Purpose of reviewThis survey takes into consideration the most recent advances in both human degenerative ataxias, disorders with a well established cerebellar origin, and discoveries from dystonia rodent models aimed at discussing the pathogenesis of dystonia.Recent findingsOne common recurrent term that emerges when describing dystonia is heterogeneity. Indeed, dystonia encompasses a wide group of hyperkinetic' movement disorders, with heterogeneous causes, classification, anatomical and physiological substrates. In addition, the clinical heterogeneity of age at onset, symptom distribution and appearance of non-motor symptoms has supported the concept of dystonia as network' disorder. Pathophysiological alterations are thought to arise from dysfunction at cortico-thalamic-basal ganglia level, whereas, more recently, a role for cerebellar pathways emerged. Results from human and animal studies thus fuel the evolving concept of the network disorder.SummaryCurrent evidence suggests the involvement of multiple brain regions and cellular mechanisms, as part of the neural dysfunction observed at system level in dystonia.

Schirinzi, T., Sciamanna, G., Mercuri, N.b., Pisani, A. (2018). Dystonia as a network disorder: A concept in evolution. CURRENT OPINION IN NEUROLOGY, 31(4), 498-503 [10.1097/WCO.0000000000000580].

Dystonia as a network disorder: A concept in evolution

Schirinzi T.;Sciamanna G.;Mercuri N. B.;Pisani A.
2018-01-01

Abstract

Purpose of reviewThis survey takes into consideration the most recent advances in both human degenerative ataxias, disorders with a well established cerebellar origin, and discoveries from dystonia rodent models aimed at discussing the pathogenesis of dystonia.Recent findingsOne common recurrent term that emerges when describing dystonia is heterogeneity. Indeed, dystonia encompasses a wide group of hyperkinetic' movement disorders, with heterogeneous causes, classification, anatomical and physiological substrates. In addition, the clinical heterogeneity of age at onset, symptom distribution and appearance of non-motor symptoms has supported the concept of dystonia as network' disorder. Pathophysiological alterations are thought to arise from dysfunction at cortico-thalamic-basal ganglia level, whereas, more recently, a role for cerebellar pathways emerged. Results from human and animal studies thus fuel the evolving concept of the network disorder.SummaryCurrent evidence suggests the involvement of multiple brain regions and cellular mechanisms, as part of the neural dysfunction observed at system level in dystonia.
2018
Pubblicato
Rilevanza internazionale
Recensione
Esperti anonimi
Settore MED/26 - NEUROLOGIA
English
ataxia; basal ganglia; cerebellum; dystonia; network; Ataxia; Dystonia; Dystonic Disorders; Humans; Nerve Net; Biological Evolution
Schirinzi, T., Sciamanna, G., Mercuri, N.b., Pisani, A. (2018). Dystonia as a network disorder: A concept in evolution. CURRENT OPINION IN NEUROLOGY, 31(4), 498-503 [10.1097/WCO.0000000000000580].
Schirinzi, T; Sciamanna, G; Mercuri, Nb; Pisani, A
Articolo su rivista
File in questo prodotto:
Non ci sono file associati a questo prodotto.

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2108/218177
Citazioni
  • ???jsp.display-item.citation.pmc??? 20
  • Scopus 54
  • ???jsp.display-item.citation.isi??? 51
social impact