Clinical and Electrophysiological Characterization of Essential Tremor in 18 Children and Adolescents.

children essential tremor myoclonic jerks quality of life

Journal

Tremor and other hyperkinetic movements (New York, N.Y.)
ISSN: 2160-8288
Titre abrégé: Tremor Other Hyperkinet Mov (N Y)
Pays: England
ID NLM: 101569493

Informations de publication

Date de publication:
2023
Historique:
received: 01 08 2023
accepted: 03 12 2023
medline: 25 12 2023
pubmed: 25 12 2023
entrez: 25 12 2023
Statut: epublish

Résumé

Essential tremor (ET) is considered the most frequent abnormal movement in the general population, with childhood onset in 5 to 30% of the patients. A multicenter, descriptive cross-sectional study enrolled patients ⩽18 years with a definite diagnosis of ET according to the International Parkinson and Movement Disorders Society criteria. Demographic data, clinical and electrophysiological characteristics of the tremor, neurological examination and impact on quality of life were collected. 9 males and 9 females were included (mean age of 13.9 years). Tremor was characterized by : upper limb onset at a mean age of 6.5 years; at enrollment, upper limbs localization, and involvement of an additional body region in 28% of the patients; kinetic tremor in all of the patients combined with postural tremor in 17 and rest tremor in 3; tremor mean frequency of 7.6 Hz, mean burst duration of 82.7 ms; identification of mild myoclonic jerks on the polymyographic recordings in 7 patients; altered quality of life with worse emotional outcomes in girls and when a disease duration >5 years was suggested. Childhood-onset ET is associated with delayed diagnosis and remarkable functional impact. Electromyographic identification of additional mild myoclonus is a new finding whose significance is discussed. ET onset involved upper limbs and at inclusion, 28% of the patients exhibited involvement of an additional body region.ET impacted quality of life for all patients.Girls and patients affected for >5 years reported worse emotional outcomes.Mild myoclonic jerks were identified on 7/17 polymyographic recordings.

Sections du résumé

Background UNASSIGNED
Essential tremor (ET) is considered the most frequent abnormal movement in the general population, with childhood onset in 5 to 30% of the patients.
Methods UNASSIGNED
A multicenter, descriptive cross-sectional study enrolled patients ⩽18 years with a definite diagnosis of ET according to the International Parkinson and Movement Disorders Society criteria. Demographic data, clinical and electrophysiological characteristics of the tremor, neurological examination and impact on quality of life were collected.
Results UNASSIGNED
9 males and 9 females were included (mean age of 13.9 years). Tremor was characterized by : upper limb onset at a mean age of 6.5 years; at enrollment, upper limbs localization, and involvement of an additional body region in 28% of the patients; kinetic tremor in all of the patients combined with postural tremor in 17 and rest tremor in 3; tremor mean frequency of 7.6 Hz, mean burst duration of 82.7 ms; identification of mild myoclonic jerks on the polymyographic recordings in 7 patients; altered quality of life with worse emotional outcomes in girls and when a disease duration >5 years was suggested.
Discussion UNASSIGNED
Childhood-onset ET is associated with delayed diagnosis and remarkable functional impact. Electromyographic identification of additional mild myoclonus is a new finding whose significance is discussed.
Highlights UNASSIGNED
ET onset involved upper limbs and at inclusion, 28% of the patients exhibited involvement of an additional body region.ET impacted quality of life for all patients.Girls and patients affected for >5 years reported worse emotional outcomes.Mild myoclonic jerks were identified on 7/17 polymyographic recordings.

Identifiants

pubmed: 38145278
doi: 10.5334/tohm.803
pmc: PMC10742103
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

46

Informations de copyright

Copyright: © 2023 The Author(s).

Déclaration de conflit d'intérêts

The authors have no competing interests to declare.

Auteurs

Julie Piarroux (J)

Department of Pediatric Neurology, Gui de Chauliac University Hospital, Montpellier, France.

Evgenia Dimopoulou (E)

Department of Pediatric Neurology, Gui de Chauliac University Hospital, Montpellier, France.

Guillaume Taieb (G)

University of Montpellier, CNRS (IGF), Department of Neurology, Montpellier, University Hospital 34295 Montpellier, France.

Sarah Souvannanorath (S)

Reference center for neuromuscular diseases, Henri-Mondor University Hospital, Assistance publique-Hôpitaux de Paris, Créteil, France.

Emmanuel Roze (E)

Department of Neurology, Salpêtrière Hospital, Sorbonne University and Assistance Publique - Hôpitaux de Paris, Paris, France.

Laurence Lion-François (L)

Department of Pediatric Neurology, Lyon University Hospital, Bron, France.

Marie-Aude Spitz (MA)

Department of Pediatry, Strasbourg University Hospitals, Strasbourg, France.

Emmanuel Broussolle (E)

Research Unit UMR 5229, Marc-Jeannerod Institute of Cognitive Science, French National Center for Scientific Research (CNRS), University of Lyon, Bron, France.
Department of Neurology C, Civil Hospices of Lyon, Pierre-Wertheimer Neurological Hospital, Bron, France.
Faculty of Medicine Lyon-Sud Charles-Mérieux, University of Lyon, Oullins, France.

Chloé Laurencin (C)

Department of Neurology C, Civil Hospices of Lyon, Pierre-Wertheimer Neurological Hospital, Bron, France.
Lyon Neuroscience Research Center (CRNL), INSERM U1028, CNRS UMR5292, University Lyon 1, Lyon F-69000, France.

Jean-Baptiste Chanson (JB)

Department of Neurology, Strasbourg University Hospitals, Strasbourg, France and Reference centre for neuromuscular diseases Nord/Est/Ile-de-France, France.

Johanna Belleville-Goffeney (J)

Departement of Pediatric Neurology, Jean-Minjoz University Hospital, Besançon, France.

Marie Céline François-Heude (MC)

Department of Pediatric Neurology, Gui de Chauliac University Hospital, Montpellier, France.

Pierre Meyer (P)

Department of Pediatric Neurology, Gui de Chauliac University Hospital, Montpellier, France.

Mirna Khalil (M)

Clinical Investigation Center, Montpellier University Hospital, France. INSERM, CIC1411, Montpellier, France.

Maelle Dereure (M)

Clinical Research and Epidemiology Unit, La Colombière University Hospital, Montpellier, France.

Diane Doummar (D)

Department of Pediatric Neurology and developmental pathologies, Sorbonne University and Trousseau University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France, APHP. SU, FHU I2D2, F-75012, Paris, France.

Hugues Chevassus (H)

Clinical Investigation Center, Montpellier University Hospital, France. INSERM, CIC1411, Montpellier, France.

Emmanuelle Apartis (E)

Department of Neurophysiology, Saint-Antoine Hospital, Assistance Publique-Hôpitaux de Paris, INSERM U1127, CNRS UMR7225, UM75, ICM, 75013 Paris, France.

Agathe Roubertie (A)

Department of Pediatric Neurology, Gui de Chauliac University Hospital, Montpellier, France.
Institute for Neurosciences of Montpellier, INSERM U 1298, University of Montpellier, Montpellier, France.

Classifications MeSH