Peripheral Disorders Flashcards
What is amyotrophic lateral sclerosis ALS
Progressive neuromuscular disease characterized by degeneration and eventual death of upper motor neurons and lower motor neurons causing weakness of the limbs, respiratory, and vulgar musculture. Lower motor neurons affect it first
What are the known risk factors of ALS
Older age, lower BMI, cigarette smoking, professional athletes are more at risk, repeated head injuries
What is the pathology of ALS
Dysfunction of the astrocytic excitatory amino acid transporter two, reduced uptake of glutamate from the synaptic cleft, leading to glutamate excitotoxity
The resulting glutamate induced excitotoxins city induces neurodegeneration through activation of calcium dependent in enzymatic pathways
What is amyotrophy
Loss of brain stem cranial nerve nuclei and anterior horn cells and spinal cord
What is lateral sclerosis
Loss of cortical motor cells causing corticospinal tract dysfunction
What is El Escorial criteria
Presence of three of these findings including evidence of lower motor neuron degeneration by clinical, electrophysiology goal or neuropathology goal exam, evidence of upper motor neuron degeneration by clinical exam, or progression of the motor syndrome within a hidden region or to other regions by history or exam
What is the most common a ALS presentation
Is limb onset of symptoms with distal to proximal progression including fasciculations, atrophy, weakness, spasticity
What form of ALS typically progresses faster
Bulbar onset occurs in 25% of cases including changes in speech, difficulty swallowing, involuntary tongue twitching, vocal cord spasms
What type of ALS is rare
Respiratory answer is rare under 3% which includes shortness of breath orthopnea, and sleep disordered breathing
What is the prognosis of ALS
Three years survival equals 50% five year survival equals 20% 10 years survival is 10%
What are negative prognostic factors for ALS
Bulbar onset, weight loss, cognitive impairment, impaired respiratory function
What are the five stages of ALS
Stage zero equals pre-symptomatic
Stage one is involvement of one clinical region
Stage two is involvement of two clinical regions
Stage three is involvement of three clinical regions
Stage four is substantial respiratory or nutritional failure
Stage five equals Death
What are the two types of medical management for ALS
Riluzole add three months to overall survival is taken for 18 months
Edaravone decreases oxidative stress in ALS
What does your examination Of historyinclude for ALS
Location of symptom onset, month or year of symptom onset, date of diagnosis, previous trauma, falls secondary to weakness, cervical and lumbar laminectomy’s, carpal tunnel releases
What are your examination items for impairments for ALS
ALS functional rating scale
Range of motion, strength, sensation, muscle tone, skin integrity, respiratory status
What are the examination items For activity limitations
Balance and gait measures based on the individuals current functional status including,, bread, gait speed, six minute walk test, tug
What are the general interventions for ALS
Disease progression is different for everyone, you are in LA, fall risk and prevention, energy conservvation and prioritization of activities
What are the early stage presentations of ALS
Muscle weakness in extremities, weakness and muscles with speech and swallowening, specificity, muscle fasciculations, atrophy, painful muscle cramping, shortness of breath, mild changes
What are the middle stage presentations of ALS
Muscle weakness and atrophy at the body, paralysis occurs in some muscles, has more of the lower motor neurons damage, to speed decreases, uncontrollable or inappropriate laughing or crying, further weakness of swallowing muscles with reports of choking, weakness of muscles of speech to the degree of requiring augmentative communication, difficulty breathing requiring noninvasive ventilation, increased cognitive decline
What are the late stage presentations ALS
Majority voluntary muscles are paralyzed
Respiratory muscle weakness resulted in significant respiratory insufficiency requiring mechanical ventilation for survival
String Clawson swallowing muscle significant enough to require gastrostomy tube for nutrition
What are epigenetic factors that influence house Health
Aging, obesity, physical activity and exercise, smoking, geographic variations, socioeconomic status, social support, environmental barriers healthcare, gender, race, ethnicity, generational differences
What is a type of myopathy
Myasthenia gravis
What is myasthenia gravis
Chronic peripheral autoimmune neuromuscular disease of the neuromuscular junction
Skeletal muscle weakness that worsens after bouts of activity and improves after rest
What are the cardinal signs of myasthenia gravis
Ptosis a.k.a. eyelid drooping which is usually asymmetrical
Muscle fatigue
What are the signs and symptoms of myasthenia gravis
Ziploc Pilea Facial weakness For ptosis Dysphasia, dysarthria Shortness of breath Weakness in neck, upper extremities and lower extremities Fatigue
What is Limb girdle muscular dystrophy (LGMD)
mutations in many different genes that provide instructions for making proteins involvled in muscle maintenance and repair type 1= dominant inheritance type 2= recessive inheritance affects promzimal muscles of UE/LE 6/1000 affected
What is early stage of LGMD
changes in walking (waddling, waking on balls of fee), hands on knees to transition from squat to sstand
What are late stages fo LGMD
may require w/c , scapular wingin (due to muscle wasting), increased lumbar lordosis, pseudohypertrophy of calf muscles ( due to fatty deposits and scar tissue)
What are PT considerations for LGMD
resistance trianing– both higher and lower intensity resistance training may improve strength in arms over 6 months
aerobic and endurance– may also improve aerobic capacity and walking
What ia fascioscapularhumeral muscular dystrophy (FSHD)
facial, scapular, and humeral muscles affects autosomal dominant inheritance mask like appearnce symptoms usually before 20 life expectancy not shortened
