Overview of young-onset dementias with research update and discussion on forensic implications

1. Young-Onset Dementias Forensic Implications AAPL 2009 Presented by: Ryan C.W. Hall, M.D. and Brian S. Appleby, M.D.

5. Cognition Short-term memory Language Praxis Visuospatial skills Executive Functioning Emotion and personality

8. No Cognitive Deficits

10. Epidemiology Harvey RJ, et al. J Neurosurg Neurol Psychiatry , 2003 Age Range Prevalence rate (per 100,000) Male Female Total 30-64 62.6 45.5 54.0 45-64 119.8 76.5 98.1

11. October 30, 2009 Causes of young-onset dementia Alzheimer disease Cerebrovascular disease Frontotemporal dementias Metabolic disorders Traumatic brain injury HIV/AIDS dementia Alcohol-related dementia Huntington disease Prion dementias Multiple sclerosis Autoimmune illnesses

12. Fujihara S, et al. Arq Neuropsiquiatr , 2004

13. Papageorgiou SG, et al ., Alzheimer Dis Assoc Disord, 2009 AD FTD

14. <45 years of age Kelley BJ, et al . Arch Neurol, 2008

16. ZBI=Zarit Burden Interview Johns Hopkins FTD/YOD Clinic

17. Delany N, et al. Int J Ger Psych , 1995 Sperlinger D, et al. Int J Ger Psych , 1994

18. Appleby BS, et al. [In Press]

19. Velakoulis D, et al . BJP 2009 The solid curve shows the percentage of patients with frontotemporal dementia presenting with schizophrenia-like psychosis; the dashed curve shows the cumulative number of patients with frontotemporal dementia

20. Appleby BS, et al . J Neuropsychiatry Clin Neurosci, 2007

22. Creutzfeldt-Jakob disease Appleby, B. S. et al. Arch Neurol 2009;66:208-215. (Log-rank test, χ 2 = 18.35, P = 0.003) Mean age=59.7

23. Referral Patterns Newens AJ, et al. Br J Gen Pract , 1994 Neurologist-> 63% Psychiatrist-> 27% Other physician->9%

24. Work-up Patterns

26. We are already seeing them! Newens AJ, et al. Br J Gen Pract , 1994

32. Frontotemporal Lobar Degeneration Frontotemporal Dementia Progressive Non-fluent Aphasia Semantic Dementia Neary D, et al. Neurology, 1998

35. @ Primitive reflexes and drives Rational thought, inhibition Illustration from Wider Than The Sky: The Phenomenal Gift Of Consciousness by Gerald Edelman

36. Phineas Gage

40. N Appleby BS, et al. Dement Geriatr Cogn Disord , 2008

42. Creutzfeldt-Jakob disease (CJD)

45. EEG Period sharp wave complexes

46. Brain MRI DWI/FLAIR Cortical ribbon Basal ganglia

48. King S. Lancet , 2008

51. CASE PRESENTATIONS

53. Brain MRI

55. Brain MRI

57. Brain MRI

59. Head CT

61. MRI , 8 months after inhalation

62. MRI , 16 months after acute phase