Neurodegenerative Diseases

Front 1

What is neurodegenerative disease?

Back 1

Disease affecting neurons in the brain

Progressive degeneration / death of nerve cells

Alzheimer’s is most common

Parkinson’s disease is the second most common

Front 2

Symptoms of neurodegenerative diseases?

Back 2

Ataxia

Dystonia (involuntary muscle contractions causing twisting movements)

Bulbar symptoms

Dementia

Front 3

Some neurodegenerative disease examples?

Back 3

Huntington’s

Prion disease

Spinocerebellar Ataxia

Front 4

Commonalities between neurodegenerative diseases?

Back 4

Oxidative stress

Mitochondrial dysfunction

Irregular protein folding

Genetic mutations

Repeat of CAG nucleotide triplet

Front 5

Epidemiology of MS?

Back 5

Onset between: 15 and 50

Affects 400,000 people in the U.S.

more common in women 2-3:1 ratio

Mainly white populations are affected

Front 6

High, Medium, and Low frequency areas?

Back 6

High: northern US, Northern Europe, southern Canada, New Zealand

Medium: closer to the equator: southern US, Europe, and Australia

Low: in tropical areas: Africa, Asia, South America,

Front 7

Etiology of MS?

Back 7

Not exactly known

Autoimmune disease caused by a viral or infectious agent.

20% of those affected have positive family history

May inherit genetic susceptibility for abnormal T cell role

Front 8

Disproved theories for MS?

Back 8

Heavy metal exposure

Allergies

Owning pets

Aspartame

Physical trauma

Front 9

Definition of MS?

Back 9

Chronic inflammatory, demyelination of the CNS

Front 10

In MS the immune response triggers:

Back 10

T lymphocytes

Macrophages

Immunoglobulins (antibodies)

Front 11

Pathophysiology of MS?

Back 11

Antigen is activates

Produces autoimmune cytotoxic effects within the CNS

Blood brain barrier fails

T lymphocytes enter and attack the myelin sheath that surrounds the nerve and all gray white matter and axons are affected

Front 12

sensory symptoms of MS?

Back 12

•hypoesthesia,numbness, paresthesia

Front 13

pain symptoms of MS?

Back 13

•dyesthesias (uncomfortable sensations), optic or trigeminal neuralgia, chronicpain, Lhermitte’s sign

Front 14

Visual symptoms of MS?

Back 14

•blurredor double vision, diminished /loss of vision, scotoma, nystagmus

Front 15

Cognitive symptoms of MS?

Back 15

•memory/recallproblems, decreased attention/concentration, problem solving, judgement,visual-spatial abilities, speed of information processing

Front 16

Motor symptoms of MS?

Back 16

•weakness/paralysis,fatigue, spasticity, incoordination, intention tremor, impaired balance, gaitdisturbance

Front 17

Speech and swallowing symptoms of MS?

Back 17

dysarthria,diminished verbal fluency, dysphagia, dysphonia

Front 18

emotional symptoms of MS?

Back 18

•Depression,pseudobulbar affect, anxiety

Front 19

bladder symptoms of MS?

Back 19

•incontinence,urgency

Front 20

bowel symptoms of MS?

Back 20

•diarrhea,constipation, incontinence

Front 21

other symptom of MS?

Back 21

•CARDIOVASCULARDYSAUTONOMIA

Front 22

Define exacerbation of MS?

Back 22

Alsocalled relapse : new and recurrent MS symptoms that last 24 hours , unrelatedto another etiology

Front 23

Factors that impact likelihood of exacerbation?

Back 23

•Healthyindividuals are more likely to relapse than overall healthy individuals

•Viralor bacterial infections (cold, flu, UTI, sinus infection)•diseasesof major organ systems (pancreatitis, hepatitis, asthma attack)

•majorand minor stress (divorce, death, trauma, dehydration, sleep depravation)

Front 24

Define Pseudoexacerbation of MS?

Back 24

•Atemporary worsening of MS symptoms

•Resolveswithin 24 hours

•heat

•humidity

•Anythingthat raises body temperature

Front 25

What are the categories of MS?

Back 25

•BenignMS - 20%

•Relapsing-RemittingMS (RRMS) – 65-85%

•Relapsing-RemittingProgressive

•PrimaryProgressive MS (PPMS) - 10%-15%

Front 26

Typical progression of MS?

Back 26

•Beginsaround age 40

•After10 years of disease:

•10%wheelchair

•50%unable to work

•Mediantime disease onset to death is 30 years

Front 27

Steps to diagnosing MS?

Back 27

•Presence of Clinicalsymptoms?

•MRI

•CSFanalysis

1.) 2 or more relapses & 2 or morelesions

2.) 1 relapse, 1 lesion &+csf& either 2+lesions on MRI or a 2ndrelapse.

3.)neurologic progression, +csf& 9 MRI lesions w 2 in spinal cord or 4-8 in brain with 1 in spinal cord

Front 28

Medical Management Options for MS?

Back 28

•Nosurgery

•Diseasemodifying drugs

•Symptommodifying drugs

•Lowimpact exercise with gradual increase in intensity, duration and frequency

•Cognitiverehab

•Lifestylechanges

•OT/PT

Front 29

When should someone with MS be referred to rehab?

Back 29

•referralto rehabilitation when there is “anabrupt or gradual worsening in function that has a significant impact onmobility, safety, independence or quality of life.”

Front 30

•Forpeople with MS, the prevalence of falls is estimated to be between ____% (Finlayson, et al , 2006)

Back 30

52-55%

Front 31

What is a major problem in MS and how should PT cater to this?

Back 31

Fatigue

•PTshould focus on energy conservation, activity pacing , patient education tomaximize outcomes

Front 32

Definition of Parkinson's Disease?

Back 32

deathof dopaminergic neurons in the substantia nigra

Front 33

Facts regarding the substantia nigra?

Back 33

•Inbasal ganglia

•Producesdopamine

•Decreaseddopamine=decreased speed, movement quality, postural stability, cognition,affect

Front 34

What is Lewy Body formation in the substantia nigra?

Back 34

Abnormal protein folding

Front 35

What are the Two Parkinson's Disease Variants?

Back 35

Tremor Dominant

Non-Tremor Dominant (rigid and postural instability)

Front 36

Prevalence of Parkinson's?

Back 36

•Prevalence1% above age 60

•2%over age 80

•HealthCare Priority *

•Moreprevalent in Europe, N. America, S. America than Africa, Asia, Arabic countries

Front 37

Phases of Parkinson's Disease?

Back 37

•Preclinical:40 ys

•Prodromal: depression anxiety, constipation. REM behavior disorder, fatigue

•Symptomatic:this is then divided into STAGES

Front 38

Stages of Parkinson's Disease?

Back 38

•I: One- sided

•II:bilateral and balance not impaired

•III:bilateral and balance impaired

•IV:functionally disabling

•V:wheelchair or bed bound

Front 39

Clinical signs and symptoms of Parkinson's Disease?

Back 39

Progressivedecline in speed, flexibility, fluidity, coordination (find and gross) ofextremities, trunk, face, voice

•Slow,insidious

•Bradykinesia

•Rigidextremities

•“Pill-rolling” tremors in the fingers aggravated bystress, fatigue, anxiety

Front 40

Tests to diagnose PD?

Back 40

•Neurologicalexam

•Bloodtests, MRI

Front 41

Non-motor symptoms of Parkinson's?

Back 41

a) Dementiaand behavioral or emotional disturbances

b) Autonomicnervous system dysfunction

c) Abnormalitiesof vision and eye movements

Front 42

OT Treatment Techniques for PD?

Back 42

•Exerciseand Physical Activity (Boxing)

•Environmentalcues, stimuli and objects

•Selfmanagement

•Cognitivebehavioral strategies

Front 43

Use of Environmental Cues in treating PD?

Back 43

•Auditory,rhythmic cues stronger than visual, tactile or other to help regulate walking.

•“One-Off”cueing: Laser

•Rhymic:set pace; metronome (in ear)

Front 44

What is ALS?

Back 44

•Disorder affecting both upper andlower motor neurons

•Sparing sensation

•Some cognitive impairment due toprefrontal neurons

Front 45

What is ALS also called?

Back 45

Lou Gherig's disease

(Famous baseball player)

Front 46

Definition of ALS?

Back 46

DegenerativeCNS disorder, with loss of upper and lower motor neurons, resulting inprogressive weakness and death from respiratory muscle failure

Front 47

Epidemiology of ALS?

Back 47

•Incidence•2-3/100,000

•~5,500new cases/year in USA

•Prevalence:3.9/100,000

•Adultillness•Meanage onset: 55-65 years

•Male:femaleratio 1.7:1 •1:1after age 65

Front 48

Rates of fatality seen in ALS?

Back 48

•Mean from onset of symptoms to death orventilator dependence is 2-5 years

•50% die within 5 years

•19-39% survive 5 years

•8-22% survive 10 years

Front 49

Two classifications for ALS?

Back 49

•90-95%sporadic

•5-10%familial

Front 50

Relationship between ALS and FTD?

Back 50

•ALS and FTD can be distinct entities

•Can be overlapping

•Associated with shorter disease duration

•Prefrontal cognitive abnormalities in upto half of all patients with ALS

Front 51

Clinical features of ALS?

Back 51

•Muscleweakness

•Focalonset (asymmetrical)

•Gradualprogression

•UMNand LMN signs

•Nopain or sensory symptoms

Front 52

UMN signs for ALS?

Back 52

•Weakness

•Strengthok

•Musclebulk preserved

•Slow,imprecise

•Spasticity

•Limbstiffness

•Increasedreflexes

Front 53

LMN signs of ALS?

Back 53

•Weakness

•Severeweakness

•Lossof muscle tissue

•Atrophy

•Fasciculations

•Decreasedreflexes

Front 54

Bulbar symptoms seen in ALS?

Back 54

Bulbarsymptoms

Dysarthria: slurred speech

Dysphagia: impaired swallowing

Front 55

Onset of muscle weakness seen in ALS patients?

Back 55

Limb 70%

Bulbar 25%

Respiratory <5%

Front 56

____ muscle involvement develops in most ALS patients and ____ failure is theusual cause of death

Back 56

Respiratory

Respiratory

Front 57

Indication of definite ALS