French recommendations for the management of adult & pediatric chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)

Authors: Fargeot, G; Gitiaux, C; Magy, L; Pereon, Y; Delmont, E; Viala, K; Echaniz-Laguna, A

Affiliations: Neurophysiology Department, Pitié-Salpêtrière Hospital, AP–HP, Paris, France ; Department of Paediatric Neurophysiology, Necker-Enfants–Malades Hospital, AP–HP, Paris University, Paris, France ; Department of Neurology, National Reference Center for ‘Rare Peripheral Neuropathies’, University Hospital of Limoges, Limoges, France ; CHU Nantes, Centre de Référence Maladies Neuromusculaires AOC, Filnemus, Euro-NMD, Explorations Fonctionnelles, Hôtel-Dieu, Nantes, France ; Reference Center for Neuromuscular Diseases and ALS Timone University Hospital, Aix-Marseille University, Marseille, France ; Neurology Department, CHU de Bicêtre, AP–HP, Le-Kremlin-Bicêtre, France ; French National Reference Center for Rare Neuropathies (NNERF), Le-Kremlin-Bicêtre, France ; Inserm U1195, Paris-Saclay University, Le-Kremlin-Bicêtre, France

Publication: Revue Neurologique ; 2022

Abstract: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare autoimmune disorder of the peripheral nervous system, primarily affecting the myelin sheath. The pathophysiology of CIDP is complex, involving both humoral and cellular immunity. The diagnosis of CIDP should be suspected in patients with symmetrical proximal and distal motor weakness and distal sensory symptoms of progressive onset, associated with decreased/abolished tendon reflexes. Treatments include intravenous immunoglobulins, steroids and plasma exchange, with usually an induction phase followed by a maintenance therapy with progressive weaning. Treatment should be rapidly initiated to prevent axonal degeneration, which may compromise recovery. CIDP outcome is variable, ranging from mild distal paresthesiae to complete loss of ambulation. There have been several breakthroughs in the diagnosis and management of CIDP the past ten years, e.g. discovery of antibodies against the node of Ranvier, contribution of nerve ultrasound and magnetic resonance imaging to diagnosis, and demonstration of subcutaneous immunoglobulins efficiency. This led us to elaborate French recommendations for the management of adult & pediatric CIDP patients. These recommendations include diagnosis assessment, treatment, and follow-up.