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Arterial stiffness and brain integrity: A review of MRI findings.

Author(s): Badji A, Sabra D, Bherer L, Cohen-Adad J, Girouard H, Gauthier CJ

Ageing Res Rev. 2019 May 04;53:100907 Authors: Badji A, Sabra D, Bherer L, Cohen-Adad J, Girouard H, Gauthier CJ

Article GUID: 31063866
The spinal and cerebral profile of adult spinal-muscular atrophy: A multimodal imaging study.

Author(s): Querin G, El Mendili MM, Lenglet T, Behin A, Stojkovic T, Salachas F, Devos D, Le Forestier N, Del Mar Amador M, Debs R, Lacomblez L, Mening...

The spinal and cerebral profile of adult spinal-muscular atrophy: A multimodal imaging study.

Neuroimage Clin. 2019;21:101618

Authors: Querin G, El Mendili MM, Lenglet T, Behin ...

Article GUID: 30522974
Title:The spinal and cerebral profile of adult spinal-muscular atrophy: A multimodal imaging study.
Authors:Querin G,  El Mendili MM,  Lenglet T,  Behin A,  Stojkovic T,  Salachas F,  Devos D,  Le Forestier N,  Del Mar Amador M,  Debs R,  Lacomblez L,  Meninger V,  Bruneteau G,  Cohen-Adad J,  Lehéricy S,  Laforêt P,  Blancho S,  Benali H,  Catala M,  Li M,  Marchand-Pauvert V,  Hogrel JY,  Bede P,  Pradat PF
Link:www.ncbi.nlm.nih.gov/pubmed/30522974?dopt=Abstract
Category:Neuroimage Clin
PMID:30522974
Dept Affiliation: NA
1 Sorbonne Université, CNRS, INSERM, Laboratoire d'Imagerie Biomédicale, Paris, France.
2 Sorbonne Université, CNRS, INSERM, Laboratoire d'Imagerie Biomédicale, Paris, France; Icahn School of Medicine at Mount Sinai, Department of Neurology, New York, USA.
3 APHP, Département de Neurologie, Hôpital Pitié-Salpêtrière, Centre référent SLA, Paris, France; APHP, Hôpital Pitié-Salpêtriere, Service d'Explorations Fonctionnelles, Paris, France.
4 APHP, Centre de Référence Maladies Neuromusculaires Paris-Est, Institut de Myologie, Hôpital Pitié-Salpêtrière, Paris, France.
5 APHP, Département de Neurologie, Hôpital Pitié-Salpêtrière, Centre référent SLA, Paris, France.
6 Department of Neurology, ALS Centre, Lille University, INSERM UMRS_1171, University Hospital Centre, LICEND COEN Centre, Lille, France; Department of Medical Pharmacology, Lille University, INSERM UMRS_1171, University Hospital Centre, LICEND COEN Centre, Lille, France.
7 APHP, Département de Neurologie, Hôpital Pitié-Salpêtrière, Centre référent SLA, Paris, France; Département de recherche en éthique, EA 1610: Etudes des sciences et techniques, Université Paris Sud/Paris Saclay, Paris, France.
8 Sorbonne Université, CNRS, INSERM, Laboratoire d'Imagerie Biomédicale, Paris, France; APHP, Département de Neurologie, Hôpital Pitié-Salpêtrière, Centre référent SLA, Paris, France.
9 Hôpital des Peupliers, Ramsay Générale de Santé, F-75013 Paris, France.
10 NeuroPoly Lab, Institute of Biomedical Engineering, Polytechnique Montreal, Montreal, QC, Canada; Functional Neuroimaging Unit, CRIUGM, Université de Montréal, Montreal, QC, Canada.
11 APHP, Hôpital Pitié-Salpêtriere, Service de Neuroradiologie, Paris, France; Sorbonne Université, UMR-S975, Inserm U975, CNRS UMR7225, Centre de recherche de l'Institut du Cerveau et de la Moelle épinière - CRICM, Centre de Neuroimagerie de Recherche - CENIR, Paris, France.
12 Neurology Department, Nord/Est/Ile de France neuromuscular center, Raymond-Poincaré Hospital, G

Description:

The spinal and cerebral profile of adult spinal-muscular atrophy: A multimodal imaging study.



Neuroimage Clin. 2019;21:101618



Authors: Querin G, El Mendili MM, Lenglet T, Behin A, Stojkovic T, Salachas F, Devos D, Le Forestier N, Del Mar Amador M, Debs R, Lacomblez L, Meninger V, Bruneteau G, Cohen-Adad J, Lehéricy S, Laforêt P, Blancho S, Benali H, Catala M, Li M, Marchand-Pauvert V, Hogrel JY, Bede P, Pradat PF



Abstract

Spinal muscular atrophy (SMA) type III and IV are autosomal recessive, slowly progressive lower motor neuron syndromes. Nevertheless, wider cerebral involvement has been consistently reported in mouse models. The objective of this study is the characterisation of spinal and cerebral pathology in adult forms of SMA using multimodal quantitative imaging.

METHODS: Twenty-five type III and IV adult SMA patients and 25 age-matched healthy controls were enrolled in a spinal cord and brain imaging study. Structural measures of grey and white matter involvement and diffusion parameters of white matter integrity were evaluated at each cervical spinal level. Whole-brain and region-of-interest analyses were also conducted in the brain to explore cortical thickness, grey matter density and tract-based white matter alterations.

RESULTS: In the spinal cord, considerable grey matter atrophy was detected between C2-C6 vertebral levels. In the brain, increased grey matter density was detected in motor and extra-motor regions of SMA patients. No white matter pathology was identified neither at brain and spinal level.

CONCLUSIONS: Adult forms of SMA are associated with selective grey matter degeneration in the spinal cord with preserved white matter integrity. The observed increased grey matter density in the motor cortex may represent adaptive reorganisation.



PMID: 30522974 [PubMed - in process]