Faculty Opinions recommendation of Deep brain stimulation for essential vocal tremor: A technical report.

Tremulous voice is a characteristic feature of a multitude of movement disorders, but when it occurs in individuals diagnosed with essential tremor, it is referred to as essential vocal tremor (EVT). For individuals with EVT, their tremulous voice is associated with significant social embarrassment and in severe cases may result in the discontinuation of employment and hobbies. Management of EVT is extremely difficult, and current behavioral and medical interventions for vocal tremor result in suboptimal outcomes. Deep brain stimulation (DBS) has been proposed as a potential therapeutic avenue for EVT, but few studies can be identified that have systematically examined improvements in EVT following DBS. The authors describe a case of awake bilateral DBS targeting the ventral intermediate nucleus for a patient suffering from severe voice and arm tremor. They also present their comprehensive, multidisciplinary methodology for definitive treatment of EVT via DBS. To the authors’ knowledge, this is the first time comprehensive intraoperative voice evaluation has been used to guide microelectrode/stimulator placement, as well as the first time that standard pre- and post-DBS assessments have been conducted, demonstrating the efficacy of this tailored DBS approach.

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Intraoperative Evaluation of Essential Vocal Tremor in Deep Brain Stimulation Surgery

American Journal of Speech-Language Pathology ◽

10.1044/2019_ajslp-19-00079 ◽

2020 ◽

Vol 29 (2) ◽

pp. 851-863

Author(s):

Elizabeth Erickson-DiRenzo ◽

C. Kwang Sung ◽

Allen L. Ho ◽

Casey H. Halpern

Keyword(s):

Deep Brain Stimulation ◽

Fundamental Frequency ◽

Brain Stimulation ◽

Objective Assessment ◽

Intensity Modulation ◽

Added Value ◽

Electrode Placement ◽

Speech Language Pathologist ◽

Vocal Tremor ◽

Deep Brain

Purpose Essential vocal tremor (EVT) is a prevalent and difficult-to-manage voice disorder. There is evidence that deep brain stimulation (DBS) of the ventral intermediate nucleus (Vim) of the thalamus may be beneficial for treating EVT. The objective of this preliminary investigation was to conduct intraoperative voice assessments during Vim-DBS implantation in order to evaluate immediate voice outcomes in medication-refractory essential tremor patients with co-occurring EVT. Method Seven adult subjects diagnosed with EVT undergoing Vim-DBS surgery participated in this investigation. Voice samples of sustained vowels were collected by a speech-language pathologist preoperatively and intraoperatively, immediately following Vim-DBS electrode placement. Voice evaluation included objective acoustic assessment of the rate and extent of EVT fundamental frequency and intensity modulation and subjective perceptual ratings of EVT severity. Results The rate of intensity modulation, extent of fundamental frequency modulation, and perceptual rating of EVT severity were significantly reduced intraoperatively as compared to preoperatively. Moderate, positive correlations were appreciated between a subset of acoustic measures and perceptual severity ratings. Conclusions The results of this study demonstrate a speech-language pathologist can conduct intra-operative evaluation of EVT during DBS surgery. Using a noninvasive, simple acoustic recording method, we were able to supplement perceptual subjective observation with objective assessment and demonstrate immediate, intraoperative improvements in EVT. The findings of this analysis inform the added value of intraoperative voice evaluation in Vim-DBS patients and contribute to the growing body of literature seeking to evaluate the efficacy of DBS as a treatment for EVT.

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Commentary: Posteromedial Hypothalamic Deep Brain Stimulation for Refractory Aggressiveness in a Patient With Weaver Syndrome: Clinical, Technical Report and Operative Video

Operative Neurosurgery ◽

10.1093/ons/opab165 ◽

2021 ◽

Author(s):

Volker A Coenen

Keyword(s):

Deep Brain Stimulation ◽

Brain Stimulation ◽

Technical Report ◽

Weaver Syndrome ◽

Deep Brain

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Posteromedial Hypothalamic Deep Brain Stimulation for Refractory Aggressiveness in a Patient With Weaver Syndrome: Clinical, Technical Report and Operative Video

Operative Neurosurgery ◽

10.1093/ons/opab149 ◽

2021 ◽

Author(s):

Guillermo Blasco García de Andoain ◽

Marta Navas García ◽

Óscar González Aduna ◽

Alvaro Bocos Portillo ◽

Elena Ezquiaga Terrazas ◽

...

Keyword(s):

Deep Brain Stimulation ◽

Brain Stimulation ◽

Clinical Presentation ◽

Genetic Disorder ◽

Behavioral Alterations ◽

First Case ◽

Technical Report ◽

Weaver Syndrome ◽

Deep Brain

Abstract BACKGROUND AND IMPORTANCE Deep brain stimulation of the posteromedial hypothalamus (PMH DBS) appears to be an effective treatment for drug-resistant aggressiveness. Weaver syndrome (WS) is a rare genetic disorder in which patients develop some degree of intellectual disability and rarely severe behavioral alterations that may benefit from this procedure. CLINICAL PRESENTATION We present the case of a 26-yr-old man diagnosed with WS presenting with uncontrollable self and heteroaggressiveness and disruptive behavior refractory to pharmacological treatment and under severe physical and mechanical restraining measures. The patient was successfully treated with bilateral PMH DBS resulting in affective improvement, greater tolerance for signs of affection, regularization in his sleep pattern and appetite disturbances at 12-mo follow-up. A detailed description and video of the procedure are presented, and a review of the clinical characteristics of WS and the utility and benefits of PMH DBS for refractory aggressiveness are reviewed. CONCLUSION To our knowledge, this is the first case of refractory aggressiveness described in WS as well as the first patient with WS successfully treated with PMH DBS.

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