A Pilot Trial of Lithium in Subjects With Progressive Supranuclear Palsy or Corticobasal Degeneration

Inclusion Criteria:

1. Able to give informed consent

2. Able to comply with the study protocol, including ability to attend follow-up studyvisits for the duration of the study

3. Diagnosis of PSP or CBD based on the following criteria:

4. Probable PSP:

• Gradually progressive akinetic disorder
• Unequivocal and prominent slowing of vertical saccades or verticalsupranuclear gaze palsy
• Early prominent postural instability or early falls
• Poor or absent response to levodopa

2. Probable CBD:

• Chronic progressive course
• Asymmetric onset
• Presence of higher cortical dysfunction (apraxia, apraxia of speech,non-fluent aphasia, cortical sensory loss, or alien limb)
• Movement disorder: rigid/akinetic syndrome resistant to levodopa andeither dystonic limb posturing or focal myoclonus in limb (spontaneous orstimulus sensitive)

4. If psychotropic or anti-parkinsonian medications are taken (e.g., anxiolytics,hypnotics, benzodiazepines, antidepressants, levodopa, amantadine), the dosage mustbe stable for 28 days prior to the screening visit and should be maintained atconstant dosages throughout the study, as possible

5. If NSAIDs, ACE-Is, ARBs, thiazide diuretics, COX-2 inhibitors or theophylline aretaken by the subject, the dosage must be stable for 28 days prior to the screeningvisit and should be maintained at constant dosages throughout the study, aspossible.

6. Creatinine clearance > 50 ml/min

7. Able to take oral medication

8. Women must not be able to become pregnant (e.g., post menopausal, surgically sterileor using adequate birth control methods for the duration of the study.)

9. Able to identify a study partner

Exclusion Criteria:

1. Evidence of other diseases that could explain the clinical presentation

2. History of known sensitivity or intolerability to lithium or to other knowningredients in the study drug

3. Exposure to any investigational agent within 28 days of the screening visit

4. Clinically significant cardiac disease or EKG findings

5. Other serious illness, including psychiatric illness ("serious illness" is definedas an illness that is unstable enough that it might jeopardize the subject's abilityto complete the study)

6. Moderate to severe ongoing depression

7. Family history of "PSP" or "CBS"

8. Clinically significant abnormalities on the screening visit laboratory results

9. Any AE ≥ Grade 3 as listed on the CTCAE, version 3.0

10. Women who are pregnant or breastfeeding

11. History of brain surgery

12. Use of other potential GSK-3β inhibitors (e.g., valproic acid)

13. Use of iodide salts [e.g., calcium iodide, hydrogen iodide (hydriodic acid), iodide,iodinated glycerol (Organidin), iodine, potassium iodide (SSKI), and sodium iodide]

14. Previous use of lithium

15. Use of Coenzyme Q10 at a dosage greater than 600 mg a day or NanoQuinon at a dosagegreater than 150mg a day or 2.5 mg/kg a day

16. Active psoriasis