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MOD 6: med. myopathy
UMN, LMN, ms atrophies
| Question | Answer |
|---|---|
| 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. |
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PTROCKS
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