title: Immune-mediated mechanisms in the pathogenesis of Hopkins syndrome.
abstract: Hopkins syndrome occurs after an acute asthma attack. An immune-mediated mechanism has been suggested. Immunoglobulin or methylprednisone pulse therapies comprise the most useful treatment. We describe a 3-year-old girl who developed severe weakness in her left arm, 7 days after an acute asthma attack. A complete blood count with autoimmune biomarkers, immunoglobulin profile, and virology study and magnetic resonance of the brain, spine, and brachial plexus produced normal results. In the cerebrospinal fluid, T lymphocytes comprised the predominant leukocyte population, and oligoclonal bands were positive. An electromyogram revealed a partial axonal lesion (normal motor nerve conduction velocity with low amplitude) of the axillary, musculocutaneus, and interosseous nerves of the left arm, with normal sensory nerve conduction and partial denervation. We began therapy with intravenous immunoglobulin for 5 consecutive days, repeated every 4 weeks for 2 months. Afterward, our patient recovered. This report contributes to understanding the role of immune-mediated mechanisms in the pathogenesis of this disease, and the importance of immunotherapy in its treatment.
authors.affiliation: Section of Neuropediatrics, Department of Pediatrics, Hospital Infantil Universitario Niño Jesús, Madrid, Spain. verocantarin@hotmail.com
authors.forename: Verónica
authors.initials: V
authors.lastname: Cantarín-Extremera
authors.forename: Luis
authors.initials: L
authors.lastname: González-Gutiérrez-Solana
authors.forename: Manuel
authors.initials: M
authors.lastname: Ramírez-Orellana
authors.forename: Laura
authors.initials: L
authors.lastname: López-Marín
authors.forename: Anna
authors.initials: A
authors.lastname: Duat-Rodríguez
authors.forename: Maria Luz
authors.initials: ML
authors.lastname: Ruíz-Falcó-Rojas
mesh_terms: D001249:Asthma / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D002675:Child, Preschool; D019066:Facies; D005260:Female; D006801:Humans; D006985:Hyperventilation / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008607:Intellectual Disability / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D011051:Poliomyelitis / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D013577:Syndrome
chemical_list: D016756:Immunoglobulins, Intravenous