Corpos de inclusão citoplasmática: estudo em diversas doenças e revisão da literatura

Scola, Rosana Herminia; Werneck, Lineu Cesar; Franco, Célia Regina Cavichiolo

doi:10.1590/S0004-282X1996000200012

Resumos

Estudamos 16 casos entre 1400 biópsias musculares que apresentavam vacúolos marginados, cujo aspecto histológico sugeria corpos de inclusão citoplasmáticos. Procuramos correlacionar os dados clínicos, laboratoriais e histopatológicos, a fim de determinar a especificidade dos corpos de inclusão citoplasmáticos para determinadas doenças. A creatinaquinase mostrou-se elevada em 10 casos. A eletromiografia foi anormal em todos os casos. A histoquímica muscular em 5 casos revelou uma miopatia, em 7 padrão misto, em dois desinervação e em 2 casos miopatia inflamatória. A microscopia eletrônica demonstrou a presença de filamentos em 8 casos (nucleares, dispersos no citoplasma ou na região subsarcolemal). Os pacientes foram classificados conforme a história clínica, hereditariedade, dados laboratoriais, eletrofisiológicos, histoquímicos e microscopia eletrônica Encontramos miosite com corpos de inclusão citoplasmática (4 casos), atrofia muscular espinhal juvenil (6 casos), miopatias distais (3 casos), distrofia de cinturas pélvica e escapular (2 casos) e polineuropatia periférica (1 caso). Apresentamos revisão sobre a patogenia, formação e possível etiologia dos vacúolos marginados e sua relação com as diversas entidades em que foram detectados, sugerindo que não são específicos para uma única doença.

corpos de inclusão citoplasmática; vacúolos marginados; miosite com corpos de inclusão citoplasmática; atrofia muscular espinhal juvenil; miopatias distais; distrofias de cinturas pélvica e escapular

Among 1400 muscle biopsies, we studied 16 cases with rimmed vacuoles, whose histology suggests cytoplasm inclusion bodies. We tried to correlate the clinical, laboratory and histopatological data in order to verify the specificity of cytoplasm inclusion bodies to certain diseases. The creatinekinase was increased in 10 cases. In all cases electromyography was abnormal. Muscle histochemistry revealed myopathy in 5 cases, mixed pattern in 7, denervation in 2 and in 2 cases, inflammatory myopathy. Electron microscopy showed the presence of filaments in 8 cases (nuclear, disseminated in cytoplasm or in the subsarcolemmal region). The patients were classified according to history, heredity, laboratory, electrophysiologic, histochemistry data and electron microscopy: in myositis with inclusion cytoplasmic bodies (4 cases), juvenile spinal muscular atrophy (6 cases), distal myopathies (3 cases), limb-girdle dystrophy (2 cases) and peripheral neuropathy (1 case). We present a revision on the pathogenesis and possible etiology of rimmed vacuoles and their relationship with several diseases.

cytoplasmic inclusion bodies; rimmed vacuoles; inclusion body myositis; juvenile spinal muscular atrophy; distal myopathies; limb-girdle muscular dystrophy

Corpos de inclusão citoplasmática: estudo em diversas doenças e revisão da literatura

Inclusion cytoplasmic bodies: a study in several diseases and a literature review

Rosana Herminia Scola^I; Lineu Cesar Werneck^II; Célia Regina Cavichiolo Franco^III

^IProfessora Assistente

^IIProfessor Titular

^IIIAuxiliar de Ensino de Biologia

RESUMO

Estudamos 16 casos entre 1400 biópsias musculares que apresentavam vacúolos marginados, cujo aspecto histológico sugeria corpos de inclusão citoplasmáticos. Procuramos correlacionar os dados clínicos, laboratoriais e histopatológicos, a fim de determinar a especificidade dos corpos de inclusão citoplasmáticos para determinadas doenças. A creatinaquinase mostrou-se elevada em 10 casos. A eletromiografia foi anormal em todos os casos. A histoquímica muscular em 5 casos revelou uma miopatia, em 7 padrão misto, em dois desinervação e em 2 casos miopatia inflamatória. A microscopia eletrônica demonstrou a presença de filamentos em 8 casos (nucleares, dispersos no citoplasma ou na região subsarcolemal). Os pacientes foram classificados conforme a história clínica, hereditariedade, dados laboratoriais, eletrofisiológicos, histoquímicos e microscopia eletrônica Encontramos miosite com corpos de inclusão citoplasmática (4 casos), atrofia muscular espinhal juvenil (6 casos), miopatias distais (3 casos), distrofia de cinturas pélvica e escapular (2 casos) e polineuropatia periférica (1 caso). Apresentamos revisão sobre a patogenia, formação e possível etiologia dos vacúolos marginados e sua relação com as diversas entidades em que foram detectados, sugerindo que não são específicos para uma única doença.

Palavras-chave: corpos de inclusão citoplasmática, vacúolos marginados, miosite com corpos de inclusão citoplasmática, atrofia muscular espinhal juvenil, miopatias distais, distrofias de cinturas pélvica e escapular.

ABSTRACT

Among 1400 muscle biopsies, we studied 16 cases with rimmed vacuoles, whose histology suggests cytoplasm inclusion bodies. We tried to correlate the clinical, laboratory and histopatological data in order to verify the specificity of cytoplasm inclusion bodies to certain diseases. The creatinekinase was increased in 10 cases. In all cases electromyography was abnormal. Muscle histochemistry revealed myopathy in 5 cases, mixed pattern in 7, denervation in 2 and in 2 cases, inflammatory myopathy. Electron microscopy showed the presence of filaments in 8 cases (nuclear, disseminated in cytoplasm or in the subsarcolemmal region). The patients were classified according to history, heredity, laboratory, electrophysiologic, histochemistry data and electron microscopy: in myositis with inclusion cytoplasmic bodies (4 cases), juvenile spinal muscular atrophy (6 cases), distal myopathies (3 cases), limb-girdle dystrophy (2 cases) and peripheral neuropathy (1 case). We present a revision on the pathogenesis and possible etiology of rimmed vacuoles and their relationship with several diseases.

Key words: cytoplasmic inclusion bodies; rimmed vacuoles, inclusion body myositis, juvenile spinal muscular atrophy, distal myopathies, limb-girdle muscular dystrophy.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Agradecimentos - Agradecemos ao Dr. Guilberto Minghetti pelo auxílio na interpretação das microscopias eletrônicas e às Sitas. Sumico Nakagawa e Angela Chiquito pela colaboração técnica.

Aceite: 12-janeiro-1996.

Estudo realizado no Serviço de Doenças Neuromusculares, Especialidade de Neurologia e Propedêutica Médica do Hospital de Clínicas e Departamento de Morfologia da Universidade Federal do Paraná, Curitiba

Dra. Rosana Herminia Scola - Serviço de Doenças Neuromusculares, Hospital de Clínicas da UFPR - Rua General Carneiro 181 3º andar - 80069-155 Curitiba PR - Brasil.

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2. Adams RD, Kakulas BA, Samaha FA. Myopathy with cellular inclusions. Trans Am Neurol Assoc 1965;90:213-216.
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Datas de Publicação

Publicação nesta coleção
06 Dez 2010
Data do Fascículo
Jun 1996

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

[1] 1. Abarbanel JM, Lichienfeld Y, Zirkin H, Louzon Z, Osimani A, Farkash P, Herishanu Y. Inclusion body myositis in post-poliomyelitis muscular atrophy, Acta Neurol Scand 1988;78:81-84.

[2] 2. Adams RD, Kakulas BA, Samaha FA. Myopathy with cellular inclusions. Trans Am Neurol Assoc 1965;90:213-216.

[3] 3. Arahata K, Engel AG. Monoclonal antibody analysis of mononuclear cells in myopathies: I. Quantitation of subsets according to diagnosis and sites of accumulation and demonstration and counts of muscle fibers invaded by T cells. Ann Neurol 1984;16:193-208.

[4] 4. Arahata K, Engel, AG. Monoclonal antibody analysis of mononuclear cells in myopathies: V. Identification and quantitation of T8+ cytotoxic and T8+ suppressor cells. Ann Neurol 1988;23:493-499.

[5] 5. Aigov Z, Yarom R. "Rimmed vacuole myopathy" sparing the quadriceps. J Neurol Sci 1984,64:33-43.

[6] 6. Askanas V, Alvarez, RB. Fast and reliable new method for electron-microscopic identification of cytoplasmic tubulo-fi laments in biopsies of patients with inclusion-body myositis. Acta Neuropathol 1992;84:335-336.

[7] 7. Askanas V, Alvarez RB, Engel WK. Amyloid precursor epitopes in muscle fibers of inclusion body myositis. Ann Neurol 1993;34:551-560.

[8] 8. Askanas V, Bilak M, Engel WK, Alvarez RB, Tome F, Leclerc A. Prion protein abnormally accumulated in inclusion-body myositis. Neuro Report 1993;5:25-28.

[9] 9. Askanas V, Engel K, Alvarez, RB. Light and electron microscopic localization of beta-amyloid protein in muscle biopsies of patients with inclusion-body myositis. Am J Pathol 1992; 141:3136.

[10] 10. Askanas V, Serdaroglu P, Engel K, Alvarez RB. Immunolocalization of ubiquitin in muscle biopsies of patients with inclusion body myositis and oculopharyngeal muscular dystrophy. Neurose Lett 1991;130:73-76.

[11] 11. Askanas V, Serdaroglu P, Engel K, Alvarez, RB. Immunocytochemical loclization of ubiquitin in inclusion body myositis allows its light-microscopic distinction from polymiositis. Neurology 1992;42:460-461.

[12] 12. Askanas V, Engel K, Alvarez RB. Beta-amyloid protein immunoreactivity in muscle of patients with inclusion-body myositis. Lancet 1992;339:560-561.

[13] 13. Benke B. Mass occurrence of multilamellar bodies in myopathy. Virchows Arch (Cell Pathol) 1976;20:77-84

[14] 14. Bhagavati S, Ehrlich G, Kula RW, Kewok S, Sninsky J, Udani V, Poeies BJ. Detection of human T-celi lymphoma/leukemia virus type I DNA and antigen in spinal fluid and blood of patiemts wih chronic progressive myelopathy. N Engl J Med 1988;318:1141-1147.

[15] 15. Bilak M, Askanas V, Engel A. Strong imumunoreactivity of alpha-antichymotrypsin co-localizes witth beta-amyloid protein and ubiquitin in vacuolated muscle fibers of inclusion-body myositis. Acta Neuropathol 1993;85:378-382.

[16] 16. Bird SJ. Inclusion body myositis: diagnostic value of repeat biobsy. Muscle & Nerve 1992;15:1206-1297.

[17] 17. Brown WF, Gilliatt RG. The physiological and technical basis of electromyography. Boston: Butterworth, 1984.

[18] 18. Buchthal F. An introduction to electromyography. Gyldendal-Kobenhanrm: Scandinavian Univ Books, 1957.

[19] 19. Calabrese LH, Mitsumoto H, Chou S. Inclusion body myositis presenting as treatment-resistant polymyositis. Arthritis Rheum 1987;30:397-403.

[20] 20. Carpenter S, Karpati G, Heller I, Eisen A. Inclusion body myositis: a distinct variety of idiopathic inflammatory myopathy. Neurology 1978;28:8-17.

[21] 21. Chad D, Good P. Adelman L, Bradley WG, Mills J. Inclusion body myositis associated with Sjogren's syndrome. Arch Neurol 1982;39:186-188.

[22] 22. Chou SM. Myxovirus-like structures in a case human of chronic polymyositis. Science 1967;158:1453.

[23] 23. Chou SM. Myxovirus-like structures and accompanying nuclear changes in chronic polymyositis. Arch Pathol 1968;86:649-658.

[24] 24. Chou SM, Mizuno Y. Inclusion body myositis: a chronic persistente mumps myositis? Hum Pathol 1986;17:765-777.

[25] 25. Cole AJ, Kuzniecky R, Karpati G, Carpenter S, Andermann E, Andermann F. Familial myopathy with changes resembling inclusion body myositis and periventricular leucoencephalopathy. Brain 1988;111:1025-1037.

[26] 26. Coquet M, Vital C, Juiien J. Presence of inclusion body myositis-like filaments in oculopharyngeal muscular dystrophy: ultrastructural study of 10 cases. Neuropathol Appl Neurobiol 1990; 16:393-400.

[27] 27. Danon JD, Reys MG, Perurena, OH, Masdeu JC, Manaligod JR. Inclusion body myositis: a corticosteroid resistant idiopathic inflamatory myopathy.Arch Neurol 1982;39:760-764.

[28] 28. Desmet JE, Borenstein S. Relatonship of spontaneous fibrillation potentials to muscle fibre segmentation in human muscular dystrophy. Nature 1975;258:531-534.

[29] 29. Dimitriu D, Newll-Eggert M. Inclusion body myositis. Am J Phys Med Rehabil. 1990;69:2-5.

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