Acute-onset chronic inflammatory demyelinating polyneuropathy with cranial nerve involvement, dysautonomia, respiratory failure, and autoantibodies.
We examined a 27-year-old woman who developed rapidly progressive quadriplegia and acute respiratory failure that required mechanical ventilation in the intensive care unit. It was unclear whether this was a presentation of Guillain-Barré syndrome (GBS) or acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP). Remarkable features included multiple cranial nerve involvement, respiratory failure, dysautonomia, and skin manifestations. Several autoantibodies were elevated, including antinuclear (ANA), anticardiolipin (aCL), thyroid, and calcium-sensing receptor (CaSR) autoantibodies. The patient was initially diagnosed with GBS and treated with intravenous immunoglobulin (IVIg). After almost complete recovery, relapse with quadriplegia and respiratory failure was observed 12 weeks after motor symptom onset. She then received IVIg and steroid pulse therapy followed by maintenance oral methylprednisolone and plasma exchange. She recovered completely 4 months after the relapse. The further clinical and serological course was consistent with systemic lupus erythematosus (SLE)-associated CIDP. Herein we evaluate the association between A-CIDP and some biological markers of autoimmunity.
Hantson
Philippe
P
Department of Intensive Care, Cliniques St-Luc, Université Catholique de Louvain, Avenue Hippocrate 10, Brussels 1200, Belgium. philippe.hantson@uclouvain.be
Kevers
Luc
L
Fabien
Nicole
N
Van Den Bergh
Peter
P
D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001323:Autoantibodies / Q000276:immunology*; D003389:Cranial Nerve Diseases / Q000276:immunology* / Q000628:therapy; D003937:Diagnosis, Differential; D018572:Disease-Free Survival; D004568:Electrodiagnosis; D005260:Female; D020275:Guillain-Barre Syndrome / Q000175:diagnosis*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D008775:Methylprednisolone / Q000627:therapeutic use; D009431:Neural Conduction / Q000502:physiology; D010951:Plasma Exchange; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D054969:Primary Dysautonomias / Q000276:immunology* / Q000628:therapy; D011782:Quadriplegia / Q000276:immunology / Q000628:therapy; D012121:Respiration, Artificial; D012131:Respiratory Insufficiency / Q000276:immunology* / Q000628:therapy; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents; D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous; D008775:Methylprednisolone