2018 年 35 巻 4 号 p. 407-410
Neuropathy is a common disease. Recent progress of neurotherapeutics enabled us to treat and cure some intractable neuropathies ; this means that neurologists are required to diagnose and pick–up “treatable neuropathy” patients properly, and to catch–up the recent progress of neuropathy treatment. In this article, I briefly present practical points of diagnosis and treatment in representative treatable neuropathies. In Guillain–Barré syndrome (GBS), first line treatments (plasmapheresis and IVIg) were established in 1980s and 1990s, respectively, and no novel therapeutic regimen has been proposed in these 25 years. However, because a substantial number of patients, especially with severe disease, showed poor recovery and residual deficits, a breakthrough therapeutic strategy has eagerly been awaited. In 2016, Japanese neurologists completed a prospective, multi–center, placebo–controlled double–blinded, randomized phase–II study to evaluate the efficacy of eculizumab in GBS and got favorable results. Future phase–III trials are expected. In chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), three first line treatments (corticosteroids, plasmapheresis, and IVIg) were already established. However, nobody knows which therapeutic regimen is most appropriate in individual patient. Recent discovery of autoantibodies in CIDP (e.g. anti–neurofascin 155) may subdivide CIDP patients, and enable us to choose best therapy in each patient. Vasculitic neuropathy needs prompt diagnosis and rapid commencement of immunotherapy. Pathological confirmation of vasculitis is most important in the diagnosis of this disorder and most of the patients show multiple mononeuropathy, but patients presenting polyneuropathy are not rare. Neuropathy due to vitamin B1 deficiency (beriberi) shows motor–dominant acute polyneuropathy and sometimes misdiagnosed as GBS.
