This Article  • Full text  • PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citing articles on HighWire  • Citing articles on ISI (4)  • Contact me when this article is cited  Related Content  • Related article  • Similar articles in this journal  Topic Collections  • Neurology, Other  • Alert me on articles by topic

Monika Warmuth-Metz, MD; Georg Becker, MD; Martin Bendszus, MD; Laszlo Solymosi, MD

Arch Neurol. 2000;57:1635-1637.

We describe 2 brothers with L-2-hydroxyglutaric aciduria who developed severe spastic tetraparesis in their 30s. They also had an underlying condition of high-grade cervical spinal canal stenosis diagnosed by magnetic resonance imaging. They were operated on to decompress the spine, and the preexisting gait disturbances slowly diminished after the decompression. Since most people with L-2-hydroxyglutaric aciduria show spastic signs in their legs and arms, we conclude that cervical spinal canal stenosis may be the underlying reason and may be linked to this rare metabolic disorder.

From the Departments of Neuroradiology (Drs Warmuth-Metz, Bendszus, and Solymosi) and Neurology (Dr Becker), University of Würzburg, Würzburg, Germany.

RELATED ARTICLE

Archives of Neurology Reader's Choice: Continuing Medical Education
Arch Neurol. 2000;57(11):1661-1662.
FULL TEXT  

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

L-2-Hydroxyglutaric Aciduria: Clinical, Neuroimaging, and Neuropathological Findings
Seijo-Martinez et al.
Arch Neurol 2005;62:666-670.
ABSTRACT | FULL TEXT  

L-2-hydroxyglutaric aciduria and brain malignant tumors: A predisposing condition?
Moroni et al.
Neurology 2004;62:1882-1884.
ABSTRACT | FULL TEXT  

L-2-hydroxyglutaric aciduria presenting as migraine
Kossoff et al.
Neurology 2001;57:1731-1732.
FULL TEXT