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Eur J Neurol. 2017 Jan;24(1):122-129. doi: 10.1111/ene.13163. Epub 2016 Oct 18.

Structural explanation of poor prognosis of amyotrophic lateral sclerosis in the non-demented state.

Author information

1
Department of Neurology, College of Medicine, Hanyang University, Seoul, Korea.
2
Department of Neurology, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea.
3
Department of Psychiatry, Center for Brain Health, NYU School of Medicine, New York, NY, USA.
4
Department of Neurology, NYU School of Medicine, New York, NY, USA.

Abstract

BACKGROUND AND PURPOSE:

Amyotrophic lateral sclerosis (ALS), a motor neuron disease, is associated with various cortical symptoms including mild cognitive decline with behavior changes, suggesting the involvement of extra-motor areas in ALS. Our aim was to investigate the specific patterns of brain atrophy in sporadic, impaired ALS patients without commonly known genetic mutations using voxel-based morphometry.

MATERIALS AND METHODS:

Forty-seven patients with sporadic ALS and 28 age-matched healthy controls were recruited. ALS participants were divided into three groups according to comprehensive neuropsychological testing: pure (ALS-pure), cognitive impairment (ALSci) and behavioral impairment (ALSbi). Quantitative comparison of brain atrophy patterns was performed amongst these three groups using voxel-based analysis. All analyses were adjusted for total intracranial volume, age, sex, disease duration and functional disability score.

RESULTS:

The ALSci group exhibited decreased volume in the left cerebellum, fusiform gyrus, optic radiations and corticospinal tracts compared to healthy controls. ALSci patient imaging showed decreased brain volume in the bilateral cerebellum, right putamen gray matter and bilateral superior longitudinal fasciculi white matter compared to pure ALS patients (P < 0.001 uncorrected, corrected for the entire volume). Compared to healthy controls, ALS-pure and ALSbi groups did not show any significant volume changes in gray and white matter.

CONCLUSIONS:

These findings also support the hypothesis that ALS pathogenesis has a dual focality of onset (cortex and anterior horn) with contiguous spread outwards. Additionally, neuropsychological features may be an important predictor of progression and survival rates in ALS.

KEYWORDS:

amyotrophic lateral sclerosis; atrophy; cognitive impairment; voxel-based morphometry

PMID:
27753163
PMCID:
PMC5436047
DOI:
10.1111/ene.13163
[Indexed for MEDLINE]
Free PMC Article

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