Neurite hipertrófica intersticial: estudo de três casos

Werneck, Lineu Cesar; Mendonça, Eudilson

doi:10.1590/S0004-282X1978000300010

Resumos

São relatados 3 casos de neuropatia hipertrófica intersticial que apresentam conduções nervosas motoras bastante reduzidas; no estudo anatomopatológico foram encontradas estruturas em forma de "casca de cebola", com proliferação de tecido conjuntivo. São abordadas as teorias sobre a formação das "cascas de cebola" e a correlação com a diminuição da velocidade de condução nervosa. Os autores concluem que somente um dos casos corresponde a descrição original de Dejerine-Sottas, sendo que os outros são provavelmente doença de Charcot-Marie-Tooth.

Three cases of hypertrophic interstitial neuropathy with severe decrease of motor nerve conduction velocity are reported; on sural nerve biopsy was found the typical onion bulb formation, with connective tissue proliferation. The pathogenesis of the onion bulb formation and the correlation with the decreased motor nerve conduction are discussed. The authors concluded that only one of the cases could be named Dejerine-Sottas disease, since the others probably are Charcot-Marie-Tooth disease cases.

Neurite hipertrófica intersticial: estudo de três casos

Hypertrophic interstitial neuropathy: report of 3 cases

Lineu Cesar Werneck^I; Eudilson Mendonça^II

^IProfessor Assistente. Disciplina de Neurologia do Departamento de Clinica Médica da Universidade Federal do Paraná, Curitiba, PR

^IIResidente. Disciplina de Neurologia do Departamento de Clinica Médica da Universidade Federal do Paraná, Curitiba, PR

RESUMO

São relatados 3 casos de neuropatia hipertrófica intersticial que apresentam conduções nervosas motoras bastante reduzidas; no estudo anatomopatológico foram encontradas estruturas em forma de "casca de cebola", com proliferação de tecido conjuntivo. São abordadas as teorias sobre a formação das "cascas de cebola" e a correlação com a diminuição da velocidade de condução nervosa. Os autores concluem que somente um dos casos corresponde a descrição original de Dejerine-Sottas, sendo que os outros são provavelmente doença de Charcot-Marie-Tooth.

SUMMARY

Three cases of hypertrophic interstitial neuropathy with severe decrease of motor nerve conduction velocity are reported; on sural nerve biopsy was found the typical onion bulb formation, with connective tissue proliferation. The pathogenesis of the onion bulb formation and the correlation with the decreased motor nerve conduction are discussed. The authors concluded that only one of the cases could be named Dejerine-Sottas disease, since the others probably are Charcot-Marie-Tooth disease cases.

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Trabalho realizado na Disciplina de Neurologia do Departamento de Clinica Médica da Universidade Federal do Paraná, Curitiba, PR.

Disciplina de Neurologia, Departamento de Clinica Médica - Hospital de Clínicas da Universidade Federal do Paraná - Rua General Carneiro s/n - 80000 Curitiba, PR - Brasil.

1. ALANOR, A. G.; NAVARRO, C; LAGE. M. M. & LIANO, H. - Maladie de Dejérine-Sottas: forme proximate pseudo myopathique. Revue Neurol. (Paris) 124:451, 1971.
2. ALEXANDER, W. S. - Phytanic acid in Refsum's syndrome. J. Neurol. Neurosurg. Psychiat. 29:412, 1966.
3. APPENZELLER, O. & KORNFELD, M. - Macrodactyly and localized hypertrophic neuropathy. Neurology (Minneapolis) 26:767, 1974.
4. AUSAIN, J. H. - Observations on the syndrome of hypertrophic neuritis: the hypertrophic interstitial radiculo-neuropathies. Medicine (Baltimore) 35:187, 1956.
5. CREUTZFELDT, H. G.; CURTIUS, F. & KRÜGER, K. H. - Zur Klinik, Histologie und gemealogle der Déjerine-Sottasschen Krankheit. Arch. Psychlat. Nervenkr 186: 341, 1951.
6. DEJERINE, J. - Névrite interstitielle hypertrophique et progressive de l'enfance. Rev. Med. 16:57, 1896.
7. DOLMAN, C.L. A ALLAN, B. M. - Relapsing hypertrophic neuritis. Arch. Neurol. (Chicago) 28:351, 1973.
8. DYK, P. J. - Experimental hypertrophic neuropathy: pathogenesis of onion-bulb formation produced by repeated tourniquet application. Arch. Neurol. (Chicago) 21:73, 1969.
9. DYCK, P. J. - Inherited neuronal degeneration and atrophy affecting peripheral motor, sensory and autonomic neurons. In Dyck, P. J.; Thomas, P. K. & LAMBERT, E. H. - Peripheral Neuropathy, pg. 825-867, W. B. Saunders Co., Philadelphia. 1975.
10. DYCK, P. J. & LAMBERT, E. H. - Lower motor and primary sensory neuron disease with peroneal muscular atrophy. Arch. Neurol. (Chicago) 18:60¾, 1968.
11. DYCK, P. J.; LAMBERT, E. H.; SANDERS, K. & O'BRIEN, P. C. - Severe hypomyelination and marked abnormality of conductoin in Déjerine-Sottas hypertrophic neuropathy: myelin thickness and compound action potential of sural nerve in vitro. Mayo Clin. Proc. 46:432, 1971.
12. GATHIER, J. C. & BRUYN, G. W. - Hypertrophic Interstitial neuropathy (Dejerine-Sottas). In Vinken, P. J. & Bruyn, G. W. - Handbook of Clinical Neurology, pg. 169-179, North Holland Publ. Co., Amsterdam, 1970.
13. GOODGOLD, J. & EBERSTEIN, A. - Electrodiagnosis of neuromuscular diseases, pg. 157-205, Williams & Wilkins Co., Baltimore, 1972.
14. HAWKES, C. H.; JEFFERSON, J. M.; JONES, E. L. & SMITH, W. T. - Hypertrophic mononeuropathy. J. Neurol. Neurosurg. Psychiat. 37:76, 1974.
15. IMAGINARIO, J. G.; COELHO, B.; THOMÉ, F. & LUIZ, M. L. S. - Névrite interstitielle hypertrophique monosymptomatique. J. Neurol. Sci. 1:340, 1964.
16. KALYMARAMAN, K.; SMITH, B. H. & SCHLAGENHAUFF, R. E. - Hereditary hypertrophic neuropathy with facial and trigeminal involvement. Arch. Neurol. (Chicago) 131:15, 1974.
17. KRÜCKE, W. - ödem und seröse EntzUndug im peripheren Nerven. Virchow Arch. Path. Anat. 308:1, 1941.
18. LAMPERT, P. W. & SCHOCHET, S. S. - Demyelination and remyelination in lead neuropathy. J. Neuropath. & Exper. Neurol. 27:527, 1968.
19. LEON, G. A. & HODGES, F. J. - Subarachnoid block and enlargement of the spinal canal in hypertrophic neuritis: J. Neurol. Sci. 28:139. 1976.
20. PASQUIER, B.; COURDEC, P. & PASQUIER, D. - Corps de Renaut et neuropathia familiale du type Déjerine-Sottas: a propos de deux observations anatomo-cliniques. Semaine des Hospitaux 31:2103, 1975.
21. RAO, C.V.G.K.; FITZ, C.R. & HARDWOOD-NASH, D.C. - Déjerine Sottas syndrome in children. Am. J. Roent. Rad. Ther. Nucl. Med. 122:70, 1974.
22. SIMPSON, D. A. & FOWLER, M. - Two cases of localized hypertrophic neurofibromatosis. J. Neurol. Neurosurg. Psychiat. 29:80, 1966.
23. WELLER, R.O. & GUPTA, T.K. - Experimental hypertrophic neuropathy: an electron microscope study. J. Neurol. Neurosurg. Psychiat. 31:34, 1968.

Datas de Publicação

Publicação nesta coleção
28 Ago 2012
Data do Fascículo
Set 1978

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. ALANOR, A. G.; NAVARRO, C; LAGE. M. M. & LIANO, H. - Maladie de Dejérine-Sottas: forme proximate pseudo myopathique. Revue Neurol. (Paris) 124:451, 1971.

[2] 2. ALEXANDER, W. S. - Phytanic acid in Refsum's syndrome. J. Neurol. Neurosurg. Psychiat. 29:412, 1966.

[3] 3. APPENZELLER, O. & KORNFELD, M. - Macrodactyly and localized hypertrophic neuropathy. Neurology (Minneapolis) 26:767, 1974.

[4] 4. AUSAIN, J. H. - Observations on the syndrome of hypertrophic neuritis: the hypertrophic interstitial radiculo-neuropathies. Medicine (Baltimore) 35:187, 1956.

[5] 5. CREUTZFELDT, H. G.; CURTIUS, F. & KRÜGER, K. H. - Zur Klinik, Histologie und gemealogle der Déjerine-Sottasschen Krankheit. Arch. Psychlat. Nervenkr 186: 341, 1951.

[6] 6. DEJERINE, J. - Névrite interstitielle hypertrophique et progressive de l'enfance. Rev. Med. 16:57, 1896.

[7] 7. DOLMAN, C.L. A ALLAN, B. M. - Relapsing hypertrophic neuritis. Arch. Neurol. (Chicago) 28:351, 1973.

[8] 8. DYK, P. J. - Experimental hypertrophic neuropathy: pathogenesis of onion-bulb formation produced by repeated tourniquet application. Arch. Neurol. (Chicago) 21:73, 1969.

[9] 9. DYCK, P. J. - Inherited neuronal degeneration and atrophy affecting peripheral motor, sensory and autonomic neurons. In Dyck, P. J.; Thomas, P. K. & LAMBERT, E. H. - Peripheral Neuropathy, pg. 825-867, W. B. Saunders Co., Philadelphia. 1975.

[10] 10. DYCK, P. J. & LAMBERT, E. H. - Lower motor and primary sensory neuron disease with peroneal muscular atrophy. Arch. Neurol. (Chicago) 18:60¾, 1968.

[11] 11. DYCK, P. J.; LAMBERT, E. H.; SANDERS, K. & O'BRIEN, P. C. - Severe hypomyelination and marked abnormality of conductoin in Déjerine-Sottas hypertrophic neuropathy: myelin thickness and compound action potential of sural nerve in vitro. Mayo Clin. Proc. 46:432, 1971.

[12] 12. GATHIER, J. C. & BRUYN, G. W. - Hypertrophic Interstitial neuropathy (Dejerine-Sottas). In Vinken, P. J. & Bruyn, G. W. - Handbook of Clinical Neurology, pg. 169-179, North Holland Publ. Co., Amsterdam, 1970.

[13] 13. GOODGOLD, J. & EBERSTEIN, A. - Electrodiagnosis of neuromuscular diseases, pg. 157-205, Williams & Wilkins Co., Baltimore, 1972.

[14] 14. HAWKES, C. H.; JEFFERSON, J. M.; JONES, E. L. & SMITH, W. T. - Hypertrophic mononeuropathy. J. Neurol. Neurosurg. Psychiat. 37:76, 1974.

[15] 15. IMAGINARIO, J. G.; COELHO, B.; THOMÉ, F. & LUIZ, M. L. S. - Névrite interstitielle hypertrophique monosymptomatique. J. Neurol. Sci. 1:340, 1964.

[16] 16. KALYMARAMAN, K.; SMITH, B. H. & SCHLAGENHAUFF, R. E. - Hereditary hypertrophic neuropathy with facial and trigeminal involvement. Arch. Neurol. (Chicago) 131:15, 1974.

[17] 17. KRÜCKE, W. - ödem und seröse EntzUndug im peripheren Nerven. Virchow Arch. Path. Anat. 308:1, 1941.

[18] 18. LAMPERT, P. W. & SCHOCHET, S. S. - Demyelination and remyelination in lead neuropathy. J. Neuropath. & Exper. Neurol. 27:527, 1968.

[19] 19. LEON, G. A. & HODGES, F. J. - Subarachnoid block and enlargement of the spinal canal in hypertrophic neuritis: J. Neurol. Sci. 28:139. 1976.

[20] 20. PASQUIER, B.; COURDEC, P. & PASQUIER, D. - Corps de Renaut et neuropathia familiale du type Déjerine-Sottas: a propos de deux observations anatomo-cliniques. Semaine des Hospitaux 31:2103, 1975.

[21] 21. RAO, C.V.G.K.; FITZ, C.R. & HARDWOOD-NASH, D.C. - Déjerine Sottas syndrome in children. Am. J. Roent. Rad. Ther. Nucl. Med. 122:70, 1974.

[22] 22. SIMPSON, D. A. & FOWLER, M. - Two cases of localized hypertrophic neurofibromatosis. J. Neurol. Neurosurg. Psychiat. 29:80, 1966.

[23] 23. WELLER, R.O. & GUPTA, T.K. - Experimental hypertrophic neuropathy: an electron microscope study. J. Neurol. Neurosurg. Psychiat. 31:34, 1968.