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Ask Ethan about the Wu Tang/muscular dystrophy connection
• A 23-year old female student notices double vision while studying for
  her examinations. She goes to see an ophthalmologist, who said she
  had a "Iazy eye." These symptoms come and go for the next 2 years
  without becoming significant. Then, she starts to have difficulties with
  chewing food, and has troubles with getting in and out of the car.
  SIowly, problems with using her arms and legs develop. She thinks she
  is out of shape and needs more exercise. She begins spilling drinks and
  missing her mouth when using utensils. Her vision is double by
  afternoon each day, and by the evening, one eye is closed. The next
  morning, her situation is improved, but again worsens by the evening.
  Finally, she goes to see a neurologist. On examination, ptosis is noted.
  The ptosis worsens when she is asked to sustain an upward gaze, and
  closing her eyes for a short period improves it. Her voice has a nasal
  quality. Sensory examination and deep tendon reflexes are normaI.
  Which is the most likely diagnosis?
A) Botulism
B) Guillain-Barré syndrome
C) Lambert-Eaton syndrome
D) Myasthenia gravis
E) Wernicke syndrome
• A 23-year old female student notices double vision while studying for
  her examinations. She goes to see an ophthalmologist, who said she
  had a "Iazy eye." These symptoms come and go for the next 2 years
  without becoming significant. Then, she starts to have difficulties with
  chewing food, and has troubles with getting in and out of the car.
  SIowly, problems with using her arms and legs develop. She thinks she
  is out of shape and needs more exercise. She begins spilling drinks and
  missing her mouth when using utensils. Her vision is double by
  afternoon each day, and by the evening, one eye is closed. The next
  morning, her situation is improved, but again worsens by the evening.
  Finally, she goes to see a neurologist. On examination, ptosis is noted.
  The ptosis worsens when she is asked to sustain an upward gaze, and
  closing her eyes for a short period improves it. Her voice has a nasal
  quality. Sensory examination and deep tendon reflexes are normaI.
  Which is the most likely diagnosis?
A) Botulism
B) Guillain-Barré syndrome
C) Lambert-Eaton syndrome
D) Myasthenia gravis
E) Wernicke syndrome
• Progressive fatigability, ptosis, bulbar
  involvement, reflex-sparing
• GBS often presents with somatosensory
  involvement
• LEMS rarely has bulbar involvement
• The underlying pathophysiology in this disease
  involves which of the following mechanisms?
A) Antibodies to nicotinic acetylcholine
   receptors
B) Antibodies to voltage-gated calcium channels
C) Bacterial toxin
D) Infection-induced autoimmune response
E) Thiamine deficiency
• The underlying pathophysiology in this disease
  involves which of the following mechanisms?
A) Antibodies to nicotinic acetylcholine
   receptors
B) Antibodies to voltage-gated calcium channels
C) Bacterial toxin
D) Infection-induced autoimmune response
E) Thiamine deficiency
• MG is progressive, autoimmune disease
  targeting nAChR
• LEMS is VGCC
• Botulinum toxin attacks SNARE
• GBS is post-infection
• Wernicke encephalopathy is associated with
  thiamine deficiency
Questions?

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Nsb review 04

  • 1. NSB Review Ask Ethan about the Wu Tang/muscular dystrophy connection
  • 2. • A 23-year old female student notices double vision while studying for her examinations. She goes to see an ophthalmologist, who said she had a "Iazy eye." These symptoms come and go for the next 2 years without becoming significant. Then, she starts to have difficulties with chewing food, and has troubles with getting in and out of the car. SIowly, problems with using her arms and legs develop. She thinks she is out of shape and needs more exercise. She begins spilling drinks and missing her mouth when using utensils. Her vision is double by afternoon each day, and by the evening, one eye is closed. The next morning, her situation is improved, but again worsens by the evening. Finally, she goes to see a neurologist. On examination, ptosis is noted. The ptosis worsens when she is asked to sustain an upward gaze, and closing her eyes for a short period improves it. Her voice has a nasal quality. Sensory examination and deep tendon reflexes are normaI. Which is the most likely diagnosis? A) Botulism B) Guillain-Barré syndrome C) Lambert-Eaton syndrome D) Myasthenia gravis E) Wernicke syndrome
  • 3. • A 23-year old female student notices double vision while studying for her examinations. She goes to see an ophthalmologist, who said she had a "Iazy eye." These symptoms come and go for the next 2 years without becoming significant. Then, she starts to have difficulties with chewing food, and has troubles with getting in and out of the car. SIowly, problems with using her arms and legs develop. She thinks she is out of shape and needs more exercise. She begins spilling drinks and missing her mouth when using utensils. Her vision is double by afternoon each day, and by the evening, one eye is closed. The next morning, her situation is improved, but again worsens by the evening. Finally, she goes to see a neurologist. On examination, ptosis is noted. The ptosis worsens when she is asked to sustain an upward gaze, and closing her eyes for a short period improves it. Her voice has a nasal quality. Sensory examination and deep tendon reflexes are normaI. Which is the most likely diagnosis? A) Botulism B) Guillain-Barré syndrome C) Lambert-Eaton syndrome D) Myasthenia gravis E) Wernicke syndrome
  • 4. • Progressive fatigability, ptosis, bulbar involvement, reflex-sparing • GBS often presents with somatosensory involvement • LEMS rarely has bulbar involvement
  • 5.
  • 6. • The underlying pathophysiology in this disease involves which of the following mechanisms? A) Antibodies to nicotinic acetylcholine receptors B) Antibodies to voltage-gated calcium channels C) Bacterial toxin D) Infection-induced autoimmune response E) Thiamine deficiency
  • 7. • The underlying pathophysiology in this disease involves which of the following mechanisms? A) Antibodies to nicotinic acetylcholine receptors B) Antibodies to voltage-gated calcium channels C) Bacterial toxin D) Infection-induced autoimmune response E) Thiamine deficiency
  • 8. • MG is progressive, autoimmune disease targeting nAChR • LEMS is VGCC • Botulinum toxin attacks SNARE • GBS is post-infection • Wernicke encephalopathy is associated with thiamine deficiency