Article Text

Download PDFPDF
Reminder of important clinical lesson
Diffusion and ADC-map images detect ongoing demyelination on subcortical white matter in an adult metachromatic leukodystrophy patient with autoimmune Hashimoto thyroiditis
  1. Akiko Miura,
  2. Yuri Kumabe,
  3. En Kimura,
  4. Satoshi Yamashita,
  5. Akihiko Ueda,
  6. Teruyuki Hirano,
  7. Makoto Uchino
  1. Department of Neurology, Kumamoto University Hospital, Kumamoto, Japan
  1. Correspondence to En Kimura, enkimura{at}kumamoto-u.ac.jp

Summary

Adult-onset metachromatic leukodystrophy (MLD) often shows schizophrenia- or encephalopathy-like symptoms at an early stage, such as behavioural abnormalities, cognitive impairment, mood disorders and hallucinations. The authors report the case of an adult woman with MLD who had been given antipsychotic medication for schizophrenia. In the differential diagnosis, screening of auto-antibodies was important for ruling out other encephalopathies as she had a euthyroid Hashimoto thyroiditis. Diagnosis was based the results of MRI, nerve conduction velocity, sensory evoked potential, motor evoked potential, lysosomal enzyme activity and gene analysis studies. Brain MRI showed diffuse demyelination spreading from the deep white matter to subcortical area as high signals at the edges of these lesions in diffusion and apparent diffusion coefficient-map images with the U-fibres conserved. The authors diagnosed adult-onset MLD coexisting with euthyroid autoimmune Hashimoto thyroiditis.

View Full Text

Statistics from Altmetric.com

Footnotes

  • Competing interests None.

  • Patient consent Obtained.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.