Chapter 12 - Frontotemporal Degeneration

Question 1

Frontotemporal dementia (FTD)

Answer 1

• a.k.a. frontotemporal lobar degeneration (FTLD) or Pick Disease
• involves problems w/ language, behavior, and/or motor findings
• has both language and behavior variants

Question 2

Epidemiology of FTD

Answer 2

• occurs before 65 y.o., usually around 50 y.o.
• definitive diagnosis done by autopsy
• survival is shorter in FTD than AZ

Question 3

Frontal Variant of FTD

Answer 3

• shrinkage of the frontal lobe causes:
  
  • executive dysfunction (impulsiveness, lack of insight, sudden criminal behavior), social and interpersonal conduct problems (antisocial behavior), and apathy and/or disinhibition

Question 4

Primary Progressive Aphasia FTD

Answer 4

• expressive aphasia w/ word finding difficult, agrammatism, and phonetic paraphasias (sub similar sounds for each other)
• difficulty expressing language
• similar to Broca’s

Question 5

Semantic FTD

Answer 5

• temporal lobe variant
• impairment of semantic verbal memory (LTM, general facts) and associative agnosia (won’t know what to do w/ similar objs)
• similar behavioral symptoms as FVFTD

Question 6

Neuropathology of FTD

Answer 6

• frontal, temporal and caudate nucleus atrophy
• nigral pallor
• microvacuolation (holes due to loss of neurons)
• gliosis in frontal and/or temporal lobes
• no reduction in ACh, but could deplete serotonin
• Pick bodies in Pick disease

Question 7

Lewy Body Disease

Answer 7

• important cause of cog decline in older adults
• resembles AZ and Parkinsons

Question 8

Patients show cognitive deficits that reflect damage to the frontal lobe, like:

Answer 8

• learning and memory (retrieval of info)
• attention and concentration
• executive functioning
• language (verbal fluency, difficulty w/ COWA)
• visuospatial functioning

Question 9

Psychiatric features of LWD

Answer 9

• visual and auditory hallucinations and delusions
• depression, anxiety and apathy
• Capgras syndrome (won’t recognize loved ones, think they’ve been replaced by an imposter)
• Reduplicative paramnesia (think their location has been duplicated)
• decrease of REM, so will dream during wake cycles

Question 10

Motor features of LWD

Answer 10

• come much later than in Parkinson’s, but are very similar to Parkinson’s
• spontaneous parkinsonism (tremors and rigidity)
• deficit in learning and mem
• symmetric akinetic-rigid syndrome
• bradykinesia (difficulty initiating movements)
• facial masking
• decreased autonomic system function (lack of sweat, urinary incontinence, etc.)

Question 11

Cretzfeldt-Jakob Disease/Prion Disease/Spongiform encephalopathy

Answer 11

• subcortical dementia characterized by quick progression (death w/n a year)
• extremely rare
• human transmission via transplants of affected neural tissue (eating affected tissue) or there is a genetic form

Question 12

Neuropathology of CJD

Answer 12

• cerebellar and cerebrum damage (uncoordinated, difficulty walking)
• astrogliosis
• amyloid plaques
• abnormal prion proteins (misfold to create the abnormal prion that spreads its own DNA, causing other prions to misfold)

Question 13

What is the hallmark symptomology of CJD?

Answer 13

• motor symptoms that are caused by damage to the cerebellum
• uncoodrinated movement, slurred speech, tremors, choreiform grimaces, blindness, difficulty swallowing