Frontotemporal dementia

1. Frontotemporal
dementia and
criminal behavior
Criteria for bvFTD, Salience
network, Moral dilemmas,
Capacity and Competency
to stand trial in bvFTD,

2. Increasing life expectancy
 In the general population the number of people
over 65 is growing rapidly.
 In 1870 there were 1 million people over 65 in the
US.
 In 2000 there were 35 million people over 65.
 The number of inmates over 65 in state and federal
prisons tripled between 1990 and 2001.

3. Dementia in prison
 40,000 inmates with dementia are currently incarcerated in US
prisons.
 It is estimated that by 2050 this number will be 250,000.
Wilson, J., & Barboza, S. The looming challenge of dementia in prisons. Correctional Care (2010) 24(2), 10–13.

4. Reasons for the graying of prison
population
 Longer incarceration periods
 Mandatory prison sentences
 Increased number of older first offenders
 People in prison age at a faster rate than the
general population

5. Older first offenders
 The new court commitments grew by 109%
between 1995-2009.
 bvFTD patients are likely to be found among this
group
Bureau of Justice Statistics. Human Rights Watch. American Civil Liberties Union. Pew Center for the States.

6. bvFTD patients prone to criminal violations
 FTD represents 5-6% of all dementias.
 54% of patients with bvFTD are involved in criminal
behavior.
 Only 12% of AD are involved in criminal behavior.

7. Frontotemporal lesions and antisocial acts
 It has been known for almost a century that
frontotemporal lesions lead to antisocial acts.

8. Hypothesis
The anterior insular cortex (AIC) and anterior
cingulate cortex (ACC), especially in the right
hemisphere, harbor pro-social cognition,
including:
 -moral emotions
 -moral drives
 -avoidance of harm to others
 -the need for fairness
 -punishment of violators
Lesions of AIC and/or ACC lead to antisocial acts.

9. The insula (insular cortex)

10. Anterior Cingulate Cortex (ACC)
Santillo AF, Mårtensson J, Lindberg O, Nilsson M, et al. (2013) Diffusion Tensor Tractography versus Volumetric
Imaging in the Diagnosis of Behavioral Variant Frontotemporal Dementia. PLoS ONE 8(7): e66932.
doi:10.1371/journal.pone.0066932 http://www.plosone.org/article/info:doi/10.1371/journal.pone.0066932

11. The hallmark – frontotemporal lobar degeneration
(FTLD)
Neuronal cytoplasmic inclusions

12. Inability to solve moral dilemmas
 Frontotemporal lobar degeneration (FTLD) in the
right AIC and ACC impairs moral cognition.
 When patients present with dysmoral behavior for
the first time, as a change from a prior pervasive
pattern of behavior, clinicians need to consider a
possible, causative brain disorder.

13. Moral dilemma 1

14. Moral dilemma 2

15. Moral dilemma 3
 Enemy soldiers have taken over your village. They
have orders to kill everyone. You have hidden in
the basement of a large house along with other
people. Outside you hear the voices of soldiers
who have come to search the house. Your baby
begins to cry loudly. You cover his mouth to block
the sound. If you remove your hand from his
mouth the soldiers will hear his crying and will kill
you, your baby, and the other people hiding in the
basement. To save yourself and the others you
must smother your child to death. Would you
smother your child in order to save yourself and
the other people?

16. Characteristics of bvFTD
 Young onset (midlife)
 Behavioral changes usually misdiagnosed as
psychiatric d/o
 20-50% of cases are hereditary
 15% autosomal dominant
 TAU protein misfolded, but not amyloid beta protein
 VENs are specifically and selectively attacked
(reduced by an average of 74%)
John M. Allman, Nicole A. Tetreault, Atiya Y. Hakeem, Kebreten F. Manaye, Katerina Semendeferi,
Joseph M. Erwin, Soyoung Park, Virginie Goubert, and Patrick R. Hof. The von Economo neurons in the
frontoinsular and anterior cingulate cortex. ANNALS OF THE NEW YORK ACADEMY OF SCIENCES, ISSN
0077-8923

17. Criteria of the 2011 International Behavioral Variant
Frontotemporal Dementia Criteria Consortium
(BVFDC)
 six clinical hallmarks, including: disinhibition, apathy/inertia,
loss of sympathy/empathy, perseverative/compulsive
behaviors, hyperorality and dysexecutive neuropsychological
profile.

 three of these features must be present for the diagnosis of
“possible” bvFTD.
 “Probable” bvFTD requires the presence of functional
disability and characteristic neuroimaging in addition to
clinical symptoms.
 “Definite” diagnosis of bvFTD needs either documentation of
frontotemporal lobar degeneration by histopathological
confirmation or a pathogenic mutation.

18. Types of bvFTD

19. White matter tractography

20. The network

21. von Economo neurons (VENs)

22. The Intrinsic connective networks
ICNs

23. SN functions

24. Criminal Behavior in Frontotemporal Dementia
and Alzheimer’s Disease
 A retrospective medical record review of 2397 patients who were
seen at the University of California, San Francisco, Memory and Aging
Center between 1999 and 2012.
 Criminal behavior can be the first manifestation of patients with FTD
 Patients with FTD were seven times more likely to present with criminal
violations
 Criminal Behavior in Frontotemporal Dementia and Alzheimer DiseaseMadeleine Liljegren, MD; Georges Naasan,
MD; Julia Temlett, MBBS; David C. Perry, MD; Katherine P. Rankin, PhD;Jennifer Merrilees, PhD; Lea T. Grinberg, MD,
PhD; WilliamW. Seeley, MD; Elisabet Englund, MD; Bruce L Miller,MD. JAMA Neurol. doi:10.1001/jamaneurol.2014.378.
Published online January 5, 2015.

25. Capacity to stand trial (CST)
 The legal standard for CST is “whether the individual has
sufficient present ability to consult with his lawyer with a
reasonable degree of rational understanding and
whether he/she has a rational as well as factual
understanding of the proceedings against him/her.”
 This standard is based on the Supreme Court decision
Dusky v. United States in which the Court affirmed a
defendant's right to have a competency evaluation
before proceeding to trial.

26. CST in bvFTD
 In bvFTD assessing capacity to stand trial and
determining culpability and suitable sentencing is
challenging because these defendants are
sometimes able to appreciate the wrongfulness of
their acts and might understand societal rules, but
may be organically incapable of regulating their
behavior .
Sapolsky RM. The frontal cortex and the criminal justice system. Philos Trans R Soc Lond B Biol Sci (2004)
359(1451):1787–96. doi:10.1098/rstb.2004.

27. “Regaining competency”
 Frequently psychiatric reviews leave accused
individuals in legal limbo, waiting to regain
competency in facilities embracing recovery
models.

29. Task force
 Neurodegeneration in state hospitals and
correctional facilities should be studied at both
federal and state level in order to help create an
adequate system of placement for this growing
segment of our population.

30. Screening
 We advocate for screening of all first offenders
55 years of age or older via neuropsychological
testing and/or positron emission tomography
(PET).

31. Placement
 We are in need of a modern system for placement
of individuals with neurodegenerative disorders.
 Such a system should be based on the palliative,
rather than recovery model.
 Such a system would benefit elderly offenders
diagnosed with dementia, while at the same time
decongest correctional facilities.