Term
| Myasthenia Gravis is a result of |
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Definition
anti-ACh receptor antibodies which block normal binding site of Ach
Post-synaptic Nicotinic receptors at motor endplate decreased in number |
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Term
| Myasthenia Gravis clinical manifestations |
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Definition
fluctuating weakness and fatigue-ability of skeletal mm.
vary from mild to severe
fluctuating weakness is evident in proximal mm
cranial mm= diploplia and ptosis, difficulty swallowing |
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Term
| Myasthenia Gravis Treatment |
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Definition
Acetylcholinesterase inhibitor: reduces weakness by making AcH available to bind
immunosuppression by corticosteroids |
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Term
| Myasthenia Gravis Prognosis/PT intervention |
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Definition
periods of remission and exacerbation, ventilatory assistance may be required
determine neuro and respiratory baseline
never swallow with neck extended
educate on signs of MG crisis, Ach inhibitor toxicity and adverse effects of immunosuppression therapy
energy conservation strategies
avoid strenuous exercise and stress
limit onset of fatique- minimize atrophy |
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Term
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Definition
ingestion of potent neurotoxin produced by colstridium botulinum
not destroyed by digestive system and readily absorbed in circulatory system
Acts as ACh antagonist at motor endplate |
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Term
| Botulism clinical manifestations and treatment |
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Definition
symmetrical flaccid paralysis
motor weakness of face and neck muscles progresses to respiratory
Antitoxim and induced vomitting if from food
respiratory death could occur in 24 hours |
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Term
| Muscular dystrophy is a result of...and clinical manifestations |
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Definition
mutation of dystrophin gene- links sarcolemma with actin
reduced muscle growth and turnover
sarcolemma susceptible to damage during contract/relax cycle
symmetrical mm wasting without neural or sensory deficits |
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Term
| X-linked muscular dystrophy |
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Definition
Duchenne's (DMD): x-linked recessive, rapid progressing, onset 1-4 years, death late teens
Beckers (BMD)x-linked recessive, slow progressive, onset 5-10 years, walking maintained past teens, death in 20s |
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Term
| Autosomal Muscular Dystrophy |
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Definition
Facioscapulohumeral- autosomal dominant, early adolescent onset, slowly progressive, loss of walking later in life, variable life expectancy
Limb-Girdle: autosomal recessive, 1 in 4 chance of exhibiting disease, late adolescent onset, slowly progressive |
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Term
| Muscular Dystrophy Clinical Manifestations |
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Definition
Muscular weakness, wasting, hypotonia
Gower's sign: hands on things and walks up legs
frequent falls, difficulty with stairs, waddle gait (proximal mm weakness)
walk on toes, increased lordosis, trendelengburg
gait deterioration followed by shoulder girld weakness (winging), progressing to respiratory and cardiac mm |
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Term
| Muscular Dystrophy Treatment (PT and pharm) |
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Definition
none known to halt progression
glucocorticoid therapy- maintain fxn in unaffected mm groups as long as possible
maintain activity level
avoid strenuous activity
monitor respiratory involvement
ambulation and pool for endurance
assistive devices |
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Term
Spinal Muscular atrophy (werdnig Hoffman)
aka progressive infantile spinal muscular atrophy (floppy infant) |
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Definition
characterized by progressive weakness, hypotonia and wasting of skeletal mm
autosomal trait resulting from gene deletions
progressive degeneration of anterior horn cells of spinal cord and selective motor nuclei of brainstem along with gliosis. |
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Term
| Spinal Muscular Atrophy Clinical Manifestations |
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Definition
developmental delays
scoliosis
feeding problems (tongue dysfxn, weak swallow)
respiratory problems
the earlier the expression, the faster the progressions
Strictly Motor |
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Term
| Spinal Muscular Atrophy PT |
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Definition
frequent position changes
promote supine
position to encourage spine and trunk control
Intellectually normal so verbal, tactile and auditory stimulation is important |
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Term
| Amyotrophic Lateral Sclerosis (ALS) is a result of |
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Definition
degeneration and scarring (gliosis)
motor neurons in lateral spinal cord, brainstem and cerebral cortex-upper motor neuron
-cerebellum and cognition are not affected
peripheral motor nerves- LMN (anterior horn)
death of peripheral nerves results in atrophy |
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Term
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Definition
1. Pseudobulbar palsy: damage to corticobulbar tract exhibiting UMN symptoms
2. progressive bulbar palsy: cranial nerve nuclei involvement resulting in weakness in facial mm, chewing and swallowing
3. primary lateral sclerosis: neuronal loss at cortex, UMN symptoms
4. Progressive muscular atrophy: loss of neurons in anterior horn starting @ cervical area- LMN symptoms |
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Term
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Definition
LMN= atrophy and absent reflexes
UMN= heightened reflexes |
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Term
| ALS prognosis and treatment |
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Definition
relentlessly progressive with death in 2-5 years due to 2ndary health conditions
Early: increasing activity to prevent atrophy, ROM, avoid fatigue
Middle: avoid contracture formation, orthotics, deep breathing
Late: edema prevention, control spasticity, potect joint, power chair and anti-pressure interventions |
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Term
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Definition
virus invades alpha motor neuron cell bodies resulting in asymmetrical paresis and paralysis
post-polio:compensated innervation cannot maintain mm fiber innervation
myalgia, weakness, fatigue, decreased endurance |
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Term
| Post Polio PT intervention |
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Definition
non-exhaustive exercise and conditioning
functional exercises
patient education of lifestyle modifications and energy conservation |
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Term
Charcot Marie Tooth
Peroneal Muscular Atrophy |
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Definition
autosomal dominant disease affecting motor and sensory nerves
can occur in childhood or adulthood
extensive demyelination followed by hypertrophic schwann cell activity resulting in enlarged peripheral nerves |
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Term
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Definition
distal symmetrical muscle weakness, atrophy and diminished DTR's
pes cavus, hammer toe, DF and evertor weakness |
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Term
|
Definition
facial nerve unilaterally affected
inflammatory response around facial nerve= demyelination and degeneration
develops overnight
taste, lacrimation, salivation,
high dose corticosteroids
recovery over several weeks |
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