https://www.tes.

com/lessons/CudugoeTIsg4-A/phineas-gage
Behavioral Variant
Frontotemporal
Dementia
Haley Creamer, Zach Finzi, and Grace Voorhees
 Who gets dementia?

https://www.irishtimes.com/life-and-style/health-family/it-s-time-to-to-insist-on-proper-care-for-people-
with-dementia-1.2695034
 Road Map
➢ Overview
○ FTLD, FTD, and bvFTD
➢ Brief History
➢ Subtypes
○ bvFTD, PPA
➢ Symptomatology
○ Signs, symptoms, and neuroanatomy
➢ Diagnosis
➢ Epidemiology & Etiology
➢ Treatment
Frontotemporal Lobar Degeneration
(FTLD)
● Affects Frontal and Temporal Lobes
○ Gives rise to FTD
○ Does not include Alzheimer’s Disease
● Causes:
○ τ-protein
○ fused-in sarcoma protein (FUS)
○ transactive response DNA-binding protein 13 (TDP)
○ dipeptide proteins (mutant gene C9ORF72)
https://www.pinterest.com/pin/803188914765317666/
Frontotemporal Dementia (FTD)
● First described in 1892 by physician Arnold
Pick, leading to it being called “Pick’s
Disease.”
● Associated with degeneration of frontal and
temporal lobes and their respective
functions.
● Characterized by 2 main subtypes of the
disease: bvFTD and PPA.

http://www.alz.org/dementia/fronto-temporal-dementia-ftd-symptoms.asp
Subtypes of FTD
1. Behavior Variant Frontotemporal Dementia (bvFTD)
○ Characterized by behavioral alterations

1. Primary Progressive Aphasia (PPA)

○ language, speaking, and writing deficiencies
○ Subtypes:
■ Semantic Variant of PPA
■ Nonfluent/agrammatic variant of PPA
Symptomatology of bvFTD
● Behavioral Symptoms
○ Hyperoral, repetitive behaviors, loss of habits, hyperactive/hypersexual behavior, and impulsivity.
○ Degeneration of frontal lobe

● Emotional Symptoms
○ Apathy, lack of insight, emotional blunting, unstable mood
○ Degeneration of frontal & temporal lobe

● Neurological Symptoms
○ Parkinsonism
Motor Disturbances in FTD
● Can occur in the presence or absence of the two variations of FTD ( bvFTD or PPA)
● Not the prominent symptom of FTD. FTD normally characterized first by changes associated with
frontal and temporal lobe degeneration.
● Subtypes
○ Amyotrophic Lateral Sclerosis (ALS)
■ Increased muscle weakness and wasting.
○ Corticobasal Syndrome
■ Unilateral paucity of movement in arms and legs, muscle rigidity and tremors.
○ Progressive Supranuclear Palsy (PSP)
■ Loss of balance, muscle stiffness, changes in posture and problems with vision and eye movement.
 Repetition???
Neuroanatomical Symptomatology
DLPFC - cognitive decline

mPFC - apathy

OFC - emotional blunting

& inhibition

ACC - apathy

ATL - emotional blunting

Anterior Insula - emotional

blunting
https://www.neuroscientificallychallenged.com/blog/know-your-
brain-preoptic-area
Diagnosis
DSM-5 Criteria: ❏ Issue: Patient unaware of changes
➔ Present with 3 or more behavioral
symptoms ❏ Early behavioral presentations
➔ Gradual Progression
➔ Sparing learning, memory, & ❏ Hyperorality, cognitive decline, motor
perceptual-motor function deficiencies present later

❏ Issue: often misdiagnosed as

Possible Frontal Behavioral Inventory &
psychiatric disorder
Middelheim Frontality Scale

Probable Genetic testing, family history

Definite CT, MRI, CSF biomarker

 Psychiatric Manifestations

● Initial diagnosis as psychiatric illness

○ Psychopathological changes absent of neurological signs

● Frequent misdiagnoses:
○ BPD, OCD, schizophrenia, psychotic depression, ect.

● Genetic predispositions
○ C9ORF72 - OCD, BPD
○ GRN - bulimia, personality changes, ritualistic behavior
Epidemiology
Incidence :
Sociological epidemiology
- 2.7 - 4.1 / 100,000 (Europe, North
● over 10,000,000 people in the
America) in individuals <70 yrs
US have a neurodegenerative
condition
Distribution :
● FTD is most common in adults
- 45-64 yrs: 2–31/ 100,000 (North
under 65 years of age
America, Asia, and Europe)
● mean onset 56 years. Range
30-82 years
● 13% of FTD cases occur in
individuals younger than 50
 Etiology
Approximately 50% of individuals with FTD report a positive family history of the disease,
thus suggesting a genetic component

● PGRN, TDP 43, and TAU mutations have been found in individuals with FTD
● TAU and TDP 43 protein mutations account for 90% of individuals with inherited forms of FTD
● Mutations of these proteins result in protein misfolding and subsequent protein build-up
Treatment
● Currently no cure for bvFTD
● Treatment of bvFTD is largely focused on disease management
○ Caregiver education and support, behavioral interventions, occupational therapy
● Some pharmacological agents are used to treat certain symptoms experienced by patients:
○ SSRIs
○ Neuroleptic drugs (antipsychotics)
References
● Banks, S. J., & Weintraub, S. (2008). Neuropsychiatric Symptoms in Behavioral Variant Frontotemporal Dementia and Primary Progressive Aphasia. Journal of Geriatric Psychiatry and Neurology, 21(2), 133–141.
http://doi.org/10.1177/0891988708316856

● Behavioral Variant FTD (bvFTD). (n.d.). Retrieved September 13, 2017, from https://www.theaftd.org/understandingftd/disorders/bv-ftd

● Bonniwell, B. & Feidt, S. (2012). The Occupational Therapy Role in the Management of Frontotemporal Dementia: A Systematic Literature Review (Master’s Thesis). Retrieved from ProQuest. 1530585

● Diehl-Schmid, J., Perneczky, R., Koch, J., Nedopil, N., & Kurz, A. (2013). Guilty by suspicion? Criminal behavior in frontotemporal lobar degeneration. Cognitive and Behavioral Neurology: Official Journal of the Society for Behavioral and Cognitive
Neurology, 26(2), 73–77. https://doi.org/10.1097/WNN.0b013e31829cff11

● Grossman, M., Dr. (2013, April 17). Overview of Frontotemporal Lobar Degeneration. Retrieved September 12, 2017, from https://www.youtube.com/watch?v=qETS3pX3Y50

● Holroyd, C. B., & Yeung, N. (2012). Motivation of extended behaviors by anterior cingulate cortex. Trends in Cognitive Sciences, 16(2), 122–128. https://doi.org/10.1016/j.tics.2011.12.008

● Kim, E.-J., Sidhu, M., Gaus, S. E., Huang, E. J., Hof, P. R., Miller, B. L., … Seeley, W. W. (2012). Selective frontoinsular von Economo neuron and fork cell loss in early behavioral variant frontotemporal dementia. Cerebral Cortex (New York, N.Y.:
1991), 22(2), 251–259. https://doi.org/10.1093/cercor/bhr004

● Kortte, K.B. & Rogalski, E.J. (2013). Behavioral interventions for Enhancing Life Participation in behavioral variant Frontotemporal Dementia and Primary Progressive Aphasia. Int Rev Psychiatry, 25(2), 237-245.
http://doi.org/10.3109/09540261.2012.751017

● Lanata, S.C. & Miller, B.L. (2016). The behavioural variant frontotemporal dementia (bvFTD) syndrome in psychiatry. J Neurol Neurosurg Psychiatry, 87(5), 501-511. http://doi.org/10.1136/jnnp-2015-310697

● Manoochehri, M. & Huey, E.D. (2012). Diagnosis and Management of Behavioral Issues in Frontotemporal Dementia. Curr Neurol Neurosci Rep, 12(5), 528-536. http://doi.org/10.1007/s11910-012-0302-7

● Morrison, J. (2015, March 09). Temporal Lobe Anatomy & Pictures | Body Maps. Retrieved September 12, 2017, from http://www.healthline.com/human-body-maps/temporal-lobe/male

● Neurocognitive Disorders | Diagnostic and Statistical Manual of Mental Disorders. (n.d.). Retrieved September 14, 2017, from http://dsm.psychiatryonline.org/doi/full/10.1176/appi.books.9780890425596.dsm17

● Pose, M., Cetkovich, M., Gleichgerrcht, E., Ibáñez, A., Torralva, T., & Manes, F. (2013). The overlap of symptomatic dimensions between frontotemporal dementia and several psychiatric disorders that appear in late adulthood. International Review of
Psychiatry, 25(2), 159–167. https://doi.org/10.3109/09540261.2013.769939

● Rankin, K. P., Gorno-Tempini, M. L., Allison, S. C., Stanley, C. M., Glenn, S., Weiner, M. W., & Miller, B. L. (2006). Structural anatomy of empathy in neurodegenerative disease. Brain: A Journal of Neurology, 129(Pt 11), 2945–2956.
https://doi.org/10.1093/brain/awl254

● Rohrer, J., Bocchetta, M., Boretska, S., Brotherhood, E., Clarke, M., Dick, K.,..., Woollacott, I. (n.d.). FACTSHEET 12: How is FTD treated?, Retrieved from http://www.ftdtalk.org

● Tekin, S., & Cummings, J. L. (2002). Frontal-subcortical neuronal circuits and clinical neuropsychiatry: an update. Journal of Psychosomatic Research, 53(2), 647–654.

● Warren, J. D., Rohrer, J. D., & Rossor, M. N. (2013, August 06). Frontotemporal dementia. Retrieved September 12, 2017, from http://www.bmj.com/content/347/bmj.f4827

● Wider, C. & Wszolek, Z.K. (2008). Etiology and Pathophysiology of Frontotemporal Dementia, Parkinson Disease and Alzheimer Disease: Lessons from Genetic Studies. Neurodegener Dis., 5(3-4), 122-125. http://doi.org/10.1159/000113680

● Woolley, J. D., Khan, B. K., Murthy, N. K., Miller, B. L., & Rankin, K. P. (2011). The diagnostic challenge of psychiatric symptoms in neurodegenerative disease; rates of and risk factors for prior psychiatric diagnosis in patients with early
neurodegenerative disease. The Journal of Clinical Psychiatry, 72(2), 126–133. https://doi.org/10.4088/JCP.10m06382oli
Any Questions?