R Zutt1, J W Elting1, J H van der Hoeven1, F Lange1, M A J Tijssen2. 1. University Groningen, University Medical Center Groningen, Department of Neurology, NL-9700 RB Groningen, The Netherlands. 2. University Groningen, University Medical Center Groningen, Department of Neurology, NL-9700 RB Groningen, The Netherlands. Electronic address: m.a.j.de.koning-tijssen@umcg.nl.
Abstract
OBJECTIVE: To evaluate the accuracy of clinical phenotyping of myoclonus patients and to determine differentiating clinical characteristics between cortical (CM), subcortical (SCM), spinal (SM), peripheral (PM) myoclonus, and functional jerks (FJ). METHODS: Clinical notes for all patients with myoclonus over an 8-year period (2006-2014) were reviewed retrospectively. We used the conclusion of electrophysiological testing as definite diagnosis of myoclonus or FJ. RESULTS: 85 patients were identified suffering from CM (34%), SCM (11%), SM (6%), PM (2%), and 47% FJ. The clinical diagnosis of myoclonus was confirmed by electrophysiological testing in 74% and its subtype in 78% of cases. CM was characterized by an early age of onset, facial myoclonus, and provocation by action. Differentiating features of FJ were an abrupt onset, preceding contributing events and provocation by a supine position. CONCLUSION: The majority of clinical myoclonic jerk cases were functional in our heterogeneous tertiary clinic cohort. CM was the main anatomical myoclonic subtype. Clinical diagnosis was accurate in the majority of cases, although electrophysiological testing was important to verify the clinical classification. SIGNIFICANCE: In patients with jerky movements a functional diagnosis should be considered. Determination of the myoclonic subtypes is important to initiate tailored treatment.
OBJECTIVE: To evaluate the accuracy of clinical phenotyping of myoclonuspatients and to determine differentiating clinical characteristics between cortical (CM), subcortical (SCM), spinal (SM), peripheral (PM) myoclonus, and functional jerks (FJ). METHODS: Clinical notes for all patients with myoclonus over an 8-year period (2006-2014) were reviewed retrospectively. We used the conclusion of electrophysiological testing as definite diagnosis of myoclonus or FJ. RESULTS: 85 patients were identified suffering from CM (34%), SCM (11%), SM (6%), PM (2%), and 47% FJ. The clinical diagnosis of myoclonus was confirmed by electrophysiological testing in 74% and its subtype in 78% of cases. CM was characterized by an early age of onset, facial myoclonus, and provocation by action. Differentiating features of FJ were an abrupt onset, preceding contributing events and provocation by a supine position. CONCLUSION: The majority of clinical myoclonic jerk cases were functional in our heterogeneous tertiary clinic cohort. CM was the main anatomical myoclonic subtype. Clinical diagnosis was accurate in the majority of cases, although electrophysiological testing was important to verify the clinical classification. SIGNIFICANCE: In patients with jerky movements a functional diagnosis should be considered. Determination of the myoclonic subtypes is important to initiate tailored treatment.
Authors: Rodi Zutt; Jan W Elting; Jonathan C van Zijl; J Han van der Hoeven; Christiaan M Roosendaal; Jeannette M Gelauff; Kathryn J Peall; Marina A J Tijssen Journal: Neurology Date: 2018-01-19 Impact factor: 9.910
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Authors: Tanja Schmitz-Hübsch; Silke Lux; Peter Bauer; Alexander U Brandt; Elena Schlapakow; Susanne Greschus; Michael Scheel; Hanna Gärtner; Mehmet E Kirlangic; Vincent Gras; Dagmar Timmann; Matthis Synofzik; Alejandro Giorgetti; Paolo Carloni; Jon N Shah; Ludger Schöls; Ute Kopp; Lisa Bußenius; Timm Oberwahrenbrock; Hanna Zimmermann; Caspar Pfueller; Ella-Maria Kadas; Maria Rönnefarth; Anne-Sophie Grosch; Matthias Endres; Katrin Amunts; Friedemann Paul; Sarah Doss; Martina Minnerop Journal: Ann Clin Transl Neurol Date: 2021-03-19 Impact factor: 4.511
Authors: Sterre van der Veen; Rodi Zutt; Christine Klein; Connie Marras; Samuel F Berkovic; John N Caviness; Hiroshi Shibasaki; Tom J de Koning; Marina A J Tijssen Journal: Mov Disord Date: 2019-10-04 Impact factor: 10.338