Transcript Frontal-Temporal Dementias

Introduction to Degenerative
Dementing Diseases
Diagnosis & Management Issues.
Marilee Monnot, Ph.D., Assistant Professor
Department of Neurology, College of Medicine
OU Physicians CENTER for MEMORY LOSS and DEMENTIA
University of Oklahoma Health Sciences Center
Dementia: A Description of Behavior




Dementia means a progressive decline in cognitive
and behavioral competence, compared to former
abilities.
Many diseases can cause dementia.
Degenerative dementia is a permanent, incurable
state caused by diseases that kill brain cells
progressively.
Each disease usually starts in a different part of the
brain, causing different initial symptoms.
Basic Diagnostic Criteria for
Degenerative Dementing Diseases



Insidious progression
Not due to another medical disorder or delirium
Multiple cognitive deficits (2 or more)






Memory (short and/or long term)
Language (aphasia, anomia, etc.)
Judgment & planning (executive functions)
Motor functions (apraxia, etc.)
Perceptual problems (agnosia, etc.)
Abstracting difficulties (analogies, pattern recognition,
etc.)
Dementia Disease Classification

Cortical dementias



Sub-cortical dementias




Alzheimer’s (familial and non-familial)
Frontotemporal & temporopariental
Corticobasal ganglionic degeneration
Huntington’s and Parkinson’s
Progressive supernuclear palsy
Mixed cortical and subcortical dementias




Vascular dementia (many small strokes)
CJD – “mad cow” disease
Alcoholic dementia
AIDS dementia
Alzheimer’s Disease (AD)





Most common and well-known of the degenerative
dementing diseases (1% at 65 yrs, but 50% at 85 yrs of age).
Starts in the entorhinal cortex, & visual processing
areas of the cortex (↓cholinergic function).
Destroys ability to lay down new memories (caregivers
can’t correct, argue with, or remind patients), but remote
memories are preserved.
Patients usually have adequate social skills until
later stages of illness, when perceptual & judgment
abilities are greatly impaired.
Progresses (last to go is the motor strip)
MRI scan of healthy (left) and AD (right)
brains showing entorhinal cortex atrophy:
Dementia with Lewy Bodies (LBD)

3 variants (pathology: Lewy Body variant of AD, dementia associated with
Parkinson’s disease, & Lewy Body pathology without Parkinson’s)




Prevalence: 1/3 the number of AD patients
Starts at earlier ages
Predominance of males
Clinical syndrome:



Persistent visual hallucinations
Marked fluctuation of cognitive functions
Fluctuating Parkinsonism with sensitivity to
neuroleptics and unresponsiveness to Parkinson
medications
Fronto-Temporal Dementias (FTD)
FTD clinical syndrome (executive dysfunction, apathy,
social misconduct, & disinhibition) - 3 main types:
1. Pick’s disease –
only one hemisphere affected
2. Primary Progressive Aphasia – left hemisphere
degeneration in right-handed patients (aphasia, anomia)
3. Semantic Dementia – left temporal lobe disease
causes fluent dysphasia (severe difficulty in naming &
word meaning comprehension), & associative agnosia
(loss of procedural knowledge)
FTD (continued)
-Right degeneration results in disinhibition.
-Left degeneration results in withdrawal.
-Bilateral degeneration:






echolalia, echopraxia (inappropriate copying behaviors)
linguistic problems (anomia)
mutism, apathy, amotivational states
affective mood syndromes
psychotic symptoms (delusions, paranoia, etc.)
 memory, but not as severe as in AD (poor recent
and remote memory)
FTD (continued)
Neuropsychological testing results:





semantic paraphasias (saying ‘spoon’ for fork)
 confrontation naming (identifying objects/pictures)
 word definition
 recognition of visually-presented objects
 poorer phonemic naming vs category naming


Phonemic = words beginning with letter ‘c’ or ‘m’
Categorical = name animals, or fruits/vegetables, etc.
Subcortical Dementias

Cortiobasal ganglionic degeneration
 Unilateral rigidity and gait problems
 Apraxia (inability to use objects properly, or perform
deliberate movements)




Alien hand syndrome (movements without conscious
intent) and tremor
Reflex myoclonus (muscle spasms or twitches)
Cortical sensory loss (poor temperature perception, etc.)
Chorea (writhing limbs & facial muscles), and/or
choreoathetosis (chronic tight muscles, lack of muscle
tension, proximal jerky involuntary movements, etc.)
Subcortical dementias (cont.)

Huntington’s Disease - traced to emigrants
(1630) from East Anglican village of Bures, England:
 Dominant inheritance (1 parent with gene) - CAG
 Choreoathetosis starting at 35-45 yrs. of age
 Dementia & emotional lability also

Parkinson’s Disease – substantia nigra &
dopamine loss
 Bradykinesia (slowness of movement)
 Resting tremor, rigidity, postural instability
 ↓ facial expression, eye blink rate, arm swing

Dementia occurs very late in the disease
Subcortical dementias (cont.)

Progressive supranuclear palsy – tauopathy results
in a triad of opthalmoplegia (eye muscle weakness), axial
dystonia (contractions), & pseudobulbar palsy (paralysis):






Balance problems & falls
Visual disturbances
Slurred speech
Dysphagia (difficulty in swallowing)
Personality changes
Starts with downward gaze problems
Mixed Dementias

Vascular Dementia (more emotional lability & depression)



Creutzfeldt-Jakob Disease (CJD) = prion disease



‘mad cow’ acquired variant (15%) & sporadic (85%)
Wernicke-Korsakoff syndrome (nutritional, acute onset)
Alcoholic dementia





Binswanger’s (many small-vessel strokes)
Multi-infarct (abrupt and/or step-wise progression)
Cerebellar and corpus callosum atrophy
Affective prosody comprehension decline
Gait problems
Abstract reasoning decline more than education knowledge
Neurosyphilis & herpes simplex viral infections
Mixed Dementias (cont.)

HIV-1 associated dementia (HAD)
 1 yr. after AIDS diagnosis = 7% are demented
 Acute, abrupt onset during secondary infection
 Mood changes & apathy (cortex atrophy)
 Distractibility, poor concentration, & forgetfulness
 Clumsiness, leg weakness, & balance problems
 Hyper-reflexia & sustention tremor
 ↓ fine motor tasks
General Behavioral Considerations





Demented patients have altered perceptions and
judgment, due to the shrinkage of the brain.
They do the best they can with a very injured brain.
They get upset & scared when they are confused.
Speak slowly, in short phrases, with a calm lowered
voice tone; wait for them to understand.
Have a familiar family member in the treatment
room, where the patient can see them during
medical or dental procedures. This helps to
reassure them and prevent agitation and
aggression.