Neuromuscular Weakness - Guillain_Barre_Syndrome_Quesmed Flashcards
Definition
GBS is an ascending inflammatory demyelinating polyneuropathy, typified by an acute onset of bilateral and roughly symmetric limb weakness.
Epidemiology
The prevalence of GBS is approximately 1-2 cases per 100,000 worldwide.
Aetiology
GBS typically occurs 1-3 weeks following an infection, with common culprits being Campylobacter, mycoplasma, and Epstein-Barr Virus (EBV). 40% of cases are idiopathic. Other triggers include CMV, HIV, Hepatitis A, or vaccinations such as tetanus, rabies, or swine flu.
Signs and symptoms
Clinical features include progressive ascending symmetrical limb weakness, lower back pain, paraesthesia, respiratory muscle involvement, cranial nerve involvement, lower motor neuron signs, and autonomic dysfunction (e.g., arrhythmias, labile BP).
Variants
Variants include Paraparetic variant, Miller-Fisher syndrome, Pure motor variant, Bilateral facial palsy with paraesthesias, Pharyngeal-brachial-cervical weakness, and Bickerstaff’s Brainstem Encephalitis.
Differential diagnosis
Differentials include vascular causes (e.g., brainstem strokes), infective/inflammatory conditions (e.g., Polio, Lyme disease, HIV, TB), traumatic/structural causes (e.g., spinal cord compression), and metabolic causes (e.g., porphyrias, electrolyte derangements).
Investigations
Key investigations include monitoring of FVC, cardiac monitoring, blood tests, serological tests (anti-ganglioside antibodies), lumbar puncture (albuminocytological dissociation), and nerve conduction studies.
Management
Management includes supportive care (monitoring FVC, VTE prophylaxis, analgesia, cardiac arrhythmia management, enteral feeding if needed) and specific treatments like IVIG or plasmapheresis for significant disability.
Prognosis
While GBS can be life-threatening, most patients experience full recovery. Prognostic indicators include speed of onset, severity at nadir, age, and the presence of preceding diarrhoeal illness.