[Peripheral neuropathy and chronic cholestasis due to paucity of interlobular bile ducts (author's transl)]

Peripheral neuropathy was observed in three patients in the course of a chronic cholestasis due to paucity of interlobular bile ducts associated with several malformations (Alagille's syndrome). In two patients, investigations showed a chronic axonal neuropathy. In a third, the low density of myelinated fibers was associated with onion-bulb proliferation on the Schwann-cells and myelin abnormalities related to an associated dominant inherited primary hypertrophic neuropathy. The responsibility of a chronic vitamin E deficiency in the development of axonal lesions is discussed.