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
What is the management of FSHD
plumonary funciton testing
testing for retinal and hearing
speech therapy
maintain flexibilty and minimize atrophy
manage pain
compensations like orthotics might be helpful
What muscles are affected early in FSHD?
hamstring and trunk muscles are affected early
What has the fasted conduction for a nerve cell?
very thick myelin = fast conduction
What has the slowest conduction?
no myelin= very slow
What type of cells make myelin in the PNS
schwann
What type of cells make myelin in the CNS
oligodendrocytes
What is neurapraxia
temporary failure of nerve conduction without structural changes. usually caused by blunt trauma, pressure, ischemia.
axon remian intace and muscle does not atrophy
What is axonotmesis
aaxon indamages due to crush, stretch, lacerating injury or disease process. Connective tissue support for the nerve remains intact
what is neurotmesis
most sever axonal loss with complete severance of the acon and its connective tissue coverings
if cell body remains viable, regerneative sprouting occurs with growth approx 3mm/day near the lesion and 1mmday further from lesion
What are the types of neuropathy?
wide careity of cause can be acquired and idiopathic bell's palsy trigeminal neuralgia icu acquired weakness chemotherapy induced neuropath
What is bell’s palsu
typicallu preceded by a viral infection
facial paralysis of one side
71% have full recovery
What is trigeminal neuralgia
brief, intense shock like burss of pain within a distribution of the trigeminal nerve
opthalmic, maxillary, mandibular
could be related to herpes zoster, inflammatory reactions, tumors, other compression of the nerve
What is ICU acquired weakness linked to
multi organ failure and ventilare >2 weeks
What is a common side affect of cancer treatment
chemotherapy induced nueropathy
How do you differentiate Bell;s Palsy from a stroke?
bell’s palsy has full one side facial paralysis
What are the 4 classifications of neuropathy
infectious
hereditary
metabolic
immune-mediated inflammatory
What are the infectious neuropathy’s
poliomyelitis
post polio syndrom (PPS)
what is a hereditary neuropathy
Charcot-Marie-Tooth disease (CMT)
what is the metabolic neuropathy
diabteic neuropathy
what type of neuropathy is Guillain-barre syndrome
immune-mediated, inflammatory
What are symptoms of polio of the early 1950
caused by virus, highly infectious, especially <5
symptoms are fever, fatigue, headached, vomiting, tiffness of the neck and pain in th limbs
it can cause toatl paralusis by affecting the anterior horn cells of the spinal cord
What kinds of impairments do people with polio have
focal and asymmetric motor impairments– anterior horn cells (possible brainstem) may be affected
absent DTRs
equal numbers recovered, had mild residular parayis, or left with moderate to severe paralysis
What mimics polio currently
acute infantile paralysis
What is post polio syndrome
new neuromuscular sumptoms that occur decades after recovery
1/4 to 1/2 develop PPS
most recovery of muscle strength is due to reinnervation of denervated muscle fibers by collateral sprouts from nearby axons
What are some interventions for PPS
non-exhaustive exercise and gneral body condition
monitor vitals
functional exercises of submaximal intensity are stressed with the goal of maintaing and improving endurance and functional capactiy
lifestyle modification
compensations
preventions
What syndrome is the most common cause of rapidly evolving motor paresis, paralusis and sensory deficits?
Guillain-Barre syndrome
What are causes of GBS?
bacterial and viral infections, surgeries and vaccines have all been implicated
GBS is an immune mediated disorder
evidence supports the view that GBS is an immune mediated disorder
What cells are the primary targets of attack
Schwann cells
What is the first symptom of GBS and acute inflammatory demyelinating polyneuropathy (AIDP)
paresthesia in toes, followed within hours or days by weakness distally in the legs
What are the symptoms of GBS/ AIDP
first symptom if often paresthesia in toes
weakness and sensory loss that is symmetric and ascending (distal to proximal)
weakness spreads to involve arms, trunk, and facial muscles
flaccid paralsis is accompained by absence of DTRs
may result in respiratoy failure
recovery takes weeks to months (body repairs myelin)
How do you diagnose GBS/AIDP
lumbar puncture for CSF analysis – albumin elevated
fibrillations if axonal damage
may have tachycardia, cardiac arrhythmias, and labile BP
What is the treatme for GBS. AIDP
aimed at controlling the immune response
plasmapheresis removes and filters plasma to remove or dilute circulating antibodies
high-doe intravenous immunoglobulin (IVIg)
What is longer length of inpatient hospitalization correlated with for GBS/AIDP
presence of muscle belly tenderness
sever lower limb weakness as measured by MMT
FIM scores at admssion
What is the acute rehab look like fo rGBS/AIDP
LOS 63 days
by discharge min to no assistance for mobility, max A at admission
LOS double when axonal dame, ventialtor dependence, and muscle belly tenderness is present
What is the PT managmenet for the descending weakness phase of GBS/AIDP?
aarom and milde resitance = avoid fatigue
progress functional trainin
consider compensatory devices
maintain jjoint ROM, and flexibility with pain tolerance
monitor vitals
hep with pan control
WHat it the PT management for outpatient of GB/AIDO
participation level goals, return to work, independence, exercise, wean from AD, fall prevention
What is CMT?
Charcat-marie-tooth disease is characterized by distal limb muscle wasting and weakness, usually skeltal deformities, distal sensory loss, and abnormalieites of DtRs
hereditary motor and seonsroy neuropathy – begins with peroneal nerve disorder progressing to foot and leg, ankle DF and EV affected
later progresses to weakness and wasting of intrinsic hand muscles
no treatment
What is rocker bottom deformity
bones of the foot may fracture and collapse
what is pes cavus
intrinsic foot and lower lef weakness resulting in imbalance of muscle action resultin in clawing of toes and high arch
What kind of foot is more common in children?
pes cavus
What is the PT management for CMT
prevent contractures,
UE function
pain control
balance
exercise– focus on unaffectec muscles and moderated
strengthen ar 40-60% 1 RM, aerobic training at 70-90%
What is diabetic peripheral neuropathy ?
meaning a demonstratable disorder, either clinically evident or subclinical, that occurs in the setting of M without other cuases for peripheral neuropathy
affect sensory and motor nerves in a distal to proximal symmetric pattern (stocking glove pattern)
Within one year of DM diagnosis have many have neuropathy
7%
What is the pathogenesis of DN
caused by the chronic metabolic distrubances that affect neurons and schwann cells
vascular changes affect peripheral nerves
What are the specific findings of DN
early detection and prophylactic foot care regimens have led to fewer foot ulcerations and amputations
greater forefoot pressure occur during gait (decreased 1sst toe and ankle mobility)
increased risk for development of metatarsal ulcers
footdrop during gait
