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

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

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

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  • Denmark
  • Delayed toxic leukoencephalopathy

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  • 1. Young-Onset Dementias Forensic Implications AAPL 2009 Presented by: Ryan C.W. Hall, M.D. and Brian S. Appleby, M.D.
  • 2. Disclosures
    • Co-Investigator of a multi-center trial of memantine for the treatment of frontotemporal dementia (Forest Laboratories)
    • Honoraria from the CJD Foundation
  • 3. Objectives
    • Demonstrate the differential diagnosis for young-onset dementias
    • Describe the neuropsychiatric complications of young-onset dementias
    • Understand the management and legal complications of young-onset dementias
  • 4. Dementia
    • Cognitive impairment
    • Intact consciousness
    • Functional impairment
  • 5. Cognition Short-term memory Language Praxis Visuospatial skills Executive Functioning Emotion and personality
  • 6. Definition
    • Cognitive and functional impairment in individuals <65 years of age
    • Further classification :
    • Early-onset: <65 years of age
    • Young-onset: <45 years of age
    Kelley BJ, et al. Arch Neurol , 2008
  • 7. Cognitive Symptoms
    • Memory Clinic, <60 years of age:
    • -55%, no cognitive deficits
    • -13%, mild cognitive complaints
    • -17%, selective cognitive deficits
    • -15%, demented
    Vraamark Elberling T, et al. Neurology , 2002
  • 8. No Cognitive Deficits
  • 9. Epidemiology (45-64 years of age)
    • Incidence: 11.5 per 100,000 individuals
    • Prevalence: 98.1 per 100,000 individuals
    Mercy L, et al . Neurology, 2009 Harvey RJ, et al . J Neurol Neurosurg Psychiatry 2003
  • 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
  • 15. Challenges
    • Diagnosis
    • Occupational
    • Financial
    • Social
    • Family-spouse and children
    • Behavioral issues
    • Better overall health and mobility
    • Disease specific-FTD, fAD, CJD
  • 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
  • 21. Delayed Presentation
    • From onset to initial evaluation
    • 3 years (Fujihara S, et al. Arq Neuropsiquiatr , 2004)
    • 3 years (Papageorgiou SG, et al. Alzheimer Dis Assoc Disord , 2009)
    • 2 years (Kelley BJ, et al. Dement Geriatr Cogn Disord , 2009)
  • 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
  • 25. Why Psychiatrists?
    • High prevalence of behavioral disorders
    • Frequently taking psychotropic meds
      • 41% on neuroleptics
    • High degree of strain and psychiatric morbidity in caregivers
    • Need for education
    Baldwin RC. Psychiatr Bull , 1994 Kelley BJ, et al. Dement Geriatr Cogn Disord , 2009 Delany N & Rosenvinge H. Int J Ger Psychiatry , 1995
  • 26. We are already seeing them! Newens AJ, et al. Br J Gen Pract , 1994
  • 27. Diagnosis History
    • Course of illness
    • Pattern of cognitive deficit
    • Family history
    • Other neurological signs/symptoms
    • Other systemic illness
    • Social/Drug history
    Ridha B & Josephs KA. The Neurologist , 2006
  • 28. Preliminary work-up
    • CBC
    • Coagulation studies
    • CMP
    • TFT’s
    • Vitamin B12 & folate
    • Fasting lipid panel
    • RPR
    • ESR & CRP
    • Urine toxicology
    • Brain MRI
    • Neuropsych testing
  • 29. Expanded work-up
    • Antibody screen
    • Ceruloplasmin
    • Iron studies
    • Heavy metal screen
    • SPEP/UPEP
    • Homocysteine
    • NH4
    • Genetic testing
    • Brain PET scan
    • EEG
    • LP
    • Whole body PET
  • 30. Alzheimer’s disease (AD)
    • Most prevalent dementia
    • Presents differently in younger patients
      • Posterior cortical atrophy
      • Driving concerns
    • Familial illness (<1%)
      • Pre-senilin-1&2
      • Amyloid precursor protein
    Tang-Wai DF, et al. Neurology, 2004
  • 31. Case
    • 57-year-old man with AD
    • Initial symptoms were visuospatial impairment and acalculia
    • Police contact for suspicions of driving while intoxicated
  • 32. Frontotemporal Lobar Degeneration Frontotemporal Dementia Progressive Non-fluent Aphasia Semantic Dementia Neary D, et al. Neurology, 1998
  • 33. FTD Core Diagnostic Features
      • Insidious onset, gradual progression
      • Early decline in social interpersonal conduct
      • Early impairment in regulation of personal conduct
      • Early emotional blunting
      • Early loss of insight
    Neary D, et al. Neurology, 1998
  • 34. FTD Supportive diagnostic features
    • Behavioral disorder
    • Speech and language disorder
    • Physical symptoms
    • Investigational findings
      • Neuropsychological testing
      • EEG
      • Brain imaging
    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
  • 37. Case
    • 55-year-old gentleman with FTD
    • First presentation at airplane security
    • Multiple family stressors
      • Social
      • Occupational
      • Financial
    • Spouse displays positive coping skills in advocacy
    • Solution : Travel letters
  • 38. Case
    • 59-year-old women with cortical basal degeneration
    • Expressive aphasia and acalculia
    • Hands blank checks to clerks
    • Young children, ages 9 and 14
    • Solution: Pre-paid credit/debit cards and online video communication
  • 39. Traumatic Brain Injury
    • Cognitive and neuropsychiatric effects
    • Coup/contre-coup preferentially affect frontal lobe nerve fibers
    • Often associated with alcohol use
    • Chicken/Egg scenario
  • 40. N Appleby BS, et al. Dement Geriatr Cogn Disord , 2008
  • 41. Creutzfeldt-Jakob disease
    • Prion disease
    • Rapidly progressive dementia
    • Symptoms
      • Dementia
      • Cerebellar impairment
      • Vision impairment
      • Pyramidal/Extrapyramidal symptoms
    • Mean age of onset=62
    • Mean survival time=4-6 months
  • 42. Creutzfeldt-Jakob disease (CJD)
  • 43. Definite CJD
    • Histology
    • Immunohistochemistry
  • 44. Probable CJD
    • Two symptoms:
      • Cerebellar and/or visual impairment
      • Myoclonus
      • Akinetic mutism
      • Pyramidal and/or Extrapyramidal symptoms
    • Diagnostic Investigations
      • Electroencephalogram (EEG)
      • Cerebrospinal 14-3-3 protein
      • Brain MRI
  • 45. EEG Period sharp wave complexes
  • 46. Brain MRI DWI/FLAIR Cortical ribbon Basal ganglia
  • 47. Case #4
    • 57-year-old man with sCJD
    • Social stressors
      • Lack of support
      • Curiosity
      • Social construction (e.g. mad-cow disease)
    • Solution : Family gives vague explanation (e.g. neurodegenerative illness)
  • 48. King S. Lancet , 2008
  • 49. Huntington’s disease
    • Trinucleotide repeat (CAG) disorder
    • Genetic anticipation
    • Atrophy of the caudate nucleus
    • Movement (e.g. chorea), cognitive, and psychiatric symptoms
    • Increased risk of suicide
  • 50. Case
    • 40-year-old male with moderate dementia and psychosis from HD attacks a medical student during an examination.
    • Risk assessment
    • Consent for treatment
  • 51. CASE PRESENTATIONS
  • 52. Case #1
    • 64 y.o. Caucasian female
    • Problems remembering dates and conversations, would repeat self
    • Gradual progression with some daily fluctuations
    • ↑ irritability, ↑ fatigue, ↓ functioning
    • Social withdrawal, suspicious at times
  • 53. Brain MRI
  • 54. Case #2
    • 51 y.o. Caucasian male
    • Trouble focusing, prioritizing, organizing
    • Increase frequency and severity of angry outbursts
    • Loss of empathy
    • Rigid and resistant to change
    • Mini-mental state exam=30/30
  • 55. Brain MRI
  • 56. Case #3
    • 39 y.o. Jordanian male
    • Painful paresthesias of his feet (Aug)
    • Depression (Sept)
    • Forgetful (Nov)
    • Inability to dress self (Jan)
    • Worsening gait, incontinence, myoclonus (Feb)
  • 57. Brain MRI
  • 58. Case #4
    • 41 y.o. Caucasian female
    • Following birth of child in her 30’s became anergic, depressed, and distractable
    • Progressed to impulsivity and more distractability (12 car accidents/5 yrs)
    • Periods of exhilaration and irritability with poor sleep
  • 59. Head CT
  • 60. Case #5
    • 43 y.o. Caucasian male
    • History of alcohol, benzo, opioid abuse
    • Found in respiratory distress, obtunded with pinpoint pupils
    • Developed depression, apathy, disinhibition, MMSE=20/30
  • 61. MRI , 8 months after inhalation
  • 62. MRI , 16 months after acute phase
  • 63. Thank you
    • Johns Hopkins FTD/YOD Clinic:
    • Chiadi Onyike
    • Mary Anne Wylie
    • Rebecca Rye
    • Kate Hicks
    • Peter Rabins
    • Kostas Lyketsos