Spinal and Bulbar Muscular Atrophy
Conditions
Interventions
Mexiletine Hydrochloride 300mg daily, for 4 weeks
placebo for 4 weeks
Sponsors
Nagoya University Graduate School of Medicine
Eligibility
Sex/Gender
Male
Inclusion criteria
Inclusion criteria: 1. Male Patients whose number of the CAG repeat in the androgen receptor gene is 38 or more 2. Patients with muscle weakness due to lower motor neuronal loss (bulbar palsy or extremity palsy) 3. Patients with muscle weakness or myotonia under cold exposure 4. Patients are between 20 and 80 years old at the time of the agreement acquisition 5. Patients who are capable of ambulatory hospital visits 6. Patients whose hepatic and renal functions meet the following criteria: Serum AST,ALT: <4.0 * Upper Limit of Institutional Reference Value Serum creatinine: <1.5 * Upper Limit of Institutional Reference Value 7. Patients with written informed consent
Exclusion criteria
Exclusion criteria: 1. Patients who have hypersensitivity for this medicine 2. Patients who have second degree atrioventricular block, third degree atrioventricular block, or left bundle branch block 3. Patients who show Brugada-type ECG 4. Patients who have past history of myocardial infarction, valvular disease, or cardiomyopathy 5. Patients whose heart rate is 50 /min or less 6. Patients who have severe liver or renal dysfunction 7. Patients who have heart failure 8. Patients whose systolic blood pressure is 90mmHg or less 9. Patients who have parkinsonism 10. Patients whose serum potassium level is 3.5mEq/l or less 11. Patients who take anti-arrhythmic drug 12. Patients who take anti-epileptic drug 13. Patient who have neurological disorders except for SBMA 14. Patients whose HbA1c(NGSP) level is 6.5% or more 15. Patients who take hypoglycemic drugs 16. Patients who are alcohol addicts 17. Patients who are drug addicts 18. Patients who take cimetidine, rifampicin, or theophylline 19. Patients who have severe complication 20. Patients whose grip power of dominant hand is less than 10kg 21. Patients who are judged to be inappropriate to participate in the trial by any other reasons
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Change of the difference in distal latencies between under room temperature and cold exposure at 4 weeks | — |
Secondary
| Measure | Time frame |
|---|---|
| (1)Quantitative muscle strength of upper limbs (grip power, pinch power, ten seconds test) (2)Peripheral nerve conduction studies (distal latency, compound muscle action potential, motor nerve conduction velocity, sensory nerve conduction velocity, area, duration time, Harvey-Masland test, skin temperature) (3)Motor function test (tongue pressure, timed walk test, modified QMG score) (4)Pulmonary function test (vital capacity, forced vital capacity, forced expiratory volume, peak expiratory flow and V50/V25, flow-volume curve) (5)ADL scales (ALSFRS-R, SBMAFRS) (6)QOL scales (36-Item Short Form Health Survey, The Individualised Neuromuscular Quality of Life questionnaire, Interactive Voice Response System) (7)Lean mass evaluation by Dual-energy X-ray Absorption (8)Blood examinations (creatine kinase, aldolase, lactate dehydrogenase, aspartate aminotransferase, alanine aminotransferase, testosterone) | — |
Countries
Japan
Contacts
Public ContactShinichiro Yamada
Nagoya University Graduate School of Medicine Neurology
Outcome results
None listed