Acessibilidade / Reportar erro

Primary amyloidosis with meningo-radiculo-neuritic involvement

In this paper two cases which were published in 1939 are reviewed, both characterized by a clinical syndrome of meningo-radiculo-neuritis, with the following symptoms: motor impairment, of flacid type, on the upper and lower extremities; disturbances of superficial sensation (beginning at the 4th cervical root) and also of deep sensibility although less marked; trophic lesions (muscular atrophies which in case 2 were accompanied by perforating ulcer of the feet) ; gastro-intestinal symptoms; impotence and sphincter disorders; disturbances of the pupils. The histopathological study showed in case 2 the existence of degenerative lesions and slight chronic inflammation of the meninges, roots and peripheral nerves. The nature of the condition was not determined; leprosy was in both cases eliminated as a possible cause. The papers of Corino de Andrade (1951-52) on "familial atypical generalized amyloidosis" brought about the revision of the cases above mentioned, not only because the symptoms were very similar to those described by Andrade, but also because both patients were Portuguese, the first of whom was born in Póvoa de Varzim, area where the author observed the majority of cases. Re-examining the slides of case 2 and with special staining methods (gentian violet), the authors were able to verify amyloid deposits in the peripheral nerves and roots as well as in the meninges. As for case 1, the authors believe it is also a case of primary amyloidosis, based on clinical symptoms and on the birthplace of the patient. Besides these two cases, the authors make reference to a third case still under observation, which is clinically characterized by peripheral neuropathy, by gastro-intestinal disturbances, by sexual and sphincter disorders. The patient is a Portuguese who had been living in Brazil for 15 years when the first symptoms appeared. In this case it was also possible to confirm the diagnosis of "atypical amyloidosis" by the histological studies (biopsies of the testis and cutaneous nerve).


Academia Brasileira de Neurologia - ABNEURO R. Vergueiro, 1353 sl.1404 - Ed. Top Towers Offices Torre Norte, 04101-000 São Paulo SP Brazil, Tel.: +55 11 5084-9463 | +55 11 5083-3876 - São Paulo - SP - Brazil
E-mail: revista.arquivos@abneuro.org