Flashcards in 14. Neuromuscular Disease Deck (40)
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1
neuropathy
damage or disease involving fibers of the nervous system. may affect sensation, movement, gland or organ function.
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myopathy
a muscular disease[1] in which the muscle fibers do not function for any one of many reasons, resulting in muscular weakness.
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neuromuscular junction
connects the nervous system to the muscular system via synapses between efferent nerve fibers and muscle fibers, also known as muscle cells.
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"dying back" neuropathy
neuropathy affecting distal neurons first; toxins or metabolic deficiencies can affect longest nerve fibers first because longer nerve fibers require more energy to maintain functin, and because there is a greater opportunity to injure a longer nerver fiber
5
Wallerian degeneration
a process that results when a nerve fiber is cut or crushed, in which the part of the axon separated from the neuron's cell body degenerates distal to the injury
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epineurium
The epineurium is the outermost layer of dense irregular connective tissue surrounding a peripheral nerve.[1] It usually surrounds multiple nerve fascicles as well as blood vessels which supply the nerve. Smaller branches of these blood vessels penetrate into the perineurium.
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perineurium
In the peripheral nervous system, nerve fibers are each wrapped in a protective sheath known as the endoneurium. These are bundled together into groups known as fascicles, each surrounded by a protective sheath known as the perineurium
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endoneurium
The endoneurium is a layer of delicate connective tissue made up of endoneurial cells that encloses the myelin sheath of a spinal cord nerve fiber.
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schwann cells
glial cells of the PNS
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entrapment neuropathy
compression of a nerve resulting in myelin disruption. could be due to mechanical abnormalities or swelling of tissues
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where is a common site for an entrapment neuropathy?
carpal tunnel
12
lateral femoral cutaneous neuropathy
aka meralgia paresthetica, entrapment between ilium and inguinal ligament.
13
other sites of compression neuropathies?
ulnar passing behind the epicondyle, deep branch of the ulnar passing wrist, radial nerve at spiral groove of upper arm, peroneal nerve at head of fibula, lateral femoral cutaneous.
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what nerves make up the lateral femoral cutaneous?
L2, L3
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what is a result of lateral femoral cutaneous entrapment?
numbness, burning pain on lateral thigh.
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polyneuropathy
Polyneuropathy is a serious, unpredictable, occasionally progressive, and life threatening neurological disorder[1] that occurs when many nerves throughout the body malfunction simultaneously.
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Charcot Marie Tooth disease
Charcot–Marie–Tooth disease (CMT), also known as Charcot–Marie–Tooth neuropathy, is a genetically and clinically heterogeneous group of inherited disorders of the peripheral nervous system characterised by progressive loss of muscle tissue and touch sensation across various parts of the body.
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Lambert-Eaton myasthenic syndrome: cause?
due to antibodies against the VG calcium channels in the terminals of motor nerves. Ca entry into these terminals is needed for release of Ach. produces weakness, diminished reflexes and autonomic dysfunction
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paraneoplastic sx
associated with cancer. consequence of cancer being in body but not directly related to local cancer cells.
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example of a paraneoplastic sx?
Cushing's, Lambert-Eaton
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myasthenia gravis
autoimmune sx in which antibodies exist against the Ach receptor. means that nerve is unable to activate the muscle.
22
nerve conduction studies do what?
evaluate the speed of nerve conduction and amount of nerve signal that gets through. can test both sensory and motor.
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electromyography
assesses electrical activity of muscle fibers, evaluates health of muscles, can detect muscle fibers that have been denervated.
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large peripheral nerve fibers convey what?
localized touch, pressure, vibration, joint pressure sense
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small nerve fibers convey what?
pain, touch, temp, autonomic
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what are a few causes of polyneuropathy?
infection, diabetes, nutritional prob, alcohol, toxins, meds, hereditary, inflammatory
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what is a hereditary cause of polyneuropathy
Charcot-Marie Tooth
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with polyneuropathy, what reflex is detected early
ankle jerk reflex
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what is a common pattern of polyneuropathy?
distal, symmetrical sensory loss (stocking/glove)
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a few causes of myopathies?
metabolic, infectious, inflammatory, genetic, drug, toxin
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myopathies usually result in what?
proximal and symmetrical weakness (hip, shoulder) and then move on to more distal muscles
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reflexes in myopathies?
reflexes are preseved until very late in disease
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sensation in myopathies?
no sensory loss. muscles may be tender/cramp
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myasthenia gravis: most commonly what population?
young females, old males
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myasthenia: sx?
fatigued muscles, due to damage to Ach receptors by antibody
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myasthenia: how affect resp?
may affect diaphragm, other resp muscles
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MG: treatmetn?
AchE inhibitors, immunosuppressives
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Lambert-Eaton: weakness where?
hip girdle muscles, decr patellar reflexes
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Lambert-Eaton: cause
sometimes small cell cancer, can also be idiopathic
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