S. Kruger et al., SENSORIMOTOR POLYNEUROPATHY AND SYSTEMIC AMYLOIDOSIS AS PARANEOPLASTIC SIGNS OF A CARCINOID-LIKE WELL-DIFFERENTIATED CARCINOMA OF THE BREAST, Deutsche Medizinische Wochenschrift, 123(7), 1998, pp. 179-184
History and admission findings: A 75-year-old woman was admitted becau
se of weakness and paraesthesias in both legs. 15 years earlier a ches
t radiogram had shown numerous round foci of uncertain cause. Physical
examination confirmed muscular weakness in all limbs, especially the
legs, as well as abnormal superficial and deep sensory perception. Inv
estigations: Electromyography registered a patchy pattern of intention
al muscular activity and marked denervation activity but largely norma
l nerve conduction. There was no evidence of inflammatory disease in t
he laboratory tests, including the CNS. Diagnosis, course and treatmen
t: The neurological findings indicated progressive axonal sensorimotor
polyneuropathy. A paraneoplastic cause was suspected, but search for
a primary tumour was unsuccessfull. The patient developed a urinary in
fection from which she died in septic shock. A carcinoid-like well-dif
ferentiated carcinoma of the breast with haematogenous metastases to l
iver, spleen and lung was discovered at autopsy. It also revealed seve
re systemic AA-type amyloidosis. involving liver, spleen heart and kid
neys. In addition there was a severe progressive axonal neuropathy and
marked neurogenic atrophy of the peripheral skeletal musculature. No
amyloid deposition was seen. Conclusion: In case of amyloidosis and po
lyneuropathy of uncertain cause a paraneoplastic pathogenesis should b
e considered in the differential diagnosis, even in the absence of pro
ven malignancy.