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Schilder’s disease
  1. Marta Magriço1,
  2. Tiago Lorga2,
  3. Filipa Serrazina1,
  4. Manuel Salavisa1
  1. 1 Department of Neurology, Hospital de Egas Moniz, Lisboa, Portugal
  2. 2 Department of Neuroradiology, Hospital de Egas Moniz, Lisboa, Portugal
  1. Correspondence to Dr Marta Magriço, Hospital de Egas Moniz Serviço de Neurologia, Lisboa, Portugal; marta.icn.magrico{at}

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Clinical case

An 18-year-old man had a 2-week history of progressive left arm weakness. He had no history of seizures or learning disabilities and no relevant family history. On examination, there was mild (grade 4) weakness of the left arm and bilateral pyramidal signs. MR scan of brain (T2/fluid-attenuated inversion recovery (FLAIR)) showed bilateral hyperintense lesions in the centrum semiovale (figure 1A), with faint peripheral enhancement of the right-sided lesion with gadolinium (figure 1B). MR spectroscopy showed a decreased N-acetyl-aspartate peak. MR scan of the spinal cord was normal. Serum tests for HIV and syphilis, anti-aquaporin-4, anti-myelin oligodendrocyte glycoprotein and very-long-chain fatty acids were all negative. Adrenal function was normal. Nerve conduction studies found no peripheral nervous system involvement. …

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  • Contributors MM: first draft of the manuscript and data collection. TL and FS: material preparation and data collection. MS: commented on previous versions of the manuscript. All authors read and approved the final manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned. Externally peer reviewed by Mark Willis, Cardiff, UK.

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