Introduction to Degenerative Dementing Diseases Diagnosis & Management Issues.

1. 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

2. 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.

3. 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.)

4. Dementia Disease Classification • Cortical dementias • Alzheimer’s (familial and non-familial) • Frontotemporal & temporopariental • Sub-cortical dementias • 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

5. 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)

6. MRI scan of healthy (left) and AD (right) brains showing entorhinal cortex atrophy:

7. 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

8. 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 causesfluent dysphasia (severe difficulty in naming & word meaning comprehension), & associative agnosia (loss of procedural knowledge)

9. 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)

10. 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.

11. 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.)

12. 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

13. 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

14. Mixed Dementias • Vascular Dementia (more emotional lability & depression) • Binswanger’s (many small-vessel strokes) • Multi-infarct (abrupt and/or step-wise progression) • Creutzfeldt-Jakob Disease (CJD) = prion disease • ‘mad cow’ acquired variant (15%) & sporadic (85%) • Wernicke-Korsakoff syndrome (nutritional, acute onset) • Alcoholic dementia • Cerebellar and corpus callosum atrophy • Affective prosody comprehension decline • Gait problems • Abstract reasoning decline more than education knowledge • Neurosyphilis & herpes simplex viral infections

15. 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

16. 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.