Chronic recurrent Guillain-Barré syndrome: report of 3 cases

Mello, Antônio Rodrigues de; Freitas, Marcos R. G. de; Chimelli, Leila

doi:10.1590/S0004-282X1989000100012

Abstracts

The classical Guillain-Barre syndrome is an acute or subacute polyradiculoneuropathy whose main clinical features are progressive weakness of the limbs, decrease or absence of tendon reflexes, and sensory changes. Although in most of the cases there is complete recovery in weeks or months, some patients have a slow and progressive relapsing course and present thickening of the peripheral nerves. In this paper we describe three cases of the chronic and relapsing variety of Guillain-Barré syndrome, two of which had prominent hypertrophic changes in the peripheral nerves with onion bulb formations The clinical and pathological features of this disease are reviewed. The three patients improved with the use of steroids.

A síndrome de Guillain-Barré clássica é polirradiculoneuropatia aguda ou sub-aguda, cujos principais aspectos clínicos são: fraqueza progressiva dos membros, redução ou ausência de reflexos tendinosos e alterações sensitivas. Embora na maioria dos casos haja recuperação completa em semanas ou meses, em alguns pacientes o curso é lento, progressivo ou recidivante e há espessamento dos nervos periféricos. No presente artigo descrevemos três casos da variedade crônica e recidivante da síndrome de Guillain-Barré, dois dos quais tinham alterações hipertróficas proeminentes nos nervos periféricos com formações em bulbo de cebola. Os aspectos clínicos e patológicos desta doença são revistos. Os três pacientes melhoraram com o uso de esteróides.

Chronic recurrent Guillain-Barré syndrome: report of 3 cases

Síndrome de Guillain-Barré crônica recorrente: registro de 3 casos

Antônio Rodrigues de Mello^I; Marcos R. G. de Freitas^II; Leila Chimelli^III

^IDisciplinas de Neurologia e de Neuropatologia, Faculdade de Medicina, Universidade Federal Fluminense: Professor Emérito da UNIRIO

^IIDisciplinas de Neurologia e de Neuropatologia, Faculdade de Medicina, Universidade Federal Fluminense: Professor Adjunto de Neurologia

^IIIDisciplinas de Neurologia e de Neuropatologia, Faculdade de Medicina, Universidade Federal Fluminense: Professor Adjunto de Neuropatologia

SUMMARY

The classical Guillain-Barre syndrome is an acute or subacute polyradiculoneuropathy whose main clinical features are progressive weakness of the limbs, decrease or absence of tendon reflexes, and sensory changes. Although in most of the cases there is complete recovery in weeks or months, some patients have a slow and progressive relapsing course and present thickening of the peripheral nerves. In this paper we describe three cases of the chronic and relapsing variety of Guillain-Barré syndrome, two of which had prominent hypertrophic changes in the peripheral nerves with onion bulb formations The clinical and pathological features of this disease are reviewed. The three patients improved with the use of steroids.

RESUMO

A síndrome de Guillain-Barré clássica é polirradiculoneuropatia aguda ou sub-aguda, cujos principais aspectos clínicos são: fraqueza progressiva dos membros, redução ou ausência de reflexos tendinosos e alterações sensitivas. Embora na maioria dos casos haja recuperação completa em semanas ou meses, em alguns pacientes o curso é lento, progressivo ou recidivante e há espessamento dos nervos periféricos. No presente artigo descrevemos três casos da variedade crônica e recidivante da síndrome de Guillain-Barré, dois dos quais tinham alterações hipertróficas proeminentes nos nervos periféricos com formações em bulbo de cebola. Os aspectos clínicos e patológicos desta doença são revistos. Os três pacientes melhoraram com o uso de esteróides.

Full text available only in PDF format.

Texto completo disponível apenas em PDF.

Acknowledgements - We should like to thank Miss Rita C. Cunha and Miss Suely M. Cunha for the technical assistance, and Miss Rosely C. Cunha and Mr. Ricardo Barreto for the photographic work.

Rua Gastão Ruch 16, apto. 1402 - 24220 Niterói RJ - Brasil

1. Adams D, Festenstein H, Gibson JD, Hughes RAC, Jarawemada J, Papasteriadis C, Sachs J, Thomas PK - HLA antigens in chronic relapsing idiopathic inflammatory polyneuropathy. J Neurol Neurosurg Psychiat 42 :184, 1979.
2. Arnason BGW - Acute inflammatory demyelinating polyradiculoneuropathies. In Dyck PJ, Thomas PK, Lambert EW, Bunge R (eds) : Peripheral Neuropathy. Ed 2. Saunders, Philadelphia, 1984, vol 2, pg 2050.
3. Austin JH - Recurrent polyneuropathies and their corticosteroid treatment. Brain 81: 157, 1958.
4. Bonduelle M, Gruner J - Polyradiculonevrite progressive. Rev Neurol 99:307, 1958.
5. Dalakas MC, Engel WK - Chronic relapsing (dysimmune) polyneuropathy: pathogenesis and treatment. Ann Neurol 9 (suppl):134, 1981.
6. DeVivo DC, Engel WK - Remarkable recovery of a steroid-responsive recurrent poly-neuropathy. J Neurol Neurosurg Psychiat 33:62, 1970.
7. Dyck PJ - Inherited neuronal degeneration and atrophy affecting peripheral motor, sensory, and autonomic neurons. In Dyck PJ, Thomas PK, Lambert EW, Bunge R (eds) : Peripheral Neuropathy. Ed 2. Saunders, Philadelphia, 1984, vol 2, pg 1600.
8. Dyck PJ, Arnason B - Chronic inflammatory demyelinating polyradiculoneuropathy. In Dyck PJ, Thomas PK, Lambert EW, Bunge R (eds) : Peripheral Neuropathy. Ed 2. Saunders, Philadelphia, 1984, vol 2, pg 2101.
9. Dyck PJ, Lais AC, Otha M, Bastron JA, Okazaki H, Groover RV - Chronic inflammatory polyradiculoneuropathy. Mayo Clin Proc 50:621, 1975.
10. Dyck PJ, O'Brien PC, Oviatt KF, Dinapoli RP, Daube JP, Bartleson JD, Mokri B, Swift T, Low PA, Windebank AJ - Prednisone improves chronic inflammatory demyelinating polyradiculoneuropathy more than no treatment. Ann Neurol 11:136, 1982.
11. Feasby TE, Gilbert JJ, Brown WF, Bolton CF, Hahn AF, Koopman WF, Zochodne DW - An acute axonal form of Guillain-Barré polyneuropathy. Brain 109:1115, 1986.
12. Freitas MRG, Nascimento OJM - Sindrome de Guillain-Barré de evolução prolongada. Neurobiology (Recife) 34:41, 1975.
13. Hinman RC, Magee KR - Guillain-Barré syndrome with slow progressive onset and persistent elevation of spinal fluid protein. Ann Int Med 67:1007, 1967.
14. Levy RL, Newkirk R, Ochoa J - Treating chronic relapsing Guillain-Barré syndrome by plasma exchange. Lancet 2:259, 1979.
15. Marchiori PE, Callegaro D, Scaff M, Zambon AA, Assis JL - Polirradiculoneurite recidivante: relato de dois casos. Arq Neuro-Psiquiat (São Paulo) 41:208, 1983.
16. Matthews WB, Howell DA, Hughes RAC - Relapsing corticosteroid-dependent poly-neuritis. J Neurol Neurosurg Psychiat 33:330, 1970.
17. Mello AR, Mattos JP, Cavalcanti JLS - Polirradiculoncurites de evolução prolongada: a propósito de dois casos corticóide dependentes. Arq Neuro-Psiquiat (São Paulo) 30:335, 1972.
18. Nattras FJ - Recurrent hypertrophic neuritis. J Neurol Psycopath (Bristol) 11:159, 1921.
19. Pollard JD, Selby G - Relapsing neuropathy due to tetanus toxoid. J Neurol Sci 37 :113, 1978.
20. Prineas JW - Pathology of the Guillain-Barry syndrome. Ann Neurol 9 (suppl):6, 1981.
21. Prineas JW, McLeod JG - Chronic relapsing polyneuritis. J Neurol Sci 27:427, 1976.
22. Server AC, Lefkowith J, Braine H, McKhann GM - Treatment of chronic relapsing inflammatory polyradiculoneuropathy by plasma exchange. Ann Neurol 6:258, 1979.
23. Stewart GJ, Pollard JD, McLeod JG, Wolmer CM - HLA antigens in the Landry-Guillain-Barré syndrome and chronic relapsing polyneuritis. Ann Neurol 4:285, 1978.
24. Torvik A, Lundar T - A case of chronic demyelinating polyneuropathy resembling the Guillain-Barré syndrome. J Neurol Scci 32:45, 1977.
25. Webster H DeF, Schroder JM, Asbury AK, Adams RD - The role of Schwann cells in the formation of onion bulbs found in chronic neuropathies. J Neuropathol 26:276, 1967.

Publication Dates

Publication in this collection
06 June 2011
Date of issue
Mar 1989

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

[1] 1. Adams D, Festenstein H, Gibson JD, Hughes RAC, Jarawemada J, Papasteriadis C, Sachs J, Thomas PK - HLA antigens in chronic relapsing idiopathic inflammatory polyneuropathy. J Neurol Neurosurg Psychiat 42 :184, 1979.

[2] 2. Arnason BGW - Acute inflammatory demyelinating polyradiculoneuropathies. In Dyck PJ, Thomas PK, Lambert EW, Bunge R (eds) : Peripheral Neuropathy. Ed 2. Saunders, Philadelphia, 1984, vol 2, pg 2050.

[3] 3. Austin JH - Recurrent polyneuropathies and their corticosteroid treatment. Brain 81: 157, 1958.

[4] 4. Bonduelle M, Gruner J - Polyradiculonevrite progressive. Rev Neurol 99:307, 1958.

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

[6] 6. DeVivo DC, Engel WK - Remarkable recovery of a steroid-responsive recurrent poly-neuropathy. J Neurol Neurosurg Psychiat 33:62, 1970.

[7] 7. Dyck PJ - Inherited neuronal degeneration and atrophy affecting peripheral motor, sensory, and autonomic neurons. In Dyck PJ, Thomas PK, Lambert EW, Bunge R (eds) : Peripheral Neuropathy. Ed 2. Saunders, Philadelphia, 1984, vol 2, pg 1600.

[8] 8. Dyck PJ, Arnason B - Chronic inflammatory demyelinating polyradiculoneuropathy. In Dyck PJ, Thomas PK, Lambert EW, Bunge R (eds) : Peripheral Neuropathy. Ed 2. Saunders, Philadelphia, 1984, vol 2, pg 2101.

[9] 9. Dyck PJ, Lais AC, Otha M, Bastron JA, Okazaki H, Groover RV - Chronic inflammatory polyradiculoneuropathy. Mayo Clin Proc 50:621, 1975.

[10] 10. Dyck PJ, O'Brien PC, Oviatt KF, Dinapoli RP, Daube JP, Bartleson JD, Mokri B, Swift T, Low PA, Windebank AJ - Prednisone improves chronic inflammatory demyelinating polyradiculoneuropathy more than no treatment. Ann Neurol 11:136, 1982.

[11] 11. Feasby TE, Gilbert JJ, Brown WF, Bolton CF, Hahn AF, Koopman WF, Zochodne DW - An acute axonal form of Guillain-Barré polyneuropathy. Brain 109:1115, 1986.

[12] 12. Freitas MRG, Nascimento OJM - Sindrome de Guillain-Barré de evolução prolongada. Neurobiology (Recife) 34:41, 1975.

[13] 13. Hinman RC, Magee KR - Guillain-Barré syndrome with slow progressive onset and persistent elevation of spinal fluid protein. Ann Int Med 67:1007, 1967.

[14] 14. Levy RL, Newkirk R, Ochoa J - Treating chronic relapsing Guillain-Barré syndrome by plasma exchange. Lancet 2:259, 1979.

[15] 15. Marchiori PE, Callegaro D, Scaff M, Zambon AA, Assis JL - Polirradiculoneurite recidivante: relato de dois casos. Arq Neuro-Psiquiat (São Paulo) 41:208, 1983.

[16] 16. Matthews WB, Howell DA, Hughes RAC - Relapsing corticosteroid-dependent poly-neuritis. J Neurol Neurosurg Psychiat 33:330, 1970.

[17] 17. Mello AR, Mattos JP, Cavalcanti JLS - Polirradiculoncurites de evolução prolongada: a propósito de dois casos corticóide dependentes. Arq Neuro-Psiquiat (São Paulo) 30:335, 1972.

[18] 18. Nattras FJ - Recurrent hypertrophic neuritis. J Neurol Psycopath (Bristol) 11:159, 1921.

[19] 19. Pollard JD, Selby G - Relapsing neuropathy due to tetanus toxoid. J Neurol Sci 37 :113, 1978.

[20] 20. Prineas JW - Pathology of the Guillain-Barry syndrome. Ann Neurol 9 (suppl):6, 1981.

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

[22] 22. Server AC, Lefkowith J, Braine H, McKhann GM - Treatment of chronic relapsing inflammatory polyradiculoneuropathy by plasma exchange. Ann Neurol 6:258, 1979.

[23] 23. Stewart GJ, Pollard JD, McLeod JG, Wolmer CM - HLA antigens in the Landry-Guillain-Barré syndrome and chronic relapsing polyneuritis. Ann Neurol 4:285, 1978.

[24] 24. Torvik A, Lundar T - A case of chronic demyelinating polyneuropathy resembling the Guillain-Barré syndrome. J Neurol Scci 32:45, 1977.

[25] 25. Webster H DeF, Schroder JM, Asbury AK, Adams RD - The role of Schwann cells in the formation of onion bulbs found in chronic neuropathies. J Neuropathol 26:276, 1967.