PSYC 361: Delirium and Dementia

Delirium 

• From the Latin delirare, “being deranged,” “madness.”

• An acute confusional state characterized by disturbances of consciousness and changes in cognition, including severe confusion, which develop over a short period of time

• Can occur in both younger and older adults

• The most important aspect in the diagnosis of delirium is differentiating it from dementia (or other conditions).

• Delirium can often be stopped or cured

Delirium causes

• It is caused by any number of conditions, including:

• Stroke

• Cardiovascular disease

• Metabolic conditions

• Dehydration

• Fever

• Medication side effects*

• Substance intoxication or withdrawal

• Exposure to toxins

• Sleep deprivation

• or a combination of factors

Delirium treatment

• removing/addressing the cause or mitigating effects

• 1/3 of cases are preventable!

• While treatable, delirium can be fatal!

• Important to differentiate delirium from dementia and depression

Dementia

• Dementia literally means “losing one’s mind”

• It is characterized by cognitive and behavioral deficits involving some form of permanent brain damage

• It is a progressive degenerative brain disorder

• Not a single disease, but a cluster of conditions

• About a dozen types of dementia have been identified.

• About 48 million people globally are affected by dementia

• At age of 65 the rate is less than 1%

• For those over 85 the rate increases to about 50%

Alzheimer's Disease (AD, also called Senile Dementia of the Alzheimer Type):

the most common form of progressive, degenerative, and fatal dementia, accounting for as much as 70% of dementia cases

Vascular Dementia (VD)

Numerous small cerebral vascular accidents

Dementia with Lewy Bodies (LBD) & Parkinson’s Disease dementia (PDD)

 Lewy-body related disorders with cognitive impairment

Fronto-temporal dementia (FTD, several variants including Pick’s Disease)

neurodegenerative disorder of the frontal and temporal lobes, characterized by changes in personality and emotion

Creutzfeldt-Jakob disease (CJD)

 rare, invariably fatal brain disorder associated with the spread of misfolded proteins

Mixed dementia

co-occurrence of 2+ types of dementia, most commonly AD and VD

Lewy body dementias

•  a cluster of dementias that include deposition of alpha-synuclein protein into Lewy bodies and neurites

• Two sub-types:

  • Parkinson Disease Dementia (PDD)

  • Dementia with Lewy Bodies (DLB)

Parkinson Disease Dementia (PDD) and Dementia with Lewy Bodies (DLB) both include

• Progressive cognitive impairment

• Parkinsonism (umbrella term for motor symptoms of PD)

• Visual hallucinations

• Sleep disturbances, including REM sleep behavior disorder

PDD and DLB differ in timing of symptoms

• PDD: PD (motor) symptoms happen first, with dementia occurring > 1 yr. Later

• DLB: cognitive symptoms happen early, before or coincident with motor symptoms

Frontotemporal Dementia (Pick’s Disease)

• Rare syndrome [~2.36 cases/100,000 people/yr (Europe)]

• Degeneration of the frontal lobes and may extend to the temporal lobes

• selective early loss of Von Economo Neurons (VENs)

• Both familial and sporadic variants

  • Familial: inherited (30-50% of cases)

  • Sporadic: random occurrence

• Earlier onset and shorter survival than AD

  • Mean age of diagnosis: 56

  • 13% diagnosed before age 50

Frontotemporal Dementia Symptoms

• Typical symptoms include:

• Changes in personality, judgment and behavior

• Difficulty with language

• Lethargy or, oppositely, disinhibition

  • Apathetic patients may become socially withdrawn and stay in bed all day or no longer take care of themselves.

  • Disinhibited patients can make inappropriate (sometimes sexual) comments or perform inappropriate acts.

• Patients become unable to perform skills that require complex planning or sequencing (problems with executive function)

Frontotemporal Dementia: sub-types

• Behavioral-variant FTD [70% of cases]

• Primary progressive aphasia (can be semantic or non-fluent) [25%]

• Right lobe variant FTD

• FTD with motor neuron disease

Frontotemporal dementia: causes

• Genetic risk factors (30-50% of cases)

• Non-genetic risk factors:

  • Neuroinflammation

  • Autoimmune disorders more vulnerable to FTD

• Head trauma

• Prior language learning disorders

• Biology: proteinopathy, related to either TDP-43 or tau misfolding and causing neuron and glia damage

Frontotemporal dementia treatments

• Currently none; management of the disease

  • Physical and occupational therapy

  • Caregiver training

• Experimental:

  • Tau-targeting antibodies or other drugs

  • Gene editing

  • Targeting neuroinflammation pathways

Creutzfeldt-Jakob Dementia: Description

• Rapidly fatal wasting disorder that impairs memory and coordination and causes behavior changes.

• Brain changes: infectious misfolded protein (prion) that causes other proteins throughout the brain to misfold and thus malfunction.

• Spongiform brain appearance

• Very rare (1 million people per year worldwide)

Creutzfeldt-Jakob Dementia: Causes

• Infectious CJD results from exposure to an external source of abnormal prion protein (1%)

• Sporadic CJD develops spontaneously for no known reason (85%)

• Familial CJD: dominant genetic changes inherited from an affected parent (14%)