Advertisement

Neurological Sciences

, Volume 38, Issue 12, pp 2223–2225 | Cite as

Wilson’s disease presenting as central pontine myelinolysis

  • Mahdi Safdarian
  • Renato P. Munhoz
  • Mahboubeh Aghaei
  • Mohammad RohaniEmail author
Letter to the Editor

Wilson’s disease (WD) is a rare autosomal recessive disease due to impaired biliary excretion of copper. Its main neurologic symptoms are dysarthria and movement disorders such as dystonia, tremor, and parkinsonism [1, 2]. Herein, we report a case of central pontine myelinolysis (CPM) presenting as an uncommon presentation of WD.

A 34-year-old woman in the third trimester of pregnancy was referred to our hospital because of acute onset quadriplegia, anarthria, and dysphagia, started 2 weeks prior to admission. According to her family, past medical history was unremarkable, except for long-standing mild upper extremities’ action tremor. Her pregnancy so far had been uneventful; there was no history of hyperemesis, vomiting, or use of medications. Family history of neurological diseases was negative. On neurologic examination, she was awake and alert but could only respond to questions with eye blinking. There was no ophthalmoplegia but saccadic eye movements were slow especially in...

Abbreviations

WD

Wilson’s disease

CPM

Central pontine myelinolysis

Notes

Compliance with ethical standards

Conflict of interest

None.

Funding source

None.

Financial disclosure

None.

Supplementary material

10072_2017_3064_MOESM1_ESM.docx (13 kb)
Supplementary Table 1 (DOCX 13 kb)
Video 1

(MPG 16702 kb)

Video 2

(MPG 18408 kb)

References

  1. 1.
    Pfeiffer RF (2016) Wilson disease. Continuum (Minneap Minn) 22(4 Movement Disorders):1246–1261Google Scholar
  2. 2.
    Sinha S, Taly AB, Ravishankar S, Prashanth LK, Vasudev MK (2007) Central pontine signal changes in Wilson's disease: distinct MRI morphology and sequential changes with de-coppering therapy. J Neuroimaging 17(4):286–291CrossRefPubMedGoogle Scholar
  3. 3.
    Prashanth LK, Sinha S, Taly AB, Vasudev MK (2010) Do MRI features distinguish Wilson’s disease from other early onset extrapyramidal disorders? An analysis of 100 cases. Mov Disord 25(6):672–678CrossRefPubMedGoogle Scholar
  4. 4.
    Imiya M, Ichikawa K, Matsushima H, Kageyama Y, Fujioka A (1992) MR of the base of the pons in Wilson’s disease. Am J Neuroradiol 13:1009–1012PubMedGoogle Scholar
  5. 5.
    Kizkin S, Sarac K, Ozisik HI et al (2004) Central pontine myelinolysis in Wilson’s disease: MR spectroscopy findings. Magn Reson Imaging 22:117-121CrossRefPubMedGoogle Scholar
  6. 6.
    Verma R, Rai D (2013) Central pontine myelinolysis associated with Wilson disease in a 7-year-old child. BMJ Case Rep. doi: 10.1136/bcr-2012-007408
  7. 7.
    Sánchez-Ferrer ML, Prieto-Sánchez MT, Orozco-Fernández R, Machado-Linde F, Nieto-Diaz A (2016) Central pontine myelinolysis during pregnancy: pathogenesis, diagnosis and management. J Obstet Gynaecol 6:1-7Google Scholar

Copyright information

© Springer-Verlag Italia S.r.l. 2017

Authors and Affiliations

  1. 1.Department of Neurology, Hazrat Rasool HospitalIran University of Medical SciencesTehranIran
  2. 2.Morton and Gloria Shulman Movement Disorders Clinic and the Edmond J. Safra Program in Parkinson’s Disease, Toronto Western Hospital and Division of NeurologyUniversity of TorontoTorontoCanada

Personalised recommendations