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DU PA Motor Neuron D

Duke PA Neuromuscular Diseases

signs and symptoms; loss of dexterity, increased muscle tone, spasticity, hyperactive DTR, babinski sign Upper Motor Neuron disease
signs and symptoms; weakness, decreased muscle tone, muscle atrophy, fasciculations, reduced or absent reflexes lower motor neuron disease
combination of upper and lower motor neuron disease Amyotrophic Lateral Sclerosis
__% of cases of ALS are sporadic (non-hereditary) 90
incidence of ALS __/100,000/year 2
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
the only FDA approved pharmacotherapy for ALS Riluzole
__% of patients with ALS die withing within 5 years of symptom onset 60
pure upper motor neuron syndrome Primary Lateral Sclerosis
leg stiffness/weakness, spasticity, legs usually affected before the arms, ultimately spreads to arms and bulbar muscles PLS
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
pure lower motor neuron syndrome Kennedy's disease
facial fasiculations, weakness of mouth and tongue, dysphagia, limb weakness, gynecomastia, diabetes mellitus, oligospermia Kennedy's disease
autoantibodies are produce against the Ach receptors on the sarcolema Myasthenia Gravis pathology
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
asymmetric proximal or distal extremity weakness, difficulty holding or lifting head, shortness of breath are generalized symptoms of __ Myasthenia Gravis
all patients suspected with myasthenia gravis should be have a CT to rule out __ thymoma
first line treatment for Myasthenia Gravis steroids
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
every patient with LEMS needs to be screened for __ cancer
paraneoplastic (small cell lung cancer), proximal weakness and autonomic symptoms such as dry mouth, hypo/absent reflexes, weakness Lambert-Eaton Myasthenic Syndrome (LEMS)
voltage gated calcium channel antibodies-pathology of Lambert-Eaton Myasthenic Syndrome (LEMS)
most potent neurotoxin known to man is produced by __ clostridium botulinum
infant botulism caused by ingestion of __ laden with spores honey
botulinum toxin leads to __ irreversible blockade of acetylcholine release
symptoms within 24 hours, diplopia, ptosis, dilated pupils, facial and respiratory weakness, descending paralysis, autonomic dysfunction, patients take months to recover botulism
inherited muscle disorders characterized by muscle weakness and wasting muscular dystrophies
muscle membrane protein needed for stabilization dystrophin
absence of dystrophin Duchenne muscular dystrophy
reduced dystrophin Becker muscular dystrophy
incidence of Duchenne and Becker 1/__ live born males 3500
calf pseudohypertrophy, loss of ambulation by early teens, cardiomyopathy, death in early 20's Duchenne muscular dystrophy
the way a child with Duchenne/Becker MD stands up, by using hands to walk his upper body up his legs Gower's sign
milder course, remain ambulatory until late teens, cardiomyopathy may be more disabling than weakness Becker muscular dystrophy
3rd most common muscular dystrophy facioscapulohumeral (FSHD)
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)
contractures of the ankles, neck, and elbows, scapular winging, deltoid sparing, 40% die from sudden cardiac death Emery-Dreifuss muscular dystrophy
2nd most common muscular dystrophy Myotonic Dystrophy type I
facial weakness (tented upper lip), weakness in distal muscles, cardiac conduction defects, frontal balding, early cataracts, diabetes mellitus, infertility, cognitive impairment myotonic dystrophy
difficult to relax the muscle after contraction myotonia
__ 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)
in patients with active __ the serum CK is invariably elevated. muscle damage
typical examples of neuromuscular diseases without upper motor neruon involvment spinal muscular atrophy, poliomyelitis, west nile virus
typical examples of neuromuscular diseases with upper motor neuron involvment ALS
typical example of a unifocal peripheral neuromuscular disease carpal tunnel syndrome
typical example of a multifocal peripheral neuromuscular disease mononeuritis multiplex (polyarteritis nodosa)
typical example of a diffuse peripheral neuromuscular disease diabetic neuropathy, charcot-marie-tooth disease
typical example of a neuromuscular junction disease myasthenia gravis
typical example of a muscle disease Duchenne's muscular dystrophy, dermatomysositis
spontaneous electromyographic activity in a resting muscle occurs in __ myotonic disorders, inflammatory myopathies, and denervated muscles
spontaneous activity of a single muscle fiber is called a __ fibrillation
spontaneous activity of part of or an entire motor unit is called a __ fasciculation
in __ repeated muscle depolarization and contraction occur despite voluntary relaxation myotonia
in the repetitive stimulation test a pattern of decrement followed by increment is characteristic of __ myasthenia gravis
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
the most common acquired motor neuron disease amyotrophic lateral sclerosis
if only the lower motor neuron is affected the term __ is used spinal muscular atrophy
__ are hereditary, progressive motor neuron disorders that begin in utero, infancy, childhood, or adult life spinal muscular atrophies
trouble lifting objects, washing or brushing hair are symptoms that point to proximal weakness
the most common site of weakness in a myopathy proximal weakness
patients who complain of a generalized global weakness or fatigue seldom have a __, particularly if the neurologic examination is normal myopathy
rarely is a muscle disease responsible for __ if strength is normal vague aches and discomfort in muscle
__ is the phenomenon of impaired relaxation of muscle after forceful voluntary contraction. myotonia
__ follows rapid muscle destruction myoglobinuria
in __ the calves enlarge as a result of pseudohypertrophy from replacement with connective tissue and fat Duchenne's and Becker's dystrophies
the __ should be normal in muscle disease sensory exam
reflexes are preserved early in muscle disease, but when muscles become extremely weak, reflexes become hypoactive or unelicitable
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.
__ are inherited myopathies characterized by progressive muscle weakness and degeneration and subsequent replacement by fibrous and fatty connective tissue muscular dystrophies
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
Created by: bwyche