PTA Neuro - ALS, Alz, GBS, CMT, PPS Flashcards
1
Q
what is the Incidence of Alzheimer’s Disease?
A
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
2
Q
what is Alzheimer’s Disease?
A
Senile Dementia
3
Q
what is Dementia?
A
Dementia = loss of cognitive functioning (thinking, memory, reasoning) and behavioral abilities ( ADLs)
4
Q
what causes Alzheimer’s?
A
- unknown etiology
- appears to include genetic and environmental factors
- three genes have been found to cause familial early- onset Alzheimer’s
5
Q
what are some risk factors for Alzheimer’s?
A
- 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
6
Q
what is the pathophysiology?
A
- 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
7
Q
how does one diagnose Alzheimer’s Disease?
A
- 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
8
Q
what are signs and symptoms of Alzheimer’s?
A
- 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
9
Q
what are complications of Alzheimer’s?
A
- 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
10
Q
what is the Physical Therapy intervention to Alzheimer’s?
A
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
11
Q
What is ALS?
A
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
12
Q
what is the incidence of ALS?
A
- One of most common neuromuscular diseases worldwide
- Onset ages 40 – 60; more men than women
13
Q
what is the etiology of ALS?
A
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
14
Q
what is the pathophysiolgy of ALS?
A
- 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
15
Q
how does one diagnose ALS?
A
!!!! 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
16
Q
what are signs and symptoms of ALS?
A
- 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
17
Q
goals of PT for ALS:
A
- 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
18
Q
interventions of PT for ALS:
A
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.
19
Q
what is peripheral neuropathy?
A
Damage to the peripheral nervous system—to the motor, sensory, and/or autonomic nerves
Like static on telephone line–distorts and sometimes interrupts messages between the brain and rest of the body
Chronic forms begin subtly, progress slowly. Symptoms may stay same for months or years. Some worsen over time.
Damage to a single nerve (mononeuropathy) or to a nerve group (polyneuropathy)
20
Q
what are the symptoms of Peripheral Neuropathy?
A
Often appear symmetrically—i.e. in both feet with gradual progression up both legs; next fingers, hands, and arms.
Wide array of symptoms:
- Paresthesia - numbness, tingling, prickling
- Sensitivity to touch
- Loss of proprioception
- Muscle weakness, wasting, or paralysis
- Muscular cramps or fasciculations
- Burning pain - often worse at night
- Loss of pain and temperature sensations
- Organ or gland dysfunction
- Inability to digest food easily, maintain safe levels of blood pressure, or sweat normally
- Loss of bowel, bladder or sexual function
21
Q
what causes Peripheral Neuropathy?
A
- can be acquired
- can be inherited - symptoms usually begin in early adulthood
22
Q
what is the most common inherited Peripheral Neuropathy?
A
Charcot-Marie-Tooth
- Group of disorders with extreme weakening and wasting of muscles in the lower legs and feet, gait abnormalities, loss of tendon reflexes and numbness in lower limbs
- Affects approximately estimated 150,000 or 1 in 2,500 people in the United States
- Also known as Hereditary Motor and Sensory Neuropathy (HMSN) or Peroneal Muscular Atrophy
23
Q
what causes CMT?
A
- Mutations in genes that produce the proteins involved in the structure and function of either the peripheral nerve axon or the myelin sheath.
- Gene mutations in CMT disease are usually inherited
- At least 40 specific gene defects have been identified
- No known cure
- !! Causes either demyelination of axons or destruction of the axon itself !!
24
Q
what are symptoms of CMT?
A
- Weakness and atrophy of the lower leg and foot muscles (due to Peroneal Nerve being affected)
- Foot drop and a steppage gait with frequent tripping or falls
- Foot deformities, such as pes cavus and hammertoes
- Decreased sensation in LE
- Balance issues
- Later in the disease, weakness and muscle atrophy may occur in the hands
- Onset of symptoms - most often in adolescence or early adulthood, gradual progression
- May have mild to severe pain, but atypical
25
what is the treatment for CMT?
- PT, OT, orthotics, surgery, pain medications
- Physical Therapy
* Stretching to prevent tightening
* Strengthening
* Endurance training, (low impact or no-impact to avoid stress on fragile muscles and joints)
* Pain management
* Often need AFOs, custom shoes
- Early treatment beneficial to delay or reduce muscle atrophy
26
what are some causes of Acquired Peripheral Neuropathy?
- Physical injury (trauma)—most common cause
- Diabetes mellitus—most common cause of polyneuropathy
- Infections (viruses and bacteria) and autoimmune disorders
- Systemic diseases (there's a big list)
27
how does the doctor diagnose an acquired Peripheral Neuropathy?
- CT scan (Computed tomography)- can identify spinal stenosis, herniated disk, tumors, vascular problems,…
- MRI (Magnetic resonance imaging)-examine muscle quality or nerve fiber compression damage.
- EMG (Electromyography)-identify muscle vs nerve damage. (needle in the muscle thing)
- NCS (Nerve conduction studies)-degree of damage in nerve fibers. (test to see how face electrical signals move through the nerve)
- Nerve or skin biopsy to examine nerve fiber damage.
28
what is the Medical Treatment of Aquired Peripheral Neuropathy?
- First treat underlying condition, then symptoms
- Strict control of blood glucose levels
- Immunosuppressive drugs
- Plasmapheresis (remove blood and cleanse of immune system cells and antibodies, then return to body)
- Immunoglobulin therapy—high does of proteins that function as antibodies
- Analgesics over the counter for pain.
- Some prescription meds
- Common ones: gabapentin (neurontin), lyrica, various antidepressants.
- Surgery—relief from mononeuropathies caused by compression or entrapment injuries (repair of herniated disk, removal of tumor, release of ligaments or tendons compressing nerves)
- Quit smoking-constriction of blood vessels that supply nutrients to nerves can worsen symptoms.
29
why don't we have a list of signs and symptoms for Acquire Peripheral Neuropathy?
because it depends on the affected nerve
30
what are the recommended PT interventions for Acquired Peripheral Neuropathy?
- control the edema! massage, ice, wraps, elevation - all those
- educate on skin checks!! be so careful with the skin.
- very often recommend NO LEG SHAVING
- obviously exercise - strengthen, keep range, prevent cramps
- balance training is real important - gotta work it
- bracing - but not KAFO. just AFO
- bracing to alleviate nerve compression - usually OT do these for the UE
- any assistive device that you feel is needed for safety
- lots of education - around the house to prevent falls - rugs, toys, lights
- and always have protective shoes/slippers on!!
31
what is Guillain Barre Syndrome?
- Acute Inflammatory Demyelinating Polyneuropathy (AIDP)
- Autoimmune disease - system attacks peripheral nerves of body
- Rare—incidence of 1 or 2 per 100,000
- Develops over course of hours or days, or may take up to 3-4 weeks
32
what are the signs and symptoms of GBS?
- Sudden onset - First symptoms include symmetrical weakness or paralysis , sometimes numbness and tingling , affecting legs first and progressing upwards to the trunk, arms, breathing, and facial muscles
- Sensory loss, usually proprioception
- Areflexia
- Pain is common; presents as deep aching pain in the weakened muscles (likened to that from over- exercising).
- May be sensitive to touch
33
what causes GBS?
- Immune response to foreign antigens that are mistargeted at host nerve tissues.
- Usually triggered by an acute infectious process (like the flu) — after symptoms of respiratory or GI viral infection
- Occasionally following surgery or immunizations.
- End result of this attack is damage to the myelin leading to muscle paralysis
- May or may not have sensory and autonomic impairment.
- 60% of cases are idiopathic
- In severe cases, axonal damage also occurs
80% have myelin loss; 20% have axon loss also
34
how is GBS diagnosed?
- Depends on findings such as rapid development of muscle paralysis, areflexia, absence of fever and a “likely inciting event”
- CSF analysis—from lumbar puncture—shows increased protein levels, but normal WBC count.
- EMG and NCV show slowing or blockage of conduction
Ruling out other possible causes
35
what is the prognosis for GBS?
- By the 3rd week of the illness, 90% of patients are at their weakest.
- Recovery usually starts after the 4th week.
- ~80% of patients recover within a few months to a year with some residual deficit. (ankle muscles first to go and last to come back, if at all)
- ~5-10% of patients have one or more late relapses and are then classified as having Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
36
what is the relationship between Acute Inflammatory Demyelinating Polyneuropathy and Chronic Inflammatory Demyelinating Polyneuropathy?
Acute is initial onset of GBS
| Chronic is the relapse of GBS (in later years)
37
what is the medical treatment for GBS?
- Need to keep body functioning during recovery—may be put on ventilator
- High dose intravenous immunoglobulins to attack invading organisms OR
- Plasmapheresis—to remove antibodies.
38
what is the Physical Therapy intervention in Acute Care for GBS?
- keep body in shape - maintain ROM
- proper positioning - tactfully educate the nurses
- breathing and coughing exercises
- educate patient about disease course
- move upright (when stable)
- IMPORTANT - do NOT overwork - do NOT cause FATIGUE in the muscles
39
what is the PT intervention for Inpatient and Outpatient Rehab for GBS?
- AROM exercises
- Strengthening exercises, progressing gradually
* Increase reps before intensity
* AVOID FATIGUING PATIENT!
- Pool is often good in early period when still very weak
- Instruction in functional mobility and activities
- Gait training on all surfaces.
- Recommend assistive and orthotic devices as necessary.
- Aerobic training at moderate RPE when okayed by MD
40
what is Polio?
- A contagious disease caused by the polio virus, which enters thru the GI system.
- Destroys anterior horn cells, leading to flaccid paralysis, often worse on one side of body.
- Causes areflexia, muscle aches, and atrophy.
- Last big outbreak in USA was in late ‘40s/early ‘50s - eradicated 1979
41
what is Post Polio Syndrome?
- Affects polio survivors 25 to 40 years after recovery from the disease
- Gradual new weakening in muscles that were previously affected; joint pain
- Incidence and prevalence unknown
- Estimates that it affects 25 %– 50% of survivors
NOT contagious (polio is contagious)
42
what are the signs and symptoms of PPS?
- Slowly progressive muscle weakness
- Fatigue (muscular and generalized)
- Muscle atrophy
- Pain
- May have breathing or swallowing problems
- Severity of symptoms appear to correlate with severity of original illness
43
what are the risk factors for PPS?
- Increased risk if contracted polio as a teen or adult vs in childhood.
- Increased risk with greater recovery from illness.
- Increased risk if more physically active over the years.
44
what is the the etiology of PPS?
- Unknown
- Theory: During initial recovery, surviving motor neurons sprout connections to muscles.
- Years of overuse stresses the neuron→ degeneration of sprouted axon → degeneration of cell body → weakness, fatigue, atrophy
- Degeneration of individual functioning motor units
- Progresses slowly with periods of relative stability followed by decline
45
what are the PT interventions for PPS?
- Carefully prescribed and monitored exercise
* More likely to benefit those muscles unaffected by original illness
* Sub-maximal exercise program; avoid fatigue
* Discontinue if additional weakness, excessive fatigue, or prolonged recovery time
- Cardiopulmonary endurance training usually more beneficial than strengthening
- Swimming or water aerobics are good.
Instruction in energy conservation, lifestyle changes, use of mobility aids
- Stay warm, as cold increases muscle fatigue.
