Decreased corticokinematic coherence in patients with Friedreich’s Ataxia.
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1
Neurology department, ULB-Hôpital Erasme, Belgium
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2
Laboratoire de Cartographie fonctionnelle du Cerveau (LCFC), ULB Neuroscience Institute, Université libre de Bruxelles (ULB), Belgium
Friedreich’s ataxia (FA) is a genetic neurodegenerative disorder characterized by a progressive loss of spino-cortical and spino-cerebellar proprioceptive neurons leading to limb ataxia and gait instability. This pilot study investigates if the corticokinematic coherence (CKC) method can be used as a potential marker of spino-cortical proprioceptive pathways degeneration in FA. CKC, which indexes the coupling between cortical magnetoencephalography (MEG) signals and movement kinematics, is indeed mainly driven by proprioceptive afferents to the primary sensorimotor (SM1) cortex.
CKC was evaluated using whole-scalp-covering MEG (Elekta) in 11 right-handed FA patients (8 females, mean age: 29 y (range: 9-46 y), mean SARA score: 21.7 (range: 14-30.5)) and five healthy adult subjects (3 females, mean age 34 (range: 30-43 y)) performing active right index finger flexion–extension. Index finger acceleration was monitored with a 3-axis accelerometer. Coherence was used to index the coupling between cortical activity and movement kinematics. Coherent brain areas were identified using dynamic imaging of coherent sources. Non-parametric permutation statistics was used to assess the statistical significance of local coherence maxima.
Movement frequency (F0) was significantly different between patients and controls (Patients, 1.4 ± 0.5 Hz; controls 2.4 ± 0.5 Hz). In both groups, significant coherence was found at movement frequency (F0) and its first harmonic (F1) in the left SM1 cortex (coherence, patients: 0.8 (F0) and 0.17 (F1); subjects: 0.22 (F0) and 0.31 (F1)). Coherence values were significantly lower in patients than in healthy subjects.
This pilot study demonstrates lower CKC levels in FA patients compared with healthy subjects. CKC might therefore represent an interesting method to evaluate spino-cortical proprioceptive pathways degeneration in FA.
Résumé en Français:
L’ataxie de Friedreich (FA) est une maladie neurodégénérative d’origine génétique caractérisée par une perte progressive des neurones essentiels au contrôle des mouvements des membres. Cette dégénérescence est responsable de l’apparition de troubles de l’équilibre et d’une perte de coordination des mouvements. Dans cette étude, nous avons étudié l’activité électrique du cortex somato-sensoriel et l’avons corrélée à la cinétique des mouvements afin de voir si une discordance pouvait être mise en évidence chez les patients FA. Nous avons mis en évidence une cohérence entre la cinétique des mouvements et l’activité cérébrale chez les patients mais moins importante que celle observée chez les volontaires sains. Cette technique d’enregistrement pourrait permettre de diagnostiquer la neurodégénérescence des voies sensorielles responsables du contrôle du mouvement.
Samenvatting in het Nederlands:
De ataxie van Friedreich (FA) is een neurodegeneratieve genetische ziekte gekenmerkt door een voortschrijdend verlies van neuronen essentieel voor de beweging van de ledematen. Dit verval is verantwoordelijk voor evenwichtsstoornissen en verlies van coördinatie van de bewegingen. In deze studie onderzochten we de elektrische activiteit van de somatosensoriele hersenschors en vergeleken we die met de kinetiek van de bewegingen, om na te gaan of er een verband kan gelegd worden bij patiënten die lijden aan FA. We vonden een verband tussen de kinetiek van de bewegingen en de hersenactiviteit bij patiënten, minder belangrijk dan bij gezonde vrijwilligers. Deze registratietechniek moet het mogelijk maken de aftakeling van de sensoriele paden die verantwoordelijk zijn voor de controle van de bewegingen beter te diagnosticeren.
Keywords:
Friedreich Ataxia,
Magnetoencephalography (MEG),
coherence analysis,
somatosensory,
Cerebellum
Conference:
6th Belgian Brain Congress, MONS, Belgium, 8 Oct - 8 Oct, 2016.
Presentation Type:
Poster Presentation
Topic:
Brain and brain diseases: between heredity and environment
Citation:
Naeije
G,
Marty
B,
Wens
V,
Bourguignon
M,
Hari
R,
Jousmäki
V,
Pandolfo
M and
De Tiège
X
(2016). Decreased corticokinematic coherence in patients with Friedreich’s Ataxia..
Conference Abstract:
6th Belgian Brain Congress.
doi: 10.3389/conf.fnagi.2016.03.00071
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Received:
14 Jul 2016;
Published Online:
14 Jul 2016.
*
Correspondence:
MD. Gilles Naeije, Neurology department, ULB-Hôpital Erasme, brussels, Belgium, gilles.naeije@erasme.ulb.ac.be
Mr. Brice Marty, Neurology department, ULB-Hôpital Erasme, brussels, Belgium, bmarty@ulb.ac.be