exam 3 nero generative disease

Amyotrophic Lateral Sclerosis (ALS)

•Progressive

•Terminal

•Demyelination (exposed nerves—-can become damaged)

Amyotrophic Lateral Sclerosis (epidemiology)

avergae age: 58-60

Men 1.5 to 2 times more frequent

when is Amyotrophic Lateral Sclerosis most commly diagnosed 

early 30s 

symptoms of Amyotrophic Lateral Sclerosis

•Fatigue or loss of exercise tolerance

•Fasciculations

•Muscle cramping

•Weakness

•Atrophy

Bulbar Symptoms

Involve activities of the oropharyngeal muscles

• dysarthria

• dysphagia

• facial, jaw

• tongue weakness

• tongue atrophy

Frontotemporal Dementia

•Behavioral and speech disruptions

•Decreased behavior, language, and movement

•Memory largely intact

•Decreased executive functioning

Up to 50% of individuals with ALS have ______

Frontotemporal Dementia

what is dignosed by exclusion

neuro degentive diseases

ALS

•Progression will be consistent with the patient

•Median survival after diagnosis is 4 years, some will live 10-15

•Life span lengthened if ventilator use and nutritional support are chosen

•Severity at onset associated with length of life

ALS useually experinces ______

muscle spasms 

Muscle Weakness and Atrophy

•Distal losses first

•Proximal losses next

•Bulbar losses

•Flexors more than extensors

Bulbar losses

•Speech, swallow, breathing

Dysarthria (ALS)

•Volume issues

•Tongue fasciculations

•Slurred speech

Dysphagia (ALS)

•Due to weakness in oral and pharyngeal musculature

•Bringing food to mouth

•Aspiration

•Saliva management

Respiratory (ALS)

•Progressive respiratory weakening

•Decreased diaphragm and intercostal strength

•Decreased cough

•Aspiration

Stage 1 (Phase 1 Independent (ALS))

Mild weakness, clumsiness, ambulatory, I in ADL

Stage 2 (Phase 1 Independent (ALS))

Moderate selective weakness, Slight ADL difficulty, ambulatory

Stage 3 (Phase 1 Independent (ALS))

Severe weakness in ankles, wrists, & hands, Mod A ADL, Easy fatigue, ambulatory, respiratory effort

Stage 4 (Phase 2- Partially Independent)

•Hanging arm syndrome

•W/C Use

•Severe lower extremity weakness

•Can perform some ADL with fatigue

Stage 5 (Phase 2- Partially Independent)

•Severe LE Weakness

•Moderate to severe UE weakness

•W/C use

•Skin breakdown

•Max A ADL

Phase 3- Dependent

•Bedbound

•Dependent in ADL

Huntington’s Disease

•A degenerative disease of the brain

•Inherited autosomal dominant disease

•Repeats of CAG (cytosine, adenine, guanine) sequence on chromosome 4

• Normal 10- 28

• Abnormal 36-120

•Usually expresses after age 30

Huntington protein is ______

very long

• Forms “clumps” in the brain

Huntington’s Disease (personality change)

First symptoms

• Depression

•Hostility

•Feeling incompetent

Huntington’s Disease (change in movment patterns)

•Excessive chorea movements (unwanted movmnets when talking/ doing activites))

•Eventual akinesia and rigidity

Huntington’s Disease (cogitive changes)

•Decreased memory

•Decreased IQ

•Language difficulties

Huntington’s Disease (skill loss)

•Difficulty with executive functions

•Balance

•Functional Mobility- Gait disturbance

•Coordination losses

•Swallowing and respiratory difficulties

•Memory deficits

Parkinson’s Disease (cause)

Genetic and environmental factors

Exposure to

• Toxins

• TBI

•10-15% Genetic

Parkinson’s Disease (epidemiology) 

more common in: 

• Men

• Older adults

Lewey Body Dementia

•Often co-occurs with Parkinson’s disease

Lewey Body Dementia (symptoms)

•Changes in thinking and reasoning

•Slowness, gait imbalance

•Well formed visual hallucinations

•Trouble interpreting visual information

•Sleep disturbance

•Memory loss

Lewey Body Dementia (systemic symptoms) 

•Excessive sweating

•Constipation

•Early Satiety

•Dandruff

•Fatigue

•Urinary frequency, urgency, & incontinence

•Hypotension

Lewey Body Dementia (sensory symptoms) 

•Pain

•Loss of taste or smell

•Hallucinations

•Vision Problems

LSVT BIG and LOUD

Technique to address the movement disorder

• Emphasis on enlarging movement

• Emphasis on increasing vocal volume

• Extra effort

• Research supported

• Specialty Certification