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MOD 6: med. myopathy

UMN, LMN, ms atrophies

QuestionAnswer
Problem is proximal to the motor neuron UMN
can involve motor cortex, internal capsule, and other brain structures through which the corticospinal tract descends, or the spinal cord UMN
weakness and loss of fine, skilled, voluntary limb movement, followed by an exaggerated muscle tone UMN
reflexes remain intact while communication and control from higher brain centers are lost UMN
Usually see hyperreflexia UMN
After several weeks, weakness becomes converted to hypertonicity UMN
where are contractures greatest with UMN flexors of UE and extensors of LE
a stroke is an example of a UMN
Problem is distal to the motor neuron LMN
disruption between the muscle and all neural input from spinal cord reflexes, including the stretch reflex LMN
with what type are Fasciculations seen (when a mm is quivering) LMN
If muscles are totally denervated, total weakness, called flaccid paralysis, occurs. LMN
Complete lesions lead to the muscles of the limbs, bowel, bladder, and genital areas becoming atonic LMN
Muscle atrophy is an outstanding feature. LMN
There is sensory loss LMN
AKA peripheral nerve injury LMN
Examples of LMN include radiculopathy and Charcot-Marie-Tooth
Progressive degeneration of spinal cord, brainstem, or both. Muscle Atrophies
Inherited polyneuropathy : Autosomal dominant Charcot-Marie-Tooth
Involves chronic, slow degeneration of peripheral nerve roots Charcot-Marie-Tooth
Onset is in late adolescence or early adult Charcot-Marie-Tooth
in which ms do you commonly see atrophy with Charcot-Marie-Tooth peroneal
Commonly affects small, distal limbs, feet and legs and hands affected and rarely below the elbows Charcot-Marie-Tooth
Bilateral equinovarus is seen with… Charcot-Marie-Tooth
“storklike” legs (inverted champagne glasses) are seen with Charcot-Marie-Tooth
Clawed hands (are seen late in this disease) Charcot-Marie-Tooth
This disease presents with non-intact DTR Charcot-Marie-Tooth
Charcot-Marie-Tooth is a type of LMN
Ataxic gait, complicated by foot drop is seen in this disease Charcot-Marie-Tooth
there is compound motor action potential on EMG of peroneal nerve in this disease Charcot-Marie-Tooth
Rx for Charcot-Marie-Tooth bracing or surgery to stabilize ankles (arthrodesis of ankle mortise), prevention of contractures, sprinting or bracing to prevent changes involved c peroneal ms atrophy and clawed hands
this is an LMN disease but it has UMN signs ALS
signs of this disease include ↑ DTR, + Babinski-when u take tip of reflex hammer to toes and see splaying of the feet ALS
In this disease, you will see muscle wasting without sensory changes ALS
this is similar to the adult form of SMA ALS
In ALS, muscle atrophy and weakness (amyotrophy) is the result of... Massive loss of anterior horn cells of the spinal cord and the motor cranial nerve nuclei in the lower brain stem
Corticopsinal tract signs of ALS include Demyelination and gliosis of the corticospinal tracts and corticobulbar tracts caused by degeneration of the Betz cells in the motor cortex result in upper motor neuron symptoms (lateral sclerosis)
Onset: middle age (3-5th decades) ALS
creatine phosphokinase levels are elevated in approximately 70% of patients with this disease ALS
50% die within first 3 yrs. of dx. And 90% die within 6 yrs. of dx. of what disease ALS
Signs and Sypmtoms of ALS include Awkward fine finger motion, Stiff fingers, Fasciculations(tongue), Hand muscle wasting and weakness, Cramping of UE (persistent with activity), Atrophic hand weakness/forearm, Leg spasticity/increased tone, Hyperreflexia in absence of sensory changes
The order of body part involvement for ALS is... UE, neck, larynx, trunk, LE
what is Px for ALS poor
Rx for ALS includes (mainly supportitive care) Fam pt ed, transfer training, gait training, ROM, walking... Strength or resistive training may be harmful, damaging the motor neurons that are left
general Rx for ms dystophies Exercise, Bracing, no direct surgical interventions
These diseases can be autosomal recessive or dominant but are more commonly recessive Limb-Girdle Dystrophy
these diseases have a slow progression and occur in late childhood and early adulthood Limb-Girdle Dystophy
Signs and Symptons of Myasthenia Gravis Fluctuant weakness of voluntary muscles, Weakness with activity, Recruitment with rest
Another Symptom of Myasthenia Gravis involves motor endplate disorders. Describe this problem. Creates antibodies to receptors at postsynaptic neuromuscular junction. ACTH is released but nothing happens
 Onset: insidious Before 40: men get this disease more than women. After 40: equal ratios of men and women. Peak onset: 20-30, often after pregnancy. Myasthenia Gravis
Muscles of eating and facial expression are affected in this disease. Myasthenia Gravis
More energy in the AM with weakness progressing throughout day is a symptom of Myasthenia Gravis
Intermittent double vision is a symptom of Myasthenia Gravis
Is Myasthenia Gravis unilateral or bilateral? Bilateral
In this disease, exacerbations occur during stressful times/pregnancy. Myasthenia Gravis
What type of disease is Guillian Barre  Autoimmune, Idiopathic infectious polyneuropathy or polyneuritis, thought to be caused by a virus
Onset: any age, but peak is 2nd and 5th-8th decades Guillian Barre
This disease presents as progressive symmetrical weakness in the LE and UE over time Guillian Barre
This specific disease can involve Areflexia (Lose DTR early.) Guillian Barre
 May involve phrenic nerve, thus causing breathing problems. May need to be ventilated. Guillian Barre
Rx for Guillian Barre includes  Supportive care, Be respectful, Initially try steroids to control inflammatory process, Monitored in ICU initially as patient’s status can change rapidly, in most severe cases try plasmaphoresis (watch the O2 saturation levels), IV immunoglobulins
Px for Guillian Barre good, some ppl recover spontaneously...3% are fatal. 10% have severe disability. 3% may relapse and have chronic form.
Created by: PTROCKS