Intranasal Insulin in Frontotemporal Dementia (FTD)

1. Male or female subject meeting international consensus criteria for probable behavioral variant frontotemporal dementia or criteria for semantic dementia (Gorno-Tempini et al., 2011; Rascovsky et al., 2011)

2. Subject has a Mini-Mental State Exam (MMSE) score ≥18.

3. Subject is > 40 and <90 years of age.

4. Female subjects are post-menopausal or have a negative pregnancy test

5. The subject must be proficient in speaking, reading and understanding English in order to comply with procedural testing of cognitive function, memory and physiology.

6. Subject has a dedicated family member/caregiver, who will be able to attend all visits and report on subject's status.

7. Subject and family member/caregiver have both provided fully informed written consent prior to participation. In the event that subject is legally unable to provide informed written consent due to deterioration in cognitive abilities, fully informed written consent must be provided by a legally authorized representative.

8. Subject must have undergone a brain computed tomography (CT) scan or magnetic resonance imaging (MRI) scan as part of receiving frontotemporal dementia (FTD) diagnosis

1. Subject has medical history and/or clinically determined evidence of other central nervous system (CNS) disorders including, but not limited to brain tumor, active subdural hematoma, seizure disorder, multiple sclerosis, Alzheimer's disease, vascular dementia, corticobasal syndrome, progressive supranuclear palsy, Parkinson's disease, multiple system atrophy, Lewy body dementia, normal pressure hydrocephalus, Huntington's disease, or Jakob-Creutzfeldt disease presenting as dementia.

2. Subject has medical history and/or clinically determined disorders: current B12 deficiency, chronic sinusitis, untreated thyroid disease, or significant head trauma.

3. Subject has history of any of the following: moderate to severe pulmonary disease, poorly controlled congestive heart failure, significant cardiovascular and/or cerebrovascular events within previous 6 months, condition known to affect absorption, distribution, metabolism, or excretion of drugs such as any hepatic, renal or gastrointestinal disease or any other clinically relevant abnormality that inclusion would pose a safety risk to the subject as determined by investigator.

4. Subject has had previous nasal and/or oto-pharyngeal surgery and severe deviated septum and/or other anomalies.

5. Subject has a history of any psychiatric illness that would pose a safety risk to the subject as determined by investigator.

6. Subject is currently taking any medications (anticholinergics, antihistamines, benzodiazepines, barbiturates, or insulin) that are clinically contraindicated as determined by investigator.

7. Subject has undergone a recent change (<1 month) in their selective serotonin reuptake inhibitors (SSRI) or anti-depressant medication.

8. Subject has current or recent drug or alcohol abuse or dependence as defined by the Diagnostic and Statistical Manual of Mental Disorders 5, Text Revision (DSM-IV TR).

9. Screening laboratory results that are medically relevant, in which inclusion would pose a safety risk to the subject as determined by investigator.

10. The subject has participated in a clinical trial investigation within 1 month of this study.

11. The subject has an insulin allergy.