INFORMATION FOR CANDIDATE: Your next patient in an emergency department is a 14year-old boy, David, who presents with a 10-day

history of progressive weakness. He is accompanied by his father. He had a flu like illness with a runny nose, cough and generally feeling unwell about 4 weeks earlier and saw his GP at the time and was told that it was just a viral infection and he was treated with panadol. However, two weeks after that he developed progressive lower-extremity weakness and difficulty walking. YOUR TASK IS TO: 1. Take further history 2. Perform a physical examination and organise appropriate investigation 3. Discuss the most likely diagnosis and management with the examiner

HOPC: as above and he also developed numbness, tingling and paraesthesia with progressive weakness extending to his upper extremities. He described some mild distal sensory loss, muscle tenderness and flaccid muscular weakness. In the last few days he developed a hoarse voice and shortness of breath and he had difficulties with passing water and did not drink as much as usual. No V+D, no fever. PHx. + FHx. + SHx.: unremarkable EXAMINATION: David looks mildly unwell, but alert and with normal mental state. BP 150/95, P 100/min, regular, RR 28, afebrile, SaO2 93% on room air. Auscultation of the lungs reveals poor air entry, normal breathsounds. His heart sounds are normal, no murmur. His strength is symmetric but diminished to 2/5 in his lower extremities and 4/5 in his upper extremities. Sensation is intact to light touch, but there is a loss of vibratory sense. He has no deep tendon reflexes in his lower extremities, diminished deep tendon reflexes in his upper extremities, and absent plantar reflexes. Cranial nerves are intact; however, he has a weak cough and gag reflex, with impaired handling of secretions. The remainder of his examination is unremarkable. INVESTIGATIONS: 1. FBE, ESR, U+Es, ABGs etc. unremarkable 2. Lumbar puncture reveals an opening pressure of 15 cm H20. The cell count is normal. CSF protein is elevated (> 0.4 g/L). 3. Magnetic resonance imaging (MRI) of the lumbosacral spine may demonstrate enhancement of the cauda equina nerve roots. This imaging modality has been described to be 83% sensitive for acute Guillain-Barr syndrome, and abnormalities are present in 95% of typical cases. 4. Electrophysiologic (motor nerve conduction) studies are the most specific and sensitive tests for confirming the diagnosis. DIAGNOSIS: Guillain Barr Syndrome (acute demyelinating polyneuropathy) Guillain-Barr syndrome is an acute, rapidly progressive, acquired inflammatory demyelinating polyradiculoneuropathy (AIDP) that affects both children and adults with peaks in young adulthood (15-35 y) and in the elderly (50-75 y). The cause of Guillain-Barr syndrome is unknown, but the disorder is thought to result from a postinfectious (respiratrory tract or gastrointestinal infection) immune-mediated process that predominantly damages the myelin sheath of peripheral nerves. A variety of infectious agents have been associated with Guillain-Barr syndrome, although Campylobacter is the most frequent. Others include cytomegalovirus, (CMV), Epstein-Barr virus (EBV), Haemophilus influenzae, Mycoplasma pneumoniae, the enterovirus family, hepatitis A and B, herpes simplex virus, and Chlamydophila (formerly Chlamydia) pneumoniae. The typical presentation of Guillain-Barr syndrome is fine paresthesias in the toes and fingertips, followed by symmetric lower-extremity weakness that may ascend, over hours to days, to involve the arms and the muscles of respiration. Pain, predominately back, lowerlimb and abdominal pain, is often a prominent feature of the syndrome. The physical examination reveals symmetric weakness, with diminished or absent reflexes and variable loss of sensation in a stocking-glove distribution. Signs of autonomic dysfunction are present in 50% of patients, and they include cardiac dysrhythmias, orthostatic

hypotension, transient or persistent hypertension, ileus, constipation, and bladder dysfunction. Deviation from the classic presentation of ascending progression of weakness is not uncommon. In what is known as the Miller-Fisher variant, cranial nerves are affected in 3040% of patients at any time in the course of the syndrome. This form of the disease is also characterized by areflexia, ataxia and ophthalmoplegia. The facial nerves are most commonly involved, resulting in bilateral facial weakness. Although the associated autonomic dysfunction may produce life-threatening complications, mortality from Guillain-Barr syndrome is largely secondary to respiratory failure associated with respiratory muscle weakness. Approximately 20% of children with Guillain-Barr syndrome require mechanical ventilation for respiratory failure. More than 90% of patients reach the nadir of their function within 4 weeks of the onset of symptoms, with return of normal function occurring slowly over the course of weeks to months. The majority of patients with Guillain-Barr syndrome achieve a full and functional recovery within 6-12onths. The clinical course of Guillain-Barr syndrome in children is shorter than it is in adults, and recovery is more complete.

DIFFERENTIAL DIAGNOSES: spinal cord lesions (such as transverse myelitis or anterior spinal artery syndrome) peripheral neuropathies (such as those caused by heavy metals) neuromuscular junction diseases (such as that caused by organophosphate pesticides) myasthenia gravis Botulism myopathies (such as dermatomyositis)

MANAGEMENT: Admit to hospital. The indications for admission to the ICU include, but are not limited to, respiratory insufficiency or failure, loss of airway-protective reflexes, and severe autonomic instability. The main modalities of therapy for Guillain-Barr syndrome include plasma exchange and intravenously administered immunoglobulin (IVIG). Corticosteroids have not been shown to be beneficial. In addition, physical and occupational therapy should be initiated early and may be beneficial in helping patients to regain their baseline functional status.