Neurocognitive Disorders

SLP Caseload

• 3rd most common diagnosis on caseload of SLPs in healthcare

Dementia Diagnosis (DSM-IV)

A clinical syndrome defined by deterioration of memory (short-term and long-term) and at least one other cognitive function that is severe enough to interfere with daily life activities.

• Abstract thinking

• Personality change

• Judgment

• Language

• Visual recognition

• Others

Dementia (DSM V)

Neurocognitive Disorders rename

Dementia (ICD-10)

Dementia is a syndrome due to disease of the brain, usually of a chronic or progressive nature, in which there is disturbance of multiple higher cortical functions, including memory, thinking, orientation, comprehension, calculation, learning capacity, language, and judgment. Consciousness is not clouded. Impairments of cognitive function are commonly accompanied, and occasionally preceded, by deterioration in emotional control, social behavior, or motivation. Dementia produces an appreciable decline intellectual functioning, and usually some interference with personal activities of daily living, such as washing, dressing, eating, personal hygiene, excretory, and toilet activities.

Dementia (ICD-10)

● Insidious onset

● Not caused by delirium, schizophrenia, or major depression

● Acquired

● Persistent

● Affects several areas of mental function

● Severe enough to interfere with work, social activities, and relationships with others

Dementia (ASHA)

● Dementia is a syndrome of functional and cognitive decline (regardless of underlying pathophysiology).

● It is characterized by progressive deterioration in memory and/or other cognitive-linguistic domains.

● Neurodegenerative and long-term.

○ Dementia symptoms generally continue to progress in severity until death

Primary Dementia

refer to cases in which dementia is the major sign of an organic brain disease (e.g., Alzheimer’s).

○ Most are caused by proteinopathies.

Secondary Dementia

refers to cases in which dementia is caused by/or related to some other recognizable disease (e.g.,

Wernicke-Korsakoff syndrome, chronic traumatic encephalopathy, chemotherapy)

Challenge in Diagnosing Dementia

early signs of dementia are exaggerated forms of minor day-to- day lapses

● Memory failure

● Disorientation

● Lapses in judgment

● Difficulty performing activities of daily life

● Difficulty performing mentally challenging tasks

● Misplacing things

● Apathy and loss of initiative

● Changes in mood

Learning and Memory Deficits

○ Episodic memory deficits (e.g., specific autobiographical experiences).

○ Short-term/working memory deficits (e.g., rapid forgetting of information).

○ Difficulty acquiring and remembering new information (e.g., appointments)

Executive Functioning Deficits

○ Difficulties setting goals and planning.

○ Impaired reasoning and problem-solving (e.g., safety decisions).

○ Difficulty multitasking.

○ Difficulty self-monitoring and correcting errors

Social and Behavioral Deficits

○ Difficulty reading facial expressions/social cues.

○ Loss of empathy, initiative, and motivation.

○ Mood changes (e.g., agitation)

Language Deficits

○ Less concise (empty) discourse.

○ Repetitious/perseverative language.

○ Word-finding difficulties (e.g., recalling names of family and friends)

○ Comprehension deficits (e.g., conversation)

Cortical Dementias

○ Alzheimer's disease (AD).

○ Pick’s Disease, now frontotemporal dementia

Subcortical Dementias

○ Parkinson's disease.

○ Huntington's disease.

Mixed Dementias

○ Vascular dementia.

○ Lewy body disease

Alzheimer’s Disease Demographics

• more common in women than men (~ 2/3 of US-americans with it are women).

• most common cause of irreversible dementia (50-70% of progressive dementia)

Risk Factors for Alzheimer’s Disease

● Age: risk, but it is not a direct cause

● Family history: risk increases if more than one family member has it.

● Genetics: Interactions of genetic and environmental factors.

● Head trauma: Especially repeated or severe injuries.

● Medical conditions. High blood pressure, diabetes, obesity, history of depression.

Alzheimer’s Disease Neuropathology

• Microscopic changes in neurons (not readily visible with MR and CT)

• Microscopic changes are not equally distributed throughout the brain. Frequently affect TPO junction and inferior temporal lobes

  • Neurofibrillary tangles: Threadlike structures normally found in neurons

  • Neuritic Plaques: Abnormal deposits amyloid beta (Aβ) proteins

  • Granulovacuolar degeneration: Small, fluid-filled cavities appear inside neurons

Stages of Alzheimer’s Disease- Mild Cognitive Impairment

• Very mild + mild decline

Mild Cognitive Impairment (MCI) General

● Initially considered a transitional stage between aging and dementia, but some people recover.

● MCI is not dementia, but it can appear before dementia dx.

● Age, sleep disorders, certain infections, and head injury can cause it.

● Symptoms: memory, language, and judgment difficulties.

● Recovery: ~16% revert to the typical range after 1 year.

● Progression: ~10 to 15% convert to dementia

Mild Cognitive Impairment (MCI) dx criteria

● Subjective complaints about cognitive changes (corroborated by family).

● Impairment +1 cognitive domains relative to age and educational level (documented by standardized testing).

● Generally, WNL activities of daily living, but with greater effort.

● Changes are mild, with no significant social or occupational impairment.

Stages of Alzheimer’s Disease- Early Stage AD

• Moderate decline to moderately severe decline

Early Stage AD Signs

● Memory loss

● Poor judgment

● Loss of spontaneity

● Taking longer to complete daily tasks

● Trouble handling money

● Wandering/getting lost

● Losing/misplacing things in odd places

● Mood/personality changes

● Increased anxiety and/or aggression

Stages of Alzheimer’s Disease- Mid Stage AD

• Severe Decline

Mid Stage AD Signs

● Increased memory loss and confusion

● Problems recognizing familiar people

● Difficulty learning new things

● Difficulty with numbers

● Difficulty organizing thoughts

● Shortened attention span

● Problems coping with new situations

● Delusions and paranoia

● Restlessness, agitation, wandering

● Impulsive behavior

● Difficulty carrying out multi step tasks

Stages of Alzheimer’s Disease- Late Stage AD

• Very Severe Decline

Late Stage AD Signs

● Significant difficulty to communicate

● Weight loss

● Seizures

● Difficulty swallowing

● Groaning, moaning, or grunting

● Increased sleeping

● Loss of bowel and bladder control

Frontotemporal Dementia- General

• type of cortical dementia (formally known as Pick’s disease)

• quite rare (less than 1% of the US population)

• makes up about 2-3% of dementia cases.

• Neuropathology:

  • The exact cause is unknown.

  • Proliferation of enlarged neurons (Pick cells).

  • Accumulation of abnormal proteins, such as tau or TDP-43.

  • It generally begins in the frontal lobes but “moves” to the temporal lobes.

Frontotemporal Dementia Types

• Behavioral Variant (bvFTD)

• Primary Progressive Aphasia

Behavioral Variant (bvFTD)

• Mainly causes problems with behavior and personality.

Frontotemporal Dementia (Cognition and Communication)

● Memory and orientation are relatively preserved until late stages.

● Language breakdown appears early and remains prominent in both variants, especially in PPA.

○ Expression – word retrieval failures, circumlocutions, echolalia.

○ Comprehension – difficulty understanding spoken and written information.

Early Frontotemporal Dementia vs. Early Alzheimer’s Disease

●Personality and language changes are more common in early FTD.

● Impulsivity, lack of social inhibition, and wandering are seen early on in FTD.

● Memory impairments are much more prevalent in early AD.

● Persons with early FTD have more significant impairment in activities of daily living (e.g., bathing).

● FTD often diagnosed earlier (before 60s)

Parkinson’s Disease General

• subcortical dementia

• The main symptoms are associated with motor functions (e.g., rigidity, resting tremors, bradykinesia).

• Dementia occurs in 10-40% of cases.

• The exact cause is unknown, but multiple factors are believed to contribute:

  • Degeneration of nerve cells in the basal ganglia (substantia nigra).

  • Genetic mutations (e.g., PARK7, PINK1, and LRRK2).

  • Exposure to certain toxins

Parkinson’s Disease Cognition

● Forgetfulness.

● Slowed Thinking.

● Difficulty Concentrating.

● Executive Function Problems.

Parkinson’s Disease Communication

● Slurred speech.

● Reduced Volume.

● Reduced Pitch variation.

● Increased speech rate.

● Voice Tremor.

● Reduced Facial Expressions.

● Limited Body Language.

● Naming difficulty

Parkinson’s Disease Motor Traits

• tremor, bradykinesia, rigidity, PIGD (postural instability and gait disorders).

Parkinson’s Disease Cognitive Traits (and motor)

attention, memory, language, visuospatial, executive function

Parkinson’s Disease Psychiatric traits (and motor)

depression, apathy

Parkinson’s Disease Dementia

● 10-20% of people with __ have MCI at diagnosis, which may develop into dementia.

● prevalence rate is ~15-20%, 5 years after diagnosis, and ~46% after 10 years.

● Dementia with Lewy bodies and ___ are Lewy body-related neurodegenerative disorders sharing common clinical findings.

● Symptoms: impaired attention, decline in memory, issues interpreting visual information, executive dysfunction, mood changes, hallucinations, delusions.

Huntington’s Disease General

• subcortical dementia

• Inherited and progressive disorder.

  • 50% chance of gene inheritance.

• Symptoms appear in middle adulthood and gradually worsen over time.

• Symptoms include:

  • Uncontrolled movements (chorea)

  • Cognitive decline

  • Mood changes

  • Behavioral changes

Huntington’s Disease: Cognition and Communication

● Dysarthria

● Chorea affects articulatory and respiratory musculature

● Dysphagia

● Language is relatively preserved unless it involves sustained attention, memory, and judgment

Vascular Dementia General

• Mixed profile

• Multi-infarct dementia, ischemic cerebrovascular disease, ischemic dementia, and vascular cognitive impairment (VCI).

• It is due to multiple cortical infarcts.

• Second leading cause of dementia, after AD (~20% of all dementia cases).

Vascular Dementia: Cognition and Communication

• Cognitive signs and symptoms are heterogeneous, depending on the lesion distribution/vascular damage.

• Cortical lesions are associated with amnesia, visual-spatial deficits, and aphasia.

• Subcortical lesions are associated with memory, attention, and motor function impairments

Lewy Body Disease- General

● Lewy body dementia is an umbrella term for dementia with Lewy bodies and Parkinson's disease dementia.

● Lewy bodies are clumps of alpha-synuclein proteins.

● Third leading cause of dementia, behind AD and VaD.

● Lewy bodies are usually found in the frontal, temporal lobes, and basal ganglia

Lewy Body Disease- Symptoms

● Fluctuating presentation of cognitive symptoms.

● Procedural memory and learning deficits may occur with subcortical pathology.

● Declarative memory systems may be impaired with cortical pathology.

● Persistent hallucinations.

● Prominent visuospatial difficulties

• movement deficits

• sleep problems

• behavior changes

Symptoms Mistaken for Dementia

• Depression: sometimes called “psuedodementia”

• Delirium: symptoms of delirium (a transient confusional state) sometimes resemble symptoms of dementia.

• Aphasia: Patients with dementia may do poorly on both verbal and nonverbal tests. May be described as having “aphasic language” or “dementia with aphasia

SLP role in Dementia Assessment

● A medical team makes the diagnosis of dementia.

● The role of the SLP is to assess the strengths and weaknesses of cognitive-communication.

○ SLPs also do education, training, and counseling.

○ An SLP and/or another member of the interdisciplinary care team can conduct screening for cognitive impairment.

SLP Role in Dementia Treatment

● Lessening and compensating for communication challenges

● Functional maintenance programs

● Fostering meaningful communication environments