Unit 18 Neuromuscular Disorders Flashcards
An injury to where in the spinal cord would a PT be ventilator dependent?
C4 and above
Where does the ascending tract begin, what is it associated with and how many tracts are there?
Begins in the spinal cord and ends in the brain, carries sensory input to higher levels in CNS
The sensory pathway
perception of touch, pressure, vibration, proprioception, pain, temperature
Describe the Descending Tracts and what tract is Parkinson’s associated with?
Associated with the motor pathway
Two tracts
1. Pyramidal tract
voluntary movement
2.Extrapyramidal tract
automatic movement, posture
(Parkinson’s associated with problems of extrapyramidal tract)
Describe the two types of motor neurons.
Upper Motor Neurons (UMN)
- entirely within the CNS
- initiates voluntary movement
Lower Motor Neurons (LMN)
- directly innervates skeletal muscles
- essential for muscle contraction
- part of peripheral nervous system
Describe UMN Lesions vs LMN Lesions
UMN lesions will have weakness below level of injury and muscle tone will be spastic.
-injuries usually above sacral spinal cord
LMN lesions will be specific to nerve root and muscle will be flaccid
What are the common groups for spinal cord injuries and most common cause?
- elderly and young males
- some form of trauma
What is the difference between complete and incomplete spinal cord injury?
Complete- loss of all sensory, proprioception, & voluntary motor activity
Incomplete- part the the spinal cord is intact
Define Tetraplegia/Quadriplegia and Paraplegia.
Tetraplegia/Quadriplegia- loss of sensation and movement in all four limbs
Paraplegia- loss of movement & sensation in lower half of body
What are the types of spinal cord injuries?
Hyperextension
Hyperflexion (going forward, chin tuck)
Compression
Flexion-rotation (usually c-spine injury)
Penetrating
What is Rx given in high doses for spinal injury and what is the contraindication?
methylprednisolone (Solu-medrol)
*contraindication for penetrating spinal injury because PT is already at risk for infection and steroid Rx will increase the likeliness.
Describe Neurogenic shock and spinal shock.
Neurogenic shock: Loss of vasomotor tone and impairment of autonomic function
The higher the injury -T6 and above you’ll usually see neurogenic shock
S/S hypotension, bradycardia, warm dry skin
Spinal shock: Loss of spinal reflexes»flaccid paralysis
-Even with UMN injury you’ll see spinal shock initially before spasticity kicks in
What is myelin?
Covering or coating around nerve cells
Describe Multiple Sclerosis.
-Chronic disease of the CNS characterized by the degeneration and loss of myelin in the brain, spinal cord, and cerebrum. T-cells attacking myelin
- autoimmune
- cause not yet known
- 25-40 most common age group
- 60% in women
Early stages: Nerve damage minimal
Progressive stage: Nerve impulses are completely blocked
What are the motor and sensory clinical manifestations of MS?
Motor:
- weakness
- paralysis
- diplopia
- spasticity of muscles
- scanning speech (pauses in speech)
- fatigue
Sensory: Numbness and tingling Patchy blindness Vertigo Tinitus (ringing in ears)
What are cerebellar and other clinical manifestations of MS?
Cerebellar:
- Ataxia (loss of full control of body movements)
- Nystagmus (repetitive uncontrolled eye movements)
- Dysarthria (slowed speech)
- Dysphagia
Other:
- Bladder dysfunction
- Sexual dysfunction
- Emotional instability
What diagnosis can be done for MS?
Health and Physical
Cat-scan: if MS is advanced enough they will see diffused white lesions
What are the drug therapy classes and medication for MS?
> Biological Response Modifiers
- Interferons *beta-1a, *beta-1b
(decrease inflammation and immune system response) - Synthetic Immunomodulator: *glatiramer acetate
> Steroid therapy
- methylprednisolone
- prednisone
> Immunosuppressants (not first choice)
*cytosin
> Antipasmodics (for spasticity)
*baclofen
What is Parkinson’s Disease?
- Impairment of dopamine-producing cells in the brain.
- more common in men
- no known cause
What is dopamine?
A neurotransmitter that is essential for normal functioning of the extrapyramidal system which controls posture, support, and autonomic movement
What are the key features to Parkinson’s Disease and how is it diagnosed?
“TRAP”
Tremors -increases at rest
Rigidity- resistance to passive range of motion
Akinesia/Bradykinesia- Slowing of automatic movement
Postural disturbances
*Dx with 3 out of 4
Parkinson’s Disease has many drug therapies, which is the top choice class/Rx and describe it.
-Dopaminergics
*carbidopa/levadopa
PT is on it for life
:::Problems:::
Paradoxical intoxication- when the Rx should work, but is to concentrated in the body so a “Medication holiday” (stop and restart) is needed to fix.
-Unpredictability of when it will decrease or stop working
What are the other drug classes/Rx’s for Parkinson’s Disease?
Dopamine agonists
-bromcriptine (helps release own supply of dopamine; mild symptoms)
Catechol O-methyltransferase (COMT) inhibitors
-entacapone (“ase”-enzyme)
usually given with carbidopa/levadopa
not for monotherapy
MAO-B inhibitors
(same as COMT inhibitors but can be given alone)
-rasagiline
Anticholinergics (blocks the neurotransmitter acetylcholine in the central and the peripheral nervous system)
-benzotropine
What is Deep Brain Stimulation (DBS)?
- a permanent stimulating lead placed in brain & connected to an impulse generator that’s implanted in PT’s chest
- controlled by radio frequency transmissions from a computer
- DBS helps interrupt “short circuits” in brain
- decreases some symptoms of Parkinson’s
What is Myasthenia Gravis?
- Disease of the neuromuscular junction characterized by fluctuating weakness of certain skeletal muscles
- PNS autoimmune disorder
- results in decrease # of ACh receptor sites at neuromuscular junction (receptor sites are blocked)
- Woman 20-30 yoA
What are M.G. diagnostics?
- History and S/S (“Look to ceiling test”)
- Tensilon test (Rx: edrophonium) (+) if PT given that can perform normally
- Blood test: Acetylcholine Receptor Antibodies (AChR)
- EMG studies
- Ct scan (for overgrown thymus)
What are the Myasthenia Gravis S/S?
-Fatiguability of skeletal muscles, especially those involved in:
Moving eyes & eyelids
Chewing
Swallowing
Speaking
Breathing
-Ptosis (drooping of eye) #1 symptom
*Majority of symptoms from diaphragm up,
PT tends to do better in the morning
How is Myasthenia Gravis managed?
- Anticholinesterase inhibitors
- pyridostigmine bromide (first line therapy for MG) - Corticosteroids
- prednisone - Immunosuppressants
- Plasmapheresis (process to remove infected plasma and replace)
- Thymus gland removal
Describe Myasthenia Crisis.
- Due to under-medication or commonly, infection.
- Will test (+) Tensilon test
- S/S increased BP, P, RR, severe resp. distress
- Bowel incontinence
Treatment: Respiratory support, give Rx’s
Describe Cholinergic Crisis.
- Due to excessive M.G medication
- Will test (-) tensilon test
- S/S abdominal cramps, diarrhea
- Increased pulmonary secretions & bronchial spasms
Treatment: *atropine (anticholinergic) and O2
What is Amyotrophic Lateral Sclerosis (ALS)?
- Degeneration of motor neurons in brain and spinal cord for unknown reasons
- electrical and chemical messages originate in brain but the dead motor neurons cannot produce or transport vital signs to muscles
- no cure
- cns associated
- after Dx average lifespan 1-2 years
- just supportive care
What are the clinical manifestations of ALS?
- Weakness
- Dysarthria (sowed speech)
- Dysphagia
- Muscle wasting
- Spastic muscles initially then eventually flaccid
- Cognitively intact
What is Guillian-Barré Syndrome (Infectious Polyneuritis)?
- autoimmune attack on the peripheral nerve myelin
- Paralysis , weakness [PNS]
- unknown reason, usually 2-3 weeks after an infection
What are the diagnosis’ of Guillian-Barré Syndrome?
- Clinical S/S
- Lumbar puncture (increase in CSF/ protein after several weeks)
- EMG (electrode test)
- Forced Vital Capacity
- Negative Inspiratory Factor (NIF)
What are the stages of Guillian-Barré Syndrome?
Initial period
Plateau period
Recovery period
*PT’s can recover from this, gets worse before it gets better
What are the two clinical variations of Guillian-Barré Syndrome?
Ascending
-starts from feet bi-laterally and goes up
Descending
-starts in head and goes down
What are the clinical manifestations of GB?
Usually associated with descending GB:
- Fatigue while talking - Facial weakness - Cranial nerve involvement - Respiratory compromise/failure - Paresthesias - Pain resembling charlie horse - Difficulty walking - Motor weakness
What is the treatment of Guillian-Barré Syndrome?
-Plasmapheresis: plasma exchange, whole blood is removed from body-RBC’s and WBC’s are separated from plasma & returned to body without plasma. Body reproduces plasma.
PT’s gets lots of fluids while this happens
6-10x of two weeks
Monitor BP
-High dose immunoglobulin therapy
What are the two kinds of Neurogenic Bladders?
Reflex/Spastic:
- Impulses cannot travel from lower spinal cord to cortex because of UMN lesion
- Reflex arc is intact
- Result >Uncontrolled expulsion of urine without complete emptying
Flaccid:
1. Message that bladder is full gets to brain but bladder cannot intercept the message to it remains flaccid.
2. Damage to reflex arc
3. Loss of sensation of bladder fullness. result >overfill and distention (urine just leaks out)
Ex: PT’s who have diabetes, injury below sacral
What is the reflex arc?
the nerve pathway involved in a reflex action
basically a sensory nerve and a motor nerve with a synapse between.
Describe bladder management/training for spastic and flaccid neurogenic bladders.
Spastic management/training:
1. Trigger reflex (stroke inner thigh or bladder)
2 Cauterization
3. Medications
Flaccid management/training:
- Valsava maneuver
(exhaling with nostrils and mouth) - Credè (make fist, massage over bladder)
- Catheterization
- Medications
What does it mean to have a Neurogenic Bladder?
Any bladder issue due to disturbance of nervous system.
What are the medications for spastic neurogenic bladders?
- Anticholinergics
- propantheline
- oxybutynin - Skeletal muscle relaxants
- baclofen
What is the Rx for flaccid neurogenic bladder?
- Cholinergics (helps stimulate muscles of bladder)
- bethanechol
What are types of bladder problems?
> Urinary calculi
Urinary retention
Autonomic Dysreflexia -exaggerated response of sympathetic nervous system occurring above T6, full bladder #1 cause
What are the clinical manifestations of Autonomic Dysreflexia and how is it usually caused?
Hypertension
Headache and/or Blurred Vision
Diaphoresis/Vasodilation (above injury level)
Bradycardia
-Caused by spinal cord injuries T6 and above
What are the interventions for Autonomic Dysreflexia?
1st HOB up as high as possible
2nd Remove stimulus
Last resort give anti-hypertensives
if it is a paste, give above level of injury because circulation is better
What is Reflex (UMN) bowel and Flaccid (LMN) bowel?
Reflex (UMN) bowel- Incontinence without warning
Flaccid (LMN) bowel- Infrequent small stools
What is the treatment for Reflex/Spastic (UMN) bowel and Flaccid (LMN) bowel?
- Consistent time
- Suppository program
- High residual foods
- High fluid intake
- Stool softener
UMN lesion >Digital stimulation
LMN lesion >Manual removal
What are problems of Impaired Mobility?
- Skin integrity
- Thrombophlebitis
- Osteoporosis
- Muscle atrophy
- Contractures
- Fatigue
- Alteration in coordination
- Pain
What are a few causes of pain and the management regarding then?
- Disruption of nerve endings
- Spasms/fatigue
- Inflammation
Pain management:
- physical therapy
- pharmacological support
- surgical intervention
What are pharmacologic therapies for pain?
- Muscle relaxants
- Anti-inflammatory drugs
- Non-narcotic drugs
- Nerve blocks
- Epidurals
An inability to regulate body temperature is associated with what?
Spinal cord injury
With the medications listed, what are the associated conditions?
- anticholinergics
- benztropine - Dopaminergics
- carbidopa/levadopa - Corticosteroids
- prednisone - Cholinergics
- pyridostigmine - Immunosuppressant (not first choice because at risk for infection)
- cytoxin
- parkinson’s
- parkinson’s
- MS
- Myasthenia
- MS, GB, Myasthenia
What happens with circulation and spinal cord injuries?
Below level of injury results in decreased circulation
What injury will result in a PT being ventilator dependent?
C4 and above
With Parkinson’s is it better for a PT to have a PEG tube or TPN and why?
PEG tube because PT’s GI is still affective
What is the Diagnosis of ALS based on?
The signs and symptoms
What is Autonomic Dysreflexia?
an acute emergency that occurs as a result of exaggerated autonomic responses to stimuli that are harmless in normal people (like a full bladder) and usually occurs in injuries above T6
