This Article  • Full text  • PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Citing articles on Web of Science (7)  • Contact me when this article is cited  Related Content  • Related articles  • Similar articles in this journal  Topic Collections  • Neuromuscular diseases  • Alert me on articles by topic  Social Bookmarking   What's this?

Jochen Karitzky, MD; Wolfgang Block, PhD; Jörg K. Mellies, MD; Frank Träber, PhD; Anne Sperfeld, MD; Hans H. Schild, MD; Peter Haller, MD; Albert C. Ludolph, MD

Arch Neurol. 1999;56:1465-1471.

Objective  To seek regional metabolite abnormalities in patients with Kennedy disease (KD) using proton magnetic resonance spectroscopy.

Design  Nine patients with KD showing the typical phenotype without clinical signs of upper motor neuron involvement were compared with 17 male, age-matched, healthy control subjects. Relative metabolite concentrations for N-acetyl (NA) groups, choline-containing groups (Cho), phosphocreatine (Cr), and lactate (Lac) were determined in the brainstem and the motor region.

Results  Pathologic Lac signals suggesting impaired energy metabolism were absent in patients and controls. In the brainstem area, patients with KD showed a significant reduction in the NA/Cho metabolite ratio (P = .01). In the motor region, NA/Cho (P = .04) and NA/Cr (P = .03) ratios were significantly reduced. The reduction of the NA/Cho ratio in the motor region mainly resulted from decreased metabolite ratios in 3 patients. Changes in metabolite ratios did not correlate with the number of trinucleotide cytosine-adenine-guanine repeats from leukocytes. Because of the relatively small sample size due to the rarity of KD, these results should be considered preliminary.

Conclusions  Spectroscopic data fail to provide further evidence for altered energy metabolism in KD. Metabolite changes in the brainstem indicate a reduction of the neuronal marker NA or elevated Cho. These findings may reflect neuronal loss or gliosis consistent with the known pathologic features. In a subset of patients, altered metabolite ratios best explained by neuronal loss suggest subclinical involvement of the motor region. The extent of metabolite changes does not correlate with the trinucleotide repeat length.

From the Departments of Neurology, University of Ulm, Ulm (Drs Karitzky, Sperfeld, and Ludolph), Radiology, University of Bonn, Bonn (Drs Block, Träber, and Schild), and Neurology, Städt. Kliniken, Osnabrück (Drs Mellies and Haller), Germany.

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?

RELATED ARTICLES

Proton Magnetic Resonance Spectroscopy: An In Vivo Window to Study Neurodegenerative Disorders
Italo Linfante and Tetsuo Ashizawa
Arch Neurol. 1999;56(12):1446-1447.
EXTRACT | FULL TEXT  

Archives of Neurology Reader's Choice: Continuing Medical Education
Arch Neurol. 1999;56(12):1538-1539.
FULL TEXT  

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Combined 3T Diffusion Tensor Tractography and 1H-MR Spectroscopy in Motor Neuron Disease
Nelles et al.
Am. J. Neuroradiol. 2008;29:1708-1714.
ABSTRACT | FULL TEXT  

Regional N-Acetylaspartate Reduction in the Hippocampus Detected With Fast Proton Magnetic Resonance Spectroscopic Imaging in Patients With Alzheimer Disease
Block et al.
Arch Neurol 2002;59:828-834.
ABSTRACT | FULL TEXT  

Proton Magnetic Resonance Spectroscopy: An In Vivo Window to Study Neurodegenerative Disorders
Linfante and Ashizawa
Arch Neurol 1999;56:1446-1447.
FULL TEXT