Immune-Mediated Disorders: LMN And Peripheral Nerve Flashcards
What are autoimmune disorders affecting the muscular system?
Myopathies
T/f: Attack of muscle fibers can be either inherited or acquired
True
What are the inherited myopathies?
Congenital
Mitochondrial
Metabolic
Muscular dystrophy (Duchene’s and Becker’s)
What are the acquired myopathies?
Autoimmune/inflammatory myopathy
Toxic
Endocrine (thyroid, parathyroid, or adrenal)
Infections (Lyme, mono)
Electrolyte
Critical illness myopathy
Medication induced
What is mitochondrial disease myopathy?
A myopathy effecting more than just muscles, but also the heart and brain causing greater risk
What are the common s/s of myopathy?
Symmetrical proximal muscle weakness
Muscle cramps, stiffness, and spasms AT REST
Fatigue with exertion
Lack of energy
Hands and feet are spared
What are the variable s/s of myopathy?
Non-progressive or very slowly progressive
In some, muscle weakness is intermittent with periods of normal strength
In children, slow development of motor skills
Trouble with oral motor muscles for swallowing and speech
How do we diagnose and differentiate types of myopathy?
Lab values
Electromyography
MRI of muscle
Genetic testing
Muscle biopsy
What labs help us diagnose myopathy?
Muscle enzymes such as creatinine kinase (CK)
Electrolyte levels
Autoimmune disease testing
Endocrine testing
Why may CK be elevated in myopathy?
Bc of the increased muscle breakdown
T/f: electrolytes are generally off with myopathies
True
What would electromyography show with myopathies?
Normal nerve conduction and impaired EMG
What is the cardinal confirming test for myopathy?
Muscle biopsy
What should be included in the examination of myopathy.
Body chart to document location and nature of symptoms
Rating symptoms using VAS
Pattern of symptoms (changes due to weather, or time of day)
Sleep disturbance documented if the symptoms wake them
When do pts with myopathy tend to have an exacerbation of symptoms?
At the end of the day
What is a key symptom of myopathies?
Symptoms that awaken them
What is an important factor to consider in interventions with myopathies?
To avoid increasing symptoms
If there is prolonged or residual loss of strength with a myopathy following our interventions, what should we do?
Report it to the physician
If muscle pain or cramps become very uncomfortable during exercise for pts with myopathy, what should we do?
Rest until symptoms subside
If there is progressive muscle weakness or pain for pts with myopathy during a treatment program, what should we do?
Discontinue the exercise program and consult with the physician
Why are pts with myopathy at risk for rhabdomyelitis?
Bc the increased muscle breakdown effects the heart
What meds put pts at increased risk for rhabdo?
Statins
If a pt with myopathy had pepsi colored pee, what should we be concerned about?
Rhabdo
What are the problems we should work on with myopathy?
Exercise avoidance
Reduced aerobic fitness and muscle strength
Impaired ROM
T/f: we can use physical agents and electrotherapeutic modalities to reduce frequency and intensity of exercise related muscle pain in myopathy?
False, physical agents and electrotherapeutic modalities DO NOT WORK
How can we reduce frequency and intensity of exercise related muscle pain in myopathy?
Pt education on exercise modifications
What can we do to improve fxnal capacity for pts with myopathy?
Aerobic and resistance exercise therapy
T/f: exercise therapy is effective for mitochondrial disease myopathy
True
Why does aerobic exercise help with mitochondrial disease myopathy?
Bc it can increase mitochondrial production and increase muscle strength
What can we do to improve ROM in myopathy?
Stretching exercises
What are the autoimmune disorders affecting the neuromuscular junction?
Myasthenia gravis, ion channel disorders
What is myasthenia gravis
An autoimmune disease where ach and autoantibodies compete for sites on ach receptors causing muscle weakness throughout the day
Muscle weakness with fatiguability
What are the ocular symptoms in MG?
Ptosis (one of the earliest signs)
Diploplia (esp at the end of the day)
What are the bulbar symptoms of MG?
Dysarthria
Dysphasia
Jaw fatigue with chewing
Dyspnea
What are the facial/neck symptoms of MG?
Loss of expression
Dropped head due to neck muscle fatigue
T/f: forward head posture in a pt with MG puts them at risk of dropping head by the end of the day due to muscle fatigue of the neck
True
What are the extremity symptoms of MG?
Proximal muscle weakness>distal muscle weakness
Pain that improves with rest and worsens with illness, stress, heat/cold, meds, fatigue, and overexertion
T/f: muscle fatigue increases throughout the day with MG
True
What are two big risks with MG?
Neurogenic fatigue
Myasthenic crisis
What are the respiratory symptoms of MG?
Weakness of muscle of respiration leading to respiratory insufficiency
What is myasthenic crisis?
Weakness and Dyspnea after exercise that doesn’t return to baseline within 5 minutes
When do we want to schedule pts with MG?
On days or parts of the day when they are not busy or exerting themselves too much
Why is MG called the snowflake disease?
Bc no two snowflakes are the same and no two pts with MG are the same
What are the differential diagnoses with MG?
GBS, ALS, MS
How do we diagnose MG?
Long (repetitive stimulation) EMG
antibody testing
Ptosis test
What is a long EMG and what does it tell us about MG?
Repetitive muscle contraction created by the EMG that shows a decreased amplitude and doesn’t come back to baseline with rest in a pt with MG
What does an antibody test tell us about in MG?
If there are AchR antibodies it is seropositive for MG
If there are no antibodies it is zero negative for MG
What are the two Ptosis tests we can do?
Edrophonium chloride test
Ice on eyes test
What does the edrophonium chloride test for Ptosis do?
Temporarily increases Ach to relieve ptosis
What does the ice in eyes test for ptosis do?
When ice is put on the eyes for 2 minutes , it temporarily reduces ptosis
T/f: there is no cure for MG
True
T/f: MG progresses significantly in the first couple years
True
T/f: treatment of MG focuses on curing the disease
False, it focuses on reliving s/s
What meds are used to treat MG?
Anticholinesterases
Corticosteroids
Immunosuppressants
What needs to be included in the MG PT eval?
Screen and assess prn for mental fxns
Screen depression and anxiety
Screen sensory fxns and pain
Screen voice and speech fxns
Monitor VS and do orthostatic assessment
RPE with 6MWT/2MWT
respiratory assessment
MMT
Balance assessment with Berg or MiniBEST test
Skin screening
What are the disease specific measures for MG?
MG composite score
MG-ADL profile
MG-QOL15
What is the MDC and MCID for the MG Composite score?
3
T/f: we use the MG Composite score a lot
False
A score of >____ in the MG composite score indicates critically significant MG
7
Is a higher score on the MG composite score better or worse?
Worse
What disease specific measure do we use with all pts with MG?
The MG-ADL profile
Is a higher score on the MG-ADL more or less severe?
More severe
A score below ___ on an item of the MG ADL profile indicates minimal effects of the disease on the ADL
2
A score of _____ or less on the MG-QOL15 indicates minimal decrease in QOL from the disease, although this number is not completely agreed upon
7
What two MG disease specific measures are usually paired together?
The MG-ADL profile and MG-QOL15
During an acute period or exacerbation of MG, should we do exercise?
No!
What should we do during an acute period or exacerbation of MG?
Focus on energy conservation, safe mobility with AD, fall prevention, and home modifications
During medically stable periods of MG should we exercise?
Yes!
T/f: consistent exercise elevates the baseline fxnal capacity to dismiss the effects of future MG exacerbations
True
When should pts with MG exercise?
During medically stable periods at the best time of the day during peak med dose
What muscles should we be careful exercising, and only do short periods of exercise with MG?
Large, proximal muscles
Why should we only exercise large proximal muscle groups for short periods of time in pts with MG?
Bc if these muscles get fatigued they will lose fxn
What level of exercise should we prescribe for pts with MG during medically stable periods?
Mild to moderate intensity exercise
HR shouldn’t elevate more than ____ bpm from baseline during exercise in pts with MG
30
T/f: pts with MG shouldn’t be still tired 2 hours after exercise if not overexerted
True
Worsening dyspnea in pts with MG can be a sign of what?
Myasthenic crisis
What kinds of exercises should be included in the exercise program of a pt with MG?
Posture exercises
Breathing exercises
Pt education
Whole body muscle cooling or targeted muscle cooling
Interventions customized to pt specific impairments
Why is postural exercise important for pts with MG ?
To support the body for breathing, speaking, and swallowing
T/f: whole body cooling can be effective in reducing muscle fatigue
True
What are important pieces of pt education for MG?
Disease specific education
Safety recommendations
Energy conservation
What are problems we can work on in pts with MG?
Muscle fatigue and weakness
Fatigue of respiratory muscles
Cv endurance
Fxnal decline
How can we minimize the effects of muscle fatigue and weakness in pts with MG?
Physical agents
Therapeutic exercise
Neuromusculoskeletal re ed
Pt ed
What physical agents can we use to minimize muscle fatigue in MG?
Cryotherapy
What therapeutic exercise can be done to minimize muscle fatigue in MG?
Progressive strength and endurance training
What is involved in neuromusculoskeletal re ed to minimize muscle fatigue in MG?
Decreasing unnecessary co contractions by improving awareness of the problem (when pts are weak they ill use co contractions for stabilize
What pt education can we use for minimizing muscle fatigue in MG?
Pacing and rest periods
What can we do to improve the efficiency of the respiratory muscles in MG?
Phased pulmonary rehab
What can we do to improve cv fitness and aerobic endurance in MG?
Alternating aerobic exercises such as biking, walking, and arm ergometer
What can we do for fxnal training in MG?
Bed mobility and transfer training as appropriate
What is phase 1 of post-myasthenic crisis pulmonary rehab?
When the pt is on the ventilator still
Focus on strengthening the trunk and proximal muscles of the upper limb muscles to enable the pt to expand and maintain sitting position
What exercises are involved in phase 1 post myasthenic pulmonary rehab?
Breathing exercises and respiratory use progressed to autonomous breathing for short periods of time
Standing and taking a few steps while connected to the respirator
What is phase 2 of post myasthenic crisis pulmonary rehab?
Ventilator weaning with a focus on more autonomous breathing exercises and disconnected from the respirator for short intervals
T/f: rhythmic auditory stimuli has positive effects on MG pacing
True
What is the neural basis of rhythmic auditory cues?
Connections bw the auditory and motor systems
What is the theory behind how rhythmic auditory stimuli works?
One system’s frequency affects the frequency of another system
What is the technique for using rhythmic auditory stimuli for MG?
Set the frequency at 110% of the pt’s fast gait speed to increase walking distance without increases in average walking HR or RPE
T/f: polyneuropathies can affect large or small fibers
True
What are the two causes of polyneuropathies?
Acquired or hereditary
What are hereditary causes of polyneuropathies?
CMT (Charcot-Marie-Tooth)
What are acquired causes of polyneuropathies?
Infections, toxins, drugs, cancers, nutritional deficiencies, and DM
Infection that leads to inflammatory disease is what?
CIDP (chronic inflammatory demyelinated polyneuropathy)
Infection that leads to autoimmune disease is what?
AIDP (autoimmune inflammatory demyelinating polyneuropathy)
What do we expect to see in multisystem presentation in persons with polyneuropathy?
Pain
Loss of sensation, dysesthesias, paresthesias, allodynia, neuropathic pain
Voice and speech dysfunction in GBS
Progressive changes in cv with activity
Illeus and urinary retention in GBS
progressive loss of reflexes
Progressive distal to proximal muscle weakness
Dysphasia in GBS
Skin breakdown
What are the 5 subtypes of GBS?
AIDP
CIDP
AMAN
AMSAN
Miller fisher syndrome
What is the most common subtype of GBS?
AIDP
What is the least common subtype of GBS?
Miller fisher syndrome
Is demyelinating or axonal GBS worse? Why?
Axonal bc it affects the nerve itself making the speed and amount of info traveling less
What are 2/3 of GBS associated with?
Bacterial or viral infection 2 weeks prior to
T/f: all cases of GBS result from either viral or bacterial infection
False, 27% of cases have no preceding trauma or illness
What is the onset of GBS?
Very acute onset of distal to proximal weakness
What is a differentiating factor of the onset of GBS?
The onset is much more acute than other polyneuropathies
What is the most common immune mediated polyradiculoneuropathy?
GBS
T/f: LBP is a common symptom of GBS due to weakness
True
What is the clinical presentation of GBS?
Muscular weakness
Electrodynamic changes
What are the characteristics of muscular weakness in GBS?
Progressive, symmetrical ascending muscular weakness to flaccid paralysis
What are the electrodynamic characteristics of GBS?
Slowed nerve conduction velocities, prolonged distal latencies, and absent H reflexes (like DTRs)
What are the EMG differences bw demyelinating GBS and axonal GBS?
Demyelinating will affect the time of conduction and just the nerve conduction velocity
Axonal will affect both time and amount of conduction
What are the key characteristics of GBS?
Diminished reflexes
Mild to total distal paralysis
Severe fatigue
Intercostal and diaphragmatic weakness
Autonomic symptoms
What is a red flag in GBS?
Dysautonomia
What does dysautonomia do?
Leads to low cardiac output, cardiac dysrhythmias, marked fluctuations in BP
cardiac arrest
Blood pooling
Illeus and urinary retention
Why is monitoring VS important in GBS?
Bc it can have dysautonomia as a symptom
What are the sensory changes in GBS?
Glove and stocking distribution of numbness and paresthesia, decreased vibratory and position senses, and neuropathic pain
What factors help us with differentials for GBS?
Acute pain and sensory changes and decreased DTRs
Will rhabdo have tingling, DTR changes, or CN involvement?
No, but GBS will
What are the medical management options for GBS?
Plasmaphoresis
IVIg
What is plasmaphoresis and how does it help with GBS?
It is a plasma exchange that cleans the plasma of attack antibodies to reduce the duration of muscle paralysis and need for ventilation by 50%
What is IVIg and how does it help GBS?
IV immunoglobulin that takes the blood from a vein, separates out the plasma, returns the blood cells w/ a plasma substitute
What is the initial phase of recovery in GBS?
Progressive demyelination phase during the first 3 weeks
What is the plateau phase of GBS progression?
Plateau in demyelination that can begin anywhere from a few days in to 2 weeks in
What is the recovery phase of GBS?
The progressive remyelination phase of GBS in 4-6 months or up to 2 years with axonal GBS
Does Axonal or demyelinating GBS tend to have a full recovery?
Demyelinating GBS
What factors can affect recovery time in GBS?
Age, severity, and how they got it
What factors predict the inability to walk with GBS?
Age over 60
Preceding diarrheal illness
Disease severity
Rapid disease progression
Need for vent support
What needs to be included in the GBS eval?
Touch, vibration, proprioception, kinesthesia tested and their interactions with movt and positions
Voice quality and quantity assessement
Coordination of speech and respiration
BP, RR, HR, O2, RPE
Continence status
Skin inspection
MMT
Jt ROM
Swallowing assessment
What is the risk of integ fxn in GBS?
Skin breakdown risk
What can we do to decrease the risk of skin breakdown in GBS?
Frequent repositioning
What is the risk with respiratory fxn in GBS?
Respiratory failure
What can we do to prevent respiratory failure in pts with GBS?
Closely monitor vitals and auscultate
What is the CV fxn risk with GBS?
Dysautonomia
How can we decrease the risk of dysautonomia in GBS?
Closely monitor BP
Why should, we check for signs of a thromboembolism in pts with GBS?
Bc they are at high risk for DVTs
What can we do to reduce fatigue relapse risk in pts with GBS?
Start with shorter exercise sessions initially (4x15 minutes)
Avoid harmful overexertion of denervated muscles
Monitor RPE in all sessions to minimize risk of overexertion
What has the greatest risk for jt contractures in acute stages of GBS?
DF of the foot and ankle
Trunk
Shoulders in ER/flex/abd
Hips in IR/ER/ext
Knees in extension
What can we do to prevent jt contractures in acute stage of GBS?
ROM BID
Are we more concerned with mobility or stability in GBS?
Stability
T/f: DO NOT do gait training with a pt with GBS until able to stand with AD with less than min A
True
Why are pts with GBS usually very anxious and fearful?
Bc of the vent, paralysis, immobility, and restricted communication
What can we do to ease fear and anxiety in pts with GBS?
Establish alternative means of communication
Make sure they are rarely alone
Try to get familiar faces to come in
What is the most common progressive neuromuscular UMN/LMN disease?
ALS
What does amytrophy mean?
Muscle wasting
T/f: the lateral cord is affected in ALS, producing motor symptoms
True
What blocks nerve impulses to muscles in ALS?
Sclerosis of the nerves
Will someone with ALS start high functioning and become low functioning or start low functioning and become high functioning?
They start high functioning and become low functioning
What is the ratio of males to females affected by ALS?
2:1
What are some of the earliest signs of ALS?
Tongue fasciculations
Muscle cramping at rest
Tripping/clumsiness
What are the LMN manifestation of ALS?
Muscle weakness, hyporeflexia, hypotonicity, atrophy, muscle cramps, fasciculations
What are the UMN manifestation of ALS?
Spasticity, pathological reflexes, hyperreflexia, muscle weakness
What are the bulbar manifestations of ALS?
Bulbar muscle weakness, dysphagia, dysarthria, sialorrhea, pseudobulbar affect
What are the respiratory manifestation of ALS?
Respiratory muscle weakness (inspiratory/expiratory), dyspnea, nocturnal respiratory difficulty, orthopnea, hypoventilation, secretion retention, ineffective cough
Can ALS have dementia like symptoms?
Yup
What are rare impairments in ALS?
Sensory impairments, B/B dysfunction, ocular palsy
What type of ALS do PTs usually catch before others?
Classic spinal onset ALS
What is the most common type of ALS?
Classic spinal onset ALS
Where is the typical onset of classic spinal onset ALS?
In the extremities from distal to proximal
T/f: classic spinal onset ALS affects the dorsal columns and CN 3,4,6 early in the disease
False, they are spared until later in the disease progression
What are the typical s/s of classic spinal onset ALS?
Complaints of tripping, foot drop, dropping items, difficulty with ADLs, cramping (esp in hands and feet), decreased fxn of the UE/LE, decreased strength of neck extensors (head drop), SOB on exertion and difficulty sleeping supine due to breathing problems, and fatigue
Which type of ALS do physicians usually catch before others?
Bulbar onset ALS
T/f: CNs 9 and 12 are affected first in bulbar onset ALS
True
What are typical s/s of bulbar onset ALS?
Cervical and diaphragmatic weakness
CN symptoms
Dysphagia and dysarthria, difficulty breathing (Swallowing, chewing, speaking, breathing)
Problems repositioning tongue or realigning lips or opening and closing mouth
Trouble projecting voice
Drooling
What are the characteristics of late disease progression in ALS?
Paralysis of voluntary muscles and progressive atrophy
Respiratory compromise
Inability to speak due to dysarthria and/or respiratory constraints
Difficulty swallowing
Progressive loss of neck extension
Cognitive impairments
Is the progression of symptoms in late stage ALS often symmetrical or asymmetrical?
Asymmetrical
What are the consequences of paralysis and muscle atrophy in ALS?
Contractures and pain
Jt subluxations
Dependent edema
Why does dependent edema often result in late stage ALS?
Bc of the paralysis of muscles, there is no pumping action of voluntary muscles
What does respiratory compromise in late progression of ALS result in?
SOB and labored breathing
Mechanical ventilator eventually
Why do pts with ALS often need an AAC device in later stages?
Bc of inability to speak due to dysarthria and/respiratory constraints
What is a major concern for pts in late stage ALS with inability to swallow?
Aspiration pneumonia that can result in death
What are the common symptoms of ALS?
Fatigue
Weakness
Imbalance, tripping
Clumsiness
Variable pain
Cramping
UMN/LMN symptoms
Fibrillations
What will a nerve conduction velocity test show on a pt with ALS?
Slowed NCV
What will lab testing for ALS show?
Increased CK and glutamate
What is stage 1 mild ALS?
Newly diagnosed
Tripping and possibly dyspnic, but still highly fxning
Don’t need assistance
What is stage 2 moderate ALS?
Mild deficits in 3 regions
OR
Moderate to severe deficits in one region
Don’t need assistance
Still fxnal
What is stage 3 severe ALS?
Need assistance bc of deficits in 2 or 3 regions
Starting to need assistance
Still fxnal
What is stage 4 severe ALS?
Non fxnal movt of at least 2 regions
AND
Moderate or non fxnal movt of a third
What is stage 5 ALS?
Death usually in 2-4 years or sometimes 15-20 years
Which stages of ALS don’t need assistance?
Stage 1 and 2
What stages of ALS are still fxnal?
Stages 1-3
What stages of ALS are not fxnal?
Stages 4,5
Pts with ALS who start with what kind of symptoms tend to have more rapid progression?
Bulbar and respiratory symptoms
Pts with ALS who start with what kind of symptoms tend to progress more slowly.?
Extremity weakness
What is the ALS fxnal rating scale?
A 12 item questionnaire scored 0-4 (no ability-normal ability) for a max score of 48 about ALS impacts on an individual
What is the MDC of the ALS fxnal rating scale? The MCID?
MDC=6.74
MCID= 20% decline
What is the only disease modifying pharmaceutical available for ALS? What does it do?
Riluzole (a glutamate antagonist)
It helps with bulbar and respiratory s/s
T/f: Riluzole (the only disease modifying pharmaceutical for ALS) has been shown to extend trach-free survival
True
What can be given to help with pain in ALS?
NSAIDs
What meds can help with spasticity in ALS?
Baclofen and Tizanidine
What is an alternative option for spasticity and pain control in ALS?
Cannabis
What meds can be given to help with drooling in ALS?
Amitriptyline and Botox
What are the focuses of PT management of ALS?
Comfort and pain reduction
Maximize fxn
Prevent secondary impairments
T/f: a majority of pts with ALS have pain and spasms
True
What is a symptoms of ALS we need get ahead of before it starts bc once the function is lost it is very hard to manage?
Neck extensor strength to prevent head drop
What can we focus on in PT for ALS to maximize fxn?
Transfers and safety
Bed mobility
Toileting
What secondary complications are we trying to prevent with PT for ALS?
ROM losses
Spasticity
Integument complications
Respiratory complications
T/f: we should begin a strengthening program for ALS in the early disease stages
True
Can individuals with ALS experience strength gains?
Yes!!!!
What level of intensity should exercise be for a pt with ALS?
Mild to moderate intensity
T/f: pts with ALS should be doing high intensity resistance training
False, it is no more effective than moderate exercise and puts individuals with ALS at risk for injury
What should we educate pts with ALS about?
Rest periods and avoiding fatigue
T/f: we should encourage pts with ALS to participate in sports and daily activities that are enjoyable early on
True
What is involved in stage 1 PT management of ALS?
Optimization and prevention
Respiratory muscle strengthening, diaphragmatic breathing, aerobic capacity
Prevention of secondary impairments
Prescription of AD
Fall risk prevention
Energy conservation
What is involved in stage 2 PT management of ALS?
BiPAP, supplemental O2, trach
Referral to multidisciplinary ALS clinic
Management of secondary MSK impairments and pain
Prescription of ATE, bracing, AD
What is involved in stage 3 PT management of ALS?
Palliative care b4 hospice
Airway clearance
AAC device (referral)
Caregiver education on positioning and skin protection, ROM, and transfers
Additional of ATE
What outcome measures tell us what ADs to prescribe a pt with ALS?
A SLS time and a 10 meter walk test
What does palliative mean?
To lessen severity of symptoms of an illness w/o curing or removing the underlying cause
What is the goal of palliative care?
To treat, prevent, or relive symptoms of a serious or progressive illness but not cure it
Where can we find the well published ways to document care of a pt with progressive worsening where the goal is maintenance?
On the APTA site
Who are palliative care providers?
Can be nurses, nurse practitioners, social worker, or other health care professional who are certified
What services do palliative care providers give?
Education and support for the pt and family surround the health condition
Coordinating interdisciplinary meetings to discuss prognostic and treatment options
Establishing goals of care
Provide expertise in managing physical and emotional symptoms, including medication management
Ongoing time for family meetings and pt/family counseling
What is the end of life role of the PT?
Pt/family management
disciplinary team consultation and education
Professional responsibility (education, advocacy, scholarship)
To maintain and optimize safe and comfortable fxn in the presence of physical decline
