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Young-Onset Dementias Forensic Implications AAPL 2009 Presented by: Ryan C.W. Hall, M.D. and Brian S. Appleby, M.D.
Disclosures ,[object Object],[object Object]
Objectives ,[object Object],[object Object],[object Object]
Dementia ,[object Object],[object Object],[object Object]
Cognition Short-term memory Language Praxis Visuospatial skills Executive Functioning Emotion and personality
Definition ,[object Object],[object Object],[object Object],[object Object],Kelley BJ,  et al.  Arch Neurol , 2008
Cognitive Symptoms ,[object Object],[object Object],[object Object],[object Object],[object Object],Vraamark Elberling T,  et al.  Neurology , 2002
No Cognitive Deficits
Epidemiology (45-64 years of age) ,[object Object],[object Object],Mercy L, et al .  Neurology, 2009 Harvey RJ, et al .  J Neurol Neurosurg Psychiatry 2003
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
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
Fujihara S,  et al.  Arq Neuropsiquiatr , 2004
Papageorgiou SG, et al ., Alzheimer Dis Assoc Disord, 2009 AD FTD
<45 years of age Kelley BJ, et al . Arch Neurol, 2008
Challenges ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
ZBI=Zarit Burden Interview Johns Hopkins FTD/YOD Clinic
Delany N,  et al.  Int J Ger Psych , 1995 Sperlinger D,  et al. Int J Ger Psych , 1994
Appleby BS,  et al.  [In Press]
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
Appleby BS, et al . J Neuropsychiatry Clin Neurosci, 2007
Delayed Presentation ,[object Object],[object Object],[object Object],[object Object]
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
Referral Patterns Newens AJ,  et al.  Br J Gen Pract , 1994 Neurologist-> 63% Psychiatrist-> 27% Other physician->9%
Work-up Patterns
Why Psychiatrists? ,[object Object],[object Object],[object Object],[object Object],[object Object],Baldwin RC.  Psychiatr Bull , 1994 Kelley BJ,  et al.  Dement Geriatr Cogn Disord , 2009 Delany N & Rosenvinge H.  Int J Ger Psychiatry , 1995
We are already seeing them! Newens AJ,  et al.  Br J Gen Pract , 1994
Diagnosis History ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Ridha B & Josephs KA.  The Neurologist , 2006
Preliminary work-up ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Expanded work-up ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Alzheimer’s disease (AD) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Tang-Wai DF,  et al.  Neurology,  2004
Case ,[object Object],[object Object],[object Object]
Frontotemporal Lobar Degeneration Frontotemporal Dementia Progressive Non-fluent Aphasia Semantic Dementia Neary D, et al.  Neurology, 1998
FTD   Core Diagnostic Features ,[object Object],[object Object],[object Object],[object Object],[object Object],Neary D, et al.  Neurology, 1998
FTD Supportive diagnostic features ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Neary D, et al.  Neurology, 1998
@ Primitive reflexes and drives Rational thought, inhibition Illustration from Wider Than The Sky: The Phenomenal Gift Of Consciousness by Gerald Edelman
Phineas Gage
Case ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Case ,[object Object],[object Object],[object Object],[object Object],[object Object]
Traumatic Brain Injury ,[object Object],[object Object],[object Object],[object Object]
N Appleby BS,  et al. Dement Geriatr Cogn Disord , 2008
Creutzfeldt-Jakob disease ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Creutzfeldt-Jakob disease (CJD)
Definite CJD ,[object Object],[object Object]
Probable CJD ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
EEG Period sharp wave complexes
Brain MRI DWI/FLAIR  Cortical ribbon Basal ganglia
Case #4 ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
King S.  Lancet , 2008
Huntington’s disease ,[object Object],[object Object],[object Object],[object Object],[object Object]
Case ,[object Object],[object Object],[object Object]
CASE PRESENTATIONS
Case #1 ,[object Object],[object Object],[object Object],[object Object],[object Object]
Brain MRI
Case #2 ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Brain MRI
Case #3 ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Brain MRI
Case #4 ,[object Object],[object Object],[object Object],[object Object]
Head CT
Case #5 ,[object Object],[object Object],[object Object],[object Object]
MRI , 8 months after inhalation
MRI , 16 months after acute phase
Thank you ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

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Research on Young-Onset Dementia and Its Implications for Criminal and Civil Forensic Cases

Notas del editor

  1. Denmark
  2. Delayed toxic leukoencephalopathy