Causes of neuropathy in patients referred as “idiopathic neuropathy”

: The etiology of neuropathy was idiopathic in 20%–30% of patients despite thorough investigation, based on results from the 1980s and 1990s. Since then, new etiologies have been recognized, and skin biopsy has been used to confirm small‐fiber neuropathy. Methods : The authors reviewed the charts of 373 patients with idiopathic neuropathy who were referred to a neuropathy center between 2002 and 2012. Results : Among the 284 eligible patients, 93 (32.7%) remained idiopathic. The most common cause was impaired glucose metabolism (72 patients, 25.3%), including diabetes in 26 and prediabetes in 46. Other etiologies were chronic inflammatory demyelinating polyneuropathy (CIDP) in 57 (20%) and monoclonal gammopathy in 20 (7%), as well as toxic, Sjögren disease, celiac disease, other immune‐mediated diseases, vitamin B 12 deficiency, amyloidosis, vitamin B 1 and B 6 deficiency, vasculitis, hypothyroidism, hereditary, Lyme disease, and anti‐sulfatide antibody. Conclusions : The major causes of undiagnosed neuropathies were impaired glucose metabolism, CIDP, and monoclonal gammopathies. Despite thorough evaluation 32.7% remained idiopathic. Muscle Nerve 53 : 856–861, 2016