Download Sherlock Holmes - Ipswich-Year2-Med-PBL-Gp-2

“Once you eliminate the impossible,
whatever remains, no matter how
improbable, must be the truth.”
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A 25 year old woman is brought to the emergency department after
tripping during a volleyball match. Her teammate notes that she had been
stumbling.
On arrival in ED, she can no longer raise her legs and labours to adjust
herself in bed. She has also begun to complain of shortness of breath.
She denies fever but states that three weeks ago the entire team suffered
from abdominal cramps and diarrhoea after a championship BBQ
Hx
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PMH: G1P1 with no complications
PSHx, All, FHx, CIGS, ALC – all nil
Examination:
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T = 36.6oC; HR 50/min; RR 26/min; BP 90/60mm Hg
CVS, ABDO, Skin: NAD
RESP: poor inspiratory effort, lungs, airways clear
CN’s intact
Neuro upper limb (P: 3,3 10 sec, R:0+)
Neuro lower limb (P:1,1, R: 0+, S: pain and light touch diminished to her knees)
“It is a capital mistake to theorise before one has data”
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Describe the accident (how did you land?)
First signs?
Duration?
Sexual Hx (?pregnancy, unsafe sex?)
Fever, sweats, night sweats, weight loss?
Urine output, fluid intake (dehydration?)
Headache, dizziness, nausea, photophobia?
Possible precipitating factors for the
gastroenteritis (type of food?, recent travel?)
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Kernig’s, Brudzinski's
Fundoscopy looking for pappiloedema
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Spirometry
Pulse O2%
LP for protein in CSF
Clostridium jejuni serum Ig and stool culture
CT/ MRI brain and spine
ECG
FBC
Urea and electrolytes
Qual. B-HCG test
Nerve conduction testing
FBC NAD, ELFTs NAD urine HCG neg,
MRI brain & spine NAD
CSF:
Appearance
clear
WBC
1 cells/mm 3 (<5) 65%lymphs, 25% PMNs, 10% monos
RBC
3 cells/mm 3 (none - few)
Protein
150mg/dL (15-40)
Glucose
65mg/dL (50 - 70 ½ to 2/3 blood glucose level)
Gram stain
negative
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Firstly, is this a LMN lesion of UMN lesion?
The woman has decreased muscle tone (including muscles of respiration),
absent muscle stretch reflexes and sensory disturbances. She is fully
conscious although she looks quite weak – it’s probably not a problem
with her brain; this suggests Lower Motor Neuron disease (if UMN
would probably be unilateral with increased reflexes and tone).
Her lower limbs seem more affected than her upper limbs – ascending
paralysis. She is not shown to be favouring one side – we can assume I
think that she has bilateral weakness.
Interestingly her cranial nerves are all intact but her autonomic nervous
system seems disrupted
Disorders of the brain that cause acute weakness include acute stroke,
space occupying lesion, or an inflammatory or infectious processes.
Stroke seems unlikely for so many reasons, and clinically, symptoms are
not unilateral and not including speech changes, and altered mental
status. A space occupying lesion would present with symptoms of
decreased level of consciousness, headache, ophthalmoparesis/ blown
pupil etc.
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Respiratory distress with poor inspiratory
effort, tachypnoea BUT bradycardia and low
BP!!!
Ascending flaccid paralysis within a short
period (days or weeks, not months)
Reflexes absent
Cytoalbumin dissociation (increased protein
with low cell count in CSF)
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Considering the woman’s history of abdominal infection/ food
poisoning, the acutely ascending bilateral flaccid paralysis and
areflexia, a diagnosis of Guillain-Barré Syndrome should be
considered. GBS affects the population in a bimodal fashion (1535yrs and 50-75yrs).
Autonomic involvement in GBS is variable, and this may account
for her bradycardia and hypotension. This diagnosis is supported
by the elevated protein and the low number of cells in CSF.
The absence of fever is important, steering the diagnosis away
from an infective one.
Pregnant ladies are at a higher risk of getting GBS, and the risk
increases after delivery for about 2 weeks.
About 40% of GBS cases are found to have positive C. jejuni serum
antibodies and/or stool cultures. There are 4 types of GBS - Acute
Inflammatory Demyelinating Polyneuropathy (AIDP), MillerFisher variant, Acute Motor Axonal Neuropathy (AMAN) and
Acute Motor-Sensory Axonal Neuropathy (AMSAN). AIDP is the
most common subtype.
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What features indicate severity of this
condition:
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Rapid progression of paralysis
Poor inspiratory effort
The condition is a medical emergency because
patient is at risk of respiratory failure as well as
cardiac arrest due to autonomic failure (can
you propose a mechanism for this?)
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Respiratory support (intubation, mechanical
ventilation)
Cardiovascular support if needed
Plasmapheresis (plasma transfusion)
Intravenous immunoglobulin (IVIG)
Prednisone is ineffective
Expect partial recovery in 2-3 weeks with
possibility of months of rehab followup
Mortality rate 2-5% (poor prognostic factors are
older age, rate of progress of disease, level of
autonomic dysfunction)
“There is nothing more deceptive than an obvious
fact”