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Guillain Barré Syndrome

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Ru Bles

on 25 April 2016

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Transcript of Guillain Barré Syndrome

Define weakness...
The inability to carry out a movement with normal force because of reduction in muscle strength. This must be differentiated form fatigue, in which repetitive actions cause diminished strength, either real or perceived.

78 yo M presenting with
a 2/52 Hx of weakness

Activities affected (functional effect)
Related symptoms
- Sudden- suggestive of CV or traumatic cause.
- Acute onset hrs -days - polyneuropathy, myelopathy, myopathy & NMJ disorders
- Unilateral - ?cortical pattern with aphasia/ neglect/ apraxia. ?facial involvement. ?peripheral nerve distribution.
- Bilateral - trauma/ known cancer,
- Generalised - ?systemic illness
- proximal or distal , bulbar
Activities affected
- Bulbar: slurring speech, drooling, difficulty smiling, swallowing
- Proximal LL: climbing stairs, getting up from seating position
- Proximal UL: difficulty reaching/ shaving/ combing hair
- Distal LL: tripping, foot drag
- EOM: diplopia, ptosis
- Hearing/ vision/ olfactory

Related SSx
- Headache
- Sensory dysfunction
- Bladder/ bowel dysfunction
- Seizures

History continued...
- Trauma, travel
- SSx febrile illness
- PMHx cancer/ OD/ drug + alcohol/DM/ CVA/ CVD/ hepatic or renal failure/ thyroid
- Medication Hx: myopath/periph neuropathy
- Similar episodes in the past
He had a 2/52 Hx of mild lower back pain after a fall, pain worsened 2/7 prior when walking dog. 1/7 unable to stand or walk properly due to lower limb weakness & altered sensation in legs. Also c/o urge incontinence and dysuria over 2/12.
C/o pain in hands and wrists bilaterally.
No headache. No visual or hearing disturbances. Denies unilateral predominance. No LOC/ no seizures/ n+v/ neck stiffness/ change in mental status.
Pt reports an episode of slurred speech 4/52 prior which resolved within hours. Did not seek care.
Denies any other recent respiratory/ bowel/ cardiac symptoms. Denies bowel incontinence.
From home with wife, independent in ADLs. Drives.
No previous Hx falls. No noted cognitive impairment. Mild OA at knees, but nil other. No PMHx CVD. No recent changes to medications. Not on benzos/ psychotripc drugs.
Socially: drinks ETOH 3 glasses wine/ day

Mr. B
HR 110 BP 135/80 T 38 O2 96% RA

Pt looked comfortable, in no obvious distress.

HS dual, nil added
Lungs clear
Abdo soft, non tender. No flank tenderness.

Upper vs lower motor neuron signs
- Increased tone
- Weakness (pyramidal
- Hyperreflexia

Acute onset
Motor predominance
Good functional baseline
Risk factor for CVA
Alcoholic myopathy, nutritional deficiency?
- Decreased tone
- Weakness of muscle groups
- Atrophy
- Arreflexia
- Fasciculations
Upper motor neuron

Lower motor neuron


- Cortical/ subcortical/ brainstem lesion
- bilateral cerebral or brainstem lesion
- Spinal cord lesion (normal MSE)
With PMHx TIA, there is a risk of Mr B having a cerebrovascular event. Considering the distribution and onset of his symptoms, however, (not sudden, proximal predominance, symmetrical, ascending pattern during admission) unlikely.
Possible spinal cord lesion with CT showing spinal mets - but MRI nil compression.
- Radiculopathy
- Plexopathy (more than one spinal/ peripheral nerve)
- Peripheral neuropathy - DM/ carcinoma/ B1 or B12 def/ drugs

- Proximal- myopathy - steroids, ETOH, polymositis, hypoK, thyroid disease
- Distal- polyneuropathy - GB
- Diffuse- [N reflex/sens] - NMJ
- UECs , electrolytes NAD
- FBP (Hb 132, WCC 5.82 - no anaemia, inflamm markers not raised)
- BSLs (8, not hypoglycaemic)
- Vitamin B12, folate, thyroid function tests, - NAD
- CK - normal
- Urine MCS - taken after ED started Ceftriaxone- clear

Febrile on admission – T 38 – started on Abx
- With dysuria on presentation, started on Ceftriaxone and metronidazole
- USS kidneys - nil
XRs – nil
CT head - nil
AWS for ETOH w/d
CT spine showed mets
MRI spine - no spinal pathology

Neuro examinations...
Normal pupil - reacting to light & accommodation & normal fundii
Normal EOMs/ No diplopia / No facial weakness/ Normal neck flexion & extension ( 5/5)/ No bulbar signs

No drift
Shoulder abd /add - 3/5 ( c/o pain in both shoulders)
Normal elbow flexion & extension ( almost 5/5) B/L
Wrist flexion & extension ( 4/5) B/L
Finger flexors - 5/5

Hip flexion & extension 3/5
Knee flexion & extension ( 4/5)
Ankle DF /PF - 4/5

Arreflexic UL & LL
Dorsal column - reduced vibration & joint position to b/l knees
No sensory level
Normal anal sphinctor tone, no saddle anaesthesia. Prostate smooth and enlarged.
Peripheral neuropathy with b/g ETOH use,
Proximal myopathy with b/g ETOH use and
Polyneuropathy which had an ascending nature during admission... likely GBS
Day 1 of admission...
Cons review: Noted increased weakness, in an ascending pattern. ?GBS
- 6-hourly peak flow measurements
- Neuro review requested

MET call for new onset AF, ongoing fevers despite Abx therapy and clear urine MCS
Increasing paralysis to entire upper limbs
Pt transferred to ICU for ongoing care with Neuro input
Guillain Barré Syndrome
Demyelinating neuropathy: predominantly motor difficulty, absence of deep tendon reflexes, parasthesias without objective sensory loss, increased CSF albumin with normal WCC

Multiple forms, most common is AIDP "Acute inflammatory demyelinating polyradiculopathy"

Risk factors: recent viral/ bacterial illness (60%), or (less commonly) immunisation (e.g. swine flu), cancer, hodgkin's lymphoma, HIV, elderly male
Weakness begins in lower limbs and ascends, and is primarily present in proximal groups then distal. Flaccid paralysis with areflexia, over days.

80% parasthesias of hands and feet
50% nadir at 1/52, rest within 4 weeks.
30% require mechanical ventilation
50% slurred speech
80% back / leg pain at onset (hence confusion with cord compression)
Facial weakness, bulbar dysfunction, EOM weakness, facial droop, ..

Dysautonomia (usually labile BPs)
As previously +
EMG - patchy demyelination, slowing of motor nerve conduction
LP - elevated protein, normal WCC [inflammation nerve roots]
LFTs - elevated aminotransferases normalise by 1-2/52 [2' to EBV/ CMV ]
6-hrly spirometry
Antibody testing- for certain variants of GBS
MRI/ CT spine - rule out other pathologies
Serology: CMV/ EBV/ H influenzae/ C jejuni
Stool culture: C jejuni
Other:[peripheral neuropathy] TFTs/ rheumatology profile/ Vit B12/ folic A/ haemoglobin A1c/ ESR/ rapid protein reagent/ tests for heavy poisoning
Plasma exchange or IVIg
- Plasma exchange has higher risks
- IVIg is contraindicated in sepsis, IgA deficiency and renal failure
- If still independently mobile, start within 2/52, otherwise, within 4/52.

Supportive Tx
6-hourly bedside spirometry - EARLY ICU monitoring/ intubation if drop in vital capacity/ poor inspiratory or expiratory pressures
Pulse, BP monitoring (esp if autonomic dysfunction of BPs)
DVT prophylaxis
Rehabilitation once past nadir
Analgesia avoid opiates as these worsen gut dysmotility/ bladder distension

Mr B...
- Cord lesions: sphinctor
symptoms, senosry level,
bilateral motor signs
- Brain stem: dysarthria,
dysphagia, horner's,
bilateral motor signs
- Motor cortex: frontal signs,
hemianopia, dysphasias
Full transcript