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Estimation of brainstem neuronal loss in amyotrophic lateral sclerosis with in vivo proton magnetic resonance spectroscopy

From the Department of Neurology (Dr. Cwik), University of Arizona, Tucson, AZ, and the Departments of Biomedical Engineering (Drs. Hanstock and Allen) and Medicine, Division of Neurology (Dr. Martin), University of Alberta, Edmonton, Alberta, Canada.

Address correspondence and reprint requests to Dr. Valerie A. Cwik, Department of Neurology, University of Arizona Health Sciences Center, Box 245023, 1501 N. Campbell Avenue, Tucson, AZ 85724.

In vivo proton magnetic resonance spectroscopy (MRS) may be used to quantify brainstem neuronal degeneration in ALS because of the neuronal localization of N-acetylaspartate and N-acetylaspartylglutamate, together termed NA, which are estimated with this technique. We measured the ratio of NA to creatine/phosphocreatine(NA/Cr) with proton MRS at 3.0 tesla (T) in a 4.3-cm³ volume in the pons and upper medulla of 12 ALS patients and 17 age-matched control subjects. Brainstem NA/Cr was reduced in ALS versus control subjects (mean± SD: 1.57 ± 0.20 versus 1.95 ± 0.14; p< 0.0001). Patients with severe spasticity or prominent bulbar weakness had the lowest NA/Cr ratios; those with predominantly lower motor neuron limb weakness had near-normal ratios. We conclude that proton MRS may quantify region-specific neuronal dysfunction in ALS.

Supported by the H.M. Toupin Medical Foundation.

Received February 12, 1997. Accepted in final form July 25, 1997.

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