Disclosed are methods of treating a disorder or disease associated with myotonic dystrophy. Methods of treating a CNS dysfunction and/or cognitive impairment associated with myotonic dystrophy in a subject comprising administering a therapeutically effective amount of a GABAA receptor antagonist or inverse agonist to the subject are disclosed. Methods of treating a myotonic dystrophy associated disease or disorder caused by mis-splicing of GABRG2 in a subject comprising administering a therapeutically effective amount of a GABAA receptor antagonist or inverse agonist to the subject are disclosed. Methods of improving cognitive function or alertness in a subject having myotonic dystrophy comprising administering a therapeutically effective amount of a GABAA receptor antagonist or inverse agonist to the subject are disclosed. Examples of the GABAA receptor antagonist or inverse agonist include flumazenil, clarithromycin, a fluoroquinolone, picrotoxin, bicuculline, gabazine, cicutoxin, oenan-thotoxin, pentylenetetrazol, Ro15-4513, sarmazenil, amentoflavone, zinc, and any combination thereof.
TREATMENT OF CONDITIONS ASSOCIATED WITH MYOTONIC DYSTROPHY