PTA Neuro - ALS, Alz, GBS, CMT, PPS Flashcards
what is the Incidence of Alzheimer’s Disease?
Approximately 5.2 million Americans
By 2050, estimate as many as 16 million
2/3 women
Most common cause of dementia in elderly
Onset usually after age 60
Most survive 4 – 8 yrs after onset, some survive up to 20 yrs
6th leading cause of death in US; 5th leading cause of death in 65 and older
what is Alzheimer’s Disease?
Senile Dementia
what is Dementia?
Dementia = loss of cognitive functioning (thinking, memory, reasoning) and behavioral abilities ( ADLs)
what causes Alzheimer’s?
- unknown etiology
- appears to include genetic and environmental factors
- three genes have been found to cause familial early- onset Alzheimer’s
what are some risk factors for Alzheimer’s?
- Age - Likelihood doubles every 5 yrs after age 65
- Family history- Parent, sibling
- Female
- Longstanding HTN, heart disease, stroke, diabetes
- History of head trauma, especially repeated trauma with loss of consciousness
- Down Syndrome-many develop early-onset Alzheimer’s (brain changes seen by age 40 in most)
- Very low vitamin D levels
what is the pathophysiology?
- Damage begins 10-20 yrs before symptoms.
- Abnormal processing of a normal cellular protein, beta amyloid → deposits form “amyloid plaques” in the neurons
- Tau proteins form “neurofibrillary tangles” in the neurons
- Neurons die → loss of synapses
- Genetic mutations in chromosomes 21, 14, and 1 have been seen in familial early-onset Alzheimer’s Disease
how does one diagnose Alzheimer’s Disease?
- History, physical exam, neurological exam, mental status exam over time
- Tests to rule out other causes-CT or MRI
- Bio-markers: increased levels of tau or decreased levels of beta-amyloid in CSF, decreased glucose uptake on PET scan, atrophy in certain areas of MRI.
- Only conclusive diagnosis is via autopsy – evidence of plaques in brain
what are signs and symptoms of Alzheimer’s?
- Forgetfulness is usually 1st symptom
- Thinking and planning is affected next
- Difficulty with language, memory, perception, emotional behavior or personality, cognitive skills
- Misplacing items, getting lost on familiar route, flat mood…. early symptoms of AD
- Forgetting events, changes in sleep patterns, difficulty reading or writing, hallucinations, violent behavior, agitation…later stages of AD.
- Problems understanding language, recognizing family, performing ADLs….severe AD
what are complications of Alzheimer’s?
- Contractures, skin breakdown, infection (especially UTI and pneumonia)
- Falls and broken bones
- Malnutrition and dehydration
- Failure of body systems
- Sundowning (confusion, restlessness, agitation & wandering in late afternoon)
- Harmful or violent behavior toward self or others
- Abuse by over-stressed caregiver
what is the Physical Therapy intervention to Alzheimer’s?
duh.
Frequently treating a secondary complication ( fall, fracture, gait abnormality)
Flexibility, balance and strengthening exercises
Maintain function and prevent falls
Cardiovascular fitness and endurance
Instruct family in managing patient
What is ALS?
Amyotrophic Lateral Sclerosis
- Also known as Lou Gehrig’s Disease
- Rapidly progressing, always fatal, motor neuron disease
- Eventually all voluntary muscles are affected
- Most people die within 2 – 5 years, usually from respiratory failure; approx 10% of patients live for 10 or more years
what is the incidence of ALS?
- One of most common neuromuscular diseases worldwide
- Onset ages 40 – 60; more men than women
what is the etiology of ALS?
Etiology unknown
- Theories:
- Gene mutation leading to accumulation of free radicals > motor neuron degeneration
- ALS patients have higher levels of glutamate in serum and spinal fluid; studies to determine what causes imbalance
- Autoimmune responses
- Environmental factors – toxic or infectious agents
what is the pathophysiolgy of ALS?
- Progressive degeneration and death of upper and lower (UPPER AND LOWER) motor neurons which control voluntary muscles.
- Affects cortex, brain stem cranial nerve nuclei, and anterior horn cells in spinal cord.
- Ability of brain to control movement is gradually lost.
- Muscles weaken, atrophy, and exhibit fasciculations
- Sensation is often spared, cognition spared
how does one diagnose ALS?
!!!! To be diagnosed with ALS, must have S/S of both UMN and LMN damage !!!
checks over time genetic testing confirm diagnosis with UMN and LMN damage rule out other stuff (list on slide) no one test that says THIS IS ALS
Rule out other diagnoses
- NCV – rule out peripheral neuropathy or myopathy
- MRI – rule out tumors, herniated disk, spondylosis
- Blood and urine testing – rule out infection, others
what are signs and symptoms of ALS?
- Subtle symptoms can start anywhere in the body (twitching, cramping, swallowing difficulty, slurred speech…)
- Asymmetrical muscle weakness progressing to paralysis
“Clumsy” gait, impaired fine motor movements, abnormal reflexes, atrophy (LMN), spasticity (UMN) - Muscle cramps and fasciculations
- Cervical extensor weakness is common
- Breathing difficulties and dysphagia
- Dysarthria and voice changes
- Weight loss
Does not affect senses ( sight, smell, hearing), rarely affects bowel and bladder function, or cognitive status
goals of PT for ALS:
- working with what’s left to try to make life better as they go along.
- knowing that we won’t stop progression, but we want them do what they can do.
the typical goals
interventions of PT for ALS:
in addition to the typical interventions:
- Improve patient’s independence and reduce fatigue - orthotics, assistive devices, W/C
- Avoid symptoms of overuse, ie. exhaustion, pain, increased fasciculations or cramping
- Instruct patients and families – HEP, progression
- Cervical collars and high-back wheelchair for cervical extensor weakness.
- Support for weak UEs; splints for wrist/hand.
- Instruct in energy conservation.
- Assist coughing
- Breathing exercises and positioning to optimize ventilation/perfusion ratios.
- Airway clearance techniques if needed.