulins, gamma-globulins, and the number and density of M-components in the c.s.F. gamma-globulin field reflect one of the crucial aspects in the pathogenesis of S.S.P.E.
Health Leeds LS2 9J T
early
University
Service,
D. MCCRACKEN
CEREBROSPINAL FLUID AND SERUM IGM IN SUBACUTE SCLEROSING PANENCEPHALITIS
SIR,-Kiessling et al.’ indicated that in subacute sclerosing panencephalitis (S.S.P.E.) measles-virus-specific IgM antibodies in the cerebrospinal fluid (c.s.F.) reflect persistence of the virus in the central nervous system. In 8 of 20 S.S.P.E. cases, the serum/c.s.F. ratio of measles-specific IgM antibodies was <1. During the past eight years, we have examined c.s.F. and serum from 25 S.S.P.E. patients (7 females and 18 males, with an average age of nine years). In 4 patients, immunoelectrophoresis revealed IgM precipitate in the c.s.F. c.s.F. was reexamined in 3 patients but only 1 had IgM precipitate in the repeat samples two months later. However, in this patient immunoelectrophoretic abnormalities in c.s.F. albumin and a2 macroglobulin precipitates and increased concentration of c.s.F. total proteins indicated an altered blood/c.s.F. barrier. Only 1 patient displayed increased IgM concentration in the corresponding serum specimen. In additon, in 4 patients with S.S.P.E., the serum-IgM was raised in absence of c.s.F. IgM
Because of the
probably
Department of Neurology, Indiana University Medical Center, Indianapolis, Indiana 46202, U.S.A.
OLDRICH J. KOLAR
NEW CUTANEOUS PHENOTYPE IN FAMILIAL MALIGNANT MELANOMA
SIR,-Familial malignant melanoma is characterised by an age of onset, an excess of multiple primary melanomas, and, in certain kindreds, an increased frequency of other
histological varieties of cancers.The frequency of the familial variety of this disease amongst all melanoma cases ranges from 3 to 10%. The mode of genetic transmission is consistent with an autosomal dominant factor. Unfortunately, evidence for melanoma susceptibility-save light pigmentation, red hair, or freckling, often Celtic ancestry, and excessive sun exposure-is lacking.1 Clark has described a cutaneous phenotype characterised 1. 2.
Lynch, H. T., and others. Surgery Gynec. Obstet. 1975, 141, 77. Clark, W. H. Discussion at course on Advanced Dermatopathology held at meetings of the American Academy of Dermatology, ember, 1976.
in
Chicago, in Dec-
precipitate. In our S.S.P.E. patients, the occurrence of the did not correlate with c.s.F. and/or
cipitate
c.s.F. serum
IgM premeasles
haemagglutination-inhibiting antibody titres c.s.F. total protein, the number of M-components in the c.s.F. gamma-globulin field, or c.s.F. gamma-globulin concentration (determined by cellulose-polyacetate electrophoresis) and the c.s.F. or serum IgG. IgM precipitates in the c.s.F. may be also demonstrated in 10-14% of patients with multiple sclerosis.23 The c.s.F. IgM in S.S.P.E., as in multiple sclerosis, probably represents antibodies against various antigenic determinants. For example, IgM antibodies against a water-soluble brain extract found in serum of multiple-sclerosis patients4 may have their equivalent in the c.s.F. In S.S.P.E. autoantibodies against -
water-soluble brain extract were demonstrated in the C.S.F.s IgG and/or IgM antibodies with possible cross-reactivity between measles virus and the encephalitogenic factor6 should be also considered. c.s.F. IgM measles antibody in S.S.P.E. seems to be a less reliable indicator of activity of the C.N.S. involvement than is, for example, the presence of C.S.F. IgM in neurosyphilis.’ The clinical significance of the c.s.F. IgM measles antibody titres may be studied by periodic c.s.F. examinations during the course of S.S.P.E. a
c.s.F.
W. R., Hall, W. W., Yung, L. L., ter Muelen, V. Lancet, 1977, i, 324. 2. Kolar, O., Ross, A. T., Herman, J. Neurology, Minneapolis, 1970, 20, 1052. 3. Dencker, S. J. Acta neurol. scandi. 1964, 40 suppl. 10, p. 57. 4. Ross, J., Schumacher, K., Mancete, E. Klin. Wschr. 1965, 43, 1324. 5. Kolar, O. Neurology, Minneapolis, 1968, 18, no. 1, part 2, p. 107. 6. McDermott, J. R., Field, E. J. Caspary, E. A. J. Neurol Neurosurg. Psychiat. 1974, 37, 382. 7. Oxelius, V. A., Rorsman, H., Laurell, A. B. Br. J. vener. Dis. 1969, 45, 121. 1.
Kiessling,
Fig. I-Skin of back of 37-year-old White female with cutaneous malignant melanoma of right malar surface. Note
numerous
reddish-brown
in
large irregularly shaped moles which were a stnking
colour.