Type I primary neuropathic amyloidosis (Andrade, Portuguese): a clinical and laboratory study of 21 cases

Azevedo, Eduardo M.; Scaff, Milberto; Canelas, Horacio M.; Spina-França, Antonio

doi:10.1590/S0004-282X1975000200001

Abstracts

The authors present a review of 21 cases with the diagnosis of type I amyloid neuropathy based on epidemiological data, clinical evolution and histopathological findings. They call attention to the possibility of cranial nerves involvement (hyposmia, diplopia, masseterian hypotrophy, peripheral facial paralysis, hypoacusis, dysphonia, laryngeal paralysis, dysphagia, and trapezium muscle hypotrophy), to the severeness of the digestive symptoms, to the precocity of the autonomic disorders, and to the rather high incidence (6 cases) of heart involvement. The electromyography showed anterior horn involvement in 3 cases. The electrocardiography showed repolarization disorders in 11 cases, left ventricular overload in 6 cases and atrioventricular block in 5 cases. The serum proteins electrophoresis showed frequent abnormalities, but no typical curve could be obtained. The barium-contrasted X-rays of the gastrointestinal tract showed no anatomical lesions, but functional abnormalities (hypo or hypermotility) were found in 14 examinations. The Schilling test showed impairment of vitamin B12 absorption in 50% of the cases. However, with the concomitant administration of intrinsic factor (3 cases) there was improvement of its absorption. This proves that the gastric mucosa plays an important role in the disease malabsorption. The test with labeled-triolein showed slow absorption in 2 cases and steatorrhea in 3 (6 tests). For the confirmation of the amyloid deposits, the best histopathological procedure was nerve biopsy. In men, when the nerve biopsy was negative, testicular biopsy has shown to be a good option.

Os autores apresentam uma revisão de 21 pacientes com diagnóstico de neuropatia amilóide tipo I, firmado sobre os dados epidemiológicos, a evolução clínica e os achados histopatológicos. Chamam a atenção para a possibilidade de comprometimento de vários nervos cranianos, para a gravidade do quadro digestivo, a precocidade dos distúrbios neurovegetativos e a incidência relativamente alta de sintomatologia cardíaca. Constituem contribuição para o melhor conhecimento da afecção, os estudos sobre a síndrome digestiva feitos através de exames radiológicos e dos testes de absorção de vitamina B12 e trioleína radioativas.

Type I primary neuropathic amyloidosis (Andrade, Portuguese). A clinical and laboratory study of 21 cases

Neuropatia amilóide primária tipo I (Andrade, Portuguesa): estudo clínico e laboratorial de 21 casos

Eduardo M. Azevedo; Milberto Scaff; Horacio M. Canelas; Antonio Spina-França

Division of Neurology, Department of Neuropsychiatry, University of São Paulo School of Medicine

SUMMARY

The authors present a review of 21 cases with the diagnosis of type I amyloid neuropathy based on epidemiological data, clinical evolution and histopathological findings.

They call attention to the possibility of cranial nerves involvement (hyposmia, diplopia, masseterian hypotrophy, peripheral facial paralysis, hypoacusis, dysphonia, laryngeal paralysis, dysphagia, and trapezium muscle hypotrophy), to the severeness of the digestive symptoms, to the precocity of the autonomic disorders, and to the rather high incidence (6 cases) of heart involvement.

The electromyography showed anterior horn involvement in 3 cases. The electrocardiography showed repolarization disorders in 11 cases, left ventricular overload in 6 cases and atrioventricular block in 5 cases. The serum proteins electrophoresis showed frequent abnormalities, but no typical curve could be obtained.

The barium-contrasted X-rays of the gastrointestinal tract showed no anatomical lesions, but functional abnormalities (hypo or hypermotility) were found in 14 examinations.

The Schilling test showed impairment of vitamin B12 absorption in 50% of the cases. However, with the concomitant administration of intrinsic factor (3 cases) there was improvement of its absorption. This proves that the gastric mucosa plays an important role in the disease malabsorption. The test with labeled-triolein showed slow absorption in 2 cases and steatorrhea in 3 (6 tests).

For the confirmation of the amyloid deposits, the best histopathological procedure was nerve biopsy. In men, when the nerve biopsy was negative, testicular biopsy has shown to be a good option.

RESUMO

Os autores apresentam uma revisão de 21 pacientes com diagnóstico de neuropatia amilóide tipo I, firmado sobre os dados epidemiológicos, a evolução clínica e os achados histopatológicos.

Chamam a atenção para a possibilidade de comprometimento de vários nervos cranianos, para a gravidade do quadro digestivo, a precocidade dos distúrbios neurovegetativos e a incidência relativamente alta de sintomatologia cardíaca.

Constituem contribuição para o melhor conhecimento da afecção, os estudos sobre a síndrome digestiva feitos através de exames radiológicos e dos testes de absorção de vitamina B12 e trioleína radioativas.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

ACNOWLEDGEMENTS

The authors intend, with this study, to pay homage to the unforgettable master of the Brazilian Neurology, Dr. Oswaldo Freitas Julião, whose pioner researches in the field of the neuro-amyloidoses have achieved international renown. We also acknowledge with thanks, Dr. Nelson Carvalho for the study of intestinal absorption with radioactive isotopes.

Clínica Neurológica Faculdade de Medicina, Universidade de São Paulo Caixa Postal 3461 01000 São Paulo SP Brasil.

1. ANDRADE, C. A peculiar form of peripheral neuropathy: a synopsis of a study to be published shortly. Acta psychiat. neurol. scand. 26:251, 1951.
2. ANDRADE, C. Note préliminaire sur une forme particulière de neuropathie périphérique. Revue neurol. (Paris) 85:302, 1951.
3. ANDRADE, C. A peculiar form of peripheral neuropathy. Brain 75:408, 1952.
4. ANDRADE, C.; ARAKI, S.; BLOCK, W. D.; COHEN, A. S.; JACKSON, C. E.; KUROIWA, Y.; McKUSICK, V. A.; NISSIM, J.; SCHAR, E. & VAN ALLEN, M. W. Hereditary amyloidosis. Arthritis Rheum. 13:902, 1970.
5. ARAKI, S.; MAWATARI, S.; OTHA, M.; NAKAJIMA, A. & KUROIWA, Y. Polyneuritic amyloidosis in a Japanese family. Arch Neurol. (Chicago) 18:593-602, 1968.
6. AUGUSTIN, P. La neuropathie amyloïde. Thèse, Paris, 1964, Cit. por P. E. Becker 7.
7. BECKER, P. E. Enfermedades con lesión predominante de los nervios periféricos y raices espinales. In P. E. Becker: Genética Humana, tomo V/1 Enfermedades del Sistema Nervioso. Toray, Barcelona, 1969, pp 469-491.
8. BECKER, P. E.; ANTUNES, L.; RIBEIRO DO ROSÁRIO, M. & BARROS, F. Paramyloidose der peripheren Nerven in Portugal. Z. mensch. Vererb. Konstit Lehre 37:329, 1964.
9. BENNHOLD, H. Eine spezifische Amyloidfärbung mit Kongorot. Münch. med. Wschr. 69:1537, 1922.
10. BERTRAND, I.; CASTAIGNE, P.; CAMBIER, J.; CATHALA, H. P.; BINET, J. L. & DRY, J. Sur deux nouveaux cas de neuropathie paramyloïde. Revue neurol. (Paris) 104:380, 1961.
11. BLOCK, W. D.; RUKAWINA, J. G. & CURTIS, A. C. Serum electrophoretic studies on patients with familial primary systemic amyloidosis. J. Lab. clin. Med. 47:357, 1956.
12. BLUM, A.; GAFNI, J.; SOHAR, E.; SHIBOLET, S. & HELLER, H. Amyloidosis as the sole manifestation of familial Mediterarnean fever (FMF): further evidence of its genetic nature. Ann. intern. Med. 57:795, 1962.
13. CANELAS, H. M.; JULIÃO; O. F.; CARVALHO, N. & SCAFF, M. Absorção de vitamina B12 na polineuropatia amiloidótica familiar. Rev. paul. Med. (São Paulo) 75:336, 1969 (Resumo).
14. CANIJO, M. & PINHO Y COSTA, P. Polineuropatia amiloidótica familiar. Revta clín. esp. 111:583, 1968.
15. CASTAIGNE, P.; CAMBIER, J. & AUGUSTIN, P. La neuropathie amyloide. Presse méd. 73:1171, 1965.
16. CHAMBERS, R. A.; MEDD, W. E. & SPENCER, H. Primary amyloidosis with special reference to involvement of the nervous system. Q. Jl. Med. 27:207, 1958.
17. COHEN, A. S. Amyloidosis. New Engl. J. Med. 277:522, 574 and 628, 1967.
18. COIMBRA, A. & ANDRADE, C. Familial amyloid polyneuropathy: an electron microscope study of peripheral nerves in five cases. Brain 94:199, 1971.
19. DICK, P. J. & LAMBERT, E. H. Dissociated sensation in amyloidosis. Arch. Neurol. (Chicago) 20:490, 1969.
20. FREDERIKSEN, T.; GOTZSCHE, H.; HARBE, N. & KIAER, W. Familial primary amyloidosis with severe amyloid heart disease. Am. J. Med. 33:328, 1962.
21. FRENCH. J. M.; HALL, G.; PARISH, D. J. & SMITH, W. T. Peripheral and autonomic nerve involvement in primary amyloidosis associated with uncontrollable diarrhoea and steatorrhoea. Am. J. Med. 39:277, 1965.
22. GILROY, J. & MEYER, J. S. Medical Neurology. Macmillan, New York, 1969, pp 266-267, 625-626, 680-681.
23. GIMENO, A; GARCIA-ALIX, C; SEGOVIA DE ARANA, J. M.; MATEUS, F. & SOTELO, M. T. Amyloidotic polyneuritis of type III (Iowa-Van Alen). Eur. Neurol. 11:46, 1974.
24. HELLER, H.; MISSMAHL, H. P.; SIHAR, E. & GAFNI, J. Amyloidosis, its differentiation into perireticulin and pericollagen types. J. Path. Bact. 88:15, 1964.
25. HELLER, H.; SOHAR, E.; GAFNI, J. & HELLER, J. Amyloidosis in familial Mediterranean fever. Arch. intern. Med. 107:539, 1961.
26. HELLER, H.; SOHAR, E. & SHERF, L. Familial Mediterranean fever. Arch. intern. Med. 102:50, 1958.
27. HENSON, R. A. & URICH, H. Metabolic neuropathies. In P. J. Vinken & G. W. Bruyn: Handbook of Clinical Neurology, Vol. 8 Diseases of Nerves Part II. North-Holland, Amsterdam, 1970, pp 4-9.
28. JULIÃO, O. F. Paramiloidose com comprometimento do sistema nervoso periférico: a propósito de duas observações. Rev. paul. Med. (São Paulo) 56:82, 1960 (Resumo).
29. JULIÃO, O. F. Aspectos clínicos da neuropatia amilóide: considerações a propósito de 14 casos. Rev. paul. Med. (São Paulo) 63:349, 1963 (Resumo).
30. JULIÃO, O. F. & MIGNONE, C. Amiloidose primária com comprometimento meningo-radículo-neurítico. Arq. Neuro-Psiquiat. (São Paulo) 13:1-12, 1955.
31. JULIAO, O. F.; QUEIRÓZ, L. S.; LOPEZ DE FARIA, J. Portuguese type of familial amyloid polyneuropathy. Eur. Neurol. 11:180, 1974.
32. KANTARJIAN, A. D. & DeJONG, R. N. Familial primary amyloidosis with nervous system involvement. Neurology (Minneapolis) 3:399, 1953.
33. KAUFMAN, H. E. & THOMAS, L. B. Vitreous opacities diagnostic of familial primary amyloidosis. New Engl J. Med. 261:1267, 1959.
34. KERNOHAN, J. W. & WOLTMAN, H. W. Amyloid neuritis. Arch. Neurol. Psychit. (Chicago) 47:132, 1942.
35. LAMPERT, P. W. Amyloid and amyloid-like deposits. In J. Minckler: Pathology of the Nervous System. Vol. 1 Part XIV Probable Metabolic Degenerations. McGraw-Hill, New York, 1968, pp 1113-1121.
36. MAGALHÃES, A. F. N. Contribuição para o estudo da fisiopatologia das alterações digestivas da polineuropatia amiloidótica familial tipo Corino de Andrade. Tese, Faculdade de Ciencias Médicas, Campinas, 1970.
37. MAHLOUDJI, M.; TEASDALL, R. D.; ADAMKIEWICZ, J. J.; HARTMANN, W. H.; LAMBIRD, P. A. & McKUSICK, V. A. The genetic amyloidosis. Medicine (Baltimore) 48:1-37, 1969.
38. McKUSICK, V. A. & GOEDMAN, R. M. Pineal calcification: observations in systemic diseases not associate with disordered calcium metabolism. J. Am., med. Ass. 179:230, 1962.
39. MELLO, A. R. Polineuropatia amiloidótica familiar. J. bras. Med. (Rio de Janeiro 1:161, 1959.
40. MERETOJA, J. & TEPPO, L. Histopathological findings of familial amyloidosis with cranial neuropathy as principal manifestation: report of three cases. Acta. path. microbiol. scand. 79:432, 1971.
41. MISSMAHL, H. P. What is the relation between the various forms of amyloidosis and the connective tissue fibers? Verh. dt. Ges. inn. Med. 65:439, 1959.
42. MISSMAHL, H. P. Fragen aus der Praxis: was versteht man unter Amyloidose und Paramyloidose? Dt. med. Wschr. 90:582, 1965.
43. MUCKLE, Th. J. Protein components of amyloid. Nature (London) 203:773, 1964.
44. MUCKLE, Th. J. & WELLS, M. Urticaria, deafness and amyloidosis: a new heredofamilial syndrome. Q. Jl Med. 31:235, 1962.
45. MUNSAT, T. L. & POUSSAINT, A. F. Clinical manifestations and diagnosis of amyloid polyneuropathy: report of three cases. Neurology, (Minneapolis) 12: 413, 1962.
46. OSTERTAG, B. Demonstration einer eigenartigen familiären Paramyloidose. Zentbl. allg. Path. path. Anat. 56:253, 1932.
47. ROSARIO, R. M.; ANTUNES, L. & BARROS, F. Contribuição para o estudo clínico e laboratorial da paramiloidose de Corino de Andrade. J. Soc. Cienc. méd. Lisboa 125:1-35, 1961.
48. RUKAVINA, J. G.; BLOCK, W. D.; JACKSON, C. E.; FALLS, H. F.; CAREY, J. H. & CURTIS, A. C. Primary systemic amyloidosis: a review and an experimental genetic and clinical study of 29 cases with particular emphasis on the familial form. Medicine (Baltimore) 35:239, 1956.
49. SCHILLING, R. F. A new test for intrinsic fator activity. J. Lab. clin. Med. 42:946, 1953.
50. SILVA-HORTA, J.; FELIPE, I. & DUARTE, S. Portuguese polyneuritic familial type of amyloidosis. Pathologia Microbiol. 27:809, 1964.
51. SPINA-FRANÇA, A. Eletroforese em papel das proteínas do líquido cefalorraqueano. Rev. paul. Med. (São Paulo) 59:420, 1961.
52. SULLIVAN, J. F.; TWITCHELL, T. E.; GHERARDI, G. J. & VANDERLAAN, W. P. Amyloid polyneuropathy. Neurology (Minneapolis) 5:847, 1955.
53. THOMAS, P. K. & KING, R. H. M. Peripheral nerve changes in amyloid neuropathy. Brain 97:395, 1974.
54. VAN ALLEN, M. W.; FROHLICH, J. A. & DAVIS, J. R. Inherited predisposition to generalized amyloidosis: clinical and pathological study of a family with neuropathy, nephropathy and peptic ulcer. Neurology (Minneapolis) 19: 10, 1969.
55. VIRCHOW, R. L. K. Cellular Pathology as based on Physiological and Pathological History. De Witt, New York, 1860, pp 409-437.
56. WOHLWILL, F. Formas atípicas da amiloidose. Amatus lusit. 1:373, 1942.
57. WONG, V. G. & Mc FARLIN, D. E. Primary familial amyloidosis. Arch. Ophtal. (New York) 78:208, 1963.

Publication Dates

Publication in this collection
09 Apr 2013
Date of issue
June 1975

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

[1] 1. ANDRADE, C. A peculiar form of peripheral neuropathy: a synopsis of a study to be published shortly. Acta psychiat. neurol. scand. 26:251, 1951.

[2] 2. ANDRADE, C. Note préliminaire sur une forme particulière de neuropathie périphérique. Revue neurol. (Paris) 85:302, 1951.

[3] 3. ANDRADE, C. A peculiar form of peripheral neuropathy. Brain 75:408, 1952.

[4] 4. ANDRADE, C.; ARAKI, S.; BLOCK, W. D.; COHEN, A. S.; JACKSON, C. E.; KUROIWA, Y.; McKUSICK, V. A.; NISSIM, J.; SCHAR, E. & VAN ALLEN, M. W. Hereditary amyloidosis. Arthritis Rheum. 13:902, 1970.

[5] 5. ARAKI, S.; MAWATARI, S.; OTHA, M.; NAKAJIMA, A. & KUROIWA, Y. Polyneuritic amyloidosis in a Japanese family. Arch Neurol. (Chicago) 18:593-602, 1968.

[6] 6. AUGUSTIN, P. La neuropathie amyloïde. Thèse, Paris, 1964, Cit. por P. E. Becker 7.

[7] 7. BECKER, P. E. Enfermedades con lesión predominante de los nervios periféricos y raices espinales. In P. E. Becker: Genética Humana, tomo V/1 Enfermedades del Sistema Nervioso. Toray, Barcelona, 1969, pp 469-491.

[8] 8. BECKER, P. E.; ANTUNES, L.; RIBEIRO DO ROSÁRIO, M. & BARROS, F. Paramyloidose der peripheren Nerven in Portugal. Z. mensch. Vererb. Konstit Lehre 37:329, 1964.

[9] 9. BENNHOLD, H. Eine spezifische Amyloidfärbung mit Kongorot. Münch. med. Wschr. 69:1537, 1922.

[10] 10. BERTRAND, I.; CASTAIGNE, P.; CAMBIER, J.; CATHALA, H. P.; BINET, J. L. & DRY, J. Sur deux nouveaux cas de neuropathie paramyloïde. Revue neurol. (Paris) 104:380, 1961.

[11] 11. BLOCK, W. D.; RUKAWINA, J. G. & CURTIS, A. C. Serum electrophoretic studies on patients with familial primary systemic amyloidosis. J. Lab. clin. Med. 47:357, 1956.

[12] 12. BLUM, A.; GAFNI, J.; SOHAR, E.; SHIBOLET, S. & HELLER, H. Amyloidosis as the sole manifestation of familial Mediterarnean fever (FMF): further evidence of its genetic nature. Ann. intern. Med. 57:795, 1962.

[13] 13. CANELAS, H. M.; JULIÃO; O. F.; CARVALHO, N. & SCAFF, M. Absorção de vitamina B12 na polineuropatia amiloidótica familiar. Rev. paul. Med. (São Paulo) 75:336, 1969 (Resumo).

[14] 14. CANIJO, M. & PINHO Y COSTA, P. Polineuropatia amiloidótica familiar. Revta clín. esp. 111:583, 1968.

[15] 15. CASTAIGNE, P.; CAMBIER, J. & AUGUSTIN, P. La neuropathie amyloide. Presse méd. 73:1171, 1965.

[16] 16. CHAMBERS, R. A.; MEDD, W. E. & SPENCER, H. Primary amyloidosis with special reference to involvement of the nervous system. Q. Jl. Med. 27:207, 1958.

[17] 17. COHEN, A. S. Amyloidosis. New Engl. J. Med. 277:522, 574 and 628, 1967.

[18] 18. COIMBRA, A. & ANDRADE, C. Familial amyloid polyneuropathy: an electron microscope study of peripheral nerves in five cases. Brain 94:199, 1971.

[19] 19. DICK, P. J. & LAMBERT, E. H. Dissociated sensation in amyloidosis. Arch. Neurol. (Chicago) 20:490, 1969.

[20] 20. FREDERIKSEN, T.; GOTZSCHE, H.; HARBE, N. & KIAER, W. Familial primary amyloidosis with severe amyloid heart disease. Am. J. Med. 33:328, 1962.

[21] 21. FRENCH. J. M.; HALL, G.; PARISH, D. J. & SMITH, W. T. Peripheral and autonomic nerve involvement in primary amyloidosis associated with uncontrollable diarrhoea and steatorrhoea. Am. J. Med. 39:277, 1965.

[22] 22. GILROY, J. & MEYER, J. S. Medical Neurology. Macmillan, New York, 1969, pp 266-267, 625-626, 680-681.

[23] 23. GIMENO, A; GARCIA-ALIX, C; SEGOVIA DE ARANA, J. M.; MATEUS, F. & SOTELO, M. T. Amyloidotic polyneuritis of type III (Iowa-Van Alen). Eur. Neurol. 11:46, 1974.

[24] 24. HELLER, H.; MISSMAHL, H. P.; SIHAR, E. & GAFNI, J. Amyloidosis, its differentiation into perireticulin and pericollagen types. J. Path. Bact. 88:15, 1964.

[25] 25. HELLER, H.; SOHAR, E.; GAFNI, J. & HELLER, J. Amyloidosis in familial Mediterranean fever. Arch. intern. Med. 107:539, 1961.

[26] 26. HELLER, H.; SOHAR, E. & SHERF, L. Familial Mediterranean fever. Arch. intern. Med. 102:50, 1958.

[27] 27. HENSON, R. A. & URICH, H. Metabolic neuropathies. In P. J. Vinken & G. W. Bruyn: Handbook of Clinical Neurology, Vol. 8 Diseases of Nerves Part II. North-Holland, Amsterdam, 1970, pp 4-9.

[28] 28. JULIÃO, O. F. Paramiloidose com comprometimento do sistema nervoso periférico: a propósito de duas observações. Rev. paul. Med. (São Paulo) 56:82, 1960 (Resumo).

[29] 29. JULIÃO, O. F. Aspectos clínicos da neuropatia amilóide: considerações a propósito de 14 casos. Rev. paul. Med. (São Paulo) 63:349, 1963 (Resumo).

[30] 30. JULIÃO, O. F. & MIGNONE, C. Amiloidose primária com comprometimento meningo-radículo-neurítico. Arq. Neuro-Psiquiat. (São Paulo) 13:1-12, 1955.

[31] 31. JULIAO, O. F.; QUEIRÓZ, L. S.; LOPEZ DE FARIA, J. Portuguese type of familial amyloid polyneuropathy. Eur. Neurol. 11:180, 1974.

[32] 32. KANTARJIAN, A. D. & DeJONG, R. N. Familial primary amyloidosis with nervous system involvement. Neurology (Minneapolis) 3:399, 1953.

[33] 33. KAUFMAN, H. E. & THOMAS, L. B. Vitreous opacities diagnostic of familial primary amyloidosis. New Engl J. Med. 261:1267, 1959.

[34] 34. KERNOHAN, J. W. & WOLTMAN, H. W. Amyloid neuritis. Arch. Neurol. Psychit. (Chicago) 47:132, 1942.

[35] 35. LAMPERT, P. W. Amyloid and amyloid-like deposits. In J. Minckler: Pathology of the Nervous System. Vol. 1 Part XIV Probable Metabolic Degenerations. McGraw-Hill, New York, 1968, pp 1113-1121.

[36] 36. MAGALHÃES, A. F. N. Contribuição para o estudo da fisiopatologia das alterações digestivas da polineuropatia amiloidótica familial tipo Corino de Andrade. Tese, Faculdade de Ciencias Médicas, Campinas, 1970.

[37] 37. MAHLOUDJI, M.; TEASDALL, R. D.; ADAMKIEWICZ, J. J.; HARTMANN, W. H.; LAMBIRD, P. A. & McKUSICK, V. A. The genetic amyloidosis. Medicine (Baltimore) 48:1-37, 1969.

[38] 38. McKUSICK, V. A. & GOEDMAN, R. M. Pineal calcification: observations in systemic diseases not associate with disordered calcium metabolism. J. Am., med. Ass. 179:230, 1962.

[39] 39. MELLO, A. R. Polineuropatia amiloidótica familiar. J. bras. Med. (Rio de Janeiro 1:161, 1959.

[40] 40. MERETOJA, J. & TEPPO, L. Histopathological findings of familial amyloidosis with cranial neuropathy as principal manifestation: report of three cases. Acta. path. microbiol. scand. 79:432, 1971.

[41] 41. MISSMAHL, H. P. What is the relation between the various forms of amyloidosis and the connective tissue fibers? Verh. dt. Ges. inn. Med. 65:439, 1959.

[42] 42. MISSMAHL, H. P. Fragen aus der Praxis: was versteht man unter Amyloidose und Paramyloidose? Dt. med. Wschr. 90:582, 1965.

[43] 43. MUCKLE, Th. J. Protein components of amyloid. Nature (London) 203:773, 1964.

[44] 44. MUCKLE, Th. J. & WELLS, M. Urticaria, deafness and amyloidosis: a new heredofamilial syndrome. Q. Jl Med. 31:235, 1962.

[45] 45. MUNSAT, T. L. & POUSSAINT, A. F. Clinical manifestations and diagnosis of amyloid polyneuropathy: report of three cases. Neurology, (Minneapolis) 12: 413, 1962.

[46] 46. OSTERTAG, B. Demonstration einer eigenartigen familiären Paramyloidose. Zentbl. allg. Path. path. Anat. 56:253, 1932.

[47] 47. ROSARIO, R. M.; ANTUNES, L. & BARROS, F. Contribuição para o estudo clínico e laboratorial da paramiloidose de Corino de Andrade. J. Soc. Cienc. méd. Lisboa 125:1-35, 1961.

[48] 48. RUKAVINA, J. G.; BLOCK, W. D.; JACKSON, C. E.; FALLS, H. F.; CAREY, J. H. & CURTIS, A. C. Primary systemic amyloidosis: a review and an experimental genetic and clinical study of 29 cases with particular emphasis on the familial form. Medicine (Baltimore) 35:239, 1956.

[49] 49. SCHILLING, R. F. A new test for intrinsic fator activity. J. Lab. clin. Med. 42:946, 1953.

[50] 50. SILVA-HORTA, J.; FELIPE, I. & DUARTE, S. Portuguese polyneuritic familial type of amyloidosis. Pathologia Microbiol. 27:809, 1964.

[51] 51. SPINA-FRANÇA, A. Eletroforese em papel das proteínas do líquido cefalorraqueano. Rev. paul. Med. (São Paulo) 59:420, 1961.

[52] 52. SULLIVAN, J. F.; TWITCHELL, T. E.; GHERARDI, G. J. & VANDERLAAN, W. P. Amyloid polyneuropathy. Neurology (Minneapolis) 5:847, 1955.

[53] 53. THOMAS, P. K. & KING, R. H. M. Peripheral nerve changes in amyloid neuropathy. Brain 97:395, 1974.

[54] 54. VAN ALLEN, M. W.; FROHLICH, J. A. & DAVIS, J. R. Inherited predisposition to generalized amyloidosis: clinical and pathological study of a family with neuropathy, nephropathy and peptic ulcer. Neurology (Minneapolis) 19: 10, 1969.

[55] 55. VIRCHOW, R. L. K. Cellular Pathology as based on Physiological and Pathological History. De Witt, New York, 1860, pp 409-437.

[56] 56. WOHLWILL, F. Formas atípicas da amiloidose. Amatus lusit. 1:373, 1942.

[57] 57. WONG, V. G. & Mc FARLIN, D. E. Primary familial amyloidosis. Arch. Ophtal. (New York) 78:208, 1963.