Robbins Ch.

27 Peripheral Nerve and Skeletal Muscle

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1.

Motor unit consists of what 3 parts?

lower motor neuron, axon, muscle fiber epineurium, perineurium, endoneurium (KNOW THESE!) disease affecting Schwann cells leading to loss of myelin destruction of axon with secondary disintegration of myelin sheath skeletal muscle atrophy myopathy axonal injury occurring as a result of focal lesion affecting distal portion motor neuron

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2.

3 major connective tissue components of peripheral nerve are what?

Thiamine deficiency is associated with axonal neuropathy. A clinical condition termed what? Axonal neuropathies also occur with deficiencies of what vitamins? When do avulsions occur? All forms of spinal muscular atrophy are associated with mutations affecting what? This feature distinguishes dystrophies from myopathies? What happens in a dystrophy? DIFFERENCE BETWEEN DYSTROPHIES AND MYOPATHIES

neuropathic beriberi

3.

What happens in segmental demyelination?

17.

Vitamins B12, B6, & E

4.

What happens in axonal degeneration?

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when tension is applied to peripheral nerve

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survival motor neuron I

5.

When does denervation atrophy occur? Primary abnormality of the muscle fiber itself is what? What is Wallerian degeneration?

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Histologically, in advanced cases muscle fibers undergo degeneration and are replaced by fibrofatty tissue and collagen. muscle fibers undergo degeneration and are replaced by fibro-fatty tissue and collagen - Muscular dystrophies: are a heterogeneous group of inherited disorders of muscle, often beginning in childhood, that lead to progressive weakness and muscle wasting. Histologically, in advanced cases muscle fibers undergo degeneration and are replaced by fibrofatty tissue and collagen. - Myopathies: are pathologic processes seen in skeletal muscle due to a primary abnormality of the muscle fiber itself. - Duchenne's Muscular Dystrophy (DMD) - Becker's Muscular Dystrophy (BMD)

8.

What determines the type of muscle (fast/slow)? When do you see type 2 atrophy?

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associated with inactivity or disuse or during therapy with steroids Guillain-Barre Syndrome

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10.

Disease characterized by weakness beginning in distal limbs, which advanced to proximal muscle function? What cells are involved in leprosy? What types of fibers are involved in Leprosy? In Charcot-Marie-Tooth disease (HMSN I), demyelinating type, usually presents in childhood or early adulthood. Where is muscle weakness seen? Type III HMSN, what parts of the body are effected? The most common peripheral neuropathy in type II diabetes involves what types of nerves?

11. 12.

Schwann cells pain fibers below the knee

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13.

2 most common forms of muscular dystrophy? What is the protein effected in muscular dystrophies?

24.

dsytrophin

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trunk and limb muscles distal sensory and motor nerves

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25.

What does dystrophin do?

forms interface between intracellular contractile apparatus and extracellular connective tissue matrix; links outside the cell to inside the cell stiffness, difficulty releasing grip,; myotonia (sustained involuntary contraction) Abnormalities of carnitine transport or deficiencies of the mitochondrial dehydrogenase enzyme systems can lead to blocks in fatty acid oxidation and accumulation of lipid droplets within muscle (lipid myopathies). Patients with these disorders develop muscle pain, tightness, and myoglobinuria following prolonged exercise or exercise during fasting states. Fatty acids provide energy for muscle contraction, especially when glycogen stores are depleted (as in fasting). With a metabolic block in fatty acid oxidation, the required energy is not available, resulting in symptoms. Concomitant cardiomyopathies and fatty liver may also occur. infectious, non-infectious, and systemic

31.

Muscle disease in which there is immune mediated loss of acetylcholine receptor? What cancer is typically associated with Lambert-Eaton myasthenic syndrome?

Myasthenia gravis small cell carcinoma of the lung

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26.

What happens clinically in myotonic dystrophy? Lipid Myopathies:

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28.

What are the 3 subgroups of inflammatory muscle disease? Grotton lesions are associated with what disease? >> What are Grotton lesions? >> What is dermatomyositis?

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dermatomyositis >> scaling erythematous eruption or dusky red patches over the knuckles, elbows, and knees

30.

an inflammatory disorder of the skin as well as skeletal muscle. It is characterized by a distinctive skin rash that may accompany or precede the onset of muscle disease. The classic rash takes the form of a lilac or heliotrope discoloration of the upper eyelids associated with periorbital edema (Fig. 27-13A). It is often accompanied by Grotton lesions. Muscle weakness is slow in onset, bilaterally symmetric, and often accompanied by myalgias. It typically affects the proximal muscles first.