Postmortem findings in primary familial amyloidosis with polyneuropathy.

摘要: The pathology of primary familial amyloidosis with polyneuropathy is described on the basis post-mortem examination six cases from Northern Sweden. Clinically disease characterized by progressive sensory and motor disturbances loss sensation, muscular wasting flaccid paralysis. Impotence, urinary bladder dysfunction, motility gastro-intestinal tract postural hypotension indicate affection autonomic nervous system as well. Malabsorption, cardiac insufficiency vitreous opacites also occur. As regards distribution amyloid, following findings seemed to be characteristic. Usually there were no gross lesions indicating amyloid disease. Histopathologically, deposits observed in great extent peripheral various parts It occurred extensively walls blood vessels calibres, perivascular collagenous connective tissue adjacent smooth musculature. Amyloid deposition was found more or less abundantly other organs tissues. No deposits, however, only insignificant amounts, central system, either parenchyma liver, islets Langerhans, bone marrow. Clinical manifestations related local substance. Our clinical pathological this particular type conformed mostly those previously described.