Dementia due to Fragile X Syndrome (Q99872)

Dementia due to Fragile X Syndrome, a rare genetic syndrome characterised by intention tremor, ataxia, parkinsonism, cognitive and behavioural changes. It is an inclusion body disease with intranuclear inclusions throughout the brain, particularly in the hippocampus and frontal cortex, as well as in the deep cerebellar nuclei. Some have characterised the dementia as one of frontal subcortical subtype.