Hereditary sensory radicular neuropathy: an electrophoretic and immunoelectrophoretic study of the blood serum

Scaff, Milberto; Ferri, Rubens G.; Canelas, Horacio M.

doi:10.1590/S0004-282X1970000400006

Abstracts

The protein metabolism in four cases of hereditary sensory radicular neuropathy was studied through the electrophoresis and immunoelectrophoresis of the blood serum. A slight increase of the gammaglobulin content was found in all cases. The immunoelectrophoresis showed an absense of IgA in a case, a slight increase of IgG in all cases, and an absence of IgM in two cases and a marked decrease of this macroglobulin in two cases of the disease and in their healthy parents. Only two patients showed an increase of beta1 lipoprotein.

Foi estudado, mediante eletroforese e imunoeletroforese do soro sanguíneo, o metabolismo protêico em 4 casos de neuropatia radicular sensitiva hereditária. Em todos os casos foi encontrado discreto aumento do teor de gamaglobulina. A imunoeletroforese evidenciou ausência de imunoglobulina A em um caso, discreto aumento de imunoglobulina G em todos os casos, ausência de imunoglobulina M em dois casos e acentuada diminuição desta macroglobulina nos dois outros casos, assim como nos outros membros da família aparentemente sadios. Somente em dois pacientes foi assinalado aumento de hipoproteina beta1.

Hereditary sensory radicular neuropathy: an electrophoretic and immunoelectrophoretic study of the blood serum

Neuropatia radicular sensitiva hereditária: estudo eletroforético e immunoeletroforético do soro sanguíneo

Milberto Scaff^I; Rubens G. Ferri^II; Horacio M. Canelas^III

^IAssistant Neurologist. Neurological Clinic. University of São Paulo School of Medicine, São Paulo SP, Brazil

^IIDoctor in Sciences, Department of Microbiology. University of São Paulo School of Medicine, São Paulo SP, Brazil

^IIIAssociate Professor, Neurological Clinic. University of São Paulo School of Medicine, São Paulo SP, Brazil

SUMMARY

The protein metabolism in four cases of hereditary sensory radicular neuropathy was studied through the electrophoresis and immunoelectrophoresis of the blood serum. A slight increase of the gammaglobulin content was found in all cases. The immunoelectrophoresis showed an absense of IgA in a case, a slight increase of IgG in all cases, and an absence of IgM in two cases and a marked decrease of this macroglobulin in two cases of the disease and in their healthy parents. Only two patients showed an increase of beta₁ lipoprotein.

RESUMO

Foi estudado, mediante eletroforese e imunoeletroforese do soro sanguíneo, o metabolismo protêico em 4 casos de neuropatia radicular sensitiva hereditária. Em todos os casos foi encontrado discreto aumento do teor de gamaglobulina. A imunoeletroforese evidenciou ausência de imunoglobulina A em um caso, discreto aumento de imunoglobulina G em todos os casos, ausência de imunoglobulina M em dois casos e acentuada diminuição desta macroglobulina nos dois outros casos, assim como nos outros membros da família aparentemente sadios. Somente em dois pacientes foi assinalado aumento de hipoproteina beta₁.

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Clínica Neurológica Faculdade de Medicina, Universidade de São Paulo Caixa Postal 3461 São Paulo, SP Brasil.

1. DENNY-BROWN, D. Hereditary sensory radicular neuropathy. J. Neurol. Neurosurg. Psychiat. 14:237-252, 1951.
2. FERRI, R. G., and COSSERMELLI, W. Analyse immunoélectrophorétique: micro et macro-méthodes. Rev. franç. Étud. clin. biol. 9:134-138, 1964.
3. GORNALL, A. G., BARDAWILL, C. J. and DAVID, M. M. Determination of serum proteins by means of the biuret reaction. J. biol. Chem. 177:751-766, 1949.
4. GRABAR, P. and WILLIAMS, C. A., Jr. Méthode permettant l'étude conjuguée des propriétés électrophorétiques et immunochimiques d'un mélange de proteines: application au sérum sanguin. Biochim. Biophys. Acta 10:193-194, 1953.
5. GRABAR, P. and WILLIAMS, C. A., Jr. Métode immunoélectrophorétique d'analyse de mélanges de substances antigéniques. Biochim. Biophys. Acta 17: 67-74, 1955.
6. GRASSMAN, W., and HANNIG, H. Paper electrophoresis. In Cramer, E.: Paper Chromatography. Macmillan, New York, 1954, pp. 39-43.
7. KISSEL, P., SCHMITT, J., and BARRUCAND, D. Les acropathies ulcéro-mutilantes. Rev. Praticien 17:1731-1742, 1967.
8. KOCH, G. Siringomielia. In Becker, P. E.: Genética Humana. Spanish translation of the German text. Toray, Barcelona, 1969, vol. V/l, pp. 121-139.
9. ORTIZ DE ZARATE, J. C. Sobre la acropatia úlcero-mutilante de Thévenard o neuropatia radicular sensitiva hereditaria de Hicks y Denny-Brown (acrosteólisis o seudosiringomielia lombosacra hereditaria). Acta Neuropsiquiat. argent. 3:15-23, 1957.
10. ORTIZ DE ZARATE, J. C. Acropathie ulcéro-mutilante familiale de Thévenard avec pieds creux et troubles endocrino-métaboliques: étude d'une famille et révision génétique d'aprčs la litterature. J. Génét. hum. 6:279-303, 1957.
11. SPILLANE, J. D., and WELLS, C. E. C. Acro-dystrophic Neuropathy. Oxford Univ. Press, London, 1969.
12. THÉVENARD, A L'acropathie ulcéro-mutilante familiale. Acta neurol. belg. 53:1-24, 1953.

Publication Dates

Publication in this collection
22 Apr 2013
Date of issue
Dec 1970

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[1] 1. DENNY-BROWN, D. Hereditary sensory radicular neuropathy. J. Neurol. Neurosurg. Psychiat. 14:237-252, 1951.

[2] 2. FERRI, R. G., and COSSERMELLI, W. Analyse immunoélectrophorétique: micro et macro-méthodes. Rev. franç. Étud. clin. biol. 9:134-138, 1964.

[3] 3. GORNALL, A. G., BARDAWILL, C. J. and DAVID, M. M. Determination of serum proteins by means of the biuret reaction. J. biol. Chem. 177:751-766, 1949.

[4] 4. GRABAR, P. and WILLIAMS, C. A., Jr. Méthode permettant l'étude conjuguée des propriétés électrophorétiques et immunochimiques d'un mélange de proteines: application au sérum sanguin. Biochim. Biophys. Acta 10:193-194, 1953.

[5] 5. GRABAR, P. and WILLIAMS, C. A., Jr. Métode immunoélectrophorétique d'analyse de mélanges de substances antigéniques. Biochim. Biophys. Acta 17: 67-74, 1955.

[6] 6. GRASSMAN, W., and HANNIG, H. Paper electrophoresis. In Cramer, E.: Paper Chromatography. Macmillan, New York, 1954, pp. 39-43.

[7] 7. KISSEL, P., SCHMITT, J., and BARRUCAND, D. Les acropathies ulcéro-mutilantes. Rev. Praticien 17:1731-1742, 1967.

[8] 8. KOCH, G. Siringomielia. In Becker, P. E.: Genética Humana. Spanish translation of the German text. Toray, Barcelona, 1969, vol. V/l, pp. 121-139.

[9] 9. ORTIZ DE ZARATE, J. C. Sobre la acropatia úlcero-mutilante de Thévenard o neuropatia radicular sensitiva hereditaria de Hicks y Denny-Brown (acrosteólisis o seudosiringomielia lombosacra hereditaria). Acta Neuropsiquiat. argent. 3:15-23, 1957.

[10] 10. ORTIZ DE ZARATE, J. C. Acropathie ulcéro-mutilante familiale de Thévenard avec pieds creux et troubles endocrino-métaboliques: étude d'une famille et révision génétique d'aprčs la litterature. J. Génét. hum. 6:279-303, 1957.

[11] 11. SPILLANE, J. D., and WELLS, C. E. C. Acro-dystrophic Neuropathy. Oxford Univ. Press, London, 1969.

[12] 12. THÉVENARD, A L'acropathie ulcéro-mutilante familiale. Acta neurol. belg. 53:1-24, 1953.