title: Low-dose guanidine and pyridostigmine: relatively safe and effective long-term symptomatic therapy in Lambert-Eaton myasthenic syndrome.
abstract: Guanidine hydrochloride is known to be highly effective in the symptomatic treatment of the Lambert-Eaton myasthenic syndrome (LEMS). However, because of its potentially dangerous side reactions of hematologic abnormalities and renal insufficiency, 3,4-diaminopyridine, which is not readily available in the United States, is recommended as the preferred drug for LEMS. We used low-dose guanidine and pyridostigmine combination therapy in 9 patients with LEMS and analyzed its long-term safety and effectiveness. In all patients, a liberal amount of pyridostigmine was used, while daily guanidine dose was kept below 1000 mg a day, and guanidine was given between pyridostigmine dosings. This combination therapy was used for 3-102 months (mean: 34.1 months) and improved clinical status in all patients. Although guanidine had to be discontinued due to severe gastrointestinal symptoms in 3 cases, no serious side reactions such as bone marrow suppressions or signs of renal insufficiency developed in any case. Thus, we conclude that low-dose guanidine therapy is relatively safe and effective for long-term symptomatic treatment of LEMS when it is combined with pyridostigmine.
authors.affiliation: Department of Neurology, University of Alabama at Birmingham 35294, USA.
authors.forename: S J
authors.initials: SJ
authors.lastname: Oh
authors.forename: D S
authors.initials: DS
authors.lastname: Kim
authors.forename: T C
authors.initials: TC
authors.lastname: Head
authors.forename: G C
authors.initials: GC
authors.lastname: Claussen
mesh_terms: D000328:Adult; D000368:Aged; D002800:Cholinesterase Inhibitors / Q000008:administration & dosage* / Q000009:adverse effects / Q000627:therapeutic use; D004305:Dose-Response Relationship, Drug; D004594:Electrophysiology; D005260:Female; D019791:Guanidine; D006146:Guanidines / Q000008:administration & dosage* / Q000009:adverse effects / Q000627:therapeutic use; D006801:Humans; D015624:Lambert-Eaton Myasthenic Syndrome / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000150:complications / Q000628:therapy; D011729:Pyridostigmine Bromide / Q000008:administration & dosage* / Q000009:adverse effects / Q000627:therapeutic use; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D002800:Cholinesterase Inhibitors; D006146:Guanidines; D019791:Guanidine; D011729:Pyridostigmine Bromide