Neurological Sciences

, Volume 33, Issue 4, pp 905–909 | Cite as

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
Case Report

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.

Keywords

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

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Copyright information

© Springer-Verlag 2011

Authors and Affiliations

  • A. Maues De Paula
    • 1
  • B. Michel
    • 2
  • D. W. Dickson
    • 3
  • Z. K. Wszolek
    • 4
  • J. F. Pellissier
    • 1
  1. 1.Service d’Anatomie Pathologique et Neuropathologie du Pr Figarella-BrangerCentre Hospitalier Universitaire La TimoneMarseille Cedex 05France
  2. 2.Unité de Neurologie Comportementale, Hôpital Sainte-MargueriteMarseilleFrance
  3. 3.Department of NeuropathologyMayo ClinicJacksonvilleUSA
  4. 4.Department of NeurologyMayo ClinicJacksonvilleUSA

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