Immune-Mediated Mechanisms in the Pathogenesis of Hopkins Syndrome

• Published in: Pediatric neurology Vol. 47; no. 5; pp. 373 - 374
• Main Authors: Cantarín-Extremera, Verónica, MD, González-Gutiérrez-Solana, Luis, MD, Ramírez-Orellana, Manuel, MD, PhD, López-Marín, Laura, MD, Duat-Rodríguez, Anna, MD, Ruíz-Falcó-Rojas, Maria Luz, MD
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01.11.2012; Elsevier
• Subjects: Asthma; Asthma - diagnosis; Asthma - drug therapy; Asthma - immunology; Autoimmune diseases; Biological and medical sciences; biomarkers; Blood; Brain; Cerebrospinal fluid; Child, Preschool; Denervation; EMG; Facies; Female; Humans; Hyperventilation - diagnosis; Hyperventilation - drug therapy; Hyperventilation - immunology; Immunoglobulins; Immunoglobulins, Intravenous - therapeutic use; Immunotherapy; Intellectual Disability - diagnosis; Intellectual Disability - drug therapy; Intellectual Disability - immunology; Intravenous administration; Leukocytes; Lymphocytes T; Malformations of the nervous system; Medical sciences; N.M.R; Nerve conduction; Neurology; Pediatrics; Poliomyelitis - diagnosis; Poliomyelitis - drug therapy; Poliomyelitis - immunology; Sensory neurons; Spine; Syndrome; Pathogenesis; Nervous system diseases
• ISSN: 0887-8994; 1873-5150
• DOI: 10.1016/j.pediatrneurol.2012.08.006

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.