Magnetic Resonance Imaging-Based Distribution and Reversibility of Lesions in Pediatric Vigabatrin-Related Brain Toxicity.

Pediatr Neurol

Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.

Published: November 2023


Category Ranking

98%

Total Visits

921

Avg Visit Duration

2 minutes

Citations

20

Article Abstract

Background: We aimed to systematically characterize the magnetic resonance imaging (MRI) findings in vigabatrin-related neurotoxicity in children and determine the reversibility of lesions based on follow-up images.

Methods: We evaluated children with a history of refractory seizures who had a brain MRI while on vigabatrin therapy. We included available brain MRI studies before vigabatrin therapy initiation, during vigabatrin treatment, and after vigabatrin was discontinued. A pediatric neuroradiologist systematically assessed images on T2/fluid-attenuated inversion recovery (FLAIR) and diffusion-weighted imaging /apparent diffusion coefficient sequences to identify hyperintense lesions and/or restricted diffusion. The frequency of abnormal signal at each location was determined, as well as the reversibility of these after vigabatrin discontinuation.

Results: MRIs of 43 patients were reviewed: 13 before vigabatrin initiation, 18 during treatment, and 12 after vigabatrin discontinuation. In the MRIs acquired during vigabatrin treatment, most lesions on T2/FLAIR occurred in the globus pallidi, thalami, and midbrain. Correspondingly, the most common locations for restricted diffusion were the globus pallidi, thalami, and subthalamic nuclei. On MRI after vigabatrin discontinuation, complete resolution of lesions on T2/FLAIR in all patients was seen in the midbrain, dentate nuclei, subthalamic nuclei, and hypothalami. Complete resolution of restricted diffusion was observed in the globus pallidi, midbrain, dentate nuclei, hippocampi, anterior commissure, and hypothalami.

Conclusion: Globus pallidi and thalami are the most commonly affected structures in vigabatrin-related toxicity, and most vigabatrin-related neuroimaging findings are reversible.

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.pediatrneurol.2023.08.012DOI Listing

Publication Analysis

Top Keywords

globus pallidi
16
restricted diffusion
12
pallidi thalami
12
vigabatrin
9
magnetic resonance
8
reversibility lesions
8
brain mri
8
mri vigabatrin
8
vigabatrin therapy
8
vigabatrin treatment
8

Similar Publications

Bilateral globi pallidi infarctions from opioid and cocaine use.

Am J Emerg Med

June 2025

Department of Emergency Medicine, University of Illinois College of Medicine, Room 469 COME, 1819 W Polk St., Chicago, IL 60612, USA. Electronic address:

The globi pallidi (GP) are paired structures present in the basal ganglia of the brain. Their primary function is to regulate motor movement. Due to their high metabolic activity, the GP are highly susceptible to injury from hypoxia and other metabolic insults.

View Article and Find Full Text PDF

Background: Biallelic pathogenic variants in the FUCA1 gene are associated with fucosidosis. This report describes a 4-year-old boy presenting with psychomotor regression, spasticity, and dystonic postures.

Methods And Results: Trio-based whole exome sequencing revealed two previously unreported loss-of-function variants in the FUCA1 gene.

View Article and Find Full Text PDF

Background: Mild traumatic brain injury (mTBI) can result in lasting brain damage that is often too subtle to detect by qualitative visual inspection on conventional MR imaging. Although a number of FDA-cleared MR neuroimaging tools have demonstrated changes associated with mTBI, they are still under-utilized in clinical practice.

Methods: We investigated a group of 65 individuals with predominantly mTBI (60 mTBI, 48 due to motor-vehicle collision, mean age 47 ± 13 years, 27 men and 38 women) with MR neuroimaging performed in a median of 37 months post-injury.

View Article and Find Full Text PDF

Role of the globus pallidus in motor and non-motor symptoms of Parkinson's disease.

Neural Regen Res

June 2025

Department of Physiology, School of Medicine, Nantong University, Nantong, Jiangsu Province, China.

The globus pallidus plays a pivotal role in the basal ganglia circuit. Parkinson's disease is characterized by degeneration of dopamine-producing cells in the substantia nigra, which leads to dopamine deficiency in the brain that subsequently manifests as various motor and non-motor symptoms. This review aims to summarize the involvement of the globus pallidus in both motor and non-motor manifestations of Parkinson's disease.

View Article and Find Full Text PDF

Further delineation of short-chain enoyl-CoA hydratase deficiency in the Pacific population.

Mol Genet Metab

July 2024

Paediatric and Adult National Metabolic Service, Te Toka Tumai, Te Whatu Ora Health New Zealand, Auckland, New Zealand.

Short-chain enoyl-coA hydratase (SCEH) deficiency due to biallelic pathogenic ECHS1 variants was first reported in 2014 in association with Leigh syndrome (LS) and increased S-(2-carboxypropyl)cysteine excretion. It is potentially treatable with a valine-restricted, high-energy diet and emergency regimen. Recently, Simon et al.

View Article and Find Full Text PDF