II. Epidemiology
- Affects 30% men with FRM1 premutation 50-60 years old
III. Mechanism
- Related to Fragile-X Mental Retardation (FMR1) gene
IV. Clinical findings
- Progressive Intention Tremor with cerebellar Ataxia
V. Presentation
- Parkinsonism
- Peripheral Neuropathy
- Dementia after age 50 years