Duke PA Neuromuscular Diseases
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| signs and symptoms; loss of dexterity, increased muscle tone, spasticity, hyperactive DTR, babinski sign | Upper Motor Neuron disease
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| signs and symptoms; weakness, decreased muscle tone, muscle atrophy, fasciculations, reduced or absent reflexes | lower motor neuron disease
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| combination of upper and lower motor neuron disease | Amyotrophic Lateral Sclerosis
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| __% of cases of ALS are sporadic (non-hereditary) | 90
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| incidence of ALS __/100,000/year | 2
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| focal, painless limb weakness that spreads to contiguous areas over several months. Distal muscles often affected first. No sensory symptoms. Head drop, foot drop, hand weakness | ALS
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| the only FDA approved pharmacotherapy for ALS | Riluzole
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| __% of patients with ALS die withing within 5 years of symptom onset | 60
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| pure upper motor neuron syndrome | Primary Lateral Sclerosis
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| leg stiffness/weakness, spasticity, legs usually affected before the arms, ultimately spreads to arms and bulbar muscles | PLS
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| hyperreflexia, unilateral onset of leg symptoms, dysphagia/dysarthria, late onset of emotional lability, impaired ocular smooth peruit, urinary urgency, subclinical abnormalities in frontal lobe function | PLS
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| pure lower motor neuron syndrome | Kennedy's disease
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| facial fasiculations, weakness of mouth and tongue, dysphagia, limb weakness, gynecomastia, diabetes mellitus, oligospermia | Kennedy's disease
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| autoantibodies are produce against the Ach receptors on the sarcolema | Myasthenia Gravis pathology
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| fluctuating weakness of specific muscles (worse with repetition, improves with rest), drooping of eyelids (ptosis), double vision, difficulty chewing or swallowing, tired facial appearance, difficulty smiling/whistling, keeping food in mouth. | Myasthenia Gravis
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| asymmetric proximal or distal extremity weakness, difficulty holding or lifting head, shortness of breath are generalized symptoms of __ | Myasthenia Gravis
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| all patients suspected with myasthenia gravis should be have a CT to rule out __ | thymoma
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| first line treatment for Myasthenia Gravis | steroids
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| medications to avoid in patients with myasthenia gravis | neuromuscular blocking agents, quinine, quinidine, procainamide, antibiotics (aminoglycosides (tobra, gent), tetracyclines, telithromycin, quinolones (cipro, levo)), botox, beta blockers, calcium channel blockers, magnesium , IV contrast
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| every patient with LEMS needs to be screened for __ | cancer
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| paraneoplastic (small cell lung cancer), proximal weakness and autonomic symptoms such as dry mouth, hypo/absent reflexes, weakness | Lambert-Eaton Myasthenic Syndrome (LEMS)
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| voltage gated calcium channel antibodies-pathology of | Lambert-Eaton Myasthenic Syndrome (LEMS)
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| most potent neurotoxin known to man is produced by __ | clostridium botulinum
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| infant botulism caused by ingestion of __ laden with spores | honey
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| botulinum toxin leads to __ | irreversible blockade of acetylcholine release
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| symptoms within 24 hours, diplopia, ptosis, dilated pupils, facial and respiratory weakness, descending paralysis, autonomic dysfunction, patients take months to recover | botulism
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| inherited muscle disorders characterized by muscle weakness and wasting | muscular dystrophies
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| muscle membrane protein needed for stabilization | dystrophin
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| absence of dystrophin | Duchenne muscular dystrophy
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| reduced dystrophin | Becker muscular dystrophy
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| incidence of Duchenne and Becker 1/__ live born males | 3500
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| calf pseudohypertrophy, loss of ambulation by early teens, cardiomyopathy, death in early 20's | Duchenne muscular dystrophy
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| the way a child with Duchenne/Becker MD stands up, by using hands to walk his upper body up his legs | Gower's sign
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| milder course, remain ambulatory until late teens, cardiomyopathy may be more disabling than weakness | Becker muscular dystrophy
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| 3rd most common muscular dystrophy | facioscapulohumeral (FSHD)
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| sensorineural hearing loss, vascular disease of the retina, epilepsy and cognitive impairment in rare cases, no effect on life expectancy, scapular winging and sleeping with eyes open are often first symptoms | facioscapulohumeral (FSHD)
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| contractures of the ankles, neck, and elbows, scapular winging, deltoid sparing, 40% die from sudden cardiac death | Emery-Dreifuss muscular dystrophy
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| 2nd most common muscular dystrophy | Myotonic Dystrophy type I
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| facial weakness (tented upper lip), weakness in distal muscles, cardiac conduction defects, frontal balding, early cataracts, diabetes mellitus, infertility, cognitive impairment | myotonic dystrophy
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| difficult to relax the muscle after contraction | myotonia
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| __ is present in high concentrations in the sarcoplasm of muscle and may leak into blood to serve as a sensitive indicator of muscle damage. | creatinine kinase (CK)
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| in patients with active __ the serum CK is invariably elevated. | muscle damage
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| typical examples of neuromuscular diseases without upper motor neruon involvment | spinal muscular atrophy, poliomyelitis, west nile virus
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| typical examples of neuromuscular diseases with upper motor neuron involvment | ALS
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| typical example of a unifocal peripheral neuromuscular disease | carpal tunnel syndrome
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| typical example of a multifocal peripheral neuromuscular disease | mononeuritis multiplex (polyarteritis nodosa)
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| typical example of a diffuse peripheral neuromuscular disease | diabetic neuropathy, charcot-marie-tooth disease
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| typical example of a neuromuscular junction disease | myasthenia gravis
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| typical example of a muscle disease | Duchenne's muscular dystrophy, dermatomysositis
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| spontaneous electromyographic activity in a resting muscle occurs in __ | myotonic disorders, inflammatory myopathies, and denervated muscles
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| spontaneous activity of a single muscle fiber is called a __ | fibrillation
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| spontaneous activity of part of or an entire motor unit is called a __ | fasciculation
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| in __ repeated muscle depolarization and contraction occur despite voluntary relaxation | myotonia
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| in the repetitive stimulation test a pattern of decrement followed by increment is characteristic of __ | myasthenia gravis
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| lower motor neurons are located in the brain stem and in the ventral spinal cord and when diseased produce __ | decreased strength, tone, and reflexes accompanied by fasciculations and atrophy
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| the most common acquired motor neuron disease | amyotrophic lateral sclerosis
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| if only the lower motor neuron is affected the term __ is used | spinal muscular atrophy
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| __ are hereditary, progressive motor neuron disorders that begin in utero, infancy, childhood, or adult life | spinal muscular atrophies
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| trouble lifting objects, washing or brushing hair are symptoms that point to | proximal weakness
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| the most common site of weakness in a myopathy | proximal weakness
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| patients who complain of a generalized global weakness or fatigue seldom have a __, particularly if the neurologic examination is normal | myopathy
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| rarely is a muscle disease responsible for __ if strength is normal | vague aches and discomfort in muscle
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| __ is the phenomenon of impaired relaxation of muscle after forceful voluntary contraction. | myotonia
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| __ follows rapid muscle destruction | myoglobinuria
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| in __ the calves enlarge as a result of pseudohypertrophy from replacement with connective tissue and fat | Duchenne's and Becker's dystrophies
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| the __ should be normal in muscle disease | sensory exam
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| reflexes are preserved early in muscle disease, but when muscles become extremely weak, reflexes become | hypoactive or unelicitable
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| most common pattern of muscle weakness in myopathies | proximal muscles of the arms and legs: a limb girdle distribution. Neck flexor and extensor muscles can also be affected.
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| __ are inherited myopathies characterized by progressive muscle weakness and degeneration and subsequent replacement by fibrous and fatty connective tissue | muscular dystrophies
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| clinical findings common in the __ are reduced muscle bulk, slender body build, and a long, narrow face, with skeletal abnormalities and absent or reduced muscle stretch reflexes | congenital myopathies
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