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Brain atrophy in primary lateral sclerosis

From the Departments of Clinical Neurological Sciences (M.C.T., A.R., K.F., M.J.S.) and Diagnostic Radiology and Nuclear Medicine Radiology (D.H.L., K.K.), London Health Sciences Centre, Canada; Memory and Aging Center (M.C.T., V.L., J.H.K.), University of California, San Francisco; and Molecular Brain Research Group (M.J.S.), Robarts Research Institute, The University of Western Ontario, London, Canada.

Address correspondence and reprint requests to Dr. Michael J. Strong, Rm C7-120, University Hospital, London Health Sciences Centre, 339 Windermere Road, London, Ontario, Canada

Background: Primary lateral sclerosis (PLS) is an idiopathic upper motor neuron degenerative disorder. The aim of this study was to compare brain volumes in patients with PLS and controls and determine whether differences were due to loss of gray matter (GM), white matter (WM), or both.

Methods: T1-weighted images were acquired in patients with PLS and controls. Freesurfer was used for volumetric segmentation of whole brain, cortical GM, precentral and postcentral cortex, WM, corpus callosum, basal ganglia, thalamus, cerebellum, and CSF. Relationships were sought between disease severity, disease duration, age and brain volumes.

Results: Eleven patients with PLS and 10 age-matched healthy controls were included in this study. Compared to control subjects, patients with PLS had significantly smaller whole brain (p = 0.043), frontal lobe (p = 0.036), precentral cortex (p = 0.016), and corpus callosum (p = 0.036) volumes. There was a trend toward a smaller thalamus (p = 0.051). Disease severity correlated with ventricular CSF volume (rho = –0.604, p = 0.025) and precentral cortex volume loss (rho = 0.599, p = 0.026). Disease duration tended to correlate with a loss of WM (rho = –0.636, p = 0.063).

Conclusions: Our results suggest that there is focal atrophy in patients with primary lateral sclerosis compared with controls especially in the precentral cortex and the corpus callosum, specifically where there is transfer of motor fibers.

ALS = amyotrophic lateral sclerosis; ALSFRS-R = ALS Functional Rating Scale; fSPGR = fast spoiled gradient echo sequence; GM = gray matter; PLS = primary lateral sclerosis; TE = echo time; TR = repetition time; WM = white matter.

mstrong{at}uwo.ca

Supported by funds from the ALS Association (ALSA) and the ALS patients of Southwestern Ontario.

Disclosure: The authors report no disclosures.

Received October 15, 2008. Accepted in final form December 30, 2008.