Adrenomedullin for CADASIL

Inclusion criteria

• Patients who have given written informed consent from the patient or the sponsor to participate in the clinical trial
• Patients aged between 20 and 90 at the time of obtaining consent
• Patients diagnosed as CADASIL after confirming NOTCH3 gene mutation by genetic testing
• Patients with Mini-mental state examination-J score of 10-27 or Trail maiking test score (age ajustment) of average + 1.5 SD (standard deviation) or higher

Exclusion criteria

• Patients who cannot perform cognitive function tests (deafness, blindness, etc., MMSE-J less than 10 points Severe cognitive impairment, etc.)
• Patients received reatment with prohibited drugs or prohibited therapy within the past 12 weeks from the time of registration
• Patients who started to take concomitant restriction drugs or changed dosage of concomitant restriction drugs within the past 4 weeks from the time of registration
• Patients whose Mini-mental state examination-J with 4 or more points improvements between the time of registration and 4 weeks or more at the time of screening (If patients who take concomitant restriction drugs)
• Patients with active infections requiring antibiotic treatment at registration
• Patients with a disability equivalent to modified Rankin Scale 5 at registration
• Patients with severe consciousness impairment (Japan Coma Scale 100 or more)
• Patients with severe renal impairment (estimated GFR less than 30 mL / min / 1.73m2) at registration
• Patients with severe liver damage (transaminase AST (GOT) or ALT (GPT) 100 IU / L or more) at registration
• Patients diagnosed as having cerebral infarction or intracranial hemorrhage or transient ischemic attack or cerebral aneurysm with high probability of rupture within the last 12 weeks from the time of registration
• Patients with occlusion or severe stenosis of the intracranial main artery or carotid artery at the time of registration
• Patients with significant ECG abnormalities (atrioventricular block of 2-3 degrees, extension of QRS interval of 120 ms or more, extension of QTcB of 450 msec or more) at registration, or past histroy of acute coronary syndrome or acute heart failure within the last 12 weeks from the time of registration
• Patients with systolic blood pressure less than 100 mmHg at registration
• Patients whose pulse rate is less than 45 beats / minute or 120 beats / minute or more at registration
• Patients with substance abuse or alcoholism
• Patients who cannot perform MRI
• Patients with active solid malignant tumors
• Patients who do not give consent to contraception from the date of obtaining consent until the end of the safety evaluation period
• Pregnant, lactating, and possibly pregnant
• Patient who participated in another trial within 24 weeks before registration
• Other patients judged by the Investigator or Investigator to be ineligible for this study