Clinical Trial of Mexiletine Hydrochloride for Spinal and Bulbar Muscular Atrophy

Inclusion Criteria:

1. Male patients with a CAG repeat count of 38 or more for the androgen receptor genein genetic testing and a confirmed diagnosis of SBMA

2. Patients with muscle weakness (limb weakness and atrophy, or bulbar palsy) due tolower motor neuron lesion

3. Patients with a total ALSFRS-R score of ≥ 24 and ≤ 42 at screening

4. Patients who are at least 18 years old and less than 80 years old at the time ofconsent

5. Patients who give their voluntary written consent after having received adequateinformation on this study (However, if the patient is unable to sign the consentform due to the condition of the disease, a person equivalent to a regalrepresentative must be present to provide written explanation, the prospectivecandidate must verbally consent to participate in the study, and a person equivalentto a regal representative must sign the consent form on behalf of the patient. Theperson who is to be the regal representative may sign the document on his/herbehalf, noting the circumstances and his/her relationship to the subject.)

Exclusion Criteria:

1. Patients who have participated or are participating in a clinical trial within 12weeks prior to enrollment

2. Patients with a history of hypersensitivity to any component of this drug product

3. Patients with a conduction disturbance (such as second- or third-degreeatrioventricular block without a pacemaker, or left bundle branch block)

4. Patients with Brugada-type ECG

5. Patients with severe heart failure or heart disease (myocardial infarction, valvulardisease, cardiomyopathy, etc.)

6. Patients with sinus bradycardia (<50 beats/minute)

7. Patients with systolic blood pressure of 90 mmHg or less

8. Patients with serum potassium level less than 3.5 mmol/L

9. Patients on antiarrhythmic drugs

10. Patients on antiepileptic drugs that affect to sodium channels

11. Patients on theophylline

12. Patients on narcotics

13. Patients who used Mexiletine within 1 month prior to enrollment or used Mexiletinefor expectations of improvement in symptoms of SBMA

14. Patients with serious complications

15. Patients who cannot agree to use contraception during the study period

16. Other Patients deemed inappropriate by the investigator or subinvestigator