Tonic pupils: an unusual autonomic involvement in chronic inflammatory demyelinating polyneuropathy (CIDP)

• Published in: Neurological sciences Vol. 40; no. 8; pp. 1725 - 1727
• Main Authors: Escorcio-Bezerra, Marcio Luiz, Manzano, Gilberto Mastrocola, Bichuetti, Denis Bernardi, Nunes, Karlo Faria, Correa, Daniele Sales Alves, Oliveira, Acary Souza Bulle, Baeta, Alex Machado
• Format: Journal Article
• Language: English
• Published: Cham Springer International Publishing 01.08.2019; Springer Nature B.V
• Subjects: Autonomic nervous system; Axons; Brief Communication; Corticosteroids; Cranial nerves; Demyelination; Diagnostic Errors; Female; Guillain-Barre syndrome; Guillain-Barre Syndrome - diagnosis; Humans; Immunoglobulins; Inflammation; Intravenous administration; Medicine; Medicine & Public Health; Neurology; Neuropathy; Neuroradiology; Neurosciences; Neurosurgery; Plasmapheresis; Polyneuropathy; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating - complications; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating - diagnosis; Psychiatry; Tonic Pupil - etiology; Young Adult; Tonic pupil; Autonomic dysfunction; Adie’s pupil; CIDP; Chronic inflammatory demyelinating polyneuropathy; Guillain-Barré syndrome
• ISSN: 1590-1874; 1590-3478
• DOI: 10.1007/s10072-019-03890-8

Background Chronic inflammatory demyelinating polyneuropathy (CIDP) is a neuropathy which affects mainly large myelinated axons and has a typically mild autonomic dysfunction mainly from postganglionic nerve fiber involvement. Case report We report here an acute onset CIDP initially diagnosed as Guillain-Barré syndrome (GBS), unresponsive to treatment with intravenous immunoglobulin (IVIg), which later responded to plasmapheresis and corticoids. The patient had a markedly distal demyelination, prominent cranial nerve involvement and, interestingly, bilateral fixed dilated pupils. Despite complete clinical recovery, this neurological sign remained. Conclusions Tonic pupils have previously been described in different neurologic conditions, including GBS, but not yet in acute onset CIDP or in variants with predominantly distal demyelination. It differs from the classical Adie’s pupil because it lacks the light-near dissociation. This case report expands the range of possible autonomic signs in acute onset CIDP, which could help physicians establish optimal treatment strategies earlier on.