A Family with a Hereditary Form of Torsion Dystonia from Northern Sweden Treated with Bilateral Pallidal Deep Brain Stimulation

To evaluate pallidal DBS in a non-DYT1 form of hereditary dystonia. We present the results of pallidal DBS in a family with non-DYT1 dystonia where DYT5 to 17 was excluded. The dystonia is following an autosomal dominant pattern. Ten members had definite dystonia and five had dystonia with minor sym...

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Main Authors: Blomstedt, P, Hariz, MI, Tisch, S, Holmberg, M, Bergenheim, TA, Forsgren, L
Format: Article in Journal/Newspaper
Language:unknown
Published: WILEY-LISS 2009
Subjects:
dystonia
DBS
pallidal stimulation
PRIMARY GENERALIZED DYSTONIA
GLOBUS-PALLIDUS
CERVICAL DYSTONIA
MEIGE-SYNDROME
Marsden
Northern Sweden
Online Access:http://discovery.ucl.ac.uk/92814/
id ftucl:oai:eprints.ucl.ac.uk.OAI2:92814
record_format openpolar
spelling ftucl:oai:eprints.ucl.ac.uk.OAI2:92814 2023-05-15T17:44:39+02:00 A Family with a Hereditary Form of Torsion Dystonia from Northern Sweden Treated with Bilateral Pallidal Deep Brain Stimulation Blomstedt, P Hariz, MI Tisch, S Holmberg, M Bergenheim, TA Forsgren, L 2009-12-15 http://discovery.ucl.ac.uk/92814/ unknown WILEY-LISS MOVEMENT DISORD , 24 (16) 2415 - 2419. (2009) dystonia DBS pallidal stimulation PRIMARY GENERALIZED DYSTONIA GLOBUS-PALLIDUS CERVICAL DYSTONIA MEIGE-SYNDROME Article 2009 ftucl 2016-01-15T02:24:05Z To evaluate pallidal DBS in a non-DYT1 form of hereditary dystonia. We present the results of pallidal DBS in a family with non-DYT1 dystonia where DYT5 to 17 was excluded. The dystonia is following an autosomal dominant pattern. Ten members had definite dystonia and five had dystonia with minor symptoms. Four patients received bilateral pallidal DBS. Mean age was 47 years. The patients were evaluated before surgery, and "on" stimulation after a mean of 2.5 years (range 1-3) using the Burke-Fahn-Marsden scale (BFM). Mean BFM score decreased by 79 % on stimulation, from 42.5 +/- 24 to 9 +/- 6.5 at the last evaluation. Cervical involvement improved by 89%. The 2 patients with oromandibular dystonia and blepharospasm demonstrated a reduction of 95% regarding these symptoms. The present study confirms the effectiveness of pallidal DBS in a new family with hereditary primary segmental and generalized dystonia. (C) 2009 Movement Disorder Society Article in Journal/Newspaper Northern Sweden University College London: UCL Discovery Marsden ENVELOPE(66.067,66.067,-67.867,-67.867)
institution Open Polar
collection University College London: UCL Discovery
op_collection_id ftucl
language unknown
topic dystonia
DBS
pallidal stimulation
PRIMARY GENERALIZED DYSTONIA
GLOBUS-PALLIDUS
CERVICAL DYSTONIA
MEIGE-SYNDROME
spellingShingle dystonia
DBS
pallidal stimulation
PRIMARY GENERALIZED DYSTONIA
GLOBUS-PALLIDUS
CERVICAL DYSTONIA
MEIGE-SYNDROME
Blomstedt, P
Hariz, MI
Tisch, S
Holmberg, M
Bergenheim, TA
Forsgren, L
A Family with a Hereditary Form of Torsion Dystonia from Northern Sweden Treated with Bilateral Pallidal Deep Brain Stimulation
topic_facet dystonia
DBS
pallidal stimulation
PRIMARY GENERALIZED DYSTONIA
GLOBUS-PALLIDUS
CERVICAL DYSTONIA
MEIGE-SYNDROME
description To evaluate pallidal DBS in a non-DYT1 form of hereditary dystonia. We present the results of pallidal DBS in a family with non-DYT1 dystonia where DYT5 to 17 was excluded. The dystonia is following an autosomal dominant pattern. Ten members had definite dystonia and five had dystonia with minor symptoms. Four patients received bilateral pallidal DBS. Mean age was 47 years. The patients were evaluated before surgery, and "on" stimulation after a mean of 2.5 years (range 1-3) using the Burke-Fahn-Marsden scale (BFM). Mean BFM score decreased by 79 % on stimulation, from 42.5 +/- 24 to 9 +/- 6.5 at the last evaluation. Cervical involvement improved by 89%. The 2 patients with oromandibular dystonia and blepharospasm demonstrated a reduction of 95% regarding these symptoms. The present study confirms the effectiveness of pallidal DBS in a new family with hereditary primary segmental and generalized dystonia. (C) 2009 Movement Disorder Society
format Article in Journal/Newspaper
author Blomstedt, P
Hariz, MI
Tisch, S
Holmberg, M
Bergenheim, TA
Forsgren, L
author_facet Blomstedt, P
Hariz, MI
Tisch, S
Holmberg, M
Bergenheim, TA
Forsgren, L
author_sort Blomstedt, P
title A Family with a Hereditary Form of Torsion Dystonia from Northern Sweden Treated with Bilateral Pallidal Deep Brain Stimulation
title_short A Family with a Hereditary Form of Torsion Dystonia from Northern Sweden Treated with Bilateral Pallidal Deep Brain Stimulation
title_full A Family with a Hereditary Form of Torsion Dystonia from Northern Sweden Treated with Bilateral Pallidal Deep Brain Stimulation
title_fullStr A Family with a Hereditary Form of Torsion Dystonia from Northern Sweden Treated with Bilateral Pallidal Deep Brain Stimulation
title_full_unstemmed A Family with a Hereditary Form of Torsion Dystonia from Northern Sweden Treated with Bilateral Pallidal Deep Brain Stimulation
title_sort family with a hereditary form of torsion dystonia from northern sweden treated with bilateral pallidal deep brain stimulation
publisher WILEY-LISS
publishDate 2009
url http://discovery.ucl.ac.uk/92814/
long_lat ENVELOPE(66.067,66.067,-67.867,-67.867)
geographic Marsden
geographic_facet Marsden
genre Northern Sweden
genre_facet Northern Sweden
op_source MOVEMENT DISORD , 24 (16) 2415 - 2419. (2009)
_version_ 1766146916043718656

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