Sporadic diffuse leucoencephalopathy with axonal spheroids: Report of a profuse and rapid cortical-spinal degeneration

A. Maues De Paula, B. Michel, D. W. Dickson, Z. K. Wszolek, J. F. Pellissier

Research output: Contribution to journalArticle

1 Scopus citations

Abstract

Diffuse leucoencephalopathy with axonal spheroids (DLS) is a rare disease affecting the white matter leading to dementia and progressive motor impairment. The neuropathological hallmark includes axonal swelling and spheroids as well as myelin loss. We report a case of a 46-year-old man with memory deficit and behavioral changes followed by a rapid cognitive decline and pyramidal syndrome. Head magnetic resonance imaging showed cortical atrophy of the brain and symmetric corticospinal tract involvement. He died 4 years after the first symptoms. Autopsy was performed and the brain revealed cortical and corpus callosum atrophy, a grayish granular appearance of the white matter and ventricular enlargement. Myelin stains showed a significant demyelination of the centrum ovale and corticospinal tract. Such degeneration was accompanied by axonal loss, axonal swelling, and numerous spheroids. There was no pigment overload or inflammation. We discuss this new DLS case with bilateral, severe, and rapid cortical-spinal involvement.

Original languageEnglish (US)
Pages (from-to)905-909
Number of pages5
JournalNeurological Sciences
Volume33
Issue number4
DOIs
StatePublished - Aug 1 2012

Keywords

  • Adult onset leucodystrophy
  • Adult-onset leucoencephalopathy with pigmented glia and spheroids
  • Diffuse leucoencephalopathy with spheroids
  • Pigmentary orthochromatic leucodystrophy

ASJC Scopus subject areas

  • Dermatology
  • Clinical Neurology
  • Psychiatry and Mental health

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