Frontotemporal Degeneration

Transcription

1 Frontotemporal Degeneration Dementia Education for the First Responder July 27, 2017 Gabriel Léger, MD Director, Young-Onset Dementia Program Cleveland Clinic Lou Ruvo Center for Brain Health

2 Frontotemporal degeneration: Overview A few monikers: Frontotemporal dementia Frontal lobe dementia Pick s disease (Arnold Pick described first cases between 1892 and 1906) Others, including subtypes Invariably involves aspects of the frontal and temporal lobes The frontal lobes are a BIG place (responsible for lots of functions) Doesn t always start the same way in everyone Different patients may have different difficulties at first

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4 The Awful, Odd Downfall of the School Librarian of the Year Successful special-education teacher Mid 50 s turned around outdated middle school library Awarded US school librarian of the year in 2013 Disciplined for lewd comments and unsolicited touching Maintains online relationships rather than real life ones Victim of Nigerian scam for thousands of dollars By 2014, wife moves out, broke, evicted In Bronx jail for fraudulent checks related to scam In 2015, living in homeless shelter Found dead, stabbed by his volatile 21 year-old roommate Story released by AP in April 2017

5 FTD: Frontotemporal Degeneration Probably 3 rd most common degenerative dementia 50% of all dementias occurring before age 65 Average age of presentation is mid 50s Only one quarter will present after age 65 Current prevalence ~ 20/ : total population in US Probably grossly underdiagnosed Highly heritable: up to 50%

6 FTD: Frontotemporal Degeneration Young -onset ( age 65) Diagnosis more difficult (re: neuropsychiatric Sx) Impact of diagnosis on family (often younger children) Impact of employment, personal finances, health insurance Quality of life Delay in diagnosis relative to AD 1 to 2 years Multiple healthcare professionals Wrong tests Wrong drugs Medications for AD and LBD probably make FTD worse Relentlessly progressive & incurable, progresses faster than AD

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8 Alzheimer s disease Frontotemporal Degeneration

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10 Diagnostic features Diagnosis: 3 of the following: Behavioral disinhibition Apathy or inertia Loss of empathy or sympathy Perseverative, stereotyped or compulsive/ritualistic behavior Hyperorality or dietary changes Executive deficits on cognitive testing Merrilees, J. (2007). A Model for Management of Behavioral Symptoms in Frontotemporal Lobar Degeneration. Alzheimer disease and associated disorders, 21(4), S64 S69.

11 Other characteristics Often profound anosognosia (lack of insight): There is nothing wrong with me More commonly profound anosodiaphoria (lack of concern): OK, OK, there something wrong, but it doesn t matter, I m fine anyway Unable to understand other peoples emotions (theory of mind) Unable to learn to avoid punishment Still understand wrong from right Can t grasp why it matters Only dementia to be associated with weight gain Many patients will develop parkinsonism or ALS

12 Patients with AD get in trouble, kind of Criminal behavior found in 8% of patients with AD Develops late in the disease Often consequence of cognitive difficulties: Traffic violations as a consequence of inattention Trespassing as a consequence of wandering Liljegren M, et. al. Criminal behavior in frontotemporal dementia and Alzheimer disease. JAMA Neurol Mar;72(3):

13 Patients with FTD really get into trouble Criminal behavior found in almost 40% of patients with FTD Occurs early in disease course presenting symptom in 14% More commonly consequence of: Impulsive & disinhibited behavior Loss of ability to avoid punishment Obsessive compulsive behaviors Criminal behaviors include: Sexual advances, theft, public urination, violence Liljegren M, et. al. Criminal behavior in frontotemporal dementia and Alzheimer disease. JAMA Neurol Mar;72(3):

14 Understanding others emotions / empathy

15 Rosen, HJ Emotional comprehension in the temporal variant of FTD - Brain

16 Learning to avoid punishment: Iowa Gambling Task Bechara, A The Iowa Gambling Task & the somatic marker hypothesis - TiCS

17 ` Torralva, T Affective decision making & ToM in FTD - Neuropsychologia

18 Understanding the difference between right and wrong? Mendez 2005

19 Mendez 2005

20 TITLE 18 - CRIMES AND CRIMINAL PROCEDURE PART I - CRIMES CHAPTER 1 - GENERAL PROVISIONS 17. Insanity defense (a) Affirmative Defense. It is an affirmative defense to a prosecution under any Federal statute that, at the time of the commission of the acts constituting the offense, the defendant, as a result of a severe mental disease or defect, was unable to appreciate the nature and quality or the wrongfulness of his acts. Mental disease or defect does not otherwise constitute a defense. (b) Burden of Proof. The defendant has the burden of proving the defense of insanity by clear and convincing evidence.

21 TITLE 18 - CRIMES AND CRIMINAL PROCEDURE PART I - CRIMES CHAPTER 1 - GENERAL PROVISIONS 17. Insanity defense (a) Affirmative Defense. It is an affirmative defense to a prosecution under any Federal statute that, at the time of the commission of the acts constituting the offense, the defendant, as a result of a severe mental disease or defect, was unable to appreciate the nature and quality or the wrongfulness of his acts. Mental disease or defect does not otherwise constitute a defense. (b) Burden of Proof. The defendant has the burden of proving the defense of insanity by clear and convincing evidence.

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25 Mendez 2005

26 FTD and criminal behavior Is a consequence of the disease Need our protection: early detection and directed to medical attention When to suspect: Middle aged adult exhibiting criminal behavior for the first time Change from baseline behaviors Still need to act according to situation and assessed risk, but Promote sedating SSRI, avoid neuroleptics to the extent possible once intervention complete consider referral to specialty clinic Recent article recommends screening of first-time offenders 55!

27 UCSF ADRC FTD Patient Handout