Neuro - Dementia

Dementia - compromise in at least 2 of the following

Language

Memory

Visuospatial

Executive function

Emotional Behavior

Personality

Dementia syndromes result from

disruption of large-scale neuronal networks

Mild cognitive impairment

a measurable cognitive problem that does NOT seriously disrupt daily activities

Patient is still able to carry out ADLs

Apolioprotein e4 allele

increases the risk of developing late onset Alz

Protein elevated in AD

Tau

Strongest RF for Dementia

Increasing age

MCC of Dementia

Alzheimer's disease

2nd MCC of Dementia

Vascular

Main categories of Dementia

Degenerative neuro disorders

Vascular

Infection

Infections that can cause Dementia

HIV

Creutzfelt-Jakob

Characteristics of Mild dementia

Short term memory/language/word finding

Aphasia

Agnosia

Apraxia

Executive function

Characterisitics of Moderate Dementia

Can't learn new

Personality changes

Psychotic sxs

Behavior disorders

Severe dementia

Can't perform basic activities

Totally dependent on others

AD - Brain atrophy begins ____ and then progresses___

medial temporal lobes -> lateral and medial parietal lobes -> frontal cortex

Characterisitic pattern of AD

Memory impairment -> language -> visuospatial -> executive dysfunction

AD treatment

acetylcholinesterase inhibitors (AChE)

-DONEPEZIL (ARICEPT)

Metamine

What significantly improves rate of decline in AD pts

Metamine + ACH inhibitor

First sx of AD is

impaired memory formation - esp recent events or newly learned information

Go over stages of dementia

*

For vascular dementia, changes in the brain are not due to AD pathology but due to

chronic, reduced blood flow to the brain

The clinical features of vascular dementia are related to

the area of the brain that has been affected

vascular dementia - if cortical area affected

speech difficulty, trouble with routine tasks, etc

vascular dementia - if subcortical area affected

gait, urinary difficulties, motor deficits, personality changes

Focal neurologic findings that can be found in vascular dementia

unilateral weakness, babinkski, unilateral hyperreflexia

DX of choice for vascular dementia

MRI - T2 weighted and flair best shows white matter changes

Can you undo vascular dementia

No, can only prevent further damage

Possible causes of Frontotemporal Dementia

Picks dz, ALS

Hallmark of Frontotemporal Dementia

progressive gradual decline in behavior and language

3 core clinical syndromes of Frontotemporal Dementia

Behavioral Variant

Primary progressive aphasia - semantic variant

Primary progressive aphasia - non fluent agrammatic varient

Frontotemporal Dementia - behavioral varient

most common

social and emotional dysfunction

most often accompanied by motor neuron diseasse

Frontotemporal Dementia - Semantic varient

lose the ability to decode a word, object or emotional meaning

Frontotemporal Dementia - non fluent agrammatic varient

profound inability to produce words, promiennt motor speech impairment

Frontotemporal Dementia - MRI shows

Frontal and anterior frotal lobe atrophy

early may have no changes

PET scan of Frontotemporal Dementia

Reveals frontal and/or anterior temporal hypometabolism (helps differentiate from AD**)

Pt takes an anticholinergic drug and it makes symptoms WORSE

think Frontotemporal Dementia

Frontotemporal Dementia treatment

No treatment expect treat symptoms

Drug that makes Frontotemporal Dementia worse

Cholinesterase inhibitors

Lewy body dementia - progressive degeneration in which LB build up in the

Cerebral cortex, midbrain & brainstem

Main diff from AD and Lewy body dementia

Movement issues**

Visual sxs

Spontaneous parkinsonism features should make you think of which type of dementia

Lewy body dementia

Treat the cognitive symptoms of Lewy body dementia

cholinesterase inhibitors

First line in Lewy body dementia

DONEPEZIL (ARICEPT) same as AD

Treat the parkinsons features in Lewy body dementia

LEVODOPA

Usually NO ____ when treating Lewy body dementia

Antipsychotics - can develop Neuroleptic Malignant syndrome

Caused by an accumulation of PrP prions

Creutzfedlt-Jakob Disease

Three major categories of Creutzfedlt-Jakob Disease

Sporadic (mc type)

Hereditary

Acquired (exposure to brain or nerbous tissue)

Rapidly progressing dementia as well as motor abnormalities

Creutzfedlt-Jakob Disease

Creutzfedlt-Jakob Disease - generally causes death within

9 months

MRI shows basal ganglia hyperintesity on T2 MRI sequence

Creutzfedlt-Jakob Disease

Cortical ribonning on MRI

Creutzfedlt-Jakob Disease

Creutzfedlt-Jakob Disease - only way to confirm dx

brain biopsy/autopsy

Creutzfedlt-Jakob Disease treatment

no cure or treatment - treat paim

Huntington's Disease - mutation of the ____ gene

HTT

Huntington's Disease - degeneration of the neruonal cells, esp in

frontal lobes, basal ganglia, caudate nucleus

Characterisitic feature of Huntington's Disease

Chorea

Treatment for Huntington's Disease

No real cure

Can treat with Dopamime antagonists --> TETRABENZINE**

What can make Huntington's Disease worse?

Levodopa and other agonists make chorea worse

SE of Tetrabenzine

Secondary parkininsim

Too much copper

Wilson's Dz

Intial feature of Wilson's Dz in children

Liver disease

Kayser-Fleisher rings

Wilson's Dz

Gold standard dx of Wilson's Dz

Liver biopsy with quantitative copper assays

Treatment for Wilson's Dz

Mainstay = Chelating agents = Trientine (Syprine)

Zinc

Wet, wacky, wobbly

Normal pressure hydrocephalus

Abnormal gait

urinary incontience

dementia

Normal pressure hydrocephalus

In contrast to AD, Normal pressure hydrocephalus c/o ____ early on

gait distrubace

CT or MRI showing enlarged lateral ventricles with little or no cortical atrophy

Normal pressure hydrocephalus

MRI for Normal pressure hydrocephalus

enlarged lateral ventricles

Normal pressure hydrocephalus - LP?

Improves sxs but CSF profile is normal

Normal pressure hydrocephalus - tx

VP shunt

Serial LPs

Normal pressure hydrocephalus - VP shunting imporves ___ more than ____

gait

cognition