Guillain Barré syndrome
Signs & Symptoms
- pain, numbness, paresthesia or weakness of the limbs
- typically rapidly progressive (may worsen within hours) and symmetric course
- nevertheless atypical presentation is possible: eg. cranial nerve involvement, dyspnea, swallowing impairment, pain syndrome
Evaluation
- GBS is a clinical diagnosis! Don’t wait with treatment untill all assessments have been done
- Neurological consult
- EMG
- Lumbar puncture after brain imaging: typically elevated protein with normal white blood cell count
- Blood analysis: CMV, mycoplasma, hepatitis E, campylobacter jejuni, anti-ganglioside antibodies
- Swallowing analysis
- Pulmonary function tests with vital capacity and maximum inspiratory/expiratory pressures; respiratory function should be evaluated 6 x pd
- ECG
Management
- IVIg therapy (0,4g/kg for 5 days)
- Discussion corticoid treatment
- Plasmapheresis (ideally 5 x with total exchange volume of 5 plasma volumes in 2 weeks)
- IVIg therapy may be repeated
- FU in accordance with local neurologists
- Consider location of care where ventilatory support is available (required in 15-30% of idiopathic cases)
- Close FU of patient: monitoring of respiratory function at least 6 x pd + (ECG) monitoring in order to detect autonomic dysfunction
- DVT prevention
Corticoid treatment discussion
Not indicated for idiopathic GBS, however some case reports (eg Wilgenhof S et al, Annals of Oncology 2011) show interesting results with ICPI induced GBS with methylprednisolone 1-2mg/kg. Among the neurology community discussion is ongoing if ICPI induced GBS is similar to chronic inflammatory demyelinating polyneuropathy (CIDP). The latter has a similar symptoms as GBS but has a slower onset and in contrast to GBS, for CIDP corticoids are indicated.
Abbrevations
CMV: Cytomegalovirus
DVT: Deep Venous Thrombosis
EEG: Electro EncephaloGram
GBS: Guillain-Barré Syndrome
ICPi: Immune Checkpoint Blockade Inhibition
EMG: ElectroMyoGram
IVIg: IntraVeneous Immunoglobulins
