SHERLOCK HOLMES“Once you eliminate the

impossible, whatever remains, no matter how improbable,

must be the truth.”

The Case of the Stumbling Sportswoman

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 PMH: G1P1 with no complications PSHx, All, FHx, CIGS, ALC – all nil

Examination: 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”

Further Questions to Ask

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?)

Possible Further Examinations

Kernig’s, Brudzinski's Fundoscopy looking for pappiloedema

What investigations are required?

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

Investigations performedFBC NAD, ELFTs NAD urine HCG neg, MRI brain & spine NADCSF:

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

Can you localise the lesion?

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.

Summary of Key Positives

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)

Guillain-Barré Syndrome 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 (15-35yrs 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), Miller-Fisher variant, Acute Motor Axonal Neuropathy (AMAN) and Acute Motor-Sensory Axonal Neuropathy (AMSAN). AIDP is the most common subtype.

How would you triage this patient?

What features indicate severity of this condition: 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?)

Briefly outline your treatment plan for this patient.

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”