Transcript Case- floppy 15 yo

Floppy Adolescent
Joshua Rocker, MD
Schneider Children’s Hospital
Long Island Jewish Medical Center
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15 y/o female presents with sub-acute
onset of ptosis, drooling, inability to
move neck, dysarthria, and generalized
fatigue.
History
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Symptoms started last night- progressively
worsened.
No recent travel
No recent illnesses
No recent bug/tick bites
No new foods
Don’t drink, smoke, drug, have sex
Yes, I am leaving something terribly important out… ha ha
(but what if she was alone and unable to communicate)
Look at numbers before
patient
37.6 C
 BP- 110/80
 HR- 84
 RR- 20, O2 sat-98%.
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Now… look at patient
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PE:
– Significant b/l ptosis, EOMI, vision intact
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(no diplopia), TMs clear, no nasal d/c, no
exudates.
CTA b/l, no retractions, no tachy or
hyperpnea
SS rrr no m,g,r
Soft, NT, ND, no masses, normoactive BS
No rashes, no tick/bug bites
Neuro exam
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Sensory
– normal
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Reflexes
– normal
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Motor
– 4-5/5 distal muscles
– 3/5 proximal muscles
– B/l ptosis, drooling, slurred speech, + gag.
Ddx
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Infections
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– Organophosphates
– Botulism
– Polio
– Tick Paralysis
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Auto-Immune
– Eaton Lambert
– Guillain-Barre
– Myasthenia Gravis
Toxins
– Heavy metals
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Genetic
– SMA (Spinal Muscular
Atrophy)
– Muscular Dystrophy
– Potassium-related
periodic paralysis
– Familial Dysautonomia
(Riley-Day Syndrome)
Exclude Stuff
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Genetic
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– Familial
– Botulism?
Dysautonomia
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Infections
– Polio?
age
– Tick? Travel?
– Potassium-related
periodic paralysis
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duration
– Muscular Dystrophy
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Acuity, age
– SMA
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Type 1-3
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Toxins
– Exposure?
Auto-immune
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Guillain-Barre
– Demylination
– Landry’s ascending
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Eaton Lambert
– M>F
– >40s
– Assoc with malign (small
gradual
– Miller-Fischer!!
– Post-viral (URI/AGE)
– Lost reflexes
– Tap, MRI, EMG
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cell CA, etc)
Proximal m weakness
Dry eyes,skin,mouth
Prickling,tingling
Strength better--worse
with use.
Cause- antibody to CC at
NM junction
Myasthenia Gravis!!!
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Yes, she was diagnosed 6 months ago
and has been non-compliant…
Myasthenia
ACh receptor antibody
 F>>M
 Transient neonatal vs juvenile
 EMG
 Tensilon test
 Avoid: succ, aminoglycosides,
macrolides
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Myasthenia
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Treatment
– Cholinesterase inhibitors
– IVIG
– Steroids
– Plasmapharesis
– Immunosuppresants
– Thymectomy
Myasthenia crisis vs
Myasthenic with Cholinergic
overdose
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Difficult to differentiate
– hx
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Crisis
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Supportive care
Acute treatment with longer acting ACholinest Inh
Cholinergic Overdose with
– SLUDGE
– Unresponsive to ACholinest Inh
Confused? Questions?
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Thank you!!!