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Follow-up examination of 6 children after acrodynia
Volume 76 Number 6
The calcification reported by South and coworkers is definitely cortical, as manifested by the easily recognizable convolutional pattern of gyri and sulci. In Schaffer's patient with herpes simplex, however, no convolutional pattern was manifested and the calcification was probably periventricular in position, typical of that seen in congenital cytomegalic inclusion disease or toxoplasmosis.S Although South and co-workers mentioned that no postmortem examination was performed, they did not state whether a biopsy of the brain was attempted for isolation of the herpesvirus. The results of three urine samples and a sample of cerebrospinal fluid were stated to be negative for cytopathic effect of the herpesvirus. Since South and co-workers demonstrated the herpesvlrus to be present only in the vesicular skin lesions of their patient, their evidence implicating the herpes simplex virus as the cause of the congenital malformation of his central nervous system is, therefore, inconclusive.
Charles A. Gooding, M.D. Departments o[ Radiology and Pediatrics University of Cali[ornia Medical Center San Francisco, Calif. 94122 REFERENCES
1. South, M. A., Tompklns, W. A. F., Morris, C. R., and Rawls, W. E.: Congenital malformation of the central nervous system associated with genital type (type 2) herpesvirus, J. PEmAT. 75: 13, 1969. 2. Schaffer, A. J.: Diseases of the newborn, ed. 2, Philadelphia, 1965, W. B. Saunders Company, p. 733. 3. Mallory, P. M., and Lowman, R. M.: The lack of specificity of the neonatal intracranial paraventricular calcifications, Radiology 80: 98, 1963.
Reply To the Editor: I have consulted with Dr. Edward Singleton, chief of radiology at Texas Children's Hospital, and with Dr. Robert Evans, neuroradiologist, Methodist Hospital in Houston, We all agree that from the single frontal projection of the skull x-ray i n Schaffer,s book, w e can't say_ for sure where the intracranial calcifications are located. I n our case, the early x-rays~ were reported as "periventricular calcification," but later x-rays in several views, together with the corroborating ev!dence. Of the transillumination and the collapse of, the bone structure of the skull, indicated
Letters to the Editor
98 1
that the calcification was indeed in shrunken cerebral hemispheres. Schaffer's case did not have this kind of additional information on which to base a judgment. We agree with Dr. Gooding that our evidence is not conclusive. We were careful to state in our article that the evidence suggests that the herpesvirus has a neurotropic teratogenic potential. A brain biopsy was not done. Additional evidence will be necessary to firmly establish an etiologic role of the virus in this unusual complex of abnormalities.
Mary Ann South, M.D. Houston, Texas
Follow-up examination of 6 children after acrodynia To the Editor: It is commonly supposed that most patients who have been affected with acrodynia are either totally free of sequelae, 1 or that this disease does not leave any visible aftereffects except the scars of the ulcerations2 or bite defects, a It was thought pertinent to re-examine all the children who were hospitalized in Krak6w after World War I I with Feer's disease and who were recorded in the Polish medical literature. Of the seven patients who were summoned to be reexamined, six presented themselves at the Centre. These six children were subjected, from 3 ~ to 12I~ years after recovery from acrodynia, to physical examination, intelligence tests carried out by A. Malinowska by means of the Wechsler Intelligence Scale for Children (WISC), and EEG tracings which were interpreted by M. Pawlik of the Provincial Centre for Mental Health, Krakdw. In each case the patient was the result of a normal full-term pregnancy, uncomplicated labor, and normal newborn period. There were no instances of previous head trauma or of any neurological symptoms. Three children were found to be dull and backward. Five patients showed considerable differences between their verbal and performance score of intelligence, performance scores being lower than the verbal. In all six children performance intelligence was lower than that accepted for the respective age:group norms. On analyzing the results Obtained by WISC, one m i g h t reach the
982
Letters to the Editor
conclusion that five of the examined children have organic brain damage somewhat localized on the right sider In all six chiIdren EEG tracings revealed rightsided alterations; two of them suffered from paroxysmal symptoms. 4 The alterations revealed in the bioelectric activity of the brain in all six cases indicate possible organic damage to the central nervous system in those parts of the brain which have a bearing upon the derivations from the right temporal regions toward the back. Negative interview findings and the investigations reported by Marie and associates5 corroborate our conclusion that the deviations from the norm revealed by means of the intelligence tests and in the EEG tracings should be attributed to Feet's disease. Three children had skin disorders of an allergic nature. Two of them also had psoriasis. That children suffering from acrodynia are subject to various disturbances of an allergic nature has been pointed out by Fanconi and Wallgren s and, among Polish authors, by Go~cifiska and associates5 Three of the examined children showed excessive nervous excitability. The mothers of four of the children have been for many years treated for so-called vegetative dystonla. The fact that children who had once suffered from acrodynia are frequently subject to vegetative dystonia has been pointed out by Fanconi and associates,a and among Polish authors, by Klepacki and associates,s Leys and Cameron 9 have stressed that most of the mothers of their child patients treated for acrodynia were mentally unstable. Four of the children had jaw and bit e irregularities; three of them had overbite and one had bilateral crossbite. These irregularities may have been caused by the acrodynia as a result of damage to buds of permanent teeth during the acute phase of the illness. In one child scarring of the tongue was observed as a vestige of an extensive ulceration which occurred in the course of the acrodynia. Marek Barbacki Provincial Centre [or the Mother and Child, Krakdw, Poland REFERENCES
I. Dekaban, A.: Neurology of infancy, Baltimore, 1959, The Williams & Wilklns Company. 2. Ford, F. R.: Diseases of the nervous system in infancy, childhood and adolescence, Springfield, Ill., 1960, Charles C Thomas, Publisher. 3. Pstragowska, W.:~Akrodynia, Pol. Tyg. lek, I4: 1170, 1959,
The Journal of Pediatrics June 1970
4. Pawlik, M., and Barbacki, M.: Alt6rations de l'61ectro-enc6phalogramme constat6es chez six enfants une longue periode apr~s acrodynie, Ann. Pediat. 7: 146, 1970. 5. Marie, J., Bricaire, H., R6mond, A., LerlqueKoechlin, A., Nekhorocheff, I., and Job, J. C.: Les alt6rations de l'61ectro-enc6phalogramme au course de l'acrodynle infantile, Sere. Hop. Paris 73: 3786, 1953. 6. Fanconi, G., and Wallgren, A.: Lehrbuch der P~idiatrie, Basel-Stuttgart, 1961, Benno Schwabe Verlag. 7. Go~eifiska, Z., Bartoszewicz, B., and Piasecka, M.: Neurological symptomatology in cases of chronic mercury poisoning in children, Pediat. Pol. 42: 297, 1967. 8. Klepacki, W., Gerkowicz, T., and Sta~kiewicz, J.: Acrodynia--Swift's and Feer's disease, Pol. Tyg. Lek. 13: 510, 1958. 9. Leys, I)., and Cameron, K.: A psychiatric study of six cases of infantile acrodynia, Brit. Med. J. 4751: 191, 1952.
Active chronic hepatitis in infancy To the Editor: The paper by Benjamins and SunshinO once again suggests that the widely held concept that active "chronic hepatitis" occurs mainly in young women and as a well-defined entity should be abandoned. It seems to be sufficiently documented that chronic or progressive liver disease is not rare, occurs in both sexes, and does not spare any age group. Systemic manifestations, however, are observed more frequently in young females. 2 The variety of names (chronic hepatitis, lupoid hepatitis, plasma-cell hepatitis, and variants thereof) attached to chronic types of liver disease is largely a tribute to our ignorance about the etiology(ies) of these conditions. Yet there is a need to classify these disorders. An etiologic classification (like "-itis") is impractical and confusing. The descriptive term "chronic active liver disease" for these conditions is perhaps preferable. Criteria such as chronicity (continuing or recurrent disease possibly leading to cirrhosis) and activity (perpetuation of the initial injury and referring to hepatocellular necrosis) seem less controversial and more meaningful from the point of view of prognosis and management.S These 2 criteria are useful to all of the liver disorders described. Ultimately, the ideal nomenclature will bear etiologic, prognostic, or therapeutic significance,a We have, indeed, few ideas about the cause(s) leading to chronic active liver disease, and factors responsible for the initial injury may not be
The calcification reported by South and coworkers is definitely cortical, as manifested by the easily recognizable convolutional pattern of gyri and sulci. In Schaffer's patient with herpes simplex, however, no convolutional pattern was manifested and the calcification was probably periventricular in position, typical of that seen in congenital cytomegalic inclusion disease or toxoplasmosis.S Although South and co-workers mentioned that no postmortem examination was performed, they did not state whether a biopsy of the brain was attempted for isolation of the herpesvirus. The results of three urine samples and a sample of cerebrospinal fluid were stated to be negative for cytopathic effect of the herpesvirus. Since South and co-workers demonstrated the herpesvlrus to be present only in the vesicular skin lesions of their patient, their evidence implicating the herpes simplex virus as the cause of the congenital malformation of his central nervous system is, therefore, inconclusive.
Charles A. Gooding, M.D. Departments o[ Radiology and Pediatrics University of Cali[ornia Medical Center San Francisco, Calif. 94122 REFERENCES
1. South, M. A., Tompklns, W. A. F., Morris, C. R., and Rawls, W. E.: Congenital malformation of the central nervous system associated with genital type (type 2) herpesvirus, J. PEmAT. 75: 13, 1969. 2. Schaffer, A. J.: Diseases of the newborn, ed. 2, Philadelphia, 1965, W. B. Saunders Company, p. 733. 3. Mallory, P. M., and Lowman, R. M.: The lack of specificity of the neonatal intracranial paraventricular calcifications, Radiology 80: 98, 1963.
Reply To the Editor: I have consulted with Dr. Edward Singleton, chief of radiology at Texas Children's Hospital, and with Dr. Robert Evans, neuroradiologist, Methodist Hospital in Houston, We all agree that from the single frontal projection of the skull x-ray i n Schaffer,s book, w e can't say_ for sure where the intracranial calcifications are located. I n our case, the early x-rays~ were reported as "periventricular calcification," but later x-rays in several views, together with the corroborating ev!dence. Of the transillumination and the collapse of, the bone structure of the skull, indicated
Letters to the Editor
98 1
that the calcification was indeed in shrunken cerebral hemispheres. Schaffer's case did not have this kind of additional information on which to base a judgment. We agree with Dr. Gooding that our evidence is not conclusive. We were careful to state in our article that the evidence suggests that the herpesvirus has a neurotropic teratogenic potential. A brain biopsy was not done. Additional evidence will be necessary to firmly establish an etiologic role of the virus in this unusual complex of abnormalities.
Mary Ann South, M.D. Houston, Texas
Follow-up examination of 6 children after acrodynia To the Editor: It is commonly supposed that most patients who have been affected with acrodynia are either totally free of sequelae, 1 or that this disease does not leave any visible aftereffects except the scars of the ulcerations2 or bite defects, a It was thought pertinent to re-examine all the children who were hospitalized in Krak6w after World War I I with Feer's disease and who were recorded in the Polish medical literature. Of the seven patients who were summoned to be reexamined, six presented themselves at the Centre. These six children were subjected, from 3 ~ to 12I~ years after recovery from acrodynia, to physical examination, intelligence tests carried out by A. Malinowska by means of the Wechsler Intelligence Scale for Children (WISC), and EEG tracings which were interpreted by M. Pawlik of the Provincial Centre for Mental Health, Krakdw. In each case the patient was the result of a normal full-term pregnancy, uncomplicated labor, and normal newborn period. There were no instances of previous head trauma or of any neurological symptoms. Three children were found to be dull and backward. Five patients showed considerable differences between their verbal and performance score of intelligence, performance scores being lower than the verbal. In all six children performance intelligence was lower than that accepted for the respective age:group norms. On analyzing the results Obtained by WISC, one m i g h t reach the
982
Letters to the Editor
conclusion that five of the examined children have organic brain damage somewhat localized on the right sider In all six chiIdren EEG tracings revealed rightsided alterations; two of them suffered from paroxysmal symptoms. 4 The alterations revealed in the bioelectric activity of the brain in all six cases indicate possible organic damage to the central nervous system in those parts of the brain which have a bearing upon the derivations from the right temporal regions toward the back. Negative interview findings and the investigations reported by Marie and associates5 corroborate our conclusion that the deviations from the norm revealed by means of the intelligence tests and in the EEG tracings should be attributed to Feet's disease. Three children had skin disorders of an allergic nature. Two of them also had psoriasis. That children suffering from acrodynia are subject to various disturbances of an allergic nature has been pointed out by Fanconi and Wallgren s and, among Polish authors, by Go~cifiska and associates5 Three of the examined children showed excessive nervous excitability. The mothers of four of the children have been for many years treated for so-called vegetative dystonla. The fact that children who had once suffered from acrodynia are frequently subject to vegetative dystonia has been pointed out by Fanconi and associates,a and among Polish authors, by Klepacki and associates,s Leys and Cameron 9 have stressed that most of the mothers of their child patients treated for acrodynia were mentally unstable. Four of the children had jaw and bit e irregularities; three of them had overbite and one had bilateral crossbite. These irregularities may have been caused by the acrodynia as a result of damage to buds of permanent teeth during the acute phase of the illness. In one child scarring of the tongue was observed as a vestige of an extensive ulceration which occurred in the course of the acrodynia. Marek Barbacki Provincial Centre [or the Mother and Child, Krakdw, Poland REFERENCES
I. Dekaban, A.: Neurology of infancy, Baltimore, 1959, The Williams & Wilklns Company. 2. Ford, F. R.: Diseases of the nervous system in infancy, childhood and adolescence, Springfield, Ill., 1960, Charles C Thomas, Publisher. 3. Pstragowska, W.:~Akrodynia, Pol. Tyg. lek, I4: 1170, 1959,
The Journal of Pediatrics June 1970
4. Pawlik, M., and Barbacki, M.: Alt6rations de l'61ectro-enc6phalogramme constat6es chez six enfants une longue periode apr~s acrodynie, Ann. Pediat. 7: 146, 1970. 5. Marie, J., Bricaire, H., R6mond, A., LerlqueKoechlin, A., Nekhorocheff, I., and Job, J. C.: Les alt6rations de l'61ectro-enc6phalogramme au course de l'acrodynle infantile, Sere. Hop. Paris 73: 3786, 1953. 6. Fanconi, G., and Wallgren, A.: Lehrbuch der P~idiatrie, Basel-Stuttgart, 1961, Benno Schwabe Verlag. 7. Go~eifiska, Z., Bartoszewicz, B., and Piasecka, M.: Neurological symptomatology in cases of chronic mercury poisoning in children, Pediat. Pol. 42: 297, 1967. 8. Klepacki, W., Gerkowicz, T., and Sta~kiewicz, J.: Acrodynia--Swift's and Feer's disease, Pol. Tyg. Lek. 13: 510, 1958. 9. Leys, I)., and Cameron, K.: A psychiatric study of six cases of infantile acrodynia, Brit. Med. J. 4751: 191, 1952.
Active chronic hepatitis in infancy To the Editor: The paper by Benjamins and SunshinO once again suggests that the widely held concept that active "chronic hepatitis" occurs mainly in young women and as a well-defined entity should be abandoned. It seems to be sufficiently documented that chronic or progressive liver disease is not rare, occurs in both sexes, and does not spare any age group. Systemic manifestations, however, are observed more frequently in young females. 2 The variety of names (chronic hepatitis, lupoid hepatitis, plasma-cell hepatitis, and variants thereof) attached to chronic types of liver disease is largely a tribute to our ignorance about the etiology(ies) of these conditions. Yet there is a need to classify these disorders. An etiologic classification (like "-itis") is impractical and confusing. The descriptive term "chronic active liver disease" for these conditions is perhaps preferable. Criteria such as chronicity (continuing or recurrent disease possibly leading to cirrhosis) and activity (perpetuation of the initial injury and referring to hepatocellular necrosis) seem less controversial and more meaningful from the point of view of prognosis and management.S These 2 criteria are useful to all of the liver disorders described. Ultimately, the ideal nomenclature will bear etiologic, prognostic, or therapeutic significance,a We have, indeed, few ideas about the cause(s) leading to chronic active liver disease, and factors responsible for the initial injury may not be