Inclusion Criteria:
- Written informed consent
- Capable of providing informed consent and complying with study procedures. Subjects who are unable to provide consent may use a Legally Authorized Representative (LAR).
- Patients between the age of 25-90 years, medically stable
- Clinical diagnosis of HD with either a confirmed family history or positive CAG repeat (CAG≥35)
- MoCA ≥ 22
- Able to perform the TMT-B in ≤240 seconds
- Total Functional Capacity 7-12
- Stable concomitant medical and/or psychiatric illnesses, in the judgement of the PI.
- QTc interval 350-460 ms, inclusive
- Participants must be willing to undergo LP at baseline and 3 months after treatment
Exclusion Criteria:
- Patients with hypokalemia, hypomagnesaemia, or long QT syndrome- QTc≥461 ms
- Concomitant drugs known to prolong the QTc interval and history of any cardiovascular disease, including myocardial infarction or cardiac failure, angina, arrhythmia
- History or presence of cardiac conditions including:
1. Cardiovascular or cerebrovascular event (e.g. myocardial infarction, unstable angina, or stroke)
2. Congestive heart failure
3. First, second- or third-degree atrioventricular block, sick sinus syndrome, or other serious cardiac rhythm disturbances
4. Any history of Torsade de Pointes
- Treatment with any of the following drugs at the time of screening or the preceding 30 days, and/or planned use over the course of the trial:
1. Treatment with Class IA or III antiarrhythmic drugs (e.g. quinidine)
2. Treatment with QT prolonging drugs (www.crediblemeds.org)- excluding Selective Serotonin Reuptake Inhibitors (SSRIs) (e.g. Citalopram, Escitalopram, Paroxetine, Sertraline, Duloxetine, Trazodone, etc.)
3. Strong CYP3A4 inhibitors (including grapefruit juice). The concomitant use of strong CYP3A4 inhibitors (e.g., ketoconazole, itraconazole, clarithromycin, atazanavir, indinavir, nefazodone, nelfinavir, ritonavir, saquinavir, telithromycin, voriconazole) must be avoided. Grapefruit products may also increase serum concentrations of Nilotinib. Should treatment with any of these agents be required, therapy with Nilotinib should be interrupted.
4. Anticoagulants, including Coumadin (warfarin), heparin, enoxaparin, daltiparin, xarelto, etc.
5. St. John's Wort and the concomitant use of strong other CYP3A4 inducers (e.g., dexamethasone, phenytoin, carbamazepine, rifampin, rifabutin, rifapentine, phenobarbital) must be avoided since these agents may reduce the concentration of Nilotinib.
- Abnormal liver function defined as AST and/or ALT > 100% the upper limit of the normal
- Renal insufficiency as defined by a serum creatinine > 1.5 times the upper limit of normal
- History of HIV, clinically significant chronic hepatitis, or other active infection
- Females must not be lactating, pregnant or with possible pregnancy
- Medical history of liver or pancreatic disease
- Clinical signs indicating syndromes other than idiopathic PD, including corticobasal degeneration, supranuclear gaze palsy, multiple system atrophy, chronic traumatic encephalopathy, signs of frontal dementia, history of stroke, head injury or encephalitis, cerebellar signs, early severe autonomic involvement, Babinski sign
.Current evidence or history in past two years of epilepsy, focal brain lesion, head injury with loss of consciousness or DSM-IV criteria for any active major psychiatric disorder including psychosis, major depression, bipolar disorder, alcohol or substance abuse
- Evidence of any significant clinical disorder or laboratory finding that renders the participant unsuitable for receiving an investigational drug including clinically significant or unstable hematologic, hepatic, cardiovascular, pulmonary, gastrointestinal, endocrine, metabolic, renal or other systemic disease or laboratory abnormality
- Active neoplastic disease, history of cancer five years prior to screening, including breast cancer (history of skin melanoma or stable prostate cancer are not exclusionary)
- Contraindications to LP: prior lumbosacral spine surgery, severe degenerative joint disease or deformity of the spine, platelets < 100,000, use of Coumadin/warfarin, or history of a bleeding disorder
- Must not be on any immunosuppressant medications (e.g. IVig)
- Must not be enrolled as an active participant in another clinical study