Class 8 - Dementia Syndrome

AD - Earliest Symptoms:

• Episodic memory deficits

• Working memory deficits

• Attention & EF impairments

• Language & communication impairments adversely affecting lexical retrieval & discourse

AD - Mid Stage:

• Negative impact on ADLs & reliance on others

• More severe memory loss, attention deficits, dramatic personality changes, visuo-spatial & visuo-constructive deficits, & expressive language deficits

• May experience wanderlust, sundowner syndrome, disorientation, & confusion

AD - Late Stage:

• Loss of motor function

• May become non-ambulatory, bedridden, incontinent, & unresponsive

• Memory, cognition, & expressive language deficits are profound

• May cause muteness & dysphagia

Vascular Dementia (VaD):

• Considered the second most common cause of dementia

• Caused by ischemic or hemorrhagic cerebrovascular disease, cardiovascular disease, or circulatory disturbances that damage brain areas

• Risk factors are similar to AD

• On average, people w/ vascular dementia may progress faster than those w/ AD

What are presenting symptoms of vascular dementia?

• confusion & episodic memory impairments

• slowed processing

• wandering or getting lost in familiar places

• rapid, shuffling gait (history of unsteadiness &/or falling)

• loss of bowel or bladder control

• emotional lability

• difficulty following instructions

• problems handling money

What does a diagnosis of VAD require?

• Objective evidence of cardiac &/or other systemic vascular conditions

• Evidence of cerebrovascular disease etiologically tied to onset of dementia symptoms

• Focal neurological s/s (e.g., difficulties in movement, sensation, or speech-language)

• Brain imaging evidence for ischemic, hemorrhagic, or white matter lesions on CT or MRI scans

What is frontotemporal lobar degeneration (FTD)?

• a heterogenous group of rare neurodegenerative disorders that result in significant impairments of behavior, personality, & distinct types of language impairment

• Most FTDs are diagnosed before the age of 65 years (Alzheimer’s Association, 2018), usually between the age of 35-75 years

• Rapidly progressive; has a 2 to 10 year disease course

• Strong genetic component; positive family history in 20-40% of cases

• Hallmark symptom: Progressive decline in behavior &/or language

(FTD - Neuropathology) What are the 2 brain regions that are the first to deteriorate in FTD?

• Progressive, focal atrophy of the frontal & anterior temporal brain regions

• Spongiform changes in the cortex

• Abnormal tau protein inclusions

What is the behavioral variant of FTD (bvFTD)?

• aka frontotemporal dementia/Pick’s disease

• the most common form of FTD

What are the hallmarks of bvFTD?

• personality changes

• apathy

• progressive decline in socially appropriate behavior, judgement, self-control (lack of inhibition), & empathy

• lack of awareness or concern for the effect of their behavior

• cognitive decline is typically less dramatic than the behavioural disturbance

Which neurological disorder is most commonly associated w/ bvFTD?

• Amyotrophic lateral sclerosis (ALS) can co-occur w/ any of the FTLD clinical variants, but is most commonly associated w/ bvFTD

• In addition to behavioral &/or language changes seen in bvFTD, people w/ FTD-ALS experience the progressive muscle weakness seen in ALS, fine jerks, & wiggling in muscles

Primary Progressive Aphasia:

• PPA is a specific type of a more general frontotemporal dementia

• It is also an unusual dementia since episodic memory functions remain largely preserved for many years

• The language impairment constitutes the most salient symptom that impacts the ADLs in the initial stages

• The cognitive decline follows the language symptoms

• In contrast to Alzheimer’s dementia, where patients tend to lose interest in recreational & social activities, some patients w/ PPA maintain & even intensify involvement in complex hobbies such as gardening, carpentry, sculpting, & painting

What is the diagnostic criteria for PPA?

• Gradual onset of language problems, isolated in initial stages of disease

• No initial prominent visuospatial or episodic memory deficits

• No initial behavioral disturbances

• Impairment not explained by stroke, tumor, TBI, or psychiatric condition

What are the 3 PPA subtypes?

• Semantic variant

• Logopenic variant

• Nonfluent variant

• Diagnosis of PPA subtype should follow PPA diagnosis

• Consensus criteria for diagnosis of PPA subtypes developed by international panel of experts

Semantic Variant PPA:

• loss of semantic knowledge due to anterior temporal lobe atrophy, greater in the language dominant

What are the core clinical features of semantic variant PPA?

• Both of the following:

  • Picture naming deficit

  • Single-word comprehension deficit

What are the supporting features of semantic variant PPA?

• At least three of the following:

  • Loss of object knowledge

  • Surface dyslexia/dysgraphia

  • Relatively preserved repetition

  • Intact grammar & motor speech

What is the disease progression of semantic PPA?

• Atrophy spreads throughout semantic network, eventually affecting frontal & parietal lobes as well

• Progressively empty speech

• Behavioral symptoms may emerge

  • Compulsions

  • Disinhibition

  • Personality changes

  • Altered eating preferences

• Worsening dysexecutive symptoms

Logopenic Variant PPA:

• Impaired phonological processing d/t temporoparietal atrophy, greater in the language dom hemisphere

What are the core clinical features of logopenic variant PPA?

1. Difficulty w/ single-word retrieval in spontaneous speech & picture naming

2. Phrase & sentence repetition deficit

What are the supporting features of logopenic variant PPA?

• At least 3 of the following:

1. Phonemic paraphasias in spontaneous speech & picture naming

2. Relatively preserved comprehension of single words & intact object knowledge

3. Lack of motor speech impairments

4. Spared syntactic processing

What is the disease progression of logopenic PPA?

• Atrophy begins to extend anteriorly into anterior temporal lobes, eventually affecting frontal lobe as well

• Jargon-like aphasic production

• Comprehension deficits emerge

  • Especially for long, complex utterances

• Episodic memory impairment

Nonfluent variant PPA:

• Impaired grammatical processing &/or apraxia of speech d/t frontoinsular atrophy, greater in the language dominant hemisphere

• (“declining” Broca’s aphasia - Pt can say 3-4 words @ a time, w/ smaller utterances) → progressive deterioration

What are the core clinical features of nonfluent variant PPA?

• At least one of the following:

1. Agrammatism

2. Apraxia of speech

What are the supporting features of Nonfluent variant PPA?

• At least two of the following:

1. Syntax comprehension deficit, particularly for complex syntax

2. Relatively preserved comprehension of single words

3. Relatively preserved object knowledge

What is the disease progression of Nonfluent variant PPA?

• May develop generalized movement disorder, including dysphagia

• Increasingly unintelligible & agrammatic

• Mutism

Motor Variant of FTD:

• Least common of the variants - most highly debated

Corticobasal degeneration (CBD):

• Includes (but not limited to) motor, cognitive, & language symptoms

Progressive Supranuclear Palsy:

• Includes (but not limited to) visual disturbances, muscle atrophy, loss of balance & gait instability, & speech language symptoms

What is dementia w/ lewy bodies (DLB)?

• biologically related to PD

• both conditions share pathological hallmark of the presence of Lewy bodies

• Lewy bodies are abnormal clumps of the neuronal protein, alpha-synuclein

• Lewy bodies accumulate in the anterior frontal & temporal cortex, the cingulate area, insula, substantia nigra, locus ceruleus, nucleus raphe dorsalis, & amygdala

What are the DLB symptoms?

• Persistent & complex visual hallucinations or other sensory hallucinations

• Visuospatial impairment

• Sleep disturbance

• Fluctuating attention & vigilance

• Gait imbalances or Parkinsonian movement features

• Reduced speech rate & fluency

• Executive function impairments - cognitive inflexibility

What is the assessment process?

• healthy aging → MCI → dementia

  • screener → screener + comprehensive assmt

Screening for Cognitive Impairment:

• Mini-Mental State Examination (MMSE)

• Montreal Cognitive Assessment (MoCA)

• Saint Louis University Status (SLUMS) Exam

• Clinical Dementia Rating Scale

• Mini-Cog Quick Screening → higher level of evidence & sensitivity than MMSE

Healthy Aging vs. Atypical Aging:

• Distinguishing between healthy / age-related cognitive decline & atypical cognitive decline is critical

• Using screeners (MMSE, MoCA, etc.) → comparing w/ norms that are age & education matched

Clinical Dementia Rating (CDR):

• Pt has diagnosis & has transitioned from home to SNF, staff using this to document decline in performance

• Assists in planning management

  • 0 (no dementia)

  • 0.5 (MCI)

  • 1.0 (mild dementia)

  • 2.0 (mod dementia)

  • 3.0 (severe dementia)

The AD8 (“Eight-item Interview to Differentiate Aging & Dementia”): The Washington University Dementia Screening Test)

• AD8 helps discriminate between signs of normal aging & mild dementia

• May be completed by the older adult or by a reliable informant, either in-person or over the phone

• Contains 8 items that test for memory, orientation, judgment, & function

• Short, simple, & quick to administer (~3 minutes) culturally sensitive

• Combining the AD8 w/ a brief performance test such as the MoCA & Min-Cog greatly enhances the ability to capture early cognitive change

Informant Questionnaire on Cognitive Decline in the Elderly (Short IQCODE):

• a short questionnaire that assesses cognitive decline & dementia in elderly people.

• Filled out by a relative or friend who has known the elderly person for 10 years or more

• Add up the score for each question & divide the # of questions

• Total score range from 1 to 5. A score of 3 means that the subject is rated on average as ‘no change’

• A score of 4 means an average of ‘a bit worse’

• A score of 5 an average of ‘much worse’

• A below 3.31-3.38: failed for dementia

After ruling out healthy aging…

• community-based screeners that are then referred to further neuropsychological testing → SLP

Arizona Battery for Cognitive-Communication Disorders, Second-Edition-Complete Kit:

• SLP must provide diagnosis that is communication-based

• you do NOT want to use this assessment further on in disease progress

• Use this test to narrow in on areas to focus on for tx planning w/ pt in earlier stages of cognitive impairment

Other Short Tests for Assessing Performance in Atypical Aging Across Cognitive Domains:

• Rivermead Behavioral Memory Test (RBMT)

• Repeatable Battery for the Assessment of Neuropsychological Status (RBANS)

• Dementia Rating Scale (DRS-2)

• Cognitive Linguistic Quick Test (CLQT+)

Atypical Aging - Diagnoses & Differential Diagnosis:

• Comprehensive assessment of cognitive components

• Using the neurological findings as supporting evidence for the findings from cognitive testing

• Ruling out other related diagnoses

• COGNITIVE COMMUNICATION DISORDER SECONDARY TO __________