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

  • Marcio Luiz Escorcio-BezerraEmail author
  • Gilberto Mastrocola Manzano
  • Denis Bernardi Bichuetti
  • Karlo Faria Nunes
  • Daniele Sales Alves Correa
  • Acary Souza Bulle Oliveira
  • Alex Machado Baeta
Brief Communication



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.


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.


Chronic inflammatory demyelinating polyneuropathy Adie’s pupil Tonic pupil Autonomic dysfunction Guillain-Barré syndrome CIDP 


Compliance with ethical standards

Conflict of interest

Denis Bernardi Bichuetti has received speaking/consulting honoraria from the Bayer Health Care, Biogen Idec, Merck, Sanofi-Genzyme, TEVA, and Roche.


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Copyright information

© Fondazione Società Italiana di Neurologia 2019

Authors and Affiliations

  • Marcio Luiz Escorcio-Bezerra
    • 1
    Email author
  • Gilberto Mastrocola Manzano
    • 1
  • Denis Bernardi Bichuetti
    • 1
  • Karlo Faria Nunes
    • 1
  • Daniele Sales Alves Correa
    • 1
  • Acary Souza Bulle Oliveira
    • 1
  • Alex Machado Baeta
    • 1
  1. 1.Department of Neurology, Escola Paulista de MedicinaUniversidade Federal de São PauloSão PauloBrazil

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