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

Payne, Mary S.; Brown, Benjamin Lyons; Rao, Jayaraman; Payne, Bertram R.

doi:10.1227/01.neu.0000156492.99035.ee

Cited by 14 publications

(2 citation statements)

References 19 publications

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“…Only a small number of cases of this tremor type have been treated with Vim-DBS (e.g., Romanelli et al, 2003), and these cases have had excellent results. Vim stimulation has also been used for other tremor forms, often due to rare diseases, such as phenylketonuria (Payne et al, 2005) or inherited cerebellar ataxia (Schramm et al, 2005).…”

Section: Dbs For Movement Disordersmentioning

confidence: 99%

Deep Brain Stimulation for Neurologic and Neuropsychiatric Disorders

Wichmann

DeLong

2006

Neuron

287

133

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In the 1960s, ablative stereotactic surgery was employed for a variety of movement disorders and psychiatric conditions. Although largely abandoned in the 1970s because of highly effective drugs, such as levodopa for Parkinson's disease (PD), and a reaction against psychosurgery, the field has undergone a virtual renaissance, guided by a better understanding of brain circuitry and the circuit abnormalities underlying movement disorders such as PD and neuropsychiatric conditions, such as obsessive compulsive disorder. High-frequency electrical deep brain stimulation (DBS) of specific targets, introduced in the early 1990s for tremor, has gained widespread acceptance because of its less invasive, reversible, and adjustable features and is now utilized for an increasing number of brain disorders. This review summarizes the rationale behind DBS and the use of this technique for a variety of movement disorders and neuropsychiatric diseases.

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Section: Dbs For Movement Disordersmentioning

confidence: 99%

Deep Brain Stimulation for Neurologic and Neuropsychiatric Disorders

Wichmann

DeLong

2006

Neuron

287

133

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“…DBS has been used rarely as a treatment option in several other IEMs, with dystonia being the most common indication. However, there have also been reports of successful management of other movement disorders such as parkinsonism (7,45,70,(81)(82)(83)(84)(85)(86)(87)(88)(89)(90)(91)(92)(93)(94)(95)(96)(97)(98)(99). Table 1 provides a summary of these cases.…”

Section: Other Neurometabolic Conditionsmentioning

confidence: 99%

Surgical treatment of movement disorders in neurometabolic conditions

Zea-Vera

Gropman

2023

Front. Neurol.

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Refractory movement disorders are a common feature of inborn errors of metabolism (IEMs), significantly impacting quality of life and potentially leading to life-threatening complications such as status dystonicus. Surgical techniques, including deep brain stimulation (DBS) and lesioning techniques, represent an additional treatment option. However, the application and benefits of these procedures in neurometabolic conditions is not well understood. This results in challenges selecting surgical candidates and counseling patients preoperatively. In this review, we explore the literature of surgical techniques for the treatment of movement disorders in IEMs. Globus pallidus internus DBS has emerged as a beneficial treatment option for dystonia in Panthotate-Kinase-associated Neurodegeneration. Additionally, several patients with Lesch–Nyhan Disease have shown improvement following pallidal stimulation, with more robust effects on self-injurious behavior than dystonia. Although there are numerous reports describing benefits of DBS for movement disorders in other IEMs, the sample sizes have generally been small, limiting meaningful conclusions. Currently, DBS is preferred to lesioning techniques. However, successful use of pallidotomy and thalamotomy in neurometabolic conditions has been reported and may have a role in selected patients. Surgical techniques have also been used successfully in patients with IEMs to treat status dystonicus. Advancing our knowledge of these treatment options could significantly improve the care for patients with neurometabolic conditions.

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