[Pathophysiological and diagnostic aspects of Guillain-Barré syndrome]. / Syndrome de Guillain-Barré : physiopathologie et aspects diagnostiques.

Guillain-Barré syndrome (GBS) is the most common cause of acute neuropathy. It usually onset with a rapidly progressive ascending bilateral weakness with sensory disturbances, and patients may require intensive treatment and close monitoring as about 30% have a respiratory muscle weakness and about 10% have autonomic dysfunction. The diagnosis of GBS is based on clinical history and examination. Complementary examinations are performed to rule out a differential diagnosis and to secondarily confirm the diagnosis. GBS is usually preceded by an infectious event in ≈ 2/3 of cases. Infection leads to an immune response directed against carbohydrate antigens located on the infectious agent and the formation of anti-ganglioside antibodies. By molecular mimicry, these antibodies can target structurally similar carbohydrates found on host's nerves. Their binding results in nerve conduction failure or/and demyelination which can lead to axonal loss. Some anti-ganglioside antibodies are associated with particular variants of GBS: the Miller-Fisher syndrome, facial diplegia and paresthesias, the pharyngo-cervico-brachial variant, the paraparetic variant, and the Bickerstaff brainstem encephalitis. Their semiological differences might be explained by a distinct expression of gangliosides among nerves. The aim of this review is to present pathophysiological aspects and the diagnostic approach of GBS and its variants.

[Glucocorticoid sensitive bilateral leg swelling in an 85-year-old woman presenting with polymyalgia rheumatica: A case report]. / Œdèmes des membres inférieurs cortico-sensibles chez une patiente âgée de 85 ans présentant une pseudopolyarthrite rhizomélique.

INTRODUCTION: Polymyalgia rheumatica (PMR) can be associated with distal swelling indicating an associated RS3PE syndrome. We report a case of PMR associated with oedema of the lower limbs, which resolved rapidly under glucocorticoid therapy. CASE REPORT: A 85-year-old woman presented with a 4 month history of PMR responding to the 2012 EULAR/ACR classification criteria. Examination of the lower limbs revealed pitting oedema bilaterally up to the knees, with mild erythema and warmth. Hypoalbuminemia (30g/L) was present. There was no cardiac, renal or hepatic cause to explain leg swelling. FDG-PET/CT demonstrated increased metabolism in the periarticular area of shoulders and hips. There was no sign of aortitis or neoplasia. Under treatment with prednisone 10mg/day leg swelling disappeared concomitantly to a weight loss of 8kg within 8days. CONCLUSION: This case, the first to report leg swelling of inflammatory origin in the context of PMR, could indicate an increased vascular permeability caused by inflammation in the elderly.