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Flashcards in Neuromuscular Junction Deck (24)
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0
Q

What kind of disorder is Lambert Eaton?

A

Lambert Eaton Is a presynaptic neuromuscular junction disorder which demonstrates post exercise facilitation meaning at least 100% increase in first response or CMAP immediately following exercise.
OFTEN ASSOCIATED WITH AUTONOMIC SYMPTOMS SUCH AS DRY MOUTH

1
Q

Where is the pathology in myasthenia gravis?

A

The postsynaptic neuromuscular junction Disorder.

Often associated with ptosis and extra ocular muscle weakness

2
Q

Why does spasticity occur in ALS?

A

Involvement of the vestibulospinal and reticulospinal tracts

3
Q

What is the median survival rate for ALS after diagnosis?

A

50% survival rate is 2.5 years after diagnosis.
Largely dependent on decision to use mechanical ventilation and/or feeding tube.
Five-year survival rate is between 4 to 30% and 10% will live for 10 years.

4
Q

How does riuzole all work?

A

Anti-glutamate agent may be effective in slowing down the disease, prolonging ventilator time, and may improve survival in patients with bulbar onset disease.

However side effects can include asthenia and the medication is expensive

5
Q

Describe SMA type 1 a.k.a. Werdnig- Hoffman disease.

A
  • acute infantile onset SMA.
  • severe disease often results in death by age 2-3 yrs.
  • Disease onset is usually 3 to 6 months
  • May never sit independently.
  • rapid and fatal secondary to respiratory failure.

-SMA 2-3 have slower progression and later onset

6
Q

What is kugelberg-Welanders disease?

A

SMA 3

  • Lower motor neuron
  • Onset typically between 2 and 15 years of age (later than type 1&2)
  • typically normal life expectancy with slower progression than the other two variance.
  • Patients usually achieve independent standing/walking.
  • Kids usually have normal intelligence.
  • Complications can include hand tremor, tongue fasciculations and areflexia.
7
Q

What is chronic Werdnig- Hoffman?

A

SMA 2

  • Disease onset is usually between two and 12 months and death (often by respiratory failure) occurs by 10 years of age.
  • These patients can usually achieve milestones including independent sitting.
  • They may be able to stand or walk with assistive devices.
8
Q

What is the incidence of ALS?

A

1.6-2.4/100,000 population

9
Q

What is the Halstead and Rossi criteria for a diagnosis of post polio syndrome?

A
  1. Confirmed history of poliomyelitis
  2. Partial to fairly complete neurologic and functional recovery.
  3. A period Of neurologic and functional stability of at least 15 years in duration.
  4. Onset of two or more of the following health problems since achieving a period of stability:
    - unaccustomed 15
    - muscle and or joint pain
    - new weakness and muscles previously affected and or unaffected
    - functional loss cold intolerance
    - new atrophy
  5. No other medical diagnosis to explain these health issues.
10
Q

How does Mestinon (pyridostigmine) work?

A

Mestinon reversibly binds to and inactivates acetylcholinesterase, which is a cholinesterase inhibitor.

Other treatment options for myasthenia gravis including thymectomy, corticosteroids, immunosuppressive agents, and plasmapheresis

11
Q

Which muscular dystrophy’s are known for cardiac involvement?

A

Beckers and Duchenne usually have an associate cardiomyopathy. cardiomyopathy may also be seen in a limb girdle muscular dystrophy. However it is not usually seen in fshd.

12
Q

Describe facial scapulohumeral dystrophy.

A

Autosomal dominant
Dx childhood early adult

Proximal muscle weakness with facial muscle weakness. 
Popeye arm
 with cataracts 
inability to whistle. 
Unable to extend the wrist often with elbow contractions
-protuberant scapula
-Frontal balding 
-testicular atrophy
-lordosis
-pelvic girdle weakness
Deafness
-fundal changes
-some patients may benefit from surgery that fixes the scapula to facilitate abduction of the arms
13
Q

What is on the differential for a muscle biopsy showing type one fiber Atrophy?

A

Myotonic dystrophy, Nemaline red myopathy, fiber type disproportion

14
Q

What is on the differential for muscle biopsy showing type 2 fiber atrophy?

A

Steroid myopathy, myasthenia gravis, deconditioning

15
Q

Describe Emery-Dreifus muscular dystrophy.

A

X-linked and slowly progressive myopathy caused by abnormality in the protein Emerin.

  • patient usually present in teenage years with early elbow flexion contracture’s ankle equinus, rigid spine and neck extension contractures.
  • also associated with cardiac arrhythmias usually by age 40 at which time all patients should have a Holter monitor and ECG.
16
Q

What muscle does Facio scapulohumeral dystrophy often spare?

A

Deltoid.

- Inability to extend the wrist is characteristic posture adopted by the patients.

17
Q

Describe central core disease

A

Autosomal dominant myopathy.

  • presents in infancy
  • *****associated with malignant hyperthermia **
  • muscle biopsy shows type one muscle fibers with absent mitochondria
  • congenital hip dislocation
  • not meeting childhood milestones
  • proximal weakness
18
Q

What is important clinical step with diagnosis of dermatomyositis?

A

Search for associated carcinoma with incidence as high as 10 to 20% in dermatomyositis cases.
specially male 40 years of age and older

19
Q

Name the three primary types of inflammatory myopathy

A

Polymyositis, dermatomyositis, and inclusion body myositis.

  • all thought to be immune mediated processes perhaps triggered by environmental factors in genetically susceptible individuals.
  • Age of onset for idiopathic inflammatory myopathy is bimodal
  • peaks occur between 10 and 15 years of age and 45 to 60 years of age.
20
Q

What condition do you think Of with proximal weakness and Heliotrope rash?

A

Primary idiopathic dermatomyositis typically present with proximal weakness and Heliotrope rash on the face. A rash can also be noted on the extensor surfaces of the knees, elbows, ankles, and dorsal surface of the hands

21
Q

Describe inclusion body myositis.

A

Inflammatory myopathy

  • asymmetric slowly progressive painless weakness
  • proximal and distal muscles
  • associated with polyneuropathy

-unknown etiology.

  • refractory to ***steroid treatment and immunosuppressive agents.
  • Treatment is usually rehabilitation with assistive devices.
22
Q

Describe polymyositis and dermatomyositis

A

Inflammatory myopathy

  • etiology: autoimmune connective tissue disorder, infection, cancer
  • clinical presentations with symmetric proximal weakness hips followed by shoulders
  • neck flexion Weakness
  • myalgias,dysphasia, dysphonia
  • no facial or ocular weakness
  • dermatomyositis
  • with Periorbital Violet rash and edema
  • gottron’s sign: red-purple patches over the knuckles elbows and knees
  • muscle biopsy in the process of type one and type two fibers and Perry fascicular atrophy
  • treat with corticosteroids, so toxic agents, plasmapheresis, rest, rehabilitation
23
Q

Is the second most common myopathy?

A

Myotonic dystrophy (Steinhert’s)