| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
From the Departments of Clinical Neurological Sciences (Drs. Strong and Grace), Psychology (Dr. Grace), and Communication Sciences and Disorders (Drs. Orange and Leeper, and C. Aere), The University of Western Ontario; and the John P. Robarts Research Institute (Drs. Strong and Menon), London, Ontario, Canada.
Address correspondence and reprint requests to Dr. Michael J. Strong, Room 7OF10, University Campus, London Health Sciences Center, 339 Windermere Road, London, Ontario, Canada N6A 5A5.
OBJECTIVE: To characterize prospectively the cognitive profile in ALS.
METHODS: Clinically definite ALS patients (11 men, 2 women), age 39.9 to 74.0 years (mean age, 54.2 ± 9.6 years; mean disease duration, 21.1 ± 10.5 months) underwent neuropsychologic, language, and speech testing followed by MR 1H spectroscopy (4 T). Five spousal control subjects completed an identical protocol. Eight ALS patients participated in follow-up studies at a 6-month interval.
RESULTS: Relative to control subjects, ALS patients showed mild impairment in word generation, recognition memory (faces), and motor-free visual perception. Bulbar-onset patients showed greater impairment in a number of measures (working memory, problem solving/cognitive flexibility, visual perception, and recognition memory for words and faces), and cognitive impairment appeared more progressive over time. ALS patients demonstrated anomia on a confrontation naming test, with no significant problems following commands or repeating. Speech motor performance scores and intelligibility scores were not significantly different. No significant declines in forced vital capacity, forced expiratory volume, or peak expiratory flow rates were observed. Although normal at initial testing (T1), MR 1H spectroscopy demonstrated a reduction of the N-acetylaspartate/creatine (NAA/Cr) ratio in the nondominant precentral motor strip across the two testing intervals. In contrast, the NAA/Cr ratio obtained from the anterior cingulate gyrus at T1 was already reduced in bulbar-onset patients (p < 0.001), whereas no deficits were observed in limb-onset individuals in the same region.
CONCLUSIONS: Bulbar-onset ALS patients with cognitive impairments and neuronal loss in the anterior cingulate gyrus subsequently developed more profound neuropsychological dysfunction whereas both language and speech capabilities remained relatively preserved. Of note, the absence of bulbar signs did not predict an absence of cognitive decline.
Key words: Cognitive impairment—ALS.
This article has been cited by other articles:
|
|
 |
|
  G Logroscino, B J Traynor, O Hardiman, A Chio', P Couratier, J D Mitchell, R J Swingler, E Beghi, and for EURALS Descriptive epidemiology of amyotrophic lateral sclerosis: new evidence and unsolved issues J. Neurol. Neurosurg. Psychiatry, January 1, 2008; 79(1): 6 - 11. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J Lakerveld, B Kotchoubey, and A Kubler Cognitive function in patients with late stage amyotrophic lateral sclerosis J. Neurol. Neurosurg. Psychiatry, January 1, 2008; 79(1): 25 - 29. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Hammad, A. Silva, J. Glass, J. T. Sladky, and M. Benatar Clinical, electrophysiologic, and pathologic evidence for sensory abnormalities in ALS Neurology, December 11, 2007; 69(24): 2236 - 2242. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 H. Mori, A. Yagishita, T. Takeda, and T. Mizutani Symmetric Temporal Abnormalities on MR Imaging in Amyotrophic Lateral Sclerosis with Dementia AJNR Am. J. Neuroradiol., September 1, 2007; 28(8): 1511 - 1516. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. Irwin, C. F. Lippa, and J.M. Swearer Cognition and Amyotrophic Lateral Sclerosis (ALS) American Journal of Alzheimer's Disease and Other Dementias, September 1, 2007; 22(4): 300 - 312. [Abstract] [PDF]
|
|
|
|
 |
|
 J. R. Duffy, R. K. Peach, and E. A. Strand Progressive Apraxia of Speech as a Sign of Motor Neuron Disease Am J Speech Lang Pathol, August 1, 2007; 16(3): 198 - 208. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D.M. Mezzapesa, A. Ceccarelli, F. Dicuonzo, A. Carella, M.F. De Caro, M. Lopez, V. Samarelli, P. Livrea, and I.L. Simone Whole-Brain and Regional Brain Atrophy in Amyotrophic Lateral Sclerosis AJNR Am. J. Neuroradiol., February 1, 2007; 28(2): 255 - 259. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. A. Karim, T. Hinterberger, J. Richter, J. Mellinger, N. Neumann, H. Flor, A. Kubler, and N. Birbaumer Neural Internet: Web Surfing with Brain Potentials for the Completely Paralyzed Neurorehabil Neural Repair, December 1, 2006; 20(4): 508 - 515. [Abstract] [PDF]
|
|
|
|
|
|
K M Robinson, S C Lacey, P Grugan, G Glosser, M Grossman, and L F McCluskey Cognitive functioning in sporadic amyotrophic lateral sclerosis: a six month longitudinal study J. Neurol. Neurosurg. Psychiatry, May 1, 2006; 77(5): 668 - 670. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. Hardy, P. Momeni, and B. J. Traynor Frontal temporal dementia: dissecting the aetiology and pathogenesis. Brain, April 1, 2006; 129(Pt 4): 830 - 831. [Full Text] [PDF]
|
|
|
|
 |
|
 G. A. Rippon, N. Scarmeas, P. H. Gordon, P. L. Murphy, S. M. Albert, H. Mitsumoto, K. Marder, L. P. Rowland, and Y. Stern An Observational Study of Cognitive Impairment in Amyotrophic Lateral Sclerosis. Arch Neurol, March 1, 2006; 63(3): 345 - 352. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. K. Olney, J. Murphy, D. Forshew, E. Garwood, B. L. Miller, S. Langmore, M. A. Kohn, and C. Lomen-Hoerth The effects of executive and behavioral dysfunction on the course of ALS Neurology, December 13, 2005; 65(11): 1774 - 1777. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G. M. Ringholz, S. H. Appel, M. Bradshaw, N. A. Cooke, D. M. Mosnik, and P. E. Schulz Prevalence and patterns of cognitive impairment in sporadic ALS Neurology, August 23, 2005; 65(4): 586 - 590. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. L. Chang, C. Lomen-Hoerth, J. Murphy, R. G. Henry, J. H. Kramer, B. L. Miller, and M. L. Gorno-Tempini A voxel-based morphometry study of patterns of brain atrophy in ALS and ALS/FTLD Neurology, July 12, 2005; 65(1): 75 - 80. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Abrahams, P. N. Leigh, and L. H. Goldstein Cognitive change in ALS: A prospective study Neurology, April 12, 2005; 64(7): 1222 - 1226. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Abrahams, L. H. Goldstein, A. Simmons, M. Brammer, S. C. R. Williams, V. Giampietro, and P. N. Leigh Word retrieval in amyotrophic lateral sclerosis: a functional magnetic resonance imaging study Brain, July 1, 2004; 127(7): 1507 - 1517. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Maekawa, S. Al-Sarraj, M. Kibble, S. Landau, J. Parnavelas, D. Cotter, I. Everall, and P. N. Leigh Cortical selective vulnerability in motor neuron disease: a morphometric study Brain, June 1, 2004; 127(6): 1237 - 1251. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
W. Yang, M. M. Sopper, C. Leystra-Lantz, and M. J. Strong Microtubule-associated tau protein positive neuronal and glial inclusions in ALS Neurology, December 23, 2003; 61(12): 1766 - 1773. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
N Neumann and N Birbaumer Predictors of successful self control during brain-computer communication J. Neurol. Neurosurg. Psychiatry, August 1, 2003; 74(8): 1117 - 1121. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. Lomen-Hoerth, J. Murphy, S. Langmore, J. H. Kramer, R. K. Olney, and B. Miller Are amyotrophic lateral sclerosis patients cognitively normal? Neurology, April 8, 2003; 60(7): 1094 - 1097. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. M. Wilson, G. M. Grace, D. G. Munoz, B. P. He, and M. J. Strong Cognitive impairment in sporadic ALS: A pathologic continuum underlying a multisystem disorder Neurology, August 28, 2001; 57(4): 651 - 657. [Abstract] [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
