What is Dementia?

• Dementia: The term means loss of cognitive abilities such as memory, language, problem solving etc. that impairs one’s ability to perform daily activities independently, first instrumental activities of daily living (i.e. shopping, paying bills, cooking, driving, etc.) and later even basic activities of daily living (dressing, grooming, toileting etc.). Dementia is also called a Major Neurocognitive Disorder. The term dementia does not imply an underlying cause/etiology. Dementia can develop due to a number of causes including a neurodegenerative process: Alzheimer’s dementia (AD), Dementia with Lewy bodies (DLB), Frontotemporal dementia (FTD), or other causes: cerebrovascular brain disease (Vascular dementia), infection (Herpes encephalitis), metabolic (vitamin deficiency: vitamin B12 or folate), or excessive alcohol intake, among others.

What are some of the common Dementia types?

•AD (Alzheimer’s disease) ~60-80%
•Mixed Dementia (AD+VaD; AD+DLB)
•VaD (Vascular Dementia) ~5-10%
•DLB (Dementia with Lewy Bodies) ~5-10%
•PDD (Parkinson’s disease dementia)
•FTLD-frontotemporal lobar degeneration ~ <10%
•NPH (Normal pressure hydrocephalus) ~<5%
•Autoimmune Dementia, i.e. NMDAR, LGI1, GABA A/B encephalitis, IgLON5)
•Prion disease i.e. CJD (Creutzfeldt-Jacob disease), rare cause

What is an Alzheimer's disease?

• Alzheimer’s disease is the most common cause of dementia. Alzheimer’s disease is defined by 2 (two) pathological brain changes: buildup of amyloid plaques (containing amyloid beta protein) and neurofibrillary tangles (containing protein tau). The lifetime risk of AD dementia in first degree relatives is ~39% if one parent and 54 % (by age 80), if both parents had AD dementia. Young onset Alzheimer’s disease (onset below 65 y/o) has 3 known gene mutations (APP, PSEN1, PSEN2). Apolipoprotein E (ApoE) is the most important genetic risk factor for late onset AD (onset of dementia above age 65 y/o). The ApoE4 isoform is associated with highest risk of AD. Other AD dementia risk factors include age (over 65), midlife hypertension, elevated cholesterol, diabetes, obesity, head injury, hearing loss, smoking, low educational attainment. AD dementia median survival ~ 8-12 years from diagnosis.

What is MCI ( Mild Cognitive Impairment)?

• MCI (Mild Cognitive Impairment) it is an in between state, between normal aging and dementia. In MCI, cognitive changes are greater than expected for age, but independence in daily activities is preserved. MCI poses greater risk of progressing towards dementia, about 10-15% annual risk, as compared to general population risk of developing dementia being 1-2 % annually.

What are the Dementia stages?

• Mild dementia stage: memory loss is evident, often word finding difficulties, loss of interest in hobbies may be seen, and difficulties with IADL (instrumental activities of daily living): driving, paying bills, medications management. Moderate
dementia stage: memory loss for personal information, more difficulties with language and comprehension, initiation of activities is difficult due to poor executive functions, cuing on basic ADL’s (activities of daily living) is often needed (i.e. showers, changing clothes). In this stage of dementia behaviors frequently emerge, including delusions (false beliefs), agitation, sundowning, and bowel or bladder incontinence. Severe dementia stage: hands on assistance is needed with basic ADL’s (clothing, baths/showers, toileting). Bowel and bladder incontinence present. Eventually complete loss of independence occurs, usually there is a limited communication, and difficulties with walking and swallowing.

What are BPSD (Behavioral and Psychological Symptoms of Dementia)?

NEGATIVE symptoms (a loss):
loss of empathy
low mood (depression)
poor motivation (apathy, social withdrawal)
circadian rhythm disturbances (insomnia, reversal of sleep wake cycle)
changes in hunger (can cause weight loss) and thirst (can cause dehydration)

POSITIVE symptoms (a gain):
irritability and anger
anxiety
agitation
restlessness
separation anxiety (shadowing)
auditory or visual hallucinations (psychosis)
delusions/paranoia (psychosis)
obsessive or compulsive behavior (rummaging, picking)
violence (hitting, aggressive, combative behavior)

What are Cognitive symptoms?

Memory loss (amnesia)
Loss of awareness (agnosia)
Apraxia (difficulties performing tasks, i.e. dressing)
Aphasia (difficulties with language)
Impaired Visuospatial skills (i.e. getting lost, wandering)
Impaired Executive functions (poor judgement, reasoning, or problem-solving skills)

What is FTD?

Frontotemporal Dementia is a neurodegenerative brain disease affecting predominantly the frontal and temporal lobes (FTLD, frontotemporal lobar degeneration). The main types of FTD include behavioral variant FTD (bvFTD) and Primary Progressive Aphasia (PPA). The subtypes of PPA include semantic variant PPA (svPPA), nonfluent/agrammatic variant PPA (nfvPPA) and logopenic variant PPA (lvPPA). FTD onset is commonly age 45-65, but about 25% of FTD can present after age 65. Prevalence of FTD is about 20 cases per 100,000. FTD pathology: FTLD-tau, FTLD-TDP, FTLD FET (Fus). 20-30% FTD is familial and 3 main genes involved are: C9orf72, GRN, MAPT.

What is DLB?

DLB (Dementia with Lewy Bodies) is a neurodegenerative brain disease related to abnormal aggregation of alpha-synuclein. Symptoms include fluctuations in cognition and alteration in alertness, hallucinations, RBD (REM behavior disorder), parkinsonism, repeated falls, constipation, orthostatic hypotension, urinary incontinence, medication sensitivity, etc. The nomenclature is complex. Lewy Body Dementia aka PDD and DLB. Lewy Body Disease aka PD, PDD,DLB. PD (Parkinson's disease), PDD (Parkinson's disease dementia), DLB (Dementia with Lewy Bodies).