Non-Alzheimer’s Type Dementias

The University of Texas at TylerNursing 5350 Advanced Pathophysiology

Fall 2014

Michelle Funderburg, BSN, RN

Image retrieved from http://huffingtonpost.co.uk

Dementia FactsDementia is not a normal part of aging.

35.6 million people live with dementia worldwide.

New dementia cases are diagnosed every 4 seconds.

$604 billion spent yearly in U.S. on dementia care.

Caregiver role strain is overwhelming.

Early diagnosis leads to improved quality of life for patient and family.

People with dementia and their families face discrimination.

Improved awareness and advocacy needed worldwide.

More research and evaluation needed.

WHO lists dementia as a public health priority.

Caregivers are emotionally, financially, and physically stressed.

Image retrieved from http://pilgrimshospices.org

(World Health Organization, 2014)

Patient May Notice• Memory Loss that disrupts daily life

• Challenges in planning or solving problems

• Difficulty in completing familiar tasks

• Confusion with time or place

• Trouble understanding visual images and spatial relationships

• Problems with words in speaking or writing

• Misplacing things and inability to retrace steps

• Decreased/poor judgment

• Withdrawal from work or social activities

• Changes in mood and personality

Signs and Symptoms of Dementia

Family May Notice:• repeatedly asks the same questions• becomes lost or disoriented in familiar places• is unable to follow directions• is disoriented as to the date or time of day• does not recognize and is confused about familiar people• has difficulty with routine tasks such as paying the bills• neglects personal safety, hygiene, and nutrition

Image retrieved from blogs.psychcentral.com

(Helpguide.org, 2014)

Vascular DementiaEtiology: Cerebrovascular brain injury

Epidemiology: Men more than women; incidence rises steeply with age; in the U.S., more African-Americans affected than others; higher rates in Japan and other Asian countries

Progression: Progresses in steps, beginning with an abrupt loss of intellectual skill. Life span after diagnosis is average of 5 years.

Prevalence: 1.2-4.2% of adults ≥ 65 years of age

Therapeutic Measures: Ensure safety of the individual affected. Assistance for families. Treat underlying depression.

Risks:• Smoking• Atrial fibrillation• Hypertension• Diabetes mellitus• Hypercholesterolemia• Obesity

Prevention:Promote healthy lifestyle and disease management• Midlife is a critical

period• Balanced nutrition in

young years for neurocognitive development

(Gorelick et al., 2014)

Dementia with Lewy Bodies

Etiology: Alpha-synuclein protein clumps found in the nerve cells of the outer layer of the brain and deep inside the midbrain and brain stem. May have plaques and tangles.

Epidemiology: unknown cause. No group more affected than others.

Progression: Gradual decline in cognition, worsening of symptoms.

Prevalence: 1.3 million individuals in the U.S.

Therapeutic Measures: Cholinesterase inhibitors, antipsychotic drugs, antidepressants, and clonazepam.

Risks:• Unknown Causes

Prevention:Unknown.

(Lewy Body Dementia Website, 2014)

Warning:Typical treatments for Parkinson’s and Alzheimer’s may worsen symptoms, leading to irreversible parkinsonism or neuroleptic malignant syndrome – which may result in death.

Parkinson’s Disease DementiaEtiology: Alpha-synuclein protein clumps, cortical thinning, may have plaques and tangles.

Epidemiology: Older adults,

Symptoms: Muffled speech, visual hallucinations, delusions, disturbed REM, and irritability & anxiety.

Progression: Gets worse over time, speed of progression varies. Cortical thinning

Prevalence: 1 million have Parkinson’s. An estimated 50-80% of them develop dementia.

Prevention: Unknown how to prevent Parkinson’s

Therapeutic Measures: Treat symptoms. Cholinesterase inhibitors, antidepressants, L-dopa, Clonazepam

Risks:• Older age at time of

Parkinson’s diagnosis• Greater severity of

motor symptoms• Mild cognitive

impairment• Hallucinations without

other dementia symptoms

• Daytime sleepiness• Postural instability and

gait disturbance.

Warning:Use antipsychotic drugs with extreme caution.

(Alzheimer’s Association, 2014e; Zaire et al., 2013)

Mixed DementiaDementia – Multifactorial

Etiology: Evidence of Alzheimer’s with another type(s) of dementia

Epidemiology:

Progression: Fast decline

Prevalence: Unknown

Therapeutic Measures: Medication combinations ineffective. Ensure safety of the individual affected. Assistance for families. Treat underlying depression.

Prevention:• Control HTN• Cholesterol levels• Body weight• Diabetes

Risks:• Unknown. Those with

mixed dementia more likely to develop symptoms.

• Cardiovascular problems

(Alzheimer’s Association, 2011; Obering & Batrash, 2009)

Etiology: Abnormal deposits of proteins in brain (Pick’s fibers). Frontal and temporal lobe atrophy. MRI detects shrinking.

Epidemiology: Usually developed by people in their 50s or early 60s. Men and women equally. 4th most common dementia.

Progression: Gradual decline, 2-20 years (typically 6-8 years). Become bedbound and mute.

Prevalence: approximately 250,000 in U.S.

Prevention: None known.

Therapeutic Measures: Treat symptoms (antidepressants and antipsychotics). Family support.

Frontotemporal DementiaPick’s Disease Risks:

• Only known risk factor is family history

Symptoms:• Disinhibition• Language• Movement disorders• Progressive

supranuclear palsy• Less memory loss,

but difficult to assess because of language problems

(McFarland, 2010; Alzheimer’s Association, 2014c)

Creutzfeldt-Jakob DiseaseEtiology: Prion protein conversion destroys brain cells.

Epidemiology: Three groupings: Sporadic, Familial, or Infectious.

Progression: Rapid. 90% die within one year.

Prevalence: Rare, approximately 1 case per one-million people per year worldwide.

Prevention: Synthetic growth hormone; destruction of surgical equipment used on brain or nervous tissue; single-use lumbar puncture kits; cattle guidelines

Therapeutic Measures: Diagnosed through EEG, MRI, or lumbar puncture. Treat symptoms, support individuals and families,

Risks:• Transmissible• Inherited• Infection from medical

procedure• Meat or other products

from cattle infected with bovine spongiform encephalopathy.

Symptoms:• Depression• Agitation, apathy• Rapidly worsening

confusion• Difficulty walking• Muscle stiffness, jerky

movement

(Alzheimer’s Association, 2014b; Centers for Disease Control, 2013)

rEtiology: Ventriculomegaly with preserved cerebral parenchyma resulting from disorder of CSF circulation

Epidemiology: People in their 60’s and 70’s, Cerebrospinal fluid increase in ventricles, yet spinal tap shows normal pressure

Progression: No cure, shunting slows progression for 1-3 years.

Prevalence: Less than 1% of all dementia causes

Prevention: Unknown

Therapeutic Measures: Ventriculoperitoneal shunting. Patient and family support. Treat symptoms.

Risks:• Mostly idiopathic• Hemorrhage• Infection• Inflammation

“Triad” of Symptoms:• Gait problems• Dementia• Urinary incontinence

(Vacca, 2007)

Etiology: Defective gene on chromosome 4 causes changes in central brain

Epidemiology: Mid 40s to early 50’s diagnosis. More in Europe, North America, and Australia than Asia.

Progression: 5 stages, from Early to Advanced

Prevalence: 5-7 people per 100,000 per year.

Prevention: None.

Therapeutic Measures: Treat symptoms. Provide support for patient and caregivers.

Huntington’s DiseaseRisks:• Autosomal dominant• 50% chance of

inheritance

Symptoms:• Clumsiness• Difficulty writing• Difficulty with some

ADLs (driving)• Excess restlessness• Nervous movement• Short-term memory

loss• Impulsiveness• Depression, apathy,

irritability

(Australia Huntington’s, 2014)

Wernicke-Korsakoff Syndrome

Etiology: Thiamine deficiency. Autopsy shows lesions in brain. Wernicke encephalopathy, Korsakoff psychosis.

Epidemiology: 80-90% of those with encephalopathy develop psychosis.

Symptoms: Mental confusion, oculomotor disturbances, ataxia.

Progression: Can be acute, life threatening. About 10-20% die. Thiamine replacement can halt progression.

Prevalence: 0.4-2.8% of reported autopsies

Prevention: Thiamine-rich diet. Do not misuse alcohol

Therapeutic Measures: Treatment with thiamine injectable for acute encephalopathy, oral for chronic psychosis. Avoid counseling. Support for families.

Risks:Thiamine (Vitamin B1) deficiency from• Alcohol misuse• Stringent dieting• Fasting• Starvation• Weight-loss surgery• Uncontrolled

vomiting• AIDS• Kidney dialysis• Chronic infection• Metastasized cancer

(Martin, Singleton, & Hiller-Sturmhofel, 2003; Alzheimer’s Association, 2014d; Family Caregiver, 2014)

Traumatic Brain InjuryEtiology: Direct traumatic injury to the brain. Within hours after injury, Beta-amyloid is present

Epidemiology: Older adults with moderate to severe TBI history, multiple mild TBI history

Symptoms: headache, dizziness, blurred vision, incoherent speech, blurred vision, tinnitus, nausea and vomiting, changes in emotion or sleep patterns

Progression: Dementia symptoms can show up immediately after injury, or years later. No cure.

Prevalence: Approximately 1.5 million people in U.S. suffer TBI annually. Dementia from TBI???

Prevention: Protective gear, minimize fall-risks, vehicle safety

Therapeutic Measures: Serious TBI requires hospitalization. Minor can be monitored at home. Dementia symptoms treated. Family support.

Risks:• Falls• Automobile accidents• Sports injuries• Indirect concussive

force• Bullet or other injury

that penetrates brain• Apolipoprotein-E???

Prevention:• Protective equipment• Vehicle maintenance• Fall prevention

(Alzheimer’s Association, 2012)

Age is the Greatest Risk Factor for Dementia

Females slightly higher prevalence than males.(Image retrieved from scienceblogs.com)

Current ResearchSeveral research studies ongoing for the numerous types of

dementia.

Clinical trials and studies• Treatment trials: test new medications to reduce symptoms of slow/stop disease

progress• Prevention trials: find ways to stop dementia from happening (e.g. vitamins, lifestyle

changes, medication)• Diagnostic studies: find new tests or procedures for diagnosis• Screening studies: find ways to diagnose dementia at the earliest stages• Quality of life studies: to find types of support, education, and training that will

support families and individuals living with dementia and improving their quality of life

As the population continues to age, more money will be spent on researching dementia, its causes and its cures.

Click here to learn more about current dementia news(Alzheimer’s Association, 2014e; Science Daily, 2014)

More on Research

Most dementia research dollars are spent on Alzheimer’s disease because it is the prevalent form of dementia worldwide.

As Alzheimer’s research is completed, findings are often more relational to other forms of dementia.

Not much is understood about how dementia develops, why it develops, or what can treat it.

There is no known cure.

Symptom management is the most that can be expected.

“The National Institutes of Health spends over $6 billion a year on cancer research, over $4 billion on heart disease research and over $3 billion on HIV/AIDS research. But it

spends only $480 million on Alzheimer's research.”

(Alzheimer’s Association, 2014a)

Question One

What is the most important treatment for all types of dementia?

A. Antipsychotic medications

B. Family/Caregiver support

C. Anticholinesterase inhibitors.

D. Diet

Question Two

Which type of medication should be used cautiously with Parkinson’s disease dementia?

A. Antipsychotic medications

B. Antidepressants

C. Anticholinesterase inhibitors.

D. Clonazepam

Question Three

Which of the following is incorrect about Creutzfeldt-Jakob disease?

A. Diagnosis is through EEG, MRI, or lumbar puncture.

B. Prion-protein mis-folds into a 3-dimensional shape.

C. Bovine spongiform encephalitis is an sporadic cause.

D. 90% of people with CJD die within one year.

Question Four

What are the classic triad of symptoms associated with normal pressure hydrocephalus?

A. Dementia, REM sleep problems, and increased headaches

B. Dementia, urinary incontinence, and gait problems

C. Dementia, seizures, and increased cerebrospinal pressure

D. Dementia, hemorrhage, and shock

Question Five

What type of dementia is associated with alcohol misuse?

A. Creutzfeldt-Jakob Disease

B. Mixed Dementia

C. Dementia with Lewy Bodies

D. Wernicke-Korsakoff Syndrome

ReferencesAlzheimer’s Association. (2014a). Boomer report. Retrieved from http://www.alz.org/boomers/

Alzheimer’s Association. (2014b). Creutzfeldt-Jakob disease. Retrieved from http://www.alz.org/dementia/creutzfeldt-jakob-disease-

cjd-symptoms.asp

Alzheimer’s Association. (2014c). Frontotemporal dementia. Retrieved from http://www.alz.org/dementia/fronto-temporal-dementia-f

td-symptoms.asp

Alzheimer’s Association. (2014d). Korsakoff syndrome. Retrieved from http://www.alz.org/dementia/wernicke-korsakoff-syndrome-

symptoms.asp

Alzheimer’s Association. (2011). Mixed dementia: Topic sheet. Retrieved from

http://www.alz.org/dementia/downloads/topicsheet_mixed.pdf

Alzheimer’s Association. (2014e). Parkinson’s disease dementia. Retrieved from http://www.alz.org/dementia/parkinsons-disease-

symptoms.asp

Alzheimer’s Association. (2012). Traumatic brain injury: Topic sheet. Retrieved from

http://www.alz.org/dementia/downloads/topicsheet_tbi.pdf

ReferencesAlzheimer’s Association. (2014e). Trial match. Retrieved from

http://www.alz.org/research/clinical_trials/find_clinical_trials_trialmatch.asp

Australian Huntington’s Disease Association. (2014). Website. Retrieved from http://www.huntingtonsnsw.org.au/

Centers for Disease Control and Prevention. (2013). CJD (Cruetzfeldt-Jakob disease classic). Retrieved from

http://www.cdc.gov/ncidod/dvrd/cjd/

Family Caregiver Alliance. (2014). Wernicke-Korsakoff syndrome. Retrieved from https://caregiver.org/wernicke-korsakoff-syndrome

Gorelick, P., Scuteri, A., Black, S., Decarli, C., Greenberg, S., Iadecola, C., & ... Seshadri, S. (2011). Vascular contributions to cognitive

impairment and dementia: a statement for healthcare professionals from the American heart association/American stroke

association. Stroke (00392499), 42(9), 2672-2713. doi:10.1161/STR.0b013e3182299496

Helpguide.org. (2014). Understanding dementia: Signs, symptoms, types, and treatment. Retrieved from

http://www.helpguide.org/articles/alzheimers-dementia/understanding-dementia.htm

Lewy Body Dementia Website. (2014). Retrieved from http://www.libda.org/

McFarland, D. (2010). The journey through Pick's Disease with a loved one: A personal account. International Nursing Review, 57(1),

142-144. doi:10.1111/j.1466-7657.2009.00743.x

ReferencesMartin, P., Singleton, C., & Hiller-Sturmhöfel, S. (2003). The role of thiamine deficiency in alcoholic brain disease. Alcohol Research &

Health, 27(2), 134-142.

Obering, C., & Batrash, A. (2009). Case report: Combined use of donepezil and galantamine in mixed dementia. Journal of The

American Geriatrics Society, 57(10), 1934-1935. doi:10.1111/j.1532-5415.2009.02450.x

Vacca, V. (2007). Diagnosis and treatment of idiopathic normal pressure hydrocephalus. Journal of Neuroscience Nursing, 39(2), 107-

111.

World Health Organization. (2014). Fact file: 10 facts on dementia. Retrieved from

http://www.who.int/features/factfiles/dementia/dementia_facts/en/index4.html

Zarei, M., Ibarretxe-Bilbao, N., Compta, Y., Hough, M., Junque, C., Bargallo, N., & ... Martí, M. (2013). Cortical thinning is associated

with disease stages and dementia in Parkinson's disease. Journal of Neurology, Neurosurgery & Psychiatry, 84(8), 875-

882. doi:10.1136/jnnp-2012-304126