Neuromuscular Diseases Pt 2

DMD

100

Pattern of inheritance of DMD

X- linked recessive disorder

200

This lab value is generally elevated 10-100x nml value in patients with DMD

Creatine Kinase

300

Age of onset of DMD

3-6y/o 

400

After the age of 10, this is how often DMD patients should be getting an ECG and an echo 

Q1 year 

500

This valvular abnormality is found in 25% of pts with DMD

Mitral Regurg 2/2 papillary muscle dysfunction  

BMD

100

This is the most likely potential complication in a BMD patient undergoing GA

Aspiration 

2/2 gastric hypomotility, increased oral secretions, respiratory muscle weakness 

200

Age of onset of BMD

40-60s

300

This sign is considered positive when arms are used to rise from a seated position on the floor

Gower's Sign 

400

If a patient with BMD has cardiac sxs, this is how often they should get a cardiac evaluation

q2 years 

If no sxs, q5 years 

500

Perioperitavely, patients with BMD are increased risk of this d/t  smooth muscle and platelet dysfunction

blood loss 

Name that disease Pt 2

100

This disease is the result of autoimmune destruction to myelin surrounding the peripheral nerves. Classically, patients present with ascending weakness, and decreased or absent deep tendon reflexes.

Guillain-Barre syndrome (GBS)

200

The most common form of muscular dystrophy

Duchenne's

Accounts for ~50% of MDs

300

This autoimmune disease begins with leg muscle weakness and progresses to weakness in eye, face and throat muscles. Symptoms include difficulty walking, talking, chewing and swallowing. About half of all cases are caused by small cell lung cancer

Lambert-Eaton Myasthenic Syndrome

400

These "Fainting Goats", suffer from this hereditary condition, causing them to stiffen or fall over when excited or startled

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Myotonia Congenita 

500

This aptly named type of muscular dystrophy affects the muscles of the upper arms, upper legs, shoulders, and hips.

Limb-girdle muscular dystrophy

HY for Advanced

100

Among the causes of transfusion related mortality, this is the most common in the United States                     

Transfusion-associated overload (TACO)

200

This may be requested by the surgical team prior to lipping of cerebral aneurysm 

Relative hypertension 

300

All inhaled anesthetics potentiate and reduce the required dose of neuromuscular blockers. This VA used today potentiates NMB the most 

• Desflurane

• des  > sevoflurane > isoflurane

400

This neurolytic block relieves pain arising from organs in the pelvis    

Superior Hypogastric Plexus Block 

500

This is indicated in patients with myxedema coma requiring emergency surgery 

IV L-triiodothyronine  (T3) 

True or False

100

Skipping meals, foods with high potassium content, cold temperatures, or resting post-exercise can exacerbate the attacks if you have this disease

Hyperkalemic Periodic Paralysis 

200

True or False:

Myotonia Congenita is associated with malignant hyperthermia (MH)

False

300

True or False:

Muscular dystrophy is not associated with malignant hyperthermia (MH)

True

400

True or False:

Myotonic Dystrophy has no association with malignant hyperthermia (MH)

True

Historically was thought to be associated but now debunked 

500

True or False:

Congenital Myopathies have no association with malignant hyperthermia (MH)

False

Eg King-Denborough syndrome, Mini-core disease