Skip to main content
SpringerLink
Log in

L-2-Hydroxyglutaric aciduria: MRI in seven cases

  • PAEDIATRIC NEURORADIOLOGY
  • Published:
Neuroradiology Aims and scope Submit manuscript

Abstract

The MRI findings in 7 patients with L-2-Hydroxyglutaric aciduria (L-2-OHG aciduria) are described and compared with previous neuroradiological reports and the only three published pathological cases. Signal abnormalities involved peripheral subcortical white matter, basal ganglia and dentate nuclei. Cerebellar atrophy was present. Although similar appearances may be seen in other metabolic disorders, the distribution of signal abnormalities in L-2-OHG aciduria is highly characteristic and may suggest the correct diagnosis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

Author information

Authors and Affiliations

  1. Department of Neuroradiology, Istituto Nazionale Neurologico “C. Besta”, Via Celoria 11, I-20133 Milan, Italy Tel.: +39-02-2 39 44 49 Fax: +39-02-70 63 82 17, , , , , , IT

    L. D'Incerti, L. Farina & M. Savoiardo

  2. Department of Child Neurology, Istituto Nazionale Neurologico “C. Besta”, Milan, Italy, , , , , , IT

    I. Moroni & G. Uziel

Authors
  1. L. D'Incerti
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. L. Farina
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. I. Moroni
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. G. Uziel
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. M. Savoiardo
    View author publications

    You can also search for this author in PubMed Google Scholar

Additional information

Received: 5 January 1998 Accepted: 22 April 1998

Rights and permissions

Reprints and permissions

About this article

Cite this article

D'Incerti, L., Farina, L., Moroni, I. et al. L-2-Hydroxyglutaric aciduria: MRI in seven cases. Neuroradiology 40, 727–733 (1998). https://doi.org/10.1007/s002340050673

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/s002340050673

Search

Navigation