Arwa Rekik1, Amina Nasri1,2,3, Saloua Mrabet1,2,3, Alya Gharbi1,2,3, Amira Souissi1,2,3, Amina Gargouri1,2,3, Imen Kacem1,2,3, Riadh Gouider4,5,6. 1. Neurology Department, Razi University Hospital, 1 rue des orangers Manouba, LR18SP032010, Tunis, Tunisia. 2. Faculty of Medicine of Tunis, University of Tunis El Manar, 15, Rue Djebel Akhdhar, La Rabta, 1007, Tunis, Tunisia. 3. Clinical Investigation Center (CIC) "Neurosciences and Mental Health", Razi University Hospital, 1 rue des orangers Manouba, 2010, Tunis, Tunisia. 4. Neurology Department, Razi University Hospital, 1 rue des orangers Manouba, LR18SP032010, Tunis, Tunisia. riadh.gouider@gnet.tn. 5. Faculty of Medicine of Tunis, University of Tunis El Manar, 15, Rue Djebel Akhdhar, La Rabta, 1007, Tunis, Tunisia. riadh.gouider@gnet.tn. 6. Clinical Investigation Center (CIC) "Neurosciences and Mental Health", Razi University Hospital, 1 rue des orangers Manouba, 2010, Tunis, Tunisia. riadh.gouider@gnet.tn.
Abstract
BACKGROUND: Midline essential tremor (Mid-ET) is a distinctive group of essential tremor (ET) in which tremor affects the neck, jaw, tongue, and/or voice. For long, it has been considered as an ultimate stage of the disease and a marker of its severity. However, recent studies pointed its complexity in terms of non-motor presentation. Thus, we aimed to investigate the non-motor signs (NMS) in Mid-ET. DESIGN: We conducted a cross-sectional study in a tertiary neurology referral center including ET patients classified into two groups based on the presence or not of midline tremor (Mid-ET vs. No-Mid-ET). We assessed NMS using the non-motor severity scale (NMSS), a large battery of cognitive tests, clinical and electrophysiological study of the autonomic nervous system along with the evaluation of sleep disturbances. RESULTS: A total of 163 patients were included: Mid-ET (n = 79) and No-Mid-ET (n = 84) matched in gender and age of onset. Mid-ET patients had higher proportion of late-onset ET (> 60 years old, p = 0.002) and more extrapyramidal signs (p = 0.005). For NMS, Mid-ET was marked with cognitive dysfunction (p = 0.008). The hallmarks of the neuropsychiatric profile of Mid-ET were executive dysfunction (p = 0.004), attention problems (p < 0.000), episodic memory impairment (p = 0.003), and greater depression (p = 0.010). The presence of RBD was a trait of Mid-ET (p = 0.039). In both Mid-ET and No-Mid-ET phenotypes, clinical and neurophysiological dysautonomia correlated with cognitive dysfunction. CONCLUSION: Mid-ET patients had greater cognitive dysfunction, depression, RBD, higher proportion of late-onset ET, and more extrapyramidal signs. Taken all together, these findings could provide a redesigned insight into the underlying physiopathology of Mid-ET indicative of a greater cerebellar dysfunction.
BACKGROUND: Midline essential tremor (Mid-ET) is a distinctive group of essential tremor (ET) in which tremor affects the neck, jaw, tongue, and/or voice. For long, it has been considered as an ultimate stage of the disease and a marker of its severity. However, recent studies pointed its complexity in terms of non-motor presentation. Thus, we aimed to investigate the non-motor signs (NMS) in Mid-ET. DESIGN: We conducted a cross-sectional study in a tertiary neurology referral center including ET patients classified into two groups based on the presence or not of midline tremor (Mid-ET vs. No-Mid-ET). We assessed NMS using the non-motor severity scale (NMSS), a large battery of cognitive tests, clinical and electrophysiological study of the autonomic nervous system along with the evaluation of sleep disturbances. RESULTS: A total of 163 patients were included: Mid-ET (n = 79) and No-Mid-ET (n = 84) matched in gender and age of onset. Mid-ET patients had higher proportion of late-onset ET (> 60 years old, p = 0.002) and more extrapyramidal signs (p = 0.005). For NMS, Mid-ET was marked with cognitive dysfunction (p = 0.008). The hallmarks of the neuropsychiatric profile of Mid-ET were executive dysfunction (p = 0.004), attention problems (p < 0.000), episodic memory impairment (p = 0.003), and greater depression (p = 0.010). The presence of RBD was a trait of Mid-ET (p = 0.039). In both Mid-ET and No-Mid-ET phenotypes, clinical and neurophysiological dysautonomia correlated with cognitive dysfunction. CONCLUSION: Mid-ET patients had greater cognitive dysfunction, depression, RBD, higher proportion of late-onset ET, and more extrapyramidal signs. Taken all together, these findings could provide a redesigned insight into the underlying physiopathology of Mid-ET indicative of a greater cerebellar dysfunction.
Authors: A Cerasa; D Messina; G Nicoletti; F Novellino; P Lanza; F Condino; G Arabia; M Salsone; A Quattrone Journal: AJNR Am J Neuroradiol Date: 2009-04-02 Impact factor: 3.825
Authors: Kailash P Bhatia; Peter Bain; Nin Bajaj; Rodger J Elble; Mark Hallett; Elan D Louis; Jan Raethjen; Maria Stamelou; Claudia M Testa; Guenther Deuschl Journal: Mov Disord Date: 2017-11-30 Impact factor: 10.338