Generalized Dystonia Treated With Deep Brain Stimulator: An Institutional Single Surgeon Experience

The implantation of deep brain stimulator electrodes is associated with infrequent complications. These complications are consistent across prospective trials and include infection, skin erosion, hemorrhage, and lead misplacement. Nuances of surgical technique can be used to minimize the risk of these commonly noted complications. Several of these technical nuances are highlighted in this video submission.The video can be found here: https://youtu.be/GL09W9p013g.

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KMT2B-Related Dystonia: Challenges in Diagnosis and Treatment

Molecular Syndromology ◽

10.1159/000518974 ◽

2021 ◽

pp. 1-6

Author(s):

Ayşe Aksoy ◽

Özlem Yayıcı Köken ◽

Ahmet Cevdet Ceylan ◽

Özge Toptaş Dedeoğlu

Keyword(s):

Clinical Status ◽

Childhood Onset ◽

Generalized Dystonia ◽

Basic Functions ◽

Turkish Case ◽

The Right ◽

Hereditary Dystonia ◽

Climbing Stairs ◽

Deep Brain

In this study, we report the first known Turkish case of a novel nonsense mutation c.2453dupT (p.M818fs*28) in the KMT2B (NM_014727.2) gene diagnosed in a male patient with KMT2B-related dystonia (DYT-KMT2B, DYT-28, Dystonia*-28), which is a complex, childhood-onset, progressive, hereditary dystonia. The patient, who is followed up from 9 to 13 years of age, had dysmorphic features, developmental delay, short stature, and microcephaly, in addition to focal dystonia and hemichorea (in the right and left lower extremities). Generalized dystonia involving bulbar and cervical muscles, in addition to dystonic cramps, myoclonus, and hemiballismus, were also observed during the course of the follow-up. While he was able to perform basic functions like eating, climbing stairs, walking, and writing with the aid of levodopa and trihexyphenidyl treatment, his clinical status gradually deteriorated secondary to progressive generalized dystonia in the 4-year follow-up. Deep brain stimulation has been shown to be effective in several patients which could be the next preferred treatment for the patient.

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Perioperative Challenges During Stereotactic Neurosurgery and Deep Brain Stimulator Placement

Essentials of Neurosurgical Anesthesia & Critical Care ◽

10.1007/978-3-030-17410-1_29 ◽

2019 ◽

pp. 189-192

Author(s):

Mitchell Y. Lee ◽

Marc J. Bloom

Keyword(s):

Stereotactic Neurosurgery ◽

Deep Brain Stimulator ◽

Deep Brain

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Ventral Intermediate Thalamic Stimulation for Monoclonal Gammopathy-Associated Tremor: Case Report

Neurosurgery ◽

10.1227/neu.0b013e3182124633 ◽

2011 ◽

Vol 68 (5) ◽

pp. E1464-E1467 ◽

Cited By ~ 2

Author(s):

Donald C. Shields ◽

Alice W. Flaherty ◽

Emad N. Eskandar ◽

Ziv M. Williams

Keyword(s):

Daily Living ◽

Monoclonal Gammopathy ◽

Clinical Presentation ◽

Sensory Loss ◽

Intention Tremor ◽

Deep Brain Stimulator ◽

Thalamic Stimulation ◽

Central Nervous System Dysfunction ◽

Stimulation Parameters ◽

Deep Brain

Abstract BACKGROUND AND IMPORTANCE: Peripheral and central sensory loss are often associated with significant tremor or sensory ataxia, which can be highly refractory to medical therapy. CLINICAL PRESENTATION: We present the case of a 67-year-old man with progressive and debilitating intention tremor from monoclonal gammopathy-associated peripheral neuropathy. The patient was implanted with bilateral thalamic deep brain stimulator electrodes under microelectrode guidance. Following optimization of stimulation parameters, the patient's appendicular tremor and gait improved, as did his general activities of daily living. CONCLUSION: These initial findings suggest that deep brain stimulation may benefit not only tremor presumed to originate from central nervous system dysfunction, but also tremor originating peripherally from neuropathy-related sensory loss.

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Subthalamic nucleus deep brain stimulation after bilateral pallidotomy in the treatment of generalized dystonia

Parkinsonism & Related Disorders ◽

10.1016/j.parkreldis.2013.09.015 ◽

2014 ◽

Vol 20 (1) ◽

pp. 131-133 ◽

Cited By ~ 6

Author(s):

Malgorzata Dec ◽

Marcin Tutaj ◽

Monika Rudzińska ◽

Andrzej Szczudlik ◽

Henryk Koziara ◽

...

Keyword(s):

Deep Brain Stimulation ◽

Subthalamic Nucleus ◽

Brain Stimulation ◽

Generalized Dystonia ◽

Deep Brain

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Patient with a Deep Brain Stimulator

Guide to the Inpatient Pain Consult ◽

10.1007/978-3-030-40449-9_4 ◽

2020 ◽

pp. 33-43

Author(s):

Rudy Garza ◽

Cory Jones ◽

Maxim S. Eckmann

Keyword(s):

Deep Brain Stimulator ◽

Deep Brain

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Deep brain stimulation treatment in dystonia: a bibliometric analysis

Arquivos de Neuro-Psiquiatria ◽

10.1590/0004-282x20200016 ◽

2020 ◽

Vol 78 (9) ◽

pp. 586-592

Author(s):

Clarice LISTIK ◽

Eduardo LISTIK ◽

Rubens Gisbert CURY ◽

Egberto Reis BARBOSA ◽

Manoel Jacobsen TEIXEIRA ◽

...

Keyword(s):

Deep Brain Stimulation ◽

Bibliometric Analysis ◽

Brain Stimulation ◽

The United States ◽

Scientific Journals ◽

Long Term Outcomes ◽

Generalized Dystonia ◽

Research Domain ◽

Deep Brain

ABSTRACT Background: Dystonia is a heterogeneous disorder that, when refractory to medical treatment, may have a favorable response to deep brain stimulation (DBS). A practical way to have an overview of a research domain is through a bibliometric analysis, as it makes it more accessible for researchers and others outside the field to have an idea of its directions and needs. Objective: To analyze the 100 most cited articles in the use of DBS for dystonia treatment in the last 30 years. Methods: The research protocol was performed in June 2019 in Elsevier’s Scopus database, by retrieving the most cited articles regarding DBS in dystonia. We analyzed authors, year of publication, country, affiliation, and targets of DBS. Results: Articles are mainly published in Movement Disorders (19%), Journal of Neurosurgery (9%), and Neurology (9%). European countries offer significant contributions (57% of our sample). France (192.5 citations/paper) and Germany (144.1 citations/paper) have the highest citation rates of all countries. The United States contributes with 31% of the articles, with 129.8 citations/paper. The publications are focused on General outcomes (46%), followed by Long-term outcomes (12.5%), and Complications (11%), and the leading type of dystonia researched is idiopathic or inherited, isolated, segmental or generalized dystonia, with 27% of articles and 204.3 citations/paper. Conclusions: DBS in dystonia research is mainly published in a handful of scientific journals and focused on the outcomes of the surgery in idiopathic or inherited, isolated, segmental or generalized dystonia, and with globus pallidus internus as the main DBS target.

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