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Amyotrophic Lateral Sclerosis

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PET and SPECT in Neurology

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Abstract

The core of the clinical syndrome of amyotrophic lateral sclerosis (ALS) is a progressive and typically rapid degeneration of a previously normally functioning motor system, comprising upper motor neurons (UMNs) of the primary motor cortex and corticospinal tract (CST), brainstem nuclei and the lower motor neurons (LMNs) arising from the anterior horns of the spinal cord. Weakness, with variable wasting, of the musculature of the limbs and of speech and swallowing ensues, with involvement of the diaphragm resulting in respiratory insufficiency and a median survival of 3 years. The marked clinical heterogeneity, frequent diagnostic delay and reliance of therapeutic trials on survival as the primary outcome measure make the discovery of biomarkers in ALS a research priority.

Whilst the spinal anterior horns and corticospinal tract superficially appear to bear the brunt of histopathology in ALS, it has been clear for over half a century that degeneration extends to involve the extra-motor brain, preferentially involving areas of the frontal and temporal lobes having clinical and genetic overlap with some forms of frontotemporal dementia (FTD).

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Abbreviations

5-HT:

5-Hydroxytriptamine (serotonin)

ALS:

Amyotrophic lateral sclerosis

CBF:

Cerebral blood flow

CST:

Corticospinal tract

DLPFC:

Dorsolateral prefrontal cortex

ECD:

Ethyl cysteinate dimer

fALS:

familial ALS

FDG:

Fluorodeoxyglucose

FTD:

Frontotemporal dementia

FU:

Follow-up

GABA:

γ-Aminobutyric acid

HC:

Healthy controls

HMPAO:

Hexamethylpropyleneamine oxime

IMP:

Iodoamphetamine

KD:

Kennedy’s disease

LMN:

Lower motor neuron

PLS:

Primary lateral sclerosis

PMA:

Progressive muscular atrophy

PMC:

Primary motor cortex

SOD1:

Superoxide dismutase (ps, presymptomatic; hom, homozygous)

TARDBP:

Transactive region DNA-binding protein

UMN:

Upper motor neuron

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Authors and Affiliations

  1. Nuffield Department of Clinical Neurosciences, West Wing Level 3, John Radcliffe Hospital, Oxford University, Oxford, OX3 9DU, UK

    Martin R. Turner

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  1. Martin R. Turner
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Correspondence to Martin R. Turner .

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Editors and Affiliations

  1. Dept. of Nuclear Medicine and Molecular Imaging, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands

    Rudi A.J.O. Dierckx

  2. Faculty of Electrical Engineering and Information Technology, University of Applied Sciences Offenburg, Offenburg, Germany

    Andreas Otte

  3. Nuclear Medicine and Molecular Imaging, University Medical Center Groningen, Groningen, The Netherlands

    Erik F.J. de Vries

  4. Nuclear Medicine and Molecular Imaging, University Medical Center Groningen, Groningen, The Netherlands

    Aren van Waarde

  5. Neurology, University Medical Center Groningen, Groningen, The Netherlands

    Klaus L. Leenders

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Turner, M.R. (2014). Amyotrophic Lateral Sclerosis. In: Dierckx, R., Otte, A., de Vries, E., van Waarde, A., Leenders, K. (eds) PET and SPECT in Neurology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-54307-4_29

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