Neuronopatia sensitiva aguda idiopática: relato de caso

Werneck, Lineu César; Correa Neto, Ylmar; Scola, Rosana Hermínia

doi:10.1590/S0004-282X1993000300018

Resumos

As neuronopatias sensitivas caracterizam-se por parestesias, ataxia sensitiva e arreflexia sem comprometimento motor, devidas a lesão no gânglio sensitivo dorsal, com degeneração axonal e do cordão posterior da medula espinhal. Descrevemos o caso de uma paciente de 21 anos de idade, cuja doença se iniciou de modo agudo com parestesias distais, astasia, abasia e hiporreflexia. Força muscular normal. A condução nervosa sensitiva estava ausente e a motora, discretamente reduzida. LCR com 2 leucócitos/mm3 e 1,06 g/dL de proteínas. A biópsia de músculo evidenciou atrofia de fibras tipo 2 e a biópsia do nervo sural, desmielinização axonal. Tratada com prednisona, permaneceu discreto déficit proprioceptivo nos pés após um ano e meio de evolução. São discutidos aspectos clínicos do envolvimento dos gânglios sensitivos dorsais, possíveis etiologias tóxicas, a relação com neoplasias e com a síndrome de Sjogren.

neuronopatia sensitiva; poliganglionopatia sensitiva; neuropatía sensitiva aguda

Pure sensory neuropathies are clinically characterized by paresthesias, sensory ataxia and areflexia without muscle weakness. We report the case of a 21 years-old female patient with acute onset of distal paresthesias, marked sensory ataxia and hyporeflexia. Motor strenght was normal. Sensory nerve conduction was absent and motor nerve conduction slightly decreased. CSF showed 2 leucocytes/mm3 and 1.06 g/dL protein. Collagen disorder and neoplasia were, not found. Type 2 fiber atrophy was observed on muscle biopsy, and axonal demyelination on sural nerve biopsy. The patient was treated with prednisone. After 1.5 years she was recovered, but a minor proprioceptive deficit persisted. A revision is made on the etiology, pathophysiology and clinical manifestations of the disease.

sensory neuronopathy; sensory polyganglionopathy; acute sensory neuropathy

Neuronopatia sensitiva aguda idiopática: relato de caso

Acute idiopathic sensory neurononathy: case report

Lineu César Werneck^I; Ylmar Correa Neto^II; Rosana Hermínia Scola^III

^IServiço de Doenças Neuromusculares (DNM), Especialidade Neurologia, Departamento da Clínica Médica, Hospital de Clínicas da Universidade Federal do Paraná, Curitiba: Professor Titular

^IIServiço de Doenças Neuromusculares (DNM), Especialidade Neurologia, Departamento da Clínica Médica, Hospital de Clínicas da Universidade Federal do Paraná, Curitiba: Residente de Neurologia (DNM)

^IIIServiço de Doenças Neuromusculares (DNM), Especialidade Neurologia, Departamento da Clínica Médica, Hospital de Clínicas da Universidade Federal do Paraná, Curitiba: Neurologista

RESUMO

As neuronopatias sensitivas caracterizam-se por parestesias, ataxia sensitiva e arreflexia sem comprometimento motor, devidas a lesão no gânglio sensitivo dorsal, com degeneração axonal e do cordão posterior da medula espinhal. Descrevemos o caso de uma paciente de 21 anos de idade, cuja doença se iniciou de modo agudo com parestesias distais, astasia, abasia e hiporreflexia. Força muscular normal. A condução nervosa sensitiva estava ausente e a motora, discretamente reduzida. LCR com 2 leucócitos/mm3 e 1,06 g/dL de proteínas. A biópsia de músculo evidenciou atrofia de fibras tipo 2 e a biópsia do nervo sural, desmielinização axonal. Tratada com prednisona, permaneceu discreto déficit proprioceptivo nos pés após um ano e meio de evolução. São discutidos aspectos clínicos do envolvimento dos gânglios sensitivos dorsais, possíveis etiologias tóxicas, a relação com neoplasias e com a síndrome de Sjogren.

Palavras-chave: neuronopatia sensitiva, poliganglionopatia sensitiva, neuropatía sensitiva aguda.

SUMMARY

Pure sensory neuropathies are clinically characterized by paresthesias, sensory ataxia and areflexia without muscle weakness. We report the case of a 21 years-old female patient with acute onset of distal paresthesias, marked sensory ataxia and hyporeflexia. Motor strenght was normal. Sensory nerve conduction was absent and motor nerve conduction slightly decreased. CSF showed 2 leucocytes/mm3 and 1.06 g/dL protein. Collagen disorder and neoplasia were, not found. Type 2 fiber atrophy was observed on muscle biopsy, and axonal demyelination on sural nerve biopsy. The patient was treated with prednisone. After 1.5 years she was recovered, but a minor proprioceptive deficit persisted. A revision is made on the etiology, pathophysiology and clinical manifestations of the disease.

Key words: sensory neuronopathy, sensory polyganglionopathy, acute sensory neuropathy.

Full text available only in PDF format.

Texto completo disponível apenas em PDF.

Aceite: 2-dezembro-1992.

Dr. Lineu César Werneck Rua Ewaldo Schiebler 780 - 82530-160 Curitiba PR - Brasil.

Trabalho realizado no Serviço de Doenças Neuromusculares (DNM), Especialidade Neurologia, Departamento da Clínica Médica, Hospital de Clínicas da Universidade Federal do Paraná, Curitiba

1. Anderson NE, Rosenblum MK, Graus FG, Wiley RG, Posner JB. Autoantibodies in paraneoplastic syndromes associated with small-cell lung cancer. Neurology 1988, 38:1391-1398.
2. Asbury AK. Sensory neuronopathy. Semin Neurol 1987, 7:58-66.
3. Brunet P, Schadec B, Moriceau M. Subacute sensory neuropathy with remission: an association with lymphoma. J Neurol Neurosurg Psychiatry 1983, 46:99-100.
4. Charron L, Peyronnard J-M, Marchand L. Sensory neuropathy associated with primary biliary cirrhosis. Arch Neurol 1980, 37:84-87.
5. Dalakas MC. Chronic idiopathic ataxic neuropathy. Ann Neurol 1986, 19:545-554.
6. Dawson DM, Samuels MA, Morris J. Sensory form of polyneuritis. Neurology 1988, 38: 1728-1731.
7. Dropcho EJ. The remote effects of cancer on the nervous system. Neurol Clin 1989, 7:579-604.
8. Graus F, Elkon KB, Cordon-Cardo C, Posner JB. Sensory neuropathy and small cell lung cancer. Am J Med 1986, 80:45-52.
9. Graus F, Elkon KB, Lloberes P, Ribalta T, Torres A, Ussetti P, Valls J, Obach J, Agusti-Vidal A. Neuronal antinuclear antibody in paraneoplastic encephalomyelitis simulating acute polyneuritis. Acta Neurol Scand 1987, 75:249-252.
10. Graus F, Pou A, Kanterewicz E, Anderson NE. Sensory neuronopathy and Sjogren's syndrome: clinical and immunologic study of two patients. Neurology 1988, 38:1637-1639.
11. Graus F, Santamarya J, Obach J, Valls J, Ribalta T, Tolosa E. Sensory neuropathy as remote effect of cancer. Neurology 1987, 37:1266.
12. Griffin JW. Predominantly sensory neuropathies. In Griffin JW: Peripheral neuropathy, Course #142, 43rd Annual Meeting. Boston: American Academy of Neurology, 1991, p 7-16.
13. Griffin JW, Cornblath DR, Alexander E, Campbell J, Low PA, Bird S, Feldman EL. Ataxic sensory neuropathy and dorsal root ganglionitis associated with Sjogren's syndrome. Ann Neurol 1990, 27:304-315.
14. Griffin JW, Cornblath DR, Dropcho E. Differential diagnosis of the pure sensory neuro-nopathies. Neurology 1989, 39(Suppl 1):391.
15. Hankey GJ, Gubbay SS. Peripheral neuropathy associated with sicca syndrome. J Neurol Neurosurg Psychiatry 1987, 50:1085.
16. Hemphill M, Pestronk A, Walsh T, Parhad I, Clark A, Rosenshein N. Sensory neuropathy in cis-platinum chemotherapy. Neurology 1980, 30:429.
17. Hull RG, Morgan SH, Harding AE, Hughes GRV. Sjogren's syndrome presenting as a severe sensory neuropathy including involvement of the trigeminal nerve. Br J Rheumatol 1984, 23:301-303.
18. Kaufman MD, Hopkins LC, Hurwitz BJ. Progressive sensory neuropathy in patients without carcinoma: la disorder with distinctive clinical and electrophysiological findings. Ann Neurol 1981, 9:237-242.
19. Kennett RP, Harding AE. Peripheral neuropathy associated with the sicca syndrome. J Neurol Neurosurg Psychiatry 1986, 49:90-92.
20. Mamoli A, Nemni R, Camerlingo M, Quattrini A, Casto L, Lorenzetti I, Canal N. A clinical, electrophysiological, morphological and immunological study of chronic sensory neuropathy with ataxia and paraesthesia. Acta Neurol Scand 1992, 85:110-115.
21. Malinow K, Yannakakis GD, Glusman SM, Edlow DW, Griffin J, Pestronk A, Powell DL, Ramsey-Goldman R, Eidelman BH, Medsger TA Jr, Alexander EL.. Subacute sensory neuronopathy secondary to dorsal root ganglionitis in primary Sjogren's syndrome. Ann Neurol 1986, 20:535-537.
22. Miralles F, Montero J, Rene R, Matos JAM. Pure sensory Guillain-Barré syndrome. J Neurol Neurosurg Psychiatry 1992, 55:411-412.
23. Mitsumono H, Wilbourn AJ, Massarweh W. Acquired pure sensory polyneuropathy: unique clinical features in 30 patients. Neurology 1985, 35(Suppl 1):295.
24. Read HJS. Subacute sensory neuropathy with remission: an association with lymphoma. J Neurol Neurosurg Psychiatry 1982, 45:83-85.
25. Richter RB. The ataxic form of polyradiculoneuritis. J Neuropathol Exp Neurol 1962, 21:171-184.
26. Sagar HJ, Read DJ. Subacute sensory neuropathy with remission: an association with lymphoma. J Neurol Neurosurg Psychiatry 1982, 45:83-85.
27. Schaumburg H, Kaplan J, Windebank A, Dick N, Rasmu S, Pleasure D, Brown MJ. Sensory neuropathy from pyridoxine abuse. N Engl J Med 1983, 309:445-448.
28. Smith BE, Windebank AJ, Dyck PJ. Nonmalignant inflammatory sensory polyganglio-nopathy. In Dyck PJ, Thomas PK, Griffin JW, Low PA, Poduslo JF (eds): Peripheral neuropathy. Ed 3. Philadelphia: Saunders, 1993.
29. Sterman AB, Schaumburg HH, Asbury AK. The acute sensory neuronopathy syndrome: a distinct clinical entity. Ann Neurol 1980, 7:354-358.
30. Taly AB, Prasad A, Viasanth A, Shankar SK, Nagaraja D. Acute ataxia neuropathy: a clinical, electrophysiological and morphological study. Acta Neurol Scand 1991, 84:398-402.
31. Tohgi H, Sano M, Sasaki K, Suzuki H, Sato H, Iwasaki T, Satodate R. Acute autonomic and sensory neuropathy: report of an autopsy case. Acta Neuropathol 1989, 77:659-663.
32. Vallat JM, Leboutet MJ, Hugon J, Loubet A, Dubeau M, Fressinaud C. Acute pure sensory paraneoplastic neuropathy with perivascular endoneurial inflammation: ultrastructural study of capillary walls. Neurology 1986, 36:1395-1399.
33. Viallat JM. Paraneoplastic sensory neuronopathy revisited. Neurology 1988, 38:508.
34. Vallat JM. Sensory Guillain-Barré syndrome. Neurology 1989, 39:879.
35. Windebank AJ, Blexrud MD, Dyck PJ, Daube JR, Karnes JL. The syndrome of acute sensory neuropathy: clinical features and electrophysiologic and pathologic changes. Neurology 1990, 40:584-591.

Datas de Publicação

Publicação nesta coleção
19 Jan 2011
Data do Fascículo
Set 1993

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

[1] 1. Anderson NE, Rosenblum MK, Graus FG, Wiley RG, Posner JB. Autoantibodies in paraneoplastic syndromes associated with small-cell lung cancer. Neurology 1988, 38:1391-1398.

[2] 2. Asbury AK. Sensory neuronopathy. Semin Neurol 1987, 7:58-66.

[3] 3. Brunet P, Schadec B, Moriceau M. Subacute sensory neuropathy with remission: an association with lymphoma. J Neurol Neurosurg Psychiatry 1983, 46:99-100.

[4] 4. Charron L, Peyronnard J-M, Marchand L. Sensory neuropathy associated with primary biliary cirrhosis. Arch Neurol 1980, 37:84-87.

[5] 5. Dalakas MC. Chronic idiopathic ataxic neuropathy. Ann Neurol 1986, 19:545-554.

[6] 6. Dawson DM, Samuels MA, Morris J. Sensory form of polyneuritis. Neurology 1988, 38: 1728-1731.

[7] 7. Dropcho EJ. The remote effects of cancer on the nervous system. Neurol Clin 1989, 7:579-604.

[8] 8. Graus F, Elkon KB, Cordon-Cardo C, Posner JB. Sensory neuropathy and small cell lung cancer. Am J Med 1986, 80:45-52.

[9] 9. Graus F, Elkon KB, Lloberes P, Ribalta T, Torres A, Ussetti P, Valls J, Obach J, Agusti-Vidal A. Neuronal antinuclear antibody in paraneoplastic encephalomyelitis simulating acute polyneuritis. Acta Neurol Scand 1987, 75:249-252.

[10] 10. Graus F, Pou A, Kanterewicz E, Anderson NE. Sensory neuronopathy and Sjogren's syndrome: clinical and immunologic study of two patients. Neurology 1988, 38:1637-1639.

[11] 11. Graus F, Santamarya J, Obach J, Valls J, Ribalta T, Tolosa E. Sensory neuropathy as remote effect of cancer. Neurology 1987, 37:1266.

[12] 12. Griffin JW. Predominantly sensory neuropathies. In Griffin JW: Peripheral neuropathy, Course #142, 43rd Annual Meeting. Boston: American Academy of Neurology, 1991, p 7-16.

[13] 13. Griffin JW, Cornblath DR, Alexander E, Campbell J, Low PA, Bird S, Feldman EL. Ataxic sensory neuropathy and dorsal root ganglionitis associated with Sjogren's syndrome. Ann Neurol 1990, 27:304-315.

[14] 14. Griffin JW, Cornblath DR, Dropcho E. Differential diagnosis of the pure sensory neuro-nopathies. Neurology 1989, 39(Suppl 1):391.

[15] 15. Hankey GJ, Gubbay SS. Peripheral neuropathy associated with sicca syndrome. J Neurol Neurosurg Psychiatry 1987, 50:1085.

[16] 16. Hemphill M, Pestronk A, Walsh T, Parhad I, Clark A, Rosenshein N. Sensory neuropathy in cis-platinum chemotherapy. Neurology 1980, 30:429.

[17] 17. Hull RG, Morgan SH, Harding AE, Hughes GRV. Sjogren's syndrome presenting as a severe sensory neuropathy including involvement of the trigeminal nerve. Br J Rheumatol 1984, 23:301-303.

[18] 18. Kaufman MD, Hopkins LC, Hurwitz BJ. Progressive sensory neuropathy in patients without carcinoma: la disorder with distinctive clinical and electrophysiological findings. Ann Neurol 1981, 9:237-242.

[19] 19. Kennett RP, Harding AE. Peripheral neuropathy associated with the sicca syndrome. J Neurol Neurosurg Psychiatry 1986, 49:90-92.

[20] 20. Mamoli A, Nemni R, Camerlingo M, Quattrini A, Casto L, Lorenzetti I, Canal N. A clinical, electrophysiological, morphological and immunological study of chronic sensory neuropathy with ataxia and paraesthesia. Acta Neurol Scand 1992, 85:110-115.

[21] 21. Malinow K, Yannakakis GD, Glusman SM, Edlow DW, Griffin J, Pestronk A, Powell DL, Ramsey-Goldman R, Eidelman BH, Medsger TA Jr, Alexander EL.. Subacute sensory neuronopathy secondary to dorsal root ganglionitis in primary Sjogren's syndrome. Ann Neurol 1986, 20:535-537.

[22] 22. Miralles F, Montero J, Rene R, Matos JAM. Pure sensory Guillain-Barré syndrome. J Neurol Neurosurg Psychiatry 1992, 55:411-412.

[23] 23. Mitsumono H, Wilbourn AJ, Massarweh W. Acquired pure sensory polyneuropathy: unique clinical features in 30 patients. Neurology 1985, 35(Suppl 1):295.

[24] 24. Read HJS. Subacute sensory neuropathy with remission: an association with lymphoma. J Neurol Neurosurg Psychiatry 1982, 45:83-85.

[25] 25. Richter RB. The ataxic form of polyradiculoneuritis. J Neuropathol Exp Neurol 1962, 21:171-184.

[26] 26. Sagar HJ, Read DJ. Subacute sensory neuropathy with remission: an association with lymphoma. J Neurol Neurosurg Psychiatry 1982, 45:83-85.

[27] 27. Schaumburg H, Kaplan J, Windebank A, Dick N, Rasmu S, Pleasure D, Brown MJ. Sensory neuropathy from pyridoxine abuse. N Engl J Med 1983, 309:445-448.

[28] 28. Smith BE, Windebank AJ, Dyck PJ. Nonmalignant inflammatory sensory polyganglio-nopathy. In Dyck PJ, Thomas PK, Griffin JW, Low PA, Poduslo JF (eds): Peripheral neuropathy. Ed 3. Philadelphia: Saunders, 1993.

[29] 29. Sterman AB, Schaumburg HH, Asbury AK. The acute sensory neuronopathy syndrome: a distinct clinical entity. Ann Neurol 1980, 7:354-358.

[30] 30. Taly AB, Prasad A, Viasanth A, Shankar SK, Nagaraja D. Acute ataxia neuropathy: a clinical, electrophysiological and morphological study. Acta Neurol Scand 1991, 84:398-402.

[31] 31. Tohgi H, Sano M, Sasaki K, Suzuki H, Sato H, Iwasaki T, Satodate R. Acute autonomic and sensory neuropathy: report of an autopsy case. Acta Neuropathol 1989, 77:659-663.

[32] 32. Vallat JM, Leboutet MJ, Hugon J, Loubet A, Dubeau M, Fressinaud C. Acute pure sensory paraneoplastic neuropathy with perivascular endoneurial inflammation: ultrastructural study of capillary walls. Neurology 1986, 36:1395-1399.

[33] 33. Viallat JM. Paraneoplastic sensory neuronopathy revisited. Neurology 1988, 38:508.

[34] 34. Vallat JM. Sensory Guillain-Barré syndrome. Neurology 1989, 39:879.

[35] 35. Windebank AJ, Blexrud MD, Dyck PJ, Daube JR, Karnes JL. The syndrome of acute sensory neuropathy: clinical features and electrophysiologic and pathologic changes. Neurology 1990, 40:584-591.