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Disease of motor neurons, neuromuscular junction, and muscle
Terms in this set (61)
In ALS, are upper or lower motor neurons affected?
What age group gets ALS?
average age is 65, peak range is 55-75, can occur in teens or 80s though
Is there an ethnic or racial predisposition to ALS?
No except increased prevalence in western pacific
Describe the symptoms of ALS.
most start with problems in one hand, awkwardness in one hand,, difficulty with fine finger movements, stiffness of the fingers, slight weakness, slight atrophy of the hand muscles, cramping and fasciculations beyond normal, weakness then gradually spreads up the arm, the other uppre extremity is affected, the ipsilateral leg, then bulbar and respiratory muslces, eye movements are affected very late
Are bowel and bladder function affected in ALS?
Are eye movements affected in ALS?
very late in the disease
What is pseudobulbar palsy?
emtional incontinence, spontaneous laughing/crying with little provocation and often no emotional component, can't stop once start
Are any sensory symptoms present in ALS?
Describe the neurologic exam findings in ALS.
muscle atrophy and fasciculations, increased muscle stretch reflexes, +Babinski signs, increased tone
Outline how the diagnosis of ALS made
made clinically when significant muscle atrophy and fasciculations are seen in several areas of the body along with increased muscle stretch reflexes, MRI and motor nerve conduction studies to rule out other causes, EMG can help confirm LMN damage
Describe the clinical course of ALS.
progressive and eventually leads to complete paralysis with inability to swallow or breath, most patients dies withing 3-5 years of diagnosis
Name the medication that prolongs survival in ALS. By how long does it prolong survival?
Riluzole, a few months
What other medication is being used in ALS (2nd line/adjuvant drug)? Does it improve survival?
Edaravone, impact on survival is not yet clear
List three diseases that result from a disorder at the neuromuscular junction.
myasthenia gravis, Lamber-Eaton myasthenic syndrome, botulism
Which neuromuscular junction disease is most common?
Describe the basic pathology of myasthenia gravis.
autoantibodies to acetylcholine receptors at the neuromuscular junction
Which muscles are typically affected first in myasthenia gravis?
What happens to the weakness in MG with activity?
What endocrine abnormality is associated with MG?
Tumors of what structure are associated with MG?
Who is most likely to get MG?
Are the pupils affected with MG?
Is there muscle atrophy with MG?
Describe how the diagnosis of MG is made.
If only eye involvement then the ice pack or Tensilon tests are used, acetylcholine receptor antibody testing is done (if negative test for muscle specific tyrosine kinase MuSK), repetitive nerve stimulation is also done (if negative do single fiber EMG)
What else imaging should be done once MG is diagnosed? Why?
CT or MRI of chest to look for thymoma
Outline the management of MG. What medication is used to treat this disorder?
anticholinesterase inhibitors (pyridostigmine), immune modulators are second line if still weak, thymectomy if patient under 60 UNLESS only MuSK antibody positive or if have thymoma
What is done to treat an MG crisis?
plasmpheresis or IVIg
Explain what neonatal MG is.
occurs in infants born to mothers with MG because the acetylchoniesterase receptor antibodies can cross the placenta leading to weakness in the infant, treat with acetylchoinesterase inhibitor, resolves in approximately 2 months
Describe the pathophysiology of LEMS (Lambert Eaton Myasthenic Syndrome).
antibody to the presynaptic voltage-gated calcium channels which prohibits the release of Ach
What happens to the weakness in LEMS with activity?
initially better with activity but then become weaker
Are the eyes involved in LEMS?
Who gets LEMS?
Males with malignancy, most commonly small cell lung cancer
What two diseases are associated with LEMS?
malignancy, small cell lung cancer
Does botulism cause an ascending or descending paralysis?
What is typically the first symptom in patients with botulism?
gasteroenteritis followed by diplopia
Why do these patients with botulism die?
weakness of respiratory muscles
How is botulism treated?
antitoxin - botulinum toxin immunoglobulin, intubation
When should you suspect botulism in an infant?
acute onset of weak suck, ptosis, inactivity, constipation
What happens to the pupils with botulism?
Describe the general signs and symptoms of a myopathy
weakness-usually proximal, sometimes cramps, decreased tone, decreased muscle stretch reflexes
Do patients with muscular dystrophies (MDs) have any sensory complaints?
Are MDs progressive?
Are MDs inherited?
What is the inheritance pattern for Duchenne Muscular Dystrophy?
What blood test helps make the diagnosis of DMD?
highly elevated CPK
What is Gower's maneuver?
using hands to push on legs when standing up indicates DMD
What are 3 things to make you think of DMD?
boys only, big calves, high CPK
Other than skeletal muscle, what two organs are affected in DMD?
dilated cardiomyopathy, bones easily fractured/scoliosis, subnormal IQ
What do the calves look like in people with DMD?
Which is more common: myotonic dystrophy or DMD?
What is the inheritance pattern for myotonic dystrophy type I?
What is myotonia?
inability to relax voluntary muscle after vigorous effort
How is myotonic dystrophy type I diagnosed?
EMG with characteristic findings with needle insertion, Genetic testing for confirmation after abnormal EMG
Does myotonic dystrophy type I affect proximal or distal musculature? Is this typical for a muscle problem?
What other organ systems are affected in myotonic dystrophy type I?
cardiovascular (arrythmias), diabetes, eyes (cataracts)
What is characteristic about the muscle biopsy of all patients with a congenital myopathy?
visible inclusions in muscle cells
Are congenital myopathies progressive? Are they fatal?
no, some are fatal
What is the basic pathology in the periodic paralysis syndromes?
mutations in genes coding for proteins in muscle potassium channels in the muscle membrane leading to an inability to excite a muscle
Which type of periodic paralysis is most common?
Hypokalemic periodic paralysis
What typically precipitates paralysis in patients with periodic paralysis?
heavy exertion, large meals, upon awakening
How long does the periodic paralysis last?
hours to days
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