Polirradiculoneuropatia desmielinizante inflamatória crônica: estudo de 18 pacientes

Calia, Leandro C.; Oliveira, Acary S. B.; Gabbai, Alberto Alain

doi:10.1590/S0004-282X1997000500006

Resumos

Neste estudo prospectivo, analisamos as características clínicas, evolução e resposta terapêutica de 18 pacientes com a forma idiopática de polirradiculoneuropatia desmielinizante inflamatória crônica, que foram acompanhados por período que variou de 4 a 127 meses. O sexo masculino predominou sobre o feminino (1,25:1) e a idade de início dos sintomas variou de 6 a 85 anos. Observamos a preponderância da forma de evolução progressiva (61,1%) sobre a forma recidivante (38,9%), bem como a baixa ocorrência de fatores predisponentes (16,7%). Todos os pacientes apresentavam comprometimento sensitivo e motor, associado a hipo ou arreflexia, enquanto apenas três (16,7%) apresentavam comprometimento de nervos cranianos. No exame do liquor, as taxas de proteínas estavam elevadas em 88,9% dos pacientes, com média de 203,4 mg/dl. A eletroneuromiografia mostrou alterações desmielinizantes em todos os pacientes, associadas a alterações axonais em 94,4% deles. Em todos os sete pacientes submetidos a biopsia de nervo sural encontramos alterações compatíveis com desmielinização/remielinização. A análise com imunofluorescência, realizada em três pacientes foi normal em um e evidenciou depósito de anticorpos anti-CD3 em dois e anti-HLA-Dr em um. Optamos pela prednisona como tratamento inicial em todos os pacientes, sendo mantida posteriormente em doses reduzidas e em dias alternados em 72,2% deles. Dois pacientes (11,1%) estão assintomáticos mesmo após retirada total da medicação e introduzimos azatioprína, associada ou não ao corticóide, nos quatro pacientes com má resposta à prednisona. Até a última avaliação, 16 pacientes (88,9%) evoluíram com melhora funcional.

polirradiculoneuropatia crônica; tratamento

This is a prospective study that describes 18 patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), idiopathic type. The patients have been followed for a period of 4 to 127 months. We evaluated the clinical characteristics, the evolution, and therapeutic response. Male patients outnumbered female patients in a proporction of 1.25:1. Symptoms first appeared at an age ranging from 6 to 85. Most of the patients denied the occurrence of preceding events and a progressive evolution prevailed over relapsing evolution. All patients had both motor and sensory dysfunction associated with loss of tendon reflexes, and only three patients (16.7%) had cranial nerve involvement. The cerebrospinal fluid protein levels were increased in 88.9% of the patients and mean level was 203.4 mg/dl. Electrophysiological studies revealed demyelination in all patients and axonal damage in 94.4%. Preponderant characteristics in the sural nerve biopsy of seven patients showed demyelination and remyelination, and changes indicative of axonal damage were often present. The anti-HLA Dr antibodies were found in the sural nerve of one patient and anti-CD3 antibodies in the sural nerve of two. All patients were first treated with prednisone. The drug was mantained in reduced doses and given in alternate days to 72.2% of the patients with success. Two patients (11.1%) are asymptomatic even after the withdrawal of all medication. We administered azathioprine, associated or not with corticoid, to the four patients who had not had a satisfactory response to the prednisone treatment. By the time of the last evaluation 16 patients (88.9%) had functional improvement.

polyradiculoneuropathy; chronic; treatment

Polirradiculoneuropatia desmielinizante inflamatória crônica: estudo de 18 pacientes

Chronic inflammatory demyelinating polyradiculoneuropathy: study of 18 patients

Leandro C. Calia^I; Acary S. B. Oliveira^II; Alberto Alain Gabbai^III

^IMestre em Neurologia pela Universidade Federal de São Paulo - Escola Paulista de Medicina (UNIFESP-EPM)

^IIDoutor em Neurologia, UNIFESP-EPM

^IIILivre Docente em Neurologia, UNIFESP-EPM

RESUMO

Neste estudo prospectivo, analisamos as características clínicas, evolução e resposta terapêutica de 18 pacientes com a forma idiopática de polirradiculoneuropatia desmielinizante inflamatória crônica, que foram acompanhados por período que variou de 4 a 127 meses. O sexo masculino predominou sobre o feminino (1,25:1) e a idade de início dos sintomas variou de 6 a 85 anos. Observamos a preponderância da forma de evolução progressiva (61,1%) sobre a forma recidivante (38,9%), bem como a baixa ocorrência de fatores predisponentes (16,7%). Todos os pacientes apresentavam comprometimento sensitivo e motor, associado a hipo ou arreflexia, enquanto apenas três (16,7%) apresentavam comprometimento de nervos cranianos. No exame do liquor, as taxas de proteínas estavam elevadas em 88,9% dos pacientes, com média de 203,4 mg/dl. A eletroneuromiografia mostrou alterações desmielinizantes em todos os pacientes, associadas a alterações axonais em 94,4% deles. Em todos os sete pacientes submetidos a biopsia de nervo sural encontramos alterações compatíveis com desmielinização/remielinização. A análise com imunofluorescência, realizada em três pacientes foi normal em um e evidenciou depósito de anticorpos anti-CD3 em dois e anti-HLA-Dr em um. Optamos pela prednisona como tratamento inicial em todos os pacientes, sendo mantida posteriormente em doses reduzidas e em dias alternados em 72,2% deles. Dois pacientes (11,1%) estão assintomáticos mesmo após retirada total da medicação e introduzimos azatioprína, associada ou não ao corticóide, nos quatro pacientes com má resposta à prednisona. Até a última avaliação, 16 pacientes (88,9%) evoluíram com melhora funcional.

Palavras-chave: polirradiculoneuropatia crônica, tratamento.

ABSTRACT

This is a prospective study that describes 18 patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), idiopathic type. The patients have been followed for a period of 4 to 127 months. We evaluated the clinical characteristics, the evolution, and therapeutic response. Male patients outnumbered female patients in a proporction of 1.25:1. Symptoms first appeared at an age ranging from 6 to 85. Most of the patients denied the occurrence of preceding events and a progressive evolution prevailed over relapsing evolution. All patients had both motor and sensory dysfunction associated with loss of tendon reflexes, and only three patients (16.7%) had cranial nerve involvement. The cerebrospinal fluid protein levels were increased in 88.9% of the patients and mean level was 203.4 mg/dl. Electrophysiological studies revealed demyelination in all patients and axonal damage in 94.4%. Preponderant characteristics in the sural nerve biopsy of seven patients showed demyelination and remyelination, and changes indicative of axonal damage were often present. The anti-HLA Dr antibodies were found in the sural nerve of one patient and anti-CD3 antibodies in the sural nerve of two. All patients were first treated with prednisone. The drug was mantained in reduced doses and given in alternate days to 72.2% of the patients with success. Two patients (11.1%) are asymptomatic even after the withdrawal of all medication. We administered azathioprine, associated or not with corticoid, to the four patients who had not had a satisfactory response to the prednisone treatment. By the time of the last evaluation 16 patients (88.9%) had functional improvement.

Key words: polyradiculoneuropathy, chronic, treatment.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Aceite: 18-julho-1997.

Dr. Leandro Cortoni Calia - Disciplina de Neurologia, Escola Paulista de Medicina, UNIFESP - Rua Botucatu 762 - 04023-900 São Paulo SP - Brasil.

1. Austin JH. Recurrent polyneuropathies and their corticosteroid treatment: five-year observations of a placebo-controlled case treated with corticotrophin, cortisone and prednisone. Brain 1958;81:157-192.
2. Azulay J-PH, Pouget J, Pellissier J-F, Blin O, Serratrice G. Polyradiculonévrites chroniques: 25 cas. Rev Neurol (Paris) 1992;148:752-761.
3. Barohn RJ, Kissel JT, Warmolts JR, Mendell JR. Chronic inflammatory demyelinating polyradiculoneuropathy: clinical characteristics, course, and recommendations for diagnostic criteria. Arch Neurol 1989;46:878-884.
4. Cornblath DR, Asbury AK, Albers JW, et al. Research criteria for diagnosis of chronic inflammatory demyelinating polyneuropathy: report from an Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force. Neurology;1991:41:617-618.
5. Cornblath DR, Chaudhry V, Griffin JW. Treatment of chronic inflammatory demyelinating polyneuropathy with intravenous immunoglobulin. Ann Neurol 1991;30:104-106.
6. Dalakas MC, Engel WK. Chronic-relapsing (dysimmune) polyneuropathy: pathogenesis and treatment. Ann Neurol 1981 ;9(Suppl.): 134-145.
7. Dolman CL, Allan BM. Relapsing hypertrophic neuritis. Arch Neurol 1973;28:351-353.
8. Donofrio PD, Tandan RUP, Albers JW. Plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve 1985;8:321-327.
9. Dyck PJ, Arnason B. Chronic inflammatory demyelinating polyradiculoneuropathy. In: Dyck PJ; Thomas PK; Lambert EH; Bunge R, (eds). Peripheral neuropathy Ed2.Philadelphia: W.B.Sauders, 1984:2101-2114.
10. Dyck PJ, Daube J, O'Brien P, et al. Plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy. N Engl J Med 1986;314:461-465.
11. Dyck PJ, Lais AC, Ohta M, et al. Chronic inflammatory polyradiculoneuropathy. Mayo Clin Proc 1975;50:621-637.
12. Dyck PJ, Litchy WJ, Kratz KM, et al. A plasma exchange versus immune globulin infusion trial in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol 1994;36:838-845.
13. Dyck PJ, O'brien PC, Oviatt KF, et al. Prednisone improves chronic inflammatory demyelinating polyradiculoneuropathy more than no treatment. Ann Neurol 1982;11:136-141.
14. Dyck PJ, O'Brien PC, Swanson C, Low P, Daube J. Combined azathioprine and prednisone in chronic inflammatory-demylinating polyneuropathy. Neurology 1985;35:1173-1176.
15. Dyck PJ, Oviatt KF, Lambert EH. Intensive evaluation of referred unclassified neuropathies yields improved diagnosis. Ann Neurol 1981;10:222-226.
16. Dyck PJ, Prineas J, Pollard J. Chronic inflammatory demyelinating polyradiculoneuropathy. In: Dyck PJ, Thomas PK, Griffin JW, Low PA, Poduslo, JF. Peripheral neuropathy. Ed3. Philadelphia: WB Saunders, 1992:1498-1517.
17. Faed JM, Day B, Pollock M, Taylor PK, Nukada H, Ammond-Tooke GD. High-dose intravenous human immunoglobulin in chronic inflammatory demyelinating polyneuropathy. Neurology 1989;39:422-425.
18. Gross MLP, Thomas PK. The treatment of chronic relapsing and chronic progressive idiopathic inflammatory polyneuropathy by plasma exchange. J Neurol Sci 1981;52:69-78.
19. Hirata MTA. Polirradiculoneurite crônica inflamatóría desmielinizante idiopática: avaliação clínica, diagnostica e terapêutica. Tese, Faculdade de Medicina da Universidade de São Paulo, São Paulo: 1994.
20. Hughes RAC. Guillain-Barré syndrome and chronic inflammatory demyelinating polyradiculoneuropathy. In Asbury AK, Thomas PK. Peripheral Nerve disorder 2. London: Butterworth-Heinemann 1995:175-204.
21. Marchiori PE, Scaff M, Callegaro D, Zanbon AA, Assis JL. Polirradiculoneurite recidivante: relato de dois casos. Arq Neuropsiquiatr 1983;41:208-211.
22. McCombe PA, Pollard JD, McLeod JG. Chronic inflammatory demyelinating polyradiculoneuropathy: a clinical and electrophysiological study of 92 cases. Brain 1987;110:1617-1630.
23. Nascimento OJM. Polirradiculoneuropatia desmielinizante inflamatória crônica (síndrome de Austin-Dyck): estudo de 45 pacientes. Tese Faculdade de Medicina da Universidade Federal Fluminense. Niterói, 1993.
24. Oh SJ. Subacute demyelinating polyneuropathy responding to corticosteroid treatment. Arch Neurol 1978;35:509-516.
25. Palmer KNV. Polyradiculoneuropathy treated with cytotoxic drugs. Lancet 1966,1:265.
26. Prineas JW, McLeod JG. Chronic relapsing polyneuritis. J Neurol Sci 1976; 27:427-458.
27. Van Doom PA, Vermeulen M, Brand A, Mulder PGH, Busch HFM. Intravenous immunoglobulin treatment in patients with chronic inflammatory demyelinating polyneuropathy. Arch Neurol 1991;48:217-220.
28. Vermeulen M, Van Doom PA, Brand A, Strengers PFW, Jennekens FGI, Busch HFM. Intravenous immunoglobulin treatment in patients with chronic inflammatory demyelinating polyneuropathy: a double blind, placebo controlled study. J Neurol Neurosurg Psychiatry 1993;56:36-39.
29. Winer JB, Hughes RAC, Anderson M. A prospective study of acute idiopathic neuropathy. J Neurol Neurosurg Psychiatry 1988;51:605-613.
30. Wrobel CJ, Watson D. Plasmapheresis in chronic demyelinating polyneuropathy. N Engl J Med 1992;326:1089-1090.

Datas de Publicação

Publicação nesta coleção
21 Out 2010
Data do Fascículo
1997

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

[1] 1. Austin JH. Recurrent polyneuropathies and their corticosteroid treatment: five-year observations of a placebo-controlled case treated with corticotrophin, cortisone and prednisone. Brain 1958;81:157-192.

[2] 2. Azulay J-PH, Pouget J, Pellissier J-F, Blin O, Serratrice G. Polyradiculonévrites chroniques: 25 cas. Rev Neurol (Paris) 1992;148:752-761.

[3] 3. Barohn RJ, Kissel JT, Warmolts JR, Mendell JR. Chronic inflammatory demyelinating polyradiculoneuropathy: clinical characteristics, course, and recommendations for diagnostic criteria. Arch Neurol 1989;46:878-884.

[4] 4. Cornblath DR, Asbury AK, Albers JW, et al. Research criteria for diagnosis of chronic inflammatory demyelinating polyneuropathy: report from an Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force. Neurology;1991:41:617-618.

[5] 5. Cornblath DR, Chaudhry V, Griffin JW. Treatment of chronic inflammatory demyelinating polyneuropathy with intravenous immunoglobulin. Ann Neurol 1991;30:104-106.

[6] 6. Dalakas MC, Engel WK. Chronic-relapsing (dysimmune) polyneuropathy: pathogenesis and treatment. Ann Neurol 1981 ;9(Suppl.): 134-145.

[7] 7. Dolman CL, Allan BM. Relapsing hypertrophic neuritis. Arch Neurol 1973;28:351-353.

[8] 8. Donofrio PD, Tandan RUP, Albers JW. Plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve 1985;8:321-327.

[9] 9. Dyck PJ, Arnason B. Chronic inflammatory demyelinating polyradiculoneuropathy. In: Dyck PJ; Thomas PK; Lambert EH; Bunge R, (eds). Peripheral neuropathy Ed2.Philadelphia: W.B.Sauders, 1984:2101-2114.

[10] 10. Dyck PJ, Daube J, O'Brien P, et al. Plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy. N Engl J Med 1986;314:461-465.

[11] 11. Dyck PJ, Lais AC, Ohta M, et al. Chronic inflammatory polyradiculoneuropathy. Mayo Clin Proc 1975;50:621-637.

[12] 12. Dyck PJ, Litchy WJ, Kratz KM, et al. A plasma exchange versus immune globulin infusion trial in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol 1994;36:838-845.

[13] 13. Dyck PJ, O'brien PC, Oviatt KF, et al. Prednisone improves chronic inflammatory demyelinating polyradiculoneuropathy more than no treatment. Ann Neurol 1982;11:136-141.

[14] 14. Dyck PJ, O'Brien PC, Swanson C, Low P, Daube J. Combined azathioprine and prednisone in chronic inflammatory-demylinating polyneuropathy. Neurology 1985;35:1173-1176.

[15] 15. Dyck PJ, Oviatt KF, Lambert EH. Intensive evaluation of referred unclassified neuropathies yields improved diagnosis. Ann Neurol 1981;10:222-226.

[16] 16. Dyck PJ, Prineas J, Pollard J. Chronic inflammatory demyelinating polyradiculoneuropathy. In: Dyck PJ, Thomas PK, Griffin JW, Low PA, Poduslo, JF. Peripheral neuropathy. Ed3. Philadelphia: WB Saunders, 1992:1498-1517.

[17] 17. Faed JM, Day B, Pollock M, Taylor PK, Nukada H, Ammond-Tooke GD. High-dose intravenous human immunoglobulin in chronic inflammatory demyelinating polyneuropathy. Neurology 1989;39:422-425.

[18] 18. Gross MLP, Thomas PK. The treatment of chronic relapsing and chronic progressive idiopathic inflammatory polyneuropathy by plasma exchange. J Neurol Sci 1981;52:69-78.

[19] 19. Hirata MTA. Polirradiculoneurite crônica inflamatóría desmielinizante idiopática: avaliação clínica, diagnostica e terapêutica. Tese, Faculdade de Medicina da Universidade de São Paulo, São Paulo: 1994.

[20] 20. Hughes RAC. Guillain-Barré syndrome and chronic inflammatory demyelinating polyradiculoneuropathy. In Asbury AK, Thomas PK. Peripheral Nerve disorder 2. London: Butterworth-Heinemann 1995:175-204.

[21] 21. Marchiori PE, Scaff M, Callegaro D, Zanbon AA, Assis JL. Polirradiculoneurite recidivante: relato de dois casos. Arq Neuropsiquiatr 1983;41:208-211.

[22] 22. McCombe PA, Pollard JD, McLeod JG. Chronic inflammatory demyelinating polyradiculoneuropathy: a clinical and electrophysiological study of 92 cases. Brain 1987;110:1617-1630.

[23] 23. Nascimento OJM. Polirradiculoneuropatia desmielinizante inflamatória crônica (síndrome de Austin-Dyck): estudo de 45 pacientes. Tese Faculdade de Medicina da Universidade Federal Fluminense. Niterói, 1993.

[24] 24. Oh SJ. Subacute demyelinating polyneuropathy responding to corticosteroid treatment. Arch Neurol 1978;35:509-516.

[25] 25. Palmer KNV. Polyradiculoneuropathy treated with cytotoxic drugs. Lancet 1966,1:265.

[26] 26. Prineas JW, McLeod JG. Chronic relapsing polyneuritis. J Neurol Sci 1976; 27:427-458.

[27] 27. Van Doom PA, Vermeulen M, Brand A, Mulder PGH, Busch HFM. Intravenous immunoglobulin treatment in patients with chronic inflammatory demyelinating polyneuropathy. Arch Neurol 1991;48:217-220.

[28] 28. Vermeulen M, Van Doom PA, Brand A, Strengers PFW, Jennekens FGI, Busch HFM. Intravenous immunoglobulin treatment in patients with chronic inflammatory demyelinating polyneuropathy: a double blind, placebo controlled study. J Neurol Neurosurg Psychiatry 1993;56:36-39.

[29] 29. Winer JB, Hughes RAC, Anderson M. A prospective study of acute idiopathic neuropathy. J Neurol Neurosurg Psychiatry 1988;51:605-613.

[30] 30. Wrobel CJ, Watson D. Plasmapheresis in chronic demyelinating polyneuropathy. N Engl J Med 1992;326:1089-1090.