Chronic idiopathic axonal polyneuropathy revisited

Objective To investigate whether several years after the diagnosis chronic idiopathic axonal polyneuropathy (CIAP), the cause of the neuropathy is still idiopathic. Methods All outpatients were included in this study in which, between 1993-2000, with the same guideline for chronic neuropathy, the diagnosis CIAP was made. We checked whether the patients had been evaluated according to this guideline. We evaluated the clinical course in all patients and repeated questions on the family history. We also re-examined these patients. After recording these data, we decided whether or not the neuropathy remained idiopathic. Results A total of 478 outpatients had been evaluated, of these 53 were diagnosed as CIAP. Of these the diagnosis remained CIAP in 27 (51%) patients. In 19 (36%) of the patients a wrong diagnosis was made, explained by not following the diagnostic guideline, and by not recognising hereditary neuropathies. Conclusion In half of the patients with the diagnosis CIAP, this diagnosis was changed after revision, including the family history of neuropathies. Therefore, in patients with the diagnosis CIAP, this diagnosis should be reconsidered. In addition the question should be whether the hereditary neuropathies have been excluded.