Treatment of Phenylketonuria-associated Tremor with Deep Brain Stimulation: Case Report

Neurosurgery ◽  
2005 ◽  
Vol 56 (4) ◽  
pp. E868-E868 ◽  
Author(s):  
Mary Say Payne ◽  
Benjamin Lyons Brown ◽  
Jayaraman Rao ◽  
Bryan Rankin Payne

Abstract OBJECTIVE AND IMPORTANCE: Phenylketonuria (PKU) is an inborn error of metabolism that causes severe neurological impairment, despite dietary treatment. We present a case of PKU-induced cerebellar tremor treated with deep brain stimulation. There have been no previously reported cases of a patient with a PKU tremor treated with deep brain stimulation. CLINICAL PRESENTATION: A 36-year-old male patient with PKU presented with signs of cerebellar disease including dysmetria, resting tremor, and intention tremor in the left upper extremity. INTERVENTION: A deep brain stimulation electrode was placed in the ventral intermediate nucleus of the right thalamus. CONCLUSION: Immediately after surgery, the patient had nearly complete resolution of intention tremor in the left arm. His resting tremor in the left hand was also greatly improved. The 30-month follow-up examination revealed maintenance of the immediate postoperative improvement.

2003 ◽  
Vol 99 (3) ◽  
pp. 566-571 ◽  
Author(s):  
Pantaleo Romanelli ◽  
Helen Bronté-Stewart ◽  
Tracy Courtney ◽  
Gary Heit

✓ Holmes tremor is characterized by resting, postural, and intention tremor. Deep brain stimulation (DBS) of both the nucleus ventralis intermedius (Vim) and the subthalamic nucleus (STN) may be required to control these three tremor components. A 79-year-old man presented with a long-standing combination of resting, postural, and intention tremor, which was associated with severe disability and was resistant to medical treatment. Neuroimaging studies failed to reveal areas of discrete brain damage. A DBS device was placed in the Vim and produced an improvement in both the intention and postural tremor, but there was residual resting tremor, as demonstrated by clinical observation and quantitative tremor analysis. Placement of an additional DBS device in the STN resolved the resting tremor. Stimulation of the Vim or STN alone failed to produce global resolution of mixed tremor, whereas combined Vim—STN stimulation produced global relief without creating noticeable side effects. Combined Vim—STN stimulation can thus be a safe and effective treatment for Holmes tremor.


2006 ◽  
Vol 104 (4) ◽  
pp. 506-512 ◽  
Author(s):  
Rajesh Pahwa ◽  
Kelly E. Lyons ◽  
Steven B. Wilkinson ◽  
Richard K. Simpson ◽  
William G. Ondo ◽  
...  

Object The effects of thalamic deep brain stimulation (DBS) on essential tremor (ET) and Parkinson disease (PD) have been well documented, but there is a paucity of long-term data. The aim of this study was to evaluate the longterm safety and efficacy of DBS of the ventralis intermedius nucleus (VIM) of the thalamus for PD and ET. Methods Thirty-eight of 45 patients enrolled at five sites completed a 5-year follow-up study. There were 26 patients with ET and 19 with PD undergoing 29 unilateral (18 ET/11 PD) and 16 bilateral (eight ET/eight PD) procedures. Patients with ET were evaluated using the Tremor Rating Scale, and patients with PD were evaluated using the Unified Parkinson’s Disease Rating Scale. The mean age of patients with ET was 70.2 years and 66.3 years in patients with PD. Unilaterally implanted patients with ET had a 75% improvement of the targeted hand tremor; those with bilateral implants had a 65% improvement in the left hand and 86% in the right compared with baseline. Parkinsonian patients with unilateral implants had an 85% improvement in the targeted hand tremor and those with bilateral implants had a 100% improvement in the left hand and 90% improvement in the right. Common DBS-related adverse events in patients receiving unilateral implants were paresthesia (45%) and pain (41%), and in patients receiving implants bilaterally dysarthria (75%) and balance difficulties (56%) occurred. Device-related surgical revisions other than IPG replacements occurred in 12 (27%) of the 45 patients. Conclusions Thalamic stimulation is safe and effective for the long-term management of essential and parkinsonian tremors. Bilateral stimulation can cause dysarthria and incoordination and should be used cautiously.


2018 ◽  
Vol 6 (4) ◽  
pp. 659-662 ◽  
Author(s):  
Hülagu Kaptan ◽  
Raif Çakmur

BACKGROUND: The primary target of this operation is Ventral Intermediate Nucleus (VIM); however VIM - Subthalamic Nucleus (STN) were tried to be reached with one electrode, adjusting the angle well, the coronal section; medial of VIM can partially reach the STN. Using the properties of the electrode; we believe we could act on a wide area.METHODS: An analysis was performed on one patient who underwent VIM Deep Brain Stimulation (DBS) in 3 periods (pre – peri - post-operation).RESULTS: A 53 – year - old woman diagnosed with Parkinson’s disease 8 years earlier including symptoms of severe tremor on the right than left underwent bilateral DBS VIM. Obtaining a satisfactory improvement of tremor, the patient did well, and postoperative complications were not observed. The patient was discharged from hospital on postoperative thirty dayCONCLUSIONS: It is certain that more research and experience are needed. However, we believe that the two targets can reach the same point and the second operations for another target can be avoided.We believe that this initiative is advantageous and promising regarding patient and cost.


2020 ◽  
pp. 93-96
Author(s):  
Julia Kroth ◽  
Susanne Schneider ◽  
Sergiu Groppa

A 78-year-old right-handed woman with a 10-year history of tremor-dominant Parkinson disease (PD) was recommended for bilateral deep brain stimulation (DBS) in the subthalamic nucleus (STN). The patient was implanted with bilateral omnidirectional STN DBS electrodes after intraoperative microelectrode recordings and intraoperative test stimulation. Immediately after implantation, the motor symptoms improved considerably, but 3 to 4 weeks later, a debilitating left-sided resting tremor re-emerged. Personalized programming at the right STN ameliorated the tremor, while painful dyskinesia and hemiballism of the ipsilateral right side of the body developed subsequently over the next few days. Adapting the stimulation parameters and the dopaminergic medication improved these symptoms only marginally. After turning off the left STN electrode, the dyskinesia and hemiballism disappeared completely. In the following weeks, the amperage of the right STN electrode was increased gradually to control the left-sided resting tremor. This was possible without the development of ipsilateral hyperkinesia. During the off phases of stimulation, a considerable direct improvement of hyperkinesia was noted, and the decision to turn off the left STN electrode was ultimately made.


2021 ◽  
Vol 10 (16) ◽  
pp. 3468
Author(s):  
Naomi I. Kremer ◽  
Rik W. J. Pauwels ◽  
Nicolò G. Pozzi ◽  
Florian Lange ◽  
Jonas Roothans ◽  
...  

Deep brain stimulation (DBS) of the thalamic ventral intermediate nucleus is one of the main advanced neurosurgical treatments for drug-resistant tremor. However, not every patient may be eligible for this procedure. Nowadays, various other functional neurosurgical procedures are available. In particular cases, radiofrequency thalamotomy, focused ultrasound and radiosurgery are proven alternatives to DBS. Besides, other DBS targets, such as the posterior subthalamic area (PSA) or the dentato-rubro-thalamic tract (DRT), may be appraised as well. In this review, the clinical characteristics and pathophysiology of tremor syndromes, as well as long-term outcomes of DBS in different targets, will be summarized. The effectiveness and safety of lesioning procedures will be discussed, and an evidence-based clinical treatment approach for patients with drug-resistant tremor will be presented. Lastly, the future directions in the treatment of severe tremor syndromes will be elaborated.


2012 ◽  
Vol 16 (3) ◽  
pp. 230-235 ◽  
Author(s):  
Takamitsu Yamamoto ◽  
Yoichi Katayama ◽  
Junichi Ushiba ◽  
Hiroko Yoshino ◽  
Toshiki Obuchi ◽  
...  

2021 ◽  
Author(s):  
Maarten Bot ◽  
Anne-Fleur van Rootselaari ◽  
Vincent Odekerken ◽  
Joke Dijk ◽  
Rob M A de Bie ◽  
...  

Abstract BACKGROUND Dentato-rubro-thalamic tract (DRT) deep brain stimulation (DBS) suppresses tremor in essential tremor (ET) patients. However, DRT depiction through tractography can vary depending on the included brain regions. Moreover, it is unclear which section of the DRT is optimal for DBS. OBJECTIVE To evaluate deterministic DRT tractography and tremor control in DBS for ET. METHODS After DBS surgery, DRT tractography was conducted in 37 trajectories (20 ET patients). Per trajectory, 5 different DRT depictions with various regions of interest (ROI) were constructed. Comparison resulted in a DRT depiction with highest correspondence to intraoperative tremor control. This DRT depiction was subsequently used for evaluation of short-term postoperative adverse and beneficial effects. RESULTS Postoperative optimized DRT tractography employing the ROI motor cortex, posterior subthalamic area (PSA), and ipsilateral superior cerebellar peduncle and dentate nucleus best corresponded with intraoperative trajectories (92%) and active DBS contacts (93%) showing optimal tremor control. DRT tractography employing a red nucleus or ventral intermediate nucleus of the thalamus (VIM) ROI often resulted in a more medial course. Optimal stimulation was located in the section between VIM and PSA. CONCLUSION This optimized deterministic DRT tractography strongly correlates with optimal tremor control. This technique is readily implementable for prospective evaluation in DBS target planning for ET.


2020 ◽  
Vol 10 (12) ◽  
pp. 925
Author(s):  
Christian Iorio-Morin ◽  
Anton Fomenko ◽  
Suneil K. Kalia

Tremor is a prevalent symptom associated with multiple conditions, including essential tremor (ET), Parkinson’s disease (PD), multiple sclerosis (MS), stroke and trauma. The surgical management of tremor evolved from stereotactic lesions to deep-brain stimulation (DBS), which allowed safe and reversible interference with specific neural networks. This paper reviews the current literature on DBS for tremor, starting with a detailed discussion of current tremor targets (ventral intermediate nucleus of the thalamus (Vim), prelemniscal radiations (Raprl), caudal zona incerta (Zi), thalamus (Vo) and subthalamic nucleus (STN)) and continuing with a discussion of results obtained when performing DBS in the various aforementioned tremor syndromes. Future directions for DBS research are then briefly discussed.


Sign in / Sign up

Export Citation Format

Share Document